WorldWideScience

Sample records for nontraumatic myositis ossificans

  1. Pediatric nontraumatic myositis ossificans of the neck

    Energy Technology Data Exchange (ETDEWEB)

    Kokkosis, Angela A. [Stony Brook University Medical Center, Stony Brook University School of Medicine, Stony Brook, NY (United States); Balsam, Dvorah [Stony Brook University Medical Center, Department of Radiology, Stony Brook, NY (United States); Lee, Thomas K. [Stony Brook University Medical Center, Department of Pediatric Surgery, Stony Brook, NY (United States); Schreiber, Z.J. [Stony Brook University Medical Center, Department of Pathology, Stony Brook, NY (United States)

    2009-04-15

    Nontraumatic myositis ossificans circumscripta (MOC) is a rarely reported benign heterotopic ossification characterized by the aberrant formation of bone in extraskeletal soft tissues. Although a history of trauma can be elicited in 75% of MOC patients, the etiology is unclear in patients without inciting injury. MOC is associated with young male athletes, and is most often localized to the muscle groups of the extremities. Rare cases have been reported in children and adolescents of nontraumatic MOC in the neck. We present a 15-year-old adolescent with a rapidly growing, painful neck mass without traumatic stimulus. (orig.)

  2. Pediatric nontraumatic myositis ossificans of the neck

    International Nuclear Information System (INIS)

    Kokkosis, Angela A.; Balsam, Dvorah; Lee, Thomas K.; Schreiber, Z.J.

    2009-01-01

    Nontraumatic myositis ossificans circumscripta (MOC) is a rarely reported benign heterotopic ossification characterized by the aberrant formation of bone in extraskeletal soft tissues. Although a history of trauma can be elicited in 75% of MOC patients, the etiology is unclear in patients without inciting injury. MOC is associated with young male athletes, and is most often localized to the muscle groups of the extremities. Rare cases have been reported in children and adolescents of nontraumatic MOC in the neck. We present a 15-year-old adolescent with a rapidly growing, painful neck mass without traumatic stimulus. (orig.)

  3. Nontraumatic myositis ossificans in the breast

    International Nuclear Information System (INIS)

    Salomonowitz, Erich; Youssefzadeh, Soraya; Reiner, Angelika; Heilbron, E.A.; Zollikofer, C.L.

    1991-01-01

    The authors report the 1st case of a healthy female who developed a rapidly growing mass in her left breast which proved to be a non-progressive form of myositis ossificans originating in fat tissue. This rare entitity may be important to radiologists because the clinical symptoms are suspicious of a malignancy. (author). 5 refs.; 3 figs

  4. Myositis Ossificans.

    Science.gov (United States)

    Walczak, Brian E; Johnson, Christopher N; Howe, B Matthew

    2015-10-01

    Myositis ossificans is a self-limiting, benign ossifying lesion that can affect any type of soft tissue, including subcutaneous fat, tendons, and nerves. It is most commonly found in muscle as a solitary lesion. Ossifying soft-tissue lesions historically have been inconsistently classified. Fundamentally, myositis ossificans can be categorized into nonhereditary and hereditary types, with the latter being a distinct entity with a separate pathophysiology and treatment approach. The etiology of myositis ossificans is variable; however, clinical presentation generally is characterized by an ossifying soft-tissue mass. Advanced cross-sectional imaging alone can be nonspecific and may appear to be similar to more sinister etiologies. Therefore, the evaluation of a suspicious soft-tissue mass often necessitates multiple imaging modalities for accurate diagnosis. When imaging is indeterminate, biopsy may be required for a histologic diagnosis. However, histopathology varies based on stage of evolution. The treatment of myositis ossificans is complex and is often made in a multidisciplinary fashion because accurate diagnosis is fundamental to a successful outcome. Copyright 2015 by the American Academy of Orthopaedic Surgeons.

  5. Non-traumatic myositis ossificans mimicking a malignant neoplasm in an 83-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Nishio Jun

    2010-08-01

    Full Text Available Abstract Introduction Myositis ossificans is a benign, self-limiting condition that usually affects young, athletically active men. To the best of our knowledge, this case report describes the oldest recorded patient with myositis ossificans. Case presentation Our patient was an 83-year-old Japanese woman who presented with a one week history of a palpable mass in the left thigh. She had a history of surgery for transverse colon cancer and lung cancer at the ages of 73 and 80, respectively. Clinical and radiological examinations suggested a malignant neoplasm such as metastatic carcinoma or extraskeletal osteosarcoma. A diagnosis of myositis ossificans was made by core needle biopsy. Our patient was asymptomatic and had no recurrence at one year follow-up. Conclusion Clinicians should consider myositis ossificans as a possible diagnosis for a soft tissue mass in the limb of an older patient, thereby avoiding unnecessarily aggressive therapy.

  6. Myositis ossificans localisata pseudomalignant Form

    International Nuclear Information System (INIS)

    Grunt, J.; Jankovich, E.; Vasovicova, M.

    1994-01-01

    Authors presents computer tomography and angiographic findings of rare pseudomalignant form of myositis ossificans. Correct diagnosis achieved by complex evaluation of ascertain findings, including biopsy, enables proper treatment with excluding too radical therapy. 3 figs., 4 refs

  7. Radiological evaluation of myositis ossificans

    International Nuclear Information System (INIS)

    Kwon, Yang Sook; Kim, Soo Han; Lim, Young Chae; Shin, Hyun Ja

    1986-01-01

    The 35 patients who have suffered from myositis ossificans were investigate for 11 years. They were post traumatic patients. They trauma was divided into 3 groups; spinal cord injury, direct injury (fracture) and mixed type involving both. We evaluated the age distribution, the frequency of myositis ossificans, predilection site, bilaterally and relation between predilection site and bilaterally to the type of trauma. The results are as follows; 1. The age distribution was highest in 4th decades (12 among 35 patients). 2. In regard to distribution of 35 patients, spinal cord injury were most common (15 cases, 43%), the fracture nextly common (14, 40%) and then mixed type (6, 17%). 3. Among 51 cases of myositis ossificans, the frequency was highest in spinal cord injury (25 cases, 49%), next was fracture (14, 27%). 4. The predilection site of myositis ossificans were thigh (18 cases, 35%), hip (17, 33%) and buttock (6, 12%). 5. In correlation between predilection site of myositis ossificans and level of spinal cord injury, hip is the most frequent site in thoracic injury. 6. The bilaterally of myositis ossificans is 39% (20 among 51 lesions).

  8. Myositis ossificans in hemophilia

    Energy Technology Data Exchange (ETDEWEB)

    Vas, W.; Cockshott, W.P.; Martin, R.F.; Pai, M.K.; Walker, I.

    1981-10-01

    A review of the radiographs of 60 hemophilia patients showed nine (15%) with ectopic new bone formation. Three of these patients had multiple sites of involvement. The high frequency discovered in this series contrasts with the paucity of descriptions to be found in the literature. This process of myositis ossificans affects the lower half of the body and probably represents dysplastic metaplasia developing at the site of an intramuscular hematoma when remote from bone, as well as ossification of hemorrhagic lesions related to the periosteum. In conventional radiographs anatomic localization of bone foci is difficult, but use of computed tomography permits precise identification of the affected muscle. There is negligible disability associated with this condition.

  9. Myositis ossificans in hemophilia

    International Nuclear Information System (INIS)

    Vas, W.; Cockshott, W.P.; Martin, R.F.; Pai, M.K.; Walker, I.

    1981-01-01

    A review of the radiographs of 60 hemophilia patients showed nine (15%) with ectopic new bone formation. Three of these patients had multiple sites of involvement. The high frequency discovered in this series contrasts with the paucity of descriptions to be found in the literature. This process of myositis ossificans affects the lower half of the body and probably represents dysplastic metaplasia developing at the site of an intramuscular hematoma when remote from bone, as well as ossification of hemorrhagic lesions related to the periosteum. In conventional radiographs anatomic localization of bone foci is difficult, but use of computed tomography permits precise identification of the affected muscle. There is negligible disability associated with this condition. (orig.)

  10. Myositis ossificans: magnetic resonance images

    International Nuclear Information System (INIS)

    Dosda, R.; Marti-Bonmati, L.; Concepcion, L.; Galant, J.

    1999-01-01

    Myositis ossificans is characterized by a benign, self-limiting, ossifying mass of the white tissue. In the present report, we describe the magnetic resonance (MR) images in three cases of myositis ossificans in pediatric patients, correlating the MR findings with those obtained with other radiological studies. The lesions were detected in three patients, two boys and one girl, ranging in age between 10 and 14 years. The nature of the lesion was confirmed histologically in all three cases. The MR images were obtained using superconductive units at 0.5 Teslas, with T1 and T2-weighted spin-echo and STIR sequences. In two patients, gadolinium-enhanced T1-weighted images were also obtained. As in any process of maturation, the proliferation/maturation ratio depends on the moment in the course of the lesion, which affects its MR features,. In acute phases, the soft tissue mass with an intraosseous, perilesional adematous reaction predominates, while annular calcification and lesser edema are characteristic of subacute episode. Myositis ossificans is very rare in children. The inflammatory response may present a radiological pattern difficult to distinguish from that of aggressive tumor or infection, especially in the acute phase. (Author) 7 refs

  11. Cervical myositis ossificans traumatica: a rare location

    International Nuclear Information System (INIS)

    Baysal, T.; Sarac, K.; Kutlu, R.; Baysal, O.; Ersoy, Y.; Elmali, N.

    1999-01-01

    An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.)

  12. Cervical myositis ossificans traumatica: a rare location

    Energy Technology Data Exchange (ETDEWEB)

    Baysal, T.; Sarac, K.; Kutlu, R. [Dept. of Radiology, Inonu University, Malatya (Turkey); Baysal, O.; Ersoy, Y. [Dept. of Physical Therapy and Rehabilitation, Inonu Univ., Malatya (Turkey); Elmali, N. [Dept. of Orthopedics and Traumatology, Inonu Univ., Malatya (Turkey)

    1999-05-01

    An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.) With 4 figs., 16 refs.

  13. Myositis ossificans in rectus abdominis muscle: case report

    International Nuclear Information System (INIS)

    Ko, Eun Sook; Na, Jae Boem

    2004-01-01

    Myositis ossificans is an ossifying inflammatory lesion occurring within skeletal muscle. Myositis ossificans usually arises in the large muscles of the extremities and this lesion is characterized by progression of mineralization from periphery to center. In the early phase, myositis ossificans simulates malignant soft tissue tumor without dense mineralization. Traumatic myositis ossificans in rectus abdominis muscle has been reported worldwide. The radiologic findings of early active myositis ossificans in rectus abdominis muscle are ill defined heterogenous hypoechoic mass on US, hemorrhage, early strong enhancement and early peripheral mineralization on CT and MR

  14. Myositis ossificans in rectus abdominis muscle: case report

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Eun Sook; Na, Jae Boem [Gyeongsang National University College of Medicine, Jinju (Korea, Republic of)

    2004-10-15

    Myositis ossificans is an ossifying inflammatory lesion occurring within skeletal muscle. Myositis ossificans usually arises in the large muscles of the extremities and this lesion is characterized by progression of mineralization from periphery to center. In the early phase, myositis ossificans simulates malignant soft tissue tumor without dense mineralization. Traumatic myositis ossificans in rectus abdominis muscle has been reported worldwide. The radiologic findings of early active myositis ossificans in rectus abdominis muscle are ill defined heterogenous hypoechoic mass on US, hemorrhage, early strong enhancement and early peripheral mineralization on CT and MR.

  15. Myositis ossificans with Ga-67 citrate positivity

    International Nuclear Information System (INIS)

    Moreno, A.J.; Yedinak, M.A.; Spicer, M.J.; Turnbull, G.L.; Byrd, B.F.; Brown, T.J.

    1985-01-01

    A 16 year-old boy presented with a firm mass within the left thigh. Ga-67 citrate and bone scintigraphy revealed soft tissue radiotracer accumulation within the mass lesion. Radiographs and xerography of the left thigh revealed calcification within the soft tissue mass typical of myositis ossificans. The patient's clinical course was uneventful

  16. Myositis Ossificans of Psoas Muscle: Magnetic Resonance Imaging Findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirvanci, M.; Ganiyusufoglu, A.K.; Karaman, K.; Tezer, M.; Hamzaoglu, A. [Univ. of Kadir Has, Istanbul (Turkey). Dept. of Radiology

    2004-08-01

    Myositis ossificans is a benign, localized, self-limiting ossifying soft tissue mass with unknown pathogenesis. It may be confused with a malignant tumor of bone or soft tissues, especially in the early stages of the disease. In this report, we present a case of myositis ossificans affecting unilateral psoas muscle, which to our knowledge is a very uncommon location. There was no history of trauma. We describe the imaging findings and clues to early diagnosis of myositis ossificans.

  17. Myositis Ossificans of Psoas Muscle: Magnetic Resonance Imaging Findings

    International Nuclear Information System (INIS)

    Sirvanci, M.; Ganiyusufoglu, A.K.; Karaman, K.; Tezer, M.; Hamzaoglu, A.

    2004-01-01

    Myositis ossificans is a benign, localized, self-limiting ossifying soft tissue mass with unknown pathogenesis. It may be confused with a malignant tumor of bone or soft tissues, especially in the early stages of the disease. In this report, we present a case of myositis ossificans affecting unilateral psoas muscle, which to our knowledge is a very uncommon location. There was no history of trauma. We describe the imaging findings and clues to early diagnosis of myositis ossificans

  18. Extraskeletal osteosarcoma arising in myositis ossificans

    Energy Technology Data Exchange (ETDEWEB)

    Konishi, Eiichi [Div. of Anatomic Pathology, Kyoto Prefectural University of Medicine (Japan); Kusuzaki, Katsuyuki; Murata, Hiroaki [Dept. of Orthopedic Surgery, Kyoto Prefectural University of Medicine (Japan); Tsuchihashi, Yasunari [Hospital Department of Pathology, Kyoto Prefectural University of Medicine (Japan); Beabout, J.W. [Dept. of Diagnostic Radiology, Mayo Clinic and Mayo Foundation, Rochester, MN (United States); Unni, K.K. [Division of Anatomic Pathology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905 (United States); Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (United States)

    2001-01-01

    A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing ''eggshell'' ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans. (orig.)

  19. Extraskeletal osteosarcoma arising in myositis ossificans

    International Nuclear Information System (INIS)

    Konishi, Eiichi; Kusuzaki, Katsuyuki; Murata, Hiroaki; Tsuchihashi, Yasunari; Beabout, J.W.; Unni, K.K.

    2001-01-01

    A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing ''eggshell'' ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans. (orig.)

  20. MYOSITIS OSSIFICANS TRAUMATICA IN A VAMPIRE BAT (DESMODUS ROTUNDUS).

    Science.gov (United States)

    Hausmann, Jennifer C; Manasse, Jorden; Churgin, Sarah; Steinberg, Howard; Clyde, Victoria L; Wallace, Roberta

    2016-09-01

    A 15-yr-old sexually intact female vampire bat ( Desmodus rotundus ) was diagnosed with myositis ossificans traumatica of the abdominal wall. The bat presented with a large ulcerated firm mass along the abdomen. Radiographs and cytology were performed, followed by surgical exploration. The mass was determined to be nonresectable and the bat was euthanized. Histopathology showed severe necrotizing, degenerative, and pyogranulomatous myositis with osseous and cartilaginous metaplasia, fibrosis, and ulceration, which were consistent with myositis ossificans traumatica. Myositis ossificans traumatica is commonly associated with previous trauma to skeletal muscle. Two years prior, this bat had an emergency Caesarian section at this site, which was postulated to elicit a marked tissue response leading to this condition. Myositis ossificans traumatica is infrequently reported in humans, dogs, cats, pigs, and horses. To the author's knowledge, this is the first report of this condition in a bat.

  1. Myositis ossificans imaging: keys to successful diagnosis

    International Nuclear Information System (INIS)

    Lacout, Alexis; Jarraya, Mohamed; Marcy, Pierre-Yves; Thariat, Juliette; Carlier, Robert Yves

    2012-01-01

    Myositis ossificans (MO) is an inflammatory pseudotumor of the muscle that may be mistaken clinically and even histologically for a malignant soft tissue tumor. The aim of this article is to report the imaging characteristics of MO, the emphasis being on the early diagnostic clues. USG can be used at an early stage to reveal the ‘zone phenomenon,’ which is highly suggestive of MO. A short course of nonsteroidal anti-inflammatory drug therapy may be an efficient treatment for early MO

  2. Myositis ossificans and the three-phase bone scan

    International Nuclear Information System (INIS)

    Drane, W.E.

    1984-01-01

    Myositis ossificans circumscripta (or paraosteoarthropathy) in paraplegics has been a well known entity since its classic description in 1918 by Dejerine and Ceillier. The disease is characterized by ectopic bone formation, primarily occurring in the particular soft tissues of the hips and knees. Hypotheses concerning its origin have been proposed, but its etiology is still unknown. Myositis ossificans can occur after traumatic injury, but develops in paraplegics without apparent injury in the involved regions. Radionuclide techniques have been used in the evaluation of myositis ossificans, particularly with serial studies to stage the activity of the disease. The author reports a case of myositis ossificans in a paraplegic that emphasizes the benefit of the three-phase bone scan in the early diagnosis of this disorder

  3. MR imaging and ultrasonography findings of early myositis ossificans: a case report

    International Nuclear Information System (INIS)

    Lee, Kyung Ryeol; Park, So Young; Jin, Wook; Won, Kyu Yeoun

    2016-01-01

    Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings. A 59-year-old woman presented with pain followed by a mass in the left thigh within a week. MR imaging and ultrasonography showed an intramuscular lesion with preserved muscle fascicles in the vastus lateralis muscle. Intralesional ossification or calcification was not seen on ultrasonography. A diagnosis of myositis ossificans was made by ultrasonographically guided biopsy. (orig.)

  4. MR imaging and ultrasonography findings of early myositis ossificans: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Ryeol [Jeju National University Hospital, Department of Radiology, Jeju-si, Jeju Special Self-Governing Province (Korea, Republic of); Park, So Young; Jin, Wook [Kyung Hee University Hospital at Gangdong, Department of Radiology, Seoul (Korea, Republic of); Won, Kyu Yeoun [Kyung Hee University Hospital at Gangdong, Department of Pathology, Seoul (Korea, Republic of)

    2016-10-15

    Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings. A 59-year-old woman presented with pain followed by a mass in the left thigh within a week. MR imaging and ultrasonography showed an intramuscular lesion with preserved muscle fascicles in the vastus lateralis muscle. Intralesional ossification or calcification was not seen on ultrasonography. A diagnosis of myositis ossificans was made by ultrasonographically guided biopsy. (orig.)

  5. Pseudomalignant myositis ossificans involving multiple masticatory muscles: Imaging evaluation

    International Nuclear Information System (INIS)

    Kamalapur, Muralidhar G; Patil, Pritam B; Joshi, Shyamsundar; Shastri, Dinesh

    2014-01-01

    Myositis ossificans is a rare cause of trismus. We present a case of pseudomalignant myositis ossificans involving medial pterygoid, lateral pterygoid, and temporalis muscles. Patient presented with gross limitation in mouth opening. There was no history of trauma. Computed tomography (CT) images revealed a bone density mass located in the region of medial and lateral pterygoid muscles on the right and temporalis muscle on the left. Magnetic resonance imaging (MRI) showed similar findings. Radiological diagnosis was pseudomalignant myositis ossificans. The masses were resected and histopathologic examination confirmed the above diagnosis. This report describes the characteristic CT and MRI features. The unique feature of this case is the absence of history of trauma with involvement of multiple masticatory muscles, which, to the best of our knowledge, has not been reported before

  6. Myositis ossificans with atypical clinical, radiographic, or pathologic findings

    International Nuclear Information System (INIS)

    Nuovo, M.A.; Chumas, J.; Ackerman, L.V.

    1992-01-01

    Myositis ossificans is a relatively rare, well-defined entity. The 23 cases sent for consultation to two of us (L.V.A. and A. N.) were reviewed. Clinical, radiologic, and microscopic information was reexamined, and special attention was given to features infrequently seen in typical myositis ossificans. Due to the uncommon location of 15 lesions and an unusual presentation in 5, the correct diagnosis was not obvious in these cases. Radiologic studies raised the possibility of a malignant bone-forming tumor in at least three instances; myositis ossificans was originally diagnosed in 6 cases radiologically. In 8 cases, histologic evidence suggested malignancy, including osteosarcoma, either parosteal or extraosseous, in 6. Other diagnoses included epithelioid sarcoma and callus formation. Presentation of these variations from the norm highlights the importance of recognizing the evolution of a nonneoplastic fibro-osseous and cartilaginous entity in which conservative treatment is curative. (orig./GDG)

  7. Gallium uptake in myositis ossificans. Potential pitfalls in diagnosis

    International Nuclear Information System (INIS)

    Salzman, L.; Lee, V.W.; Grant, P.

    1987-01-01

    Seven cases of gallium uptake in myositis ossificans are described. Gallium scans are done frequently in paraplegics, quadriplegics, and comatose patients to look for occult infection. It is important to be aware of possible gallium uptake in myositis ossificans, particularly in the extremities, which is frequent in these patients. Gallium uptake may be present prior to any abnormalities seen on plain films or CT scans. It is important to correlate roentgenograms with abnormal gallium scans, particularly in the extremities, to avoid potential pitfalls in diagnosis and prevent unnecessary antibiotic treatment. A bone scan should be obtained whenever possible, particularly when roentgenograms are negative, to confirm the diagnosis

  8. Regional blood flow in experimental myositis ossificans

    International Nuclear Information System (INIS)

    Hierton, C.

    1983-01-01

    In a recent model for heterotopic bone formation, muscular oedema, swelling and necrosis is seen in the quadriceps muscle of rabbit hind limbs immobilized for at least 2 weeks when, from the second week, the immobilized limb is subjected to dayly forcible mobilization lasting about 5 min. According to this model, heterotopic calcification develops gradually from the second week of forcible mobilization and is located in the vastus intermedius region. Between the fourth and fifth week of immobilization and forcible mobilization, heterotopic bone formation is seen in virtually all cases. The histological findings are similar to those in human ectopic bone formation. In the present investigation the labelled microsphere technique was used to study the regional blood flow effects in the early development of myositis ossificans with this model. The results are quite different from those reported by other investigators on immobilization alone and point to a causal relation between regional blood flow and forcible mobilization of the immobilized rabbit hind limp. Prostaglandins as mediators between the traumatic inflammation, a part of the circulatory effects observed and the induction of a new bone is suggested. (author)

  9. FDG PET/CT findings in a case of myositis ossificans circumscripta of the forearm.

    Science.gov (United States)

    Clarençon, Frédéric; Larousserie, Frédérique; Babinet, Antoine; Zylbersztein, Christophe; Talbot, Jean-Noël; Kerrou, Khaldoun

    2011-01-01

    Myositis ossificans circumscripta (MOC) is a rare benign neoplasm located in soft tissues that, most of the time, appears after a local trauma. The positive diagnosis of MOC may be challenging on CT or MRI findings. We report on an atypical case of a spontaneous nontraumatic MOC in a 54-year-old man, located in the longus supinatus muscle diagnosed with MRI and F-18 FDG PET/CT findings. Rarely described F-18 FDG PET/CT features in MOC are presented. Pattern of avid FDG focus on PET/CT, that may wrongly suggest osteosarcoma, is presented.

  10. Magnetic resonance imaging of myositis ossificans: Analysis of seven cases

    International Nuclear Information System (INIS)

    De Smet, A.A.; Norris, M.A.; Fisher, D.R.

    1992-01-01

    Since magnetic resonance imaging (MRI) is commonly used to evaluate soft tissue masses, we analyzed eight MR examinations in seven patients with myositis ossificans to determine if typical patterns were present. One acute lesion had homogeneous intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Two subacute lesions had low signal intensity margins with slightly increased signal intensity centers on T1-weighted images and very high signal intensity on T2-weighted images. Five chronic lesions had two different patterns. All five were well-defined with low signal intensity borders. Three had signal intensity patterns characteristic of fat on T1-weighted and T2-weighted images. The other two lesions had intermediate signal intensity on T1-weighted images and slightly increased signal intensity on T2-weighted images. We conclude that typical MR appearances of myositis ossificans do exist. A low signal intensity rim is a common finding. However, these patterns are not unique to myositis ossificans and resemble those that have been reported in other lesions. It is important to be aware of the spectrum of MR findings of myositis ossificans when considering the differential diagnosis of a soft tissue mass. (orig./MG)

  11. Myositis Ossificans in a 4-year-old Child

    Directory of Open Access Journals (Sweden)

    HW Boon

    2010-11-01

    Full Text Available Sprengel’s shoulder and myositis ossificans (MO are rarely seen concomitantly. This report is about a rare case in a 4 year-old girl who presented with right shoulder deformity and pain associated with right proximal arm swelling.

  12. Myositis ossificans within the intercondylar notch treated arthroscopically

    International Nuclear Information System (INIS)

    Leung, Allen H.; Desai, Panna; Rybak, Leon D.; Rose, Donald J.

    2010-01-01

    We present a case of intraarticular myositis ossificans in the right knee of a child. Myositis ossificans (MO), though relatively rare in childhood and even more uncommon within a joint, should be included in the differential diagnosis of an intra-articular mass when indicated by the typical clinical, radiographic, and histologic findings. An 11-year-old male presented with a history of trauma to his right knee. Four weeks after the initial injury, an MRI demonstrated evidence of an ACL rupture with a ''cystic mass'' within the intercondylar notch along the anterior surface of the torn ligament. At subsequent arthroscopy, the mass noted on MRI was removed. The histology was consistent with MO. The authors believe this to be the first case of MO in the intercondylar notch detected by MRI, treated by arthroscopy, and confirmed by histology. (orig.)

  13. Myositis ossificans within the intercondylar notch treated arthroscopically

    Energy Technology Data Exchange (ETDEWEB)

    Leung, Allen H.; Desai, Panna [Hospital for Joint Diseases/New York University, Department of Pathology, New York, NY (United States); Rybak, Leon D. [Hospital for Joint Diseases/New York University, Department of Radiology, New York, NY (United States); Rose, Donald J. [Hospital for Joint Diseases/New York University, Department of Orthopedic Surgery, New York, NY (United States)

    2010-09-15

    We present a case of intraarticular myositis ossificans in the right knee of a child. Myositis ossificans (MO), though relatively rare in childhood and even more uncommon within a joint, should be included in the differential diagnosis of an intra-articular mass when indicated by the typical clinical, radiographic, and histologic findings. An 11-year-old male presented with a history of trauma to his right knee. Four weeks after the initial injury, an MRI demonstrated evidence of an ACL rupture with a ''cystic mass'' within the intercondylar notch along the anterior surface of the torn ligament. At subsequent arthroscopy, the mass noted on MRI was removed. The histology was consistent with MO. The authors believe this to be the first case of MO in the intercondylar notch detected by MRI, treated by arthroscopy, and confirmed by histology. (orig.)

  14. Myositis ossificans traumatica causing ankylosis of the elbow.

    Science.gov (United States)

    Kanthimathi, B; Udhaya Shankar, S; Arun Kumar, K; Narayanan, V L

    2014-12-01

    Myositis ossificans traumatica is an unusual complication following a muscle contusion injury. A significantly large myositic mass causing ankylosis of the elbow is even rarer. We report a 13-year-old boy who presented with a 14-month history of a fixed elbow with no movement and a palpable bony mass in the anterior aspect of the elbow. He had sustained significant trauma to the affected limb 1 month prior to onset of symptoms, which was managed by native massage and bandaging for 4 weeks. The clinicoradiological diagnosis was suggestive of myositis ossificans, and the myositic mass was completely excised. Histopathology revealed lamellar bone. The 2-year follow-up showed full function of the affected limb and no signs of recurrence. We report this case of clinical interest due to the unusually large myositic mass.

  15. Myositis ossificans progressiva : a report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Eun Ah; Lee, Sun Wha [Ewha Womans Univ., Seoul (Korea, Republic of). Coll. of Medicine; Han, Tae Il [Kyung Hee University, Seoul (Korea, Republic of). Coll. of Medicine

    1997-12-01

    Myositis ossificans progressive is rare hereditable disorder characterized progressiva heterotopic bone formation in connective tissue and muscles in association with congenital skeletal anomalies. We report the plain radiologic and MR findings of myositis ossificants progressiva in two children. One case showed discrete ossification in the right buttock, neck, and both chest walls on plain radiographs, while the other showed diffuse swelling of the left posterior neck, back, and buttock muscles, which was demonstrated on MR images. Both case showed associated anomalies in the hands and feet. (author). 8 refs., 2 figs.

  16. Myositis ossificans progressiva : a report of two cases

    International Nuclear Information System (INIS)

    Kim, Eun Ah; Lee, Sun Wha; Han, Tae Il

    1997-01-01

    Myositis ossificans progressive is rare hereditable disorder characterized progressiva heterotopic bone formation in connective tissue and muscles in association with congenital skeletal anomalies. We report the plain radiologic and MR findings of myositis ossificants progressiva in two children. One case showed discrete ossification in the right buttock, neck, and both chest walls on plain radiographs, while the other showed diffuse swelling of the left posterior neck, back, and buttock muscles, which was demonstrated on MR images. Both case showed associated anomalies in the hands and feet. (author). 8 refs., 2 figs

  17. Myositis Ossificans Progressiva in the Whole Spine: A Case Report

    Directory of Open Access Journals (Sweden)

    Ebrahim Ghayem Hasankhani

    2018-02-01

    Full Text Available Myositis ossificans progressiva is a rare inherited disease characterized by progressive ectopic ossifications associated with thumb and big toe anomalies. Ossification usually progresses from central to the peripheral, proximal to distal, cranial to caudal, and from dorsal to ventral directions and leading to activity limitation, significant eating disability, recurrent pulmonary infection, and atelectasis. In this report, we present a 7-year-old boy with a total spine stiffness (wooden spine seriously limited his activity of daily living.

  18. A Rare Case Of Non Traumatic Myositis Ossificans Circumscripta

    OpenAIRE

    Mahale, Yashwant J.; Vyawahare, Chaitanya S.; Dravid, Nandkishore V.; Upase, Aditya; Rathi, Romil

    2015-01-01

    Introduction: Myositis ossificans circumscripta is a benign non neoplastic ossifying tumor presenting with bone like osteoid tissue extraskelletaly amidst the muscle planes. This condition when not associated with trauma is very trivial and considering the way it mimics certain characteristics, it may be misunderstood as a malignant neoplasm, abscess or antibioma. The aetiology of this atraumatic condition is still indistinct and remains a question unsolved. We would like to report such a cas...

  19. Myositis ossificans circumscripta, secondary to high-velocity gunshot and fragment wound that causes sciatica.

    Science.gov (United States)

    Gokkus, Kemal; Sagtas, Ergin; Suslu, Feride Ekimler; Aydin, Ahmet Turan

    2013-10-17

    This report concerns an unusual cause of sciatica. The case presented is of a young man with myositis ossificans that resulted in sciatica and was treated with en bloc excision and low-dose radiotherapy and indomethacine. The aim of this study was to explain the different diagnostic properties of myositis ossificans around the hip and non-classic causes of sciatica.

  20. Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

    Energy Technology Data Exchange (ETDEWEB)

    Koob, Meriam; Durckel, Jean; Dosch, Jean-Claude; Dietemann, Jean-Louis [Hopital de Hautepierre, Service de Radiologie II, Hopitaux Universitaires, Strasbourg Cedex (France); Entz-Werle, Natacha [Hopitaux Universitaires, Hopital de Hautepierre, Service d' Onco-hematologie pediatrique, Strasbourg Cedex (France)

    2010-12-15

    Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

  1. Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

    International Nuclear Information System (INIS)

    Koob, Meriam; Durckel, Jean; Dosch, Jean-Claude; Dietemann, Jean-Louis; Entz-Werle, Natacha

    2010-01-01

    Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

  2. Myositis Ossificans Traumatica Causing Ankylosis of the Elbow

    OpenAIRE

    Kanthimathi, B.; Udhaya Shankar, S.; Arun Kumar, K.; Narayanan, V. L.

    2014-01-01

    Myositis ossificans traumatica is an unusual complication following a muscle contusion injury. A significantly large myositic mass causing ankylosis of the elbow is even rarer. We report a 13-year-old boy who presented with a 14-month history of a fixed elbow with no movement and a palpable bony mass in the anterior aspect of the elbow. He had sustained significant trauma to the affected limb 1 month prior to onset of symptoms, which was managed by native massage and bandaging for 4 weeks. Th...

  3. Myositis Ossificans of the Psoas Muscle After Compression Fracture of Lumbar Spine: CT and MR Imaging Findings

    International Nuclear Information System (INIS)

    Choi, Mi Nyong; Lee, Gyung Kyu; Suh, Kyung Jin

    2010-01-01

    Myositis ossificans is a benign, self-limiting and non-neoplastic development of heterotopic bone in skeletal muscle following trauma. Although myositis ossificans can occur anywhere in the body, psoas muscle involvement is very rare. To the best of our knowledge, CT and MR imaging findings of myositis ossificans in the psoas muscle secondary to lumbar spine fracture have not been reported in the radiological literature. In this article, we describe the CT and MR imaging findings of myositis ossificans of the psoas muscle after lumbar spine fracture in a 64-year-old man, and conduct a review of the relevant literature

  4. Myositis Ossificans of the Psoas Muscle After Compression Fracture of Lumbar Spine: CT and MR Imaging Findings

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Mi Nyong; Lee, Gyung Kyu [Hallym University College of Medicine, Hangang Sacred Heart Hospital, Seoul (Korea, Republic of); Suh, Kyung Jin [Dongguk University College of Medicine, Gyungju Hospital, Gyeongju (Korea, Republic of)

    2010-02-15

    Myositis ossificans is a benign, self-limiting and non-neoplastic development of heterotopic bone in skeletal muscle following trauma. Although myositis ossificans can occur anywhere in the body, psoas muscle involvement is very rare. To the best of our knowledge, CT and MR imaging findings of myositis ossificans in the psoas muscle secondary to lumbar spine fracture have not been reported in the radiological literature. In this article, we describe the CT and MR imaging findings of myositis ossificans of the psoas muscle after lumbar spine fracture in a 64-year-old man, and conduct a review of the relevant literature

  5. Myositis ossificans: radiologic evaluation of two cases with diagnostic computed tomograms

    International Nuclear Information System (INIS)

    Zeanah, W.R.; Hudson, T.M.

    1982-01-01

    Although most physicians associated myositis ossificans with recent, acute trauma, only 40%-60% of patients give such a history. The appearance of a soft tissue mass without a clear history of trauma may suggest a diagnosis of sarcoma, especially because results of a biopsy of the central portion of an area of myositis ossificans may yield immature, undifferentiated tissue resembling a sarcoma. Pain and rapid growth of a mass are more usual in myositis ossificans than in sarcomas, and careful inquiry may reveal stretching injury or chronic trauma associated with normal, vigorous, physical activities. Recognizing the characteristic histologic zoning phenomenon (immature tissue centrally surrounded by more mature tissue and a peripheral shell of benign bone) during the biopsy procedure permits the correct diagnosis of myositis ossificans. Plain radiographs or conventional tomograms may reflect this histologic zoning by demonstrating the typical, mature, outer shell of bone. Although additional radiographic studies are not usually necessary, they may be obtained when the mass is suspected to be a sarcoma. In two patients computed tomographic scans clearly demonstrated well-defined, peripheral shells of mature bone, diagnostic of myositis ossificans

  6. Surgical Treatment of Traumatic Myositis Ossificans of the Extensor Carpi Radialis Muscle in a Dog.

    Science.gov (United States)

    Morton, Bridget A; Hettlich, Bianca F; Pool, Roy R

    2015-07-01

    To report clinical signs, diagnostic imaging findings, and outcome in a dog with traumatic myositis ossificans of the origin of the extensor carpi radialis muscle. Clinical report. An 8-month-old intact female Irish Setter Dog. After radiographic and computed tomographic evaluation of an osseous proliferation arising from the cranial cortex of the right distal humeral diaphysis, the protruding bone was surgically removed and evaluated by histopathology. Traumatic myositis ossificans was successfully treated with surgical removal of the osseous proliferation resulting in improved postoperative range of motion of the right elbow joint. There was no evidence of lameness or abnormal bone regrowth associated with the surgical site radiographically at follow up. Surgical removal of a traumatic myositis ossificans lesion resulted in full return to function in a young, competitive show dog. © Copyright 2014 by The American College of Veterinary Surgeons.

  7. Adult Niemann-Pick disease type B with myositis ossificans: a case report

    Directory of Open Access Journals (Sweden)

    Russka Shumnalieva

    2016-07-01

    Full Text Available Niemann-Pick Disease (NPD is a rare autosomal recessive lysosomal lipid storage disorder. The disease is caused by gene mutations that affect the metabolism of sphingolipids. The dysfunctions cause sphingomyelin to accumulate in different organs. NPD includes forms with low and high levels of sphingomyelin. We report a case of a 34 year-old man with a family history of NPD type B who presented with hepatosplenomegaly, neurological deficiency, bone abnormalities, and myositis ossificans. The clinical, biochemical, and imaging data confirmed the combined diagnosis of NPD type B with myositis ossificans.

  8. Myositis ossificans around shoulder following military training programme

    Directory of Open Access Journals (Sweden)

    Mustafa C Kir

    2011-01-01

    Full Text Available The myositis ossificans around shoulder in military recruits are not reported yet. Three young male soldiers presented with complaints of palpable mass at the anterior aspect of shoulder; tenderness around the superior part of deltopectoral groove close to acromioclavicular joint; and restriction of shoulder motion. They also noticed ecchymosis and pain around the coracoid process and anterior shoulder region during regular firing exercises. Plain X-rays and computerized tomography showed extra-capsular, dense, irregular structure in the space between pectoralis and deltoid muscles which correlated with heterotopic bone. One patient refused surgical intervention because of the completion of his military serving period. Surgical excision was performed for the other two patients. During surgical exploration, both ossified masses were found in deltopectoral region and mostly in fibers of clavicular and acromial parts of deltoid muscle. Pathological reports confirmed the structure of masses as mature trabecular bone. Postoperatively indomethacin treatment and active shoulder exercises were started until the full range of motion was regained. Mini soft bag was used on the rifle contact area of the shoulder. No complications or recurrences were observed during the 24 months of followup period.

  9. A fatal case of metastatic squamous cell carcinoma in a patient with myositis ossificans traumatica.

    NARCIS (Netherlands)

    Vlasveld, I N; Scheper, H; Stalenhoef, J; Baas, J M; van Dissel, J

    Myositis ossificans traumatica is a rare disease associated with chronic wounds and fistulae. Chronic ulcers, fistulae and wounds can transform into squamous cell carcinoma, the so-called Marjolin's ulcer. We describe a rapid, progressive and fulminant course of a metastatic squamous cell carcinoma

  10. The use of intraosseous anesthesia in a patient with myositis ossificans progressiva.

    Science.gov (United States)

    Webb, M D; Wilson, C

    1996-01-01

    The case of a pediatric patient with myositis ossificans progressiva in whom it became increasingly difficult to obtain local anesthesia is presented. Intraosseous anesthesia was successful in allowing pain-free dental treatment to be completed. This approach should be considered in other patients who have limited mouth-opening ability due to injury or disease.

  11. Three-dimensional CT diagnosis of myositis ossificans of the sacrospinous ligament

    International Nuclear Information System (INIS)

    Agrons, G.A.; Markowitz, R.I.; Bronson, W.E.

    1993-01-01

    We present the case of a 4-year-old female with a complex fracture of the left hemipelvis who, on follow-up CT imaging, developed new ossific densities within the peripelvic soft tissues of the contralateral side. Three-dimensional surface reformations of the pelvis demonstrated myositis ossificans along the course of the right sacrospinous ligament, thus elucidating unsuspected ligamentous injury and implying prior instability. (orig.)

  12. Heterotopic ossification (myositis ossificans) in acquired immune deficiency syndrome. Detection by gallium scintigraphy

    International Nuclear Information System (INIS)

    Drane, W.E.; Tipler, B.M.

    1987-01-01

    A case of heterotopic ossification (myositis ossificans) secondary to the central nervous system complications of acquired immune deficiency syndrome (AIDS) is reported. Because of the overwhelming suspicion of infection in this patient, this diagnosis was not considered until a gallium scan revealed the typical findings of heterotopic ossification. Because of the increasing utilization of gallium imaging in the AIDS population, every imaging specialist should be aware of this potential disorder

  13. Three-dimensional CT diagnosis of myositis ossificans of the sacrospinous ligament

    Energy Technology Data Exchange (ETDEWEB)

    Agrons, G.A. (Children' s Hospital of Philadelphia, PA (United States)); Markowitz, R.I. (Children' s Hospital of Philadelphia, PA (United States)); Bronson, W.E. (Children' s Hospital of Philadelphia, PA (United States))

    1993-04-01

    We present the case of a 4-year-old female with a complex fracture of the left hemipelvis who, on follow-up CT imaging, developed new ossific densities within the peripelvic soft tissues of the contralateral side. Three-dimensional surface reformations of the pelvis demonstrated myositis ossificans along the course of the right sacrospinous ligament, thus elucidating unsuspected ligamentous injury and implying prior instability. (orig.)

  14. Ossificans myositis: inflammatory changes and contrast enhancement of adjacent bone shown by MR imaging

    International Nuclear Information System (INIS)

    David, H.; Jolles, E.; Le Friant, G.; Silvestre, A.; Sarrazin, J.L.; Gordoliani, Y.S.

    1995-01-01

    The authors report a case of ossificans myositis, in which magnetic resonance imaging (MRI) showed inflammatory changes of the adjacent bone. T 1 weighted fat saturation sequence with gadolinium injection showed enhancement of medullary and cortical bone. This potentially mistaking pattern must be known, to avoid mis diagnosing with malignant osseous tumor, specially before achievement of the characteristic pattern of zonal maturation and its calcified rim. (authors). 15 refs., 6 figs

  15. Early diagnosis of myositis ossificans with Tc-99m diphosphonate imaging

    International Nuclear Information System (INIS)

    Tyler, J.L.; Derbekyan, V.; Lisbona, R.

    1984-01-01

    Myositis ossificans is primarily a disorder of young adults, whereby an area of muscle mass undergoes progressive ossification. The authors review a case in which the patient's presentation was somewhat atypical, and where the course of disease was unusually prolonged. Examination of the soft tissue lesion using Tc-99m diphosphonate bone scans was helpful in establishing the diagnosis and in determining the full extent of the process early in its evolution

  16. Merits of magnetic resonance imaging (MRI) for the diagnosis of myositis ossificans circumscripta

    International Nuclear Information System (INIS)

    Bouchardy, L.; Garcia, J.

    1994-01-01

    A retrospective study of 5 cases, 4 of myositis ossificans circumscripta (MOC) and 1 of non-ossificans myositis, is presented. The clinical presentation was a painful soft-tissues swelling, and the final diagnosis was established by biopsy in 3 cases and clinical evolution in 2 cases. Different types of imaging techniques were performed: 4 MRI, 2 arteriographies, 3 Tc 99m scintigraphies, 3 US, 3 CT and plain film radiographs for all patients. MOC is a benign process (as opposed to myositis ossificans progressiva, which is an hereditary pathology with a fatal prognosis) with 3 phases of evolution: an acute or pseudo-inflammatory phase, a sub-acute or pseudo-tumoral phase and a chronic phase with a spontaneous healing. The radiologic diagnostic findings are dependent of the phase of the disease. The calcifications are seen earlier with scintigraphy than plain films, with US being less helpful. A heterogenous mass and calcifications are seen with CT. MRI allows the characterization of oedema during the acute phase and sometimes can exclude a malignant process. MRI is the best method for an early diagnosis, the differential diagnosis and to follow the evolution. (authors). 26 refs., 8 figs

  17. MRI features of myositis ossificans with X-ray and CT findings

    International Nuclear Information System (INIS)

    Gu Xiang; Bai Rongjie; Qu Hui; Cheng Xiaoguang; Li Yuang

    2009-01-01

    Objective: To investigate the MRI features of myositis ossificans, and to address the correlation with X-ray radiography and CT findings. Methods: X-ray films, CT and MRI of 36 documented cases of myositis ossificans were retrospectively analyzed, and the literatures were reviewed. Results: Of the 36 cases, 4 cases occurred in the elbow joint, 4 in the shoulder joint, 15 in the hip joint, 6 in the tibiofibula, 5 in the femur, 1 in the metatarsal bones, and 1 in the ilium, respectively. Irregular patchy or lamellar high density calcification or ossification could be seen in the soft tissue parenchym on X-ray films and CT scan. Cortical bone integrity was preserved in diaphysis. CT enhanced scan showed that the swollen parenchyma was not enhanced and there was no parenchyma mass. On the early and middle stages, MR T 1 WI and T 2 WI showed slice-shaped low signal in the peripheral parenchyma, but patchy high signal was found around the low signal on T 2 WI. STIR showed mixed high and low signals in the swollen parenchyma with unclear demarcation. The lesions showed low signal on MR T 1 WI and T 2 WI in the late stage, and there was no edema in peripheral parenehyma. MRI enhanced scan found that the swollen parenchyma showed no enhancement in all stages. Conclusions: The imaging features of myositis ossificans have some characteristics. Misdiagnosis could be avoided when the disease was evaluated with the course. (authors)

  18. Case report 488: Post-traumatic myositis ossificans mimicking a soft tissue neoplasm

    International Nuclear Information System (INIS)

    Ackerman, L.; Ramamurthy, S.; Jablokow, V.; Van Drunen, M.; Kaplan, E.

    1988-01-01

    A case of post-traumatic myosotis ossificans (MO) in a young man with only a vague history of preceding physical trauma to the area of the lesion was presented. Imaging modalities, including plain film radiography, CT, contrast angiography and bone scintigraphy, could not exclude a malignancy with a reliable degree of certainty. The biopsy specimen was consistent with MO but could easily be misinterpreted as a sarcomatous lesion. A diagnosis of MO was only established by a scrupulous analysis of all clinical, radiological, scintigraphic and microscopic findings and unnecessary extensive surgery for a malignancy was avoided. The correlative radiological and pathological features in distinguishing post-traumatic myositis ossificans from such lesions as soft tissue osteosarcoma or parosteal osteosarcoma were described and the meaning of the term zoning phenomenon was elucidated. It was emphasized that utilizing the zonal phenomenon (both pathologically and radiologically) ensures a correct diagnosis most of the time. However, it must be emphasized that this zoning phenomenon is not established in the early stages of post-traumatic myositis ossificans. (oerig./MG)

  19. Myositis ossificans progressiva: A clinico-radiological evaluation-Case report with brief review of literature

    Directory of Open Access Journals (Sweden)

    Nikhil Rathee

    2016-01-01

    Full Text Available Myositis ossificans progressiva/fibrodysplasia ossificans progressiva (MOP, is an autosomal dominant mesodermal tissue disorder, characterized by an initial period of inflammation and subsequent proliferation of fibrous tissue with the formation of ectopic bone tissue. The incidence of MOP is one case per two million people. The ectopic bone tissue formed is located in soft tissue mainly in the connective tissue of striated musculature. We report MOP in an 18-year old female who presented with multiple tender, hard swelling in various parts of the body associated with stiffness and limitations of movements. A literature review of the subject showed few similar case reports in the literature. We revisit the criteria for diagnosis and the essentials of management and treatment of MOP as it is rare being a rare condition, and treatment guidelines are not clear.

  20. Computed Tomography in the Diagnosis of Myositis Ossificans – Case Report

    International Nuclear Information System (INIS)

    Nitek, Żaneta; Czwojdziński, Adam; Wolf-Kuś, Alicja; Walecki, Jerzy

    2014-01-01

    The term myositis ossificans refers to the formation of ossifications in the muscles, ligaments and fascias, usually as a result of trauma. Fibrodysplasia ossificans progressiva is a rare genetic disease in which heterotopic ossifications appear in early childhood and are accompanied by feet and spine defects. We present a case of a 31-year-old woman with massive heterotopic ossifications who suffered multiple injuries. We would like to emphasize the role of computed tomography in the exact localization of ossifications. Thanks to the volume rendering techniques and 3D image reconstructions, it is possible to precisely determine the position of ossifications in relation to the internal organs and blood vessels, allowing to schedule the surgery to remove the lesions

  1. Heterotopic bone formation (myositis ossificans) and lower-extremity swelling mimicking deep-venous disease

    International Nuclear Information System (INIS)

    Orzel, J.A.; Rudd, T.G.; Nelp, W.B.

    1984-01-01

    A quadriplegic patient with a swollen leg was suspected of having deep-venous thrombosis, and was studied with radionuclide venography (RNV) and contrast venography. Focal narrowing of the femoral vein, seen on RNV, was due to extrinsic compression. Although soft-tissue radiographs were normal, Tc-99m diphosphonate imaging established the diagnosis of early heterotopic bone formation (myositis ossificans), which was responsible for the venous compression. Clinically this inflammatory process can mimic deep-venous thrombosis, and should be considered in evaluating patients at risk for both heterotopic bone formation and deep-venous thrombosis

  2. A case of myositis ossificans in the upper cervical spine of a young child.

    Science.gov (United States)

    Findlay, Iain; Lakkireddi, Prabhat Reddy; Gangone, Ravinder; Marsh, Gavin

    2010-12-01

    Case report. We present a case of myositis ossificans (MO) of the upper cervical spine in a young child. The literature is reviewed with the classification, etiology, and treatment of MO discussed. Calcification of joint capsule, muscle, cartilage, and ligaments is a well-known phenomenon and is known as myositis ossificans. It is very rarely seen in the head and neck, with no reports of MO of the soft tissues surrounding the first 2 cervical vertebrae. An 8-year-old boy presented with severe neck pain after a fall. He had had a similar neck injury 4 years before, but made a full recovery. Radiographs showed a large ossified lesion between the posterior elements of C1 and C2. After further imaging, a diagnosis of MO was made. The child was treated with simple analgesia and observation. With no evidence of neurologic compromise and minimal symptoms, there was no indication for surgical intervention. Although rare, MO should be suspected as one of the possible causes of persistent pain following cervical spine injury in children. We would advise a low threshold for cervical spine imaging in the child presenting with persistent neck pain and stiffness, even years after injury.

  3. Myositis ossificans circumscripta of the psoas muscle due to overuse in an adolescent gymnast.

    Science.gov (United States)

    Masquijo, Julio Javier; Sartori, Federico

    2014-11-01

    Myositis ossificans is a pseudoinflammatory tumour that originates from skeletal muscle and corresponds to a heterotopic, metaplastic, nonmalignant bone tumour. The purpose of this article is to report the case of myositis ossificans circumscripta (MOC) of the psoas muscle due to overuse in an adolescent gymnast. A 16-year-old female athlete presented at our outpatient orthopaedic clinic for evaluation of a 1-month history of low back pain. Initial plain radiographs were initially interpreted as negative, and laboratory values were normal. MRI imaging demonstrated a circumscribed mass with associated oedema in the psoas muscle. Computed tomography-guided percutaneous biopsy was performed and histology confirmed the diagnosis of MOC. Conservative treatment was initiated with rest and anti-inflammatory drugs (indomethacin). The patient had a resolution of pain and function after 3 months of conservative treatment. At 6 months' follow-up, MRI demonstrated complete resolution of the lesion and she gradually returned to her sports activity. At last follow-up she was asymptomatic. MOC is a rare lesion in the paediatric-adolescent population. To our knowledge, this is the first report of MOC in the psoas muscle produced by overuse. MRI is very sensitive in detecting oedema during the acute phase of the lesion. Conservative treatment should be considered, especially at the early stage of the disease. Spontaneous resolution can be expected in most cases.

  4. Myositis ossificans traumatica in young children: report of three cases and review of the literature

    International Nuclear Information System (INIS)

    Gindele, A.; Benz-Bohm, G.; Schwamborn, D.; Tsironis, K.

    2000-01-01

    Myositis ossificans traumatica (MOT) is a rare musculoskeletal disorder in young children. Clinical and imaging presentation in the early stage of disease makes it difficult to differentiate between infection and musculoskeletal neoplasms, particularly in the absence of a history of trauma. Three cases of MOT in children under the age of 10 years, two with inferential trauma, are presented and the findings on different imaging modalities are discussed with reference to the existing literature. While findings based on a single imaging technique, including MRI, may be rather non-specific and even misleading, the combination of different modalities can assist in the consideration of MOT as a possible diagnosis. For example, the demonstration of soft-tissue haematoma on US would suggest the traumatic origin. A rational imaging approach is proposed. (orig.)

  5. Myositis ossificans traumatica in young children: report of three cases and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Gindele, A.; Benz-Bohm, G. [Department of Diagnostic Radiology, Pediatric Radiology, University of Cologne, Medical School, Kerpener Strasse 68, 50 924 Cologne (Germany); Schwamborn, D. [Paediatric Oncology, University Children' s Hospital, Cologne (Germany); Tsironis, K. [Department of Surgery, Leverkusen Hospital, Cologne (Germany)

    2000-07-01

    Myositis ossificans traumatica (MOT) is a rare musculoskeletal disorder in young children. Clinical and imaging presentation in the early stage of disease makes it difficult to differentiate between infection and musculoskeletal neoplasms, particularly in the absence of a history of trauma. Three cases of MOT in children under the age of 10 years, two with inferential trauma, are presented and the findings on different imaging modalities are discussed with reference to the existing literature. While findings based on a single imaging technique, including MRI, may be rather non-specific and even misleading, the combination of different modalities can assist in the consideration of MOT as a possible diagnosis. For example, the demonstration of soft-tissue haematoma on US would suggest the traumatic origin. A rational imaging approach is proposed. (orig.)

  6. Myositis ossificans versus osteosarcoma: Is it possible to achieve differential diagnosis by skeletal scintiscanning?

    International Nuclear Information System (INIS)

    Toman, A.; Enderle, A.; Munz, D.L.

    1989-01-01

    The authors report the cases of two juvenile patients suffering for about three weeks from a painful swelling in the humerus region, who were to be examined for suspected osteosarcoma. In one patient, the X-ray picture at that time showed a discrete solidification in the soft tissue, the findings in the other patient were normal. Three-phase skeletal scintiscanning with Tc-99m MDP in the perfusion and in the blood pool phase revealed an only slightly enhanced, localised activity accumulation in the affected area. The image taken in the late static phase revealed a clearly defined, clearly enhanced activity accumulation, which was characterised by a marginal accentuation with relatively lower accumulation of the radiopharmaceutical in the center. This characteristic pattern of activity distribution can only be detected by means of a subtle, computer-aided control system. The specific distribution pattern indicated the possibility of myositis ossificans localisata. X-ray diagnostics and especially computed tomography verified this diagnosis. (orig./MG) [de

  7. Myositis ossificans: a false positive of malignancy in PET - CT F.D.G. and in osseous scintigraphy with HMDP 99Tc

    International Nuclear Information System (INIS)

    Hassler, S.; Bourahla, K.

    2010-01-01

    Myositis ossificans is a difficult differential diagnosis and a false positive for malignancy in both 99m Tc H.M.D.P. (hydroxy-methylene diphosphonate) scintigraphy and in exploration PET-F.D.G. This hypothesis should be mentioned in case of young patients, despite the unusual scintigraphic appearance in a benign process. (N.C.)

  8. Ossificans myositis: inflammatory changes and contrast enhancement of adjacent bone shown by MR imaging; Myosite ossifiante circonscrite: remaniements osseux deceles en IRM

    Energy Technology Data Exchange (ETDEWEB)

    David, H.; Jolles, E.; Le Friant, G.; Silvestre, A.; Sarrazin, J.L.; Gordoliani, Y.S. [Hopital des Armees du Val-de-Grace, 75 - Paris (France)

    1995-07-01

    The authors report a case of ossificans myositis, in which magnetic resonance imaging (MRI) showed inflammatory changes of the adjacent bone. T 1 weighted fat saturation sequence with gadolinium injection showed enhancement of medullary and cortical bone. This potentially mistaking pattern must be known, to avoid mis diagnosing with malignant osseous tumor, specially before achievement of the characteristic pattern of zonal maturation and its calcified rim. (authors). 15 refs., 6 figs.

  9. Frequency of USP6 rearrangements in myositis ossificans, brown tumor, and cherubism: molecular cytogenetic evidence that a subset of ''myositis ossificans-like lesions'' are the early phases in the formation of soft-tissue aneurysmal bone cyst

    International Nuclear Information System (INIS)

    Sukov, William R.; Erickson-Johnson, Michele; Unni, K.K.; Wang, Xiaoke; Oliveira, Andre M.; Franco, Marcello F.; Chou, Margaret M.; Wenger, Doris E.

    2008-01-01

    USP6 rearrangements with several partner genes have been identified recently in primary but not in secondary aneurysmal bone cysts (ABCs). Several lesions show histologic features that may overlap with ABC, including myositis ossificans (MO), brown tumor, and cherubism. The objective of this study was to assess whether these lesions harbored USP6 rearrangements. Twelve patients with classic radiologic and histologic features of MO, 6 with brown tumors, and 5 with cherubism diagnosed at our institution were studied for the presence of USP6 rearrangements using fluorescence in situ hybridization with probes flanking the USP6 locus on chromosome 17p13. In addition, conventional cytogenetic analysis was performed in 2 patients with cherubism. USP6 rearrangements were identified in 2 patients with radiologic and histologic features consistent with MO. None of the patients with brown tumor or cherubism demonstrated USP6 rearrangements. Cytogenetic analysis of the cherubism patients demonstrated normal karyotypes. These findings indicate that a subset of cases with apparent classic histologic and imaging features of MO are rather better classified as being soft-tissue ABC with clonal USP6 rearrangements. In contrast, no USP6 rearrangements were found in patients with cherubism or brown tumor, supporting the prevailing view that these lesions are distinct biologic entities. (orig.)

  10. Focal myositis

    International Nuclear Information System (INIS)

    Kransdorf, M.J.; Temple, H.T.; Sweet, D.E.

    1998-01-01

    Focal myositis is a pseudotumor of soft tissue that typically occurs in the deep soft tissue of the extremities, and is a relatively rare lesion. There is a wide clinical spectrum, with approximately one-third of patients with focal myositis subsequently developing polymyositis, and clinical symptoms of generalized weakness, fever, myalgia, and weight loss, with elevation of creatine phosphokinase. We report the case of a patient with focal myositis who subsequently developed myositis ossificans-like features. (orig.)

  11. Myositis

    Science.gov (United States)

    Myositis means inflammation of the muscles that you use to move your body. An injury, infection, or ... weakness, plus a skin rash. Other symptoms of myositis may include Fatigue after walking or standing Tripping ...

  12. Focal myositis

    Energy Technology Data Exchange (ETDEWEB)

    Kransdorf, M.J. [Saint Mary`s Hospital, Richmond, VA (United States). Dept. of Radiol.]|[Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States); Temple, H.T. [Department of Orthopedic Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia (United States)]|[Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States); Sweet, D.E. [Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States)

    1998-05-01

    Focal myositis is a pseudotumor of soft tissue that typically occurs in the deep soft tissue of the extremities, and is a relatively rare lesion. There is a wide clinical spectrum, with approximately one-third of patients with focal myositis subsequently developing polymyositis, and clinical symptoms of generalized weakness, fever, myalgia, and weight loss, with elevation of creatine phosphokinase. We report the case of a patient with focal myositis who subsequently developed myositis ossificans-like features. (orig.) With 3 figs., 25 refs.

  13. Merits of magnetic resonance imaging (MRI) for the diagnosis of myositis ossificans circumscripta. Apport de l'imagerie par resonance magnetique (IRM) dans le diagnostic de la myosite ossifiante circonscrite (MOC)

    Energy Technology Data Exchange (ETDEWEB)

    Bouchardy, L.; Garcia, J. (Hopital Cantonal Geneve, Geneva (Switzerland))

    1994-02-01

    A retrospective study of 5 cases, 4 of myositis ossificans circumscripta (MOC) and 1 of non-ossificans myositis, is presented. The clinical presentation was a painful soft-tissues swelling, and the final diagnosis was established by biopsy in 3 cases and clinical evolution in 2 cases. Different types of imaging techniques were performed: 4 MRI, 2 arteriographies, 3 Tc 99m scintigraphies, 3 US, 3 CT and plain film radiographs for all patients. MOC is a benign process (as opposed to myositis ossificans progressiva, which is an hereditary pathology with a fatal prognosis) with 3 phases of evolution: an acute or pseudo-inflammatory phase, a sub-acute or pseudo-tumoral phase and a chronic phase with a spontaneous healing. The radiologic diagnostic findings are dependent of the phase of the disease. The calcifications are seen earlier with scintigraphy than plain films, with US being less helpful. A heterogenous mass and calcifications are seen with CT. MRI allows the characterization of oedema during the acute phase and sometimes can exclude a malignant process. MRI is the best method for an early diagnosis, the differential diagnosis and to follow the evolution. (authors). 26 refs., 8 figs.

  14. Myositis ossificans: a false positive of malignancy in PET - CT F.D.G. and in osseous scintigraphy with HMDP {sup 99}Tc;La myosite ossifiante: un faux positif de malignite en TEP-TDM FDG et en scintigraphie osseuse au HMDP Tc99

    Energy Technology Data Exchange (ETDEWEB)

    Hassler, S.; Bourahla, K. [CLCC Paul-Strauss, Service de medecine nucleaire, 67 - Strasbourg (France)

    2010-05-15

    Myositis ossificans is a difficult differential diagnosis and a false positive for malignancy in both {sup 99m}Tc H.M.D.P. (hydroxy-methylene diphosphonate) scintigraphy and in exploration PET-F.D.G. This hypothesis should be mentioned in case of young patients, despite the unusual scintigraphic appearance in a benign process. (N.C.)

  15. Frequency of USP6 rearrangements in myositis ossificans, brown tumor, and cherubism: molecular cytogenetic evidence that a subset of ''myositis ossificans-like lesions'' are the early phases in the formation of soft-tissue aneurysmal bone cyst

    Energy Technology Data Exchange (ETDEWEB)

    Sukov, William R.; Erickson-Johnson, Michele; Unni, K.K.; Wang, Xiaoke; Oliveira, Andre M. [Mayo Clinic, Department of Laboratory Medicine and Pathology, Rochester, MN (United States); Franco, Marcello F. [Universidade Federal do Estado de Sao Paulo (UNEFESP), Departamento de Patologia, Sao Paulo (Brazil); Chou, Margaret M. [University of Pennsylvania School of Medicine, Philadelphia, PA (United States); Wenger, Doris E. [Mayo Clinic, Department of Radiology, Rochester, MN (United States)

    2008-04-15

    USP6 rearrangements with several partner genes have been identified recently in primary but not in secondary aneurysmal bone cysts (ABCs). Several lesions show histologic features that may overlap with ABC, including myositis ossificans (MO), brown tumor, and cherubism. The objective of this study was to assess whether these lesions harbored USP6 rearrangements. Twelve patients with classic radiologic and histologic features of MO, 6 with brown tumors, and 5 with cherubism diagnosed at our institution were studied for the presence of USP6 rearrangements using fluorescence in situ hybridization with probes flanking the USP6 locus on chromosome 17p13. In addition, conventional cytogenetic analysis was performed in 2 patients with cherubism. USP6 rearrangements were identified in 2 patients with radiologic and histologic features consistent with MO. None of the patients with brown tumor or cherubism demonstrated USP6 rearrangements. Cytogenetic analysis of the cherubism patients demonstrated normal karyotypes. These findings indicate that a subset of cases with apparent classic histologic and imaging features of MO are rather better classified as being soft-tissue ABC with clonal USP6 rearrangements. In contrast, no USP6 rearrangements were found in patients with cherubism or brown tumor, supporting the prevailing view that these lesions are distinct biologic entities. (orig.)

  16. Fibrodysplasia ossificans progressiva

    OpenAIRE

    Tonholo-Silva, Edward R.; Adachi, Elza Aquimi; Tafner, Maria Salete; Yoshinaga, Lucia

    1994-01-01

    Fibrodisplasia (miosite) ossificante progressiva (FOP) é doença rara, de herança autossômica dominante, na qual ocorre ossificação ectópica progressiva e malformação esquelética, principalmente no tecido conectivo dos músculos. O diagnóstico é baseado nos achados clínicos e demonstração radiologica das malformações esqueléticas. Relatamos o caso de uma menina de 5 anos de idade com FOP. Fibrodysplasia (myositis) ossificans progressiva (FOP) is a rare autosomal dominant disorder in which th...

  17. Inflammatory focal myositis of the sternomastoid muscle: is there an absolute indication for biopsy? A case report and review of the literature

    NARCIS (Netherlands)

    Georgalas, Christos; Kapoor, Lekha; Chau, Ha; Bhattacharyya, Abir

    2006-01-01

    Focal myositis is a localised inflammatory process affecting skeletal muscles belonging to the pathological group of inflammatory pseudo tumours of soft tissue that includes myositis ossificans, proliferative myositis and nodular pseudosarcomatous fasciitis. Very rarely, it may affect one of the

  18. Post-traumatic myositis ossificans

    African Journals Online (AJOL)

    2014-12-09

    Dec 9, 2014 ... These features represent bone marrow surrounded by a low-signal-intensity ... radiographic or computed tomography (CT) evolution and MRI ... This is an important distinguishing feature from non-benign bone lesions.1.

  19. Periostitis ossificans.

    Science.gov (United States)

    VanDoorne, I; Soubry, R; Wackens, G; Goossens, A

    1995-09-01

    Mandibular periostitis ossificans was assessed in a 18-year-old black boy. This form of chronic osteomyelitis (Garré osteomyelitis) resulted from a periostitis on the 4.8 in association with an infected and ankylosed 4.7. Clinically a firm swelling of the right mandibular angle was noticeable. The diagnosis was confirmed by computed tomography and biopsy. After extraction of the causative teeth, in combination with an antibiotic treatment, a good evolution was obtained. The possible pathogenesis and the differential diagnosis are discussed.

  20. Fibrodysplasia ossificans circumscripta of the masseter muscle.

    Science.gov (United States)

    Geist, J R; Bhatti, P; Plezia, R A; Wesley, R K

    1998-05-01

    Fibrodysplasia ossificans circumscripta (FOC) is a lesion characterized by localized calcification of the investing fascia of skeletal muscle. It is often related to repeated trauma and was formerly known as traumatic myositis ossificans. Surgical procedures involving muscles are also believed to be a factor in the origin of the lesion. When FOC develops in the muscles of mastication it can lead to severe trismus. A case is reported of FOC in the left masseter muscle of a 44-year-old man who presented with marked limitation of opening. It was believed that previous fractures of the left maxilla and mandible and/or the subsequent surgical treatments were responsible for the onset of FOC. The radiographic and microscopic features of FOC are discussed in relation to recent theories on the etiology, pathogenesis, and treatment of the lesion.

  1. 5. Myositis Ossificans – Two Case Presentations

    African Journals Online (AJOL)

    Esem

    By time of presentation, ossification is extensive and the .... He also noticed that he was having problems in walking fast or to run. ... blood vessels in the operation field on the other; and .... of hip dislocation and increased to 34% when open. 2.

  2. NONBACTERIAL MYOSITIS

    OpenAIRE

    Crum-Cianflone, Nancy F.

    2010-01-01

    Infectious myositis is defined as an infection of a skeletal muscle. Infectious myositis is most commonly caused by bacteria; however, a variety of viral, parasitic, and fungal agents may also cause myositis. The pathogenesis of nonbacterial infectious myositis is via direct infection of the musculature or immune mechanisms. Symptoms typically include muscular pain, tenderness, swelling, and/or weakness. The diagnosis of the specific microbe is often suggested by the presence of concordant cl...

  3. An unusual case of lumbar paravertebral miositis ossificans mimicking muscular skeletal tumor.

    Science.gov (United States)

    Zoccali, C; Chichierchia, G; Covello, R

    2013-12-01

    Several lesions have clinical and radiological characteristics mimicking muscular skeletal tumor. Myositis ossificans usually presents a typical pattern making biopsy unnecessary; nevertheless, in rare cases, neoplasm must be ruled out. Biopsy is often sufficient to allow a diagnosis and a correct related treatment, but, unfortunately, sometimes it may lead to erroneous treatment. We report an unusual case of a lumbar paravertebral mass that had an MRI aspect similar to a chondrosarcoma, a histology pattern based on biopsy compatible with neurinoma and a definitive diagnosis of myosistis ossificans.

  4. [New insights of myositis-specific and -associated autoantibodies in juvenile and adult type myositis].

    Science.gov (United States)

    Váncsa, Andrea; Dankó, Katalin

    2016-07-01

    Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179-1184.

  5. Myositis Mimics.

    Science.gov (United States)

    Michelle, E Harlan; Mammen, Andrew L

    2015-10-01

    Patients with autoimmune myositis typically present with muscle weakness, elevated serum levels of muscle enzymes, and abnormal muscle biopsies. However, patients with other acquired myopathies or genetic muscle diseases may have remarkably similar presentations. Making the correct diagnosis of another muscle disease can prevent these patients from being exposed to the risks of immunosuppressive medications, which benefit those with myositis, but not those with other types of muscle disease. Here, we review some of the most common acquired and inherited muscle diseases that can mimic autoimmune myositis, including inclusion body myositis, limb girdle muscular dystrophies, metabolic myopathies, mitochondrial myopathies, and endocrine myopathies. We emphasize aspects of the medical history, physical exam, laboratory evaluation, and muscle biopsy analysis that can help clinicians distinguish myositis mimics from true autoimmune myositis.

  6. NONBACTERIAL MYOSITIS

    Science.gov (United States)

    Crum-Cianflone, Nancy F.

    2010-01-01

    Infectious myositis is defined as an infection of a skeletal muscle. Infectious myositis is most commonly caused by bacteria; however, a variety of viral, parasitic, and fungal agents may also cause myositis. The pathogenesis of nonbacterial infectious myositis is via direct infection of the musculature or immune mechanisms. Symptoms typically include muscular pain, tenderness, swelling, and/or weakness. The diagnosis of the specific microbe is often suggested by the presence of concordant clinical signs and symptoms, a detailed medical/travel history, and laboratory data. For example, immunocompromised hosts have a heightened risk of fungal myositis, whereas the presence of a travel history to an endemic location and/or eosinophilia may suggest a parasitic cause. Definitive diagnosis requires detecting the organism by specific laboratory testing including serologies, histopathology, and/or cultures. Treatment entails antimicrobial agents against the pathogen, with consideration for surgical drainage for focal purulent collections within the musculature. PMID:21308520

  7. Fibrodysplasia ossificans progressiva

    Energy Technology Data Exchange (ETDEWEB)

    Mahboubi, S. [Dept. of Radiology, Children' s Hospital of Philadelphia, PA (United States); Children Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States); Glaser, D.L. [Dept. of Orthopedic Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States); Shore, E.M. [Dept. of Orthopedic Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States); Dept. of Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States); Kaplan, F.S. [Dept. of Orthopedic Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States); Department of Medicine, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania (United States)

    2001-05-01

    Fibrodysplasia ossificans progressiva (FOP) is an extremely rare and disabling genetic disorder of connective tissue. The condition is characterized by congenital malformation of the great toes and by progressive heterotopic ossification of the tendons, ligaments, fasciae, and striated muscles. Fibrodysplasia ossificans progressiva occurs sporadically and is transmitted as a dominant trait with variable expression and complete penetrance. Reproductive fitness is low. There are fewer than 150 known patients with the disorder in the United States. A point prevalence of one affected patient in every 2 million of population has been observed. There is no sexual, racial, or ethnic predilection. The disease presents in early life; its course is unavoidably progressive. Most patients are confined to a wheelchair by the third decade of life and often succumb to pulmonary complications in the 5th/6th decade of life. At present there is no effective prevention or treatment. The recent discovery of overproduction of bone morphogenetic protein-4 in lesional cells and lymphocytic cells of affected patients provides a clue to both the underlying pathophysiology and potential therapy. The FOP gene has recently been mapped to human chromosome 4 q 27-31. (orig.)

  8. Fibrodysplasia ossificans progressiva

    International Nuclear Information System (INIS)

    Mahboubi, S.; Glaser, D.L.; Shore, E.M.; Kaplan, F.S.

    2001-01-01

    Fibrodysplasia ossificans progressiva (FOP) is an extremely rare and disabling genetic disorder of connective tissue. The condition is characterized by congenital malformation of the great toes and by progressive heterotopic ossification of the tendons, ligaments, fasciae, and striated muscles. Fibrodysplasia ossificans progressiva occurs sporadically and is transmitted as a dominant trait with variable expression and complete penetrance. Reproductive fitness is low. There are fewer than 150 known patients with the disorder in the United States. A point prevalence of one affected patient in every 2 million of population has been observed. There is no sexual, racial, or ethnic predilection. The disease presents in early life; its course is unavoidably progressive. Most patients are confined to a wheelchair by the third decade of life and often succumb to pulmonary complications in the 5th/6th decade of life. At present there is no effective prevention or treatment. The recent discovery of overproduction of bone morphogenetic protein-4 in lesional cells and lymphocytic cells of affected patients provides a clue to both the underlying pathophysiology and potential therapy. The FOP gene has recently been mapped to human chromosome 4 q 27-31. (orig.)

  9. Focal myositis

    International Nuclear Information System (INIS)

    Galloway, H.R.; Dahlstrom, J.E.; Bennett, G.M.

    2001-01-01

    Focal myositis is a rare, benign focal inflammation of muscle. The lesion often presents as a mass that may be mistaken for a soft tissue sarcoma. This report describes the MRI and histopathological features of a case and illustrates how the diagnosis may be suspected on the basis of the MR findings. Copyright (2001) Blackwell Science Pty Ltd

  10. Fibrodysplasia ossificans progressiva (FOP): watch the great toes!

    Science.gov (United States)

    Kartal-Kaess, Mutlu; Shore, Eileen M; Xu, Meiqi; Schwering, Ludwig; Uhl, Markus; Korinthenberg, Rudolf; Niemeyer, Charlotte; Kaplan, Frederick S; Lauten, Melchior

    2010-11-01

    Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder and the most disabling condition of heterotopic (extraskeletal) ossification in humans. Extraskeletal bone formation associated with inflammation preceding the osseous conversion usually begins in the first decade, predominantly in the head, neck, and shoulders. All patients have malformed great toes. Most patients have a spontaneous mutation of the ACVR1 gene. We report a 17-year-old girl with malformed great toes who had her first episode of heterotopic ossification and impaired mobility of the left hip at the age of 13 years. No inflammatory fibroproliferative masses preceded the onset of heterotopic ossification. Radiographic studies demonstrated myositis ossificans, but failure to associate the great toe malformation with heterotopic ossification led to a failure to diagnose FOP. She underwent repeated and unnecessary operative procedures to remove a recurrent lesion. FOP was finally suspected when the great toe malformation was correlated with the trauma-induced heterotopic ossification. Genetic analysis confirmed the presence of the classic FOP mutation (ACVR1 c.617G>A; R206H). This case highlights the importance of examining the great toes in anyone with heterotopic ossification. The association of malformations of the great toe with heterotopic ossification in all cases of classic FOP will lead to prompt clinical diagnosis and the prevention of iatrogenic harm.

  11. Pre- and post-therapy MR imaging in fibrodysplasia ossificans progressiva

    Energy Technology Data Exchange (ETDEWEB)

    Merchant, Rashid; Walawalkar, Avinash [Dr. Balabhai Nanavati Hospital and Research Centre, Department of Pediatrics, Mumbai (India); Sainani, Nisha I.; Lawande, Malini A.; Pungavkar, Sona A.; Patkar, Deepak P. [Dr. Balabhai Nanavati Hospital and Research Centre, Department of MRI, Mammography and BMD, Mumbai (India)

    2006-10-15

    Fibrodysplasia ossificans progressiva, also known as myositis ossificans progressiva, is characterized by congenital skeletal malformations and progressive ectopic bone formation in connective tissues. The disorder presents as rapidly growing masses usually in the neck or paraspinal region with stiffness in the adjoining joints. The preosseous lesions involve the fascia, ligaments, tendons, and skeletal muscle. These lesions occasionally resolve but more often progress to form ectopic ossification. We present a boy who had a characteristic clinical presentation. Magnetic resonance (MR) imaging conducted in the preosseous stage of the lesion revealed the pathology, resulting in early therapy and resolution of the preosseous lesion without progression to ossification. To the best of our knowledge, post-therapy follow-up MR imaging in such a case has not been reported. (orig.)

  12. An atypical presentation of myositis ossificans | Bultheel | South ...

    African Journals Online (AJOL)

    South African Journal of Sports Medicine. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 28, No 1 (2016) >. Log in or Register to get access to full text downloads.

  13. Case study An atypical presentation of myositis ossificans

    African Journals Online (AJOL)

    formation of heterotopic non-neoplastic bone in muscle or soft tissue.[1] ... aspect of the lesion upon imaging the diagnosis of end stage MO was made. ... 1 Anteroposterior (A) and frog-leg lateral (B) radiographs of the right hip showing mature ...

  14. Self-resolving focal non-ossifying myositis: a poorly known clinical and imaging entity diagnosed with MRI

    International Nuclear Information System (INIS)

    Perlepe, Vasiliki; Dallaudière, Benjamin; Omoumi, Patrick; Hristova, Lora; Rezzazadeh, Afshin; Vande Berg, Bruno; Malghem, Jacques; Lecouvet, Frederic

    2015-01-01

    Focal myositis is a rare benign inflammatory pseudotumor, presenting as a painful nodular mass within a muscle, and characterized by spontaneous resolution within weeks. To assess the clinical and imaging findings of focal nodular myositis simulating a neoplasm at clinical examination, with no history of trauma. This study describes the locations and appearance at ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) of this condition in a series of five patients. MRI and US displayed a solid intramuscular “tumor” and suggested a continuum between the proximal and distal muscle fibers that appeared thickened within the nodular lesion, a sign that has been reported in myositis ossificans. MRI showed edema in adjacent muscles and soft tissues, as well as intense enhancement of the mass. Intense vascular flows were seen at Doppler analysis. CT did not reveal the appearance of peripheral ossifications, ruling out the diagnosis of myositis ossificans. In some patients, the diagnosis of sarcoma had been suggested as possible by the radiologist. Imaging follow-up with MRI showed complete resolution of the masses over several weeks, thus avoiding a biopsy; no recurrence was observed at long-term follow-up (more than 24 months). This paper highlights MRI and US findings in focal non-ossifying myositis, and emphasizes the role of MRI in suggesting this diagnosis, leading to the careful follow-up of the lesion until its resolution, and ruling out more aggressive lesions

  15. Fibrodysplasia ossificans progressiva: case report

    Directory of Open Access Journals (Sweden)

    NUCCI ANAMARLI

    2000-01-01

    Full Text Available Fibrodysplasia ossificans progressiva is a rare genetic disease characterized by widespread soft tissue ossification and congenital stigmata of the extremities. We report on a male child followed for ten years since the age of 3 years and 9 months, when the diagnosis was made. He was born with bilateral hypoplasic hallux valgus and ventricular septal defect, corrected by transsternal approach when 32 months old. Restriction of neck mobility followed and foci of ectopic ossification appeared. Four crises of disease exacerbation were treated with oral prednisone and/or other antiinflammatory drugs. Sodium etidronate 5 to 10 mg/kg/day was prescribed intermittently during about six years but was discontinued due to osteopenia. The disease course has been relentless, with severe movement restriction including the chest wall. A review showed few similar case reports in the Brazilian literature. We revisit the criteria for diagnosis and the essentials of management and treatment.

  16. Nontraumatic terminal ileal perforation

    Directory of Open Access Journals (Sweden)

    Wani Rauf A

    2006-03-01

    Full Text Available Abstract Background There is still confusion and controversy over the diagnosis and optimal surgical treatment of non traumatic terminal ileal perforation-a cause of obscure peritonitis. Methods This study was a prospective study aimed at evaluating the clinical profile, etiology and optimal surgical management of patients with nontraumatic terminal ileal perforation. Results There were 79 cases of nontraumatic terminal ileal perforation; the causes for perforation were enteric fever(62%, nonspecific inflammation(26%, obstruction(6%, tuberculosis(4% and radiation enteritis (1%. Simple closure of the perforation (49% and end to side ileotransverse anastomosis(42% were the mainstay of the surgical management. Conclusion Terminal ileal perforation should be suspected in all cases of peritonitis especially in developing countries and surgical treatment should be optimized taking various accounts like etiology, delay in surgery and operative findings into consideration to reduce the incidence of deadly complications like fecal fistula.

  17. Nontraumatic orbital roof encephalocele.

    Science.gov (United States)

    Hoang, Amber; Maugans, Todd; Ngo, Thang; Ikeda, Jamie

    2017-02-01

    Intraorbital meningoencephaloceles occur most commonly as a complication of traumatic orbital roof fractures. Nontraumatic congenital orbital meningoncephaloceles are very rare, with most secondary to destructive processes affecting the orbit and primary skull defects. Treatment for intraorbital meningoencephaloceles is surgical repair, involving the excision of herniated brain parenchyma and meninges and reconstruction of the osseous defect. Most congenital lesions present in infancy with obvious globe and orbital deformities; we report an orbital meningoencephalocele in a 3-year-old girl who presented with ptosis. Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  18. Recurrent Bilateral Focal Myositis.

    Science.gov (United States)

    Nagafuchi, Hiroko; Nakano, Hiromasa; Ooka, Seido; Takakuwa, Yukiko; Yamada, Hidehiro; Tadokoro, Mamoru; Shimojo, Sadatomo; Ozaki, Shoichi

    This report describes a rare case of recurrent bilateral focal myositis and its successful treatment via methotrexate. A 38-year-old man presented myalgia of the right gastrocnemius in May 2005. Magnetic resonance imaging showed very high signal intensity in the right gastrocnemius on short-tau inversion recovery images. A muscle biopsy revealed inflammatory CD4+ cell-dominant myogenic change. Focal myositis was diagnosed. The first steroid treatment was effective. Tapering of prednisolone, however, repeatedly induced myositis relapse, which progressed to multiple muscle lesions of both lower limbs. Initiation of methotrexate finally allowed successful tapering of prednisolone, with no relapse in the past 4 years.

  19. Fibrodysplasia ossificans progressiva: a case report | Baidoo ...

    African Journals Online (AJOL)

    ... in unyielding new bone leading to disability and ultimately death from cardiorespiratory failure. The present case brings to light the delays and potential pitfalls in diagnosis as a result of the rarity of the condition. Keywords: Fibrodysplasia Ossificans Progressiva, ACVR1, Extra-skeletal ossification, Hallux valgus, Steroids.

  20. Inclusion-Body Myositis: Diagnosis

    Science.gov (United States)

    ... for MDA Blog Donate Search MDA.org Close Inclusion-Body Myositis (IBM) Diagnosis As with other muscle diseases, a doctor diagnoses inclusion-body myositis (IBM) by considering the individual’s personal ...

  1. Idiopathic inflammatory myositis.

    Science.gov (United States)

    Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

    2016-02-01

    Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. Crown Copyright © 2016. Published by Elsevier Ltd. All rights reserved.

  2. Benign acute childhood myositis.

    Science.gov (United States)

    Rajajee, Sarala; Ezhilarasi, S; Rajarajan, K

    2005-05-01

    To describe the clinical and laboratory features of benign acute childhood myositis. 40 children of BACM were seen during October 2001 to February 2002, 22 (52%) were male with mean age of 5.3 years. Duration of illness was 3.97 days. Preceding symptoms included fever, leg pain, vomiting and inability to walk. A provisional diagnosis of viral myositis was made in 26 (66%). Guillian Barre Syndrome was the most common referral diagnosis. 11 (27.5%) children had leucopenia with lymphocytic response and 16 (40%) had thrombocytopenia. CRP was negative in 32 (80%). CPK was markedly elevated (more than 1000 IU/l) in 18 (45%) and more than 500 IU/l in 11 (27.5%) remaining between 200 to 500 IU/l. Associated features were hepatitis (elevated SGOT & SGPT) in 28 (70%) and shock in 5 (12.5%). Serological test were indicative of dengue virus (Elisa PAN BIO) in 20 (50%) of which 8 (25%) were primary dengue and 12 (30%) were secondary dengue. The outcome of therapy mainly supportive were excellent. Benign acute myositis occurs often in association with viral infection. In the present study, Dengue virus was positive in 20 (50%) children. Benign acute myositis can be differentiated from more serious causes of walking difficulty by presence of calf and thigh muscle tenderness on stretching, normal power and deep tendon reflex and elevated CPK.

  3. [Autoantibody profile in myositis].

    Science.gov (United States)

    Allenbach, Y; Benveniste, O

    2014-07-01

    Patients suffering from muscular symptoms or with an increase of creatine kinase levels may present a myopathy. In such situations, clinicians have to confirm the existence of a myopathy and determine if it is an acquired or a genetic muscular disease. In the presence of an acquired myopathy after having ruled out an infectious, a toxic agent or an endocrine cause, physicians must identify which type of idiopathic myopathy the patient is presenting: either a myositis including polymyositis, dermatomyositis, and inclusion body myositis, or an immune-mediated necrotizing myopathy. Histopathology examination of a muscle biopsy is determinant but detection of autoantibody is now also crucial. The myositis-specific antibodies and myositis-associated antibodies lead to a serologic approach complementary to the histological classification, because strong associations of myositis-specific antibodies with clinical features and survival have been documented. The presence of anti-synthetase antibodies is associated with an original histopathologic pattern between polymyositis and dermatomyositis, and defines a syndrome where interstitial lung disease drives the prognosis. Anti-MDA-5 antibody are specifically associated with dermatomyositis, and define a skin-lung syndrome with a frequent severe disease course. Anti-TIF1-γ is also associated with dermatomyositis but its presence is frequently predictive of a cancer association whereas anti-MI2 is associated with the classical dermatomyositis. Two specific antibodies, anti-SRP and anti-HMGCR, are observed in patients with immune-mediated necrotizing myopathies and may be very useful to distinguish acquired myopathies from dystrophic muscular diseases in case of a slow onset and to allow the initiation of effective therapy. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  4. Inclusion body myositis.

    OpenAIRE

    Garlepp, M J; Mastaglia, F L

    1996-01-01

    The idiopathic inflammatory myopathies (IIM) are a heterogenous group of rare disorders that share many similarities. In addition to sporadic inclusion body myositis (IBM), these include dematomyositis (DM), polymyositis (PM), and autoimmune necrotizing myopathy (NM). For discussion of later three disorders, the reader is referred to the IIM review in this issue. IBM is the most common IIM after age 50. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric mus...

  5. Viral myositis in children.

    Science.gov (United States)

    Magee, Haley; Goldman, Ran D

    2017-05-01

    Question I recently evaluated a child in my clinic after an emergency department visit where she presented having woken up that morning refusing to walk and was crawling around the house. The parents reported she was getting over a cold, and I recall similar cases of myositis during the H1N1 influenza epidemic a few years ago. What are the key features of myositis that I should recognize? Which investigations are needed to confirm the diagnosis and how should affected patients be managed? Answer Benign acute childhood myositis is a mild and self-limited sudden onset of lower extremity pain during or following recovery from a viral illness. Presentation can include tiptoe gait or refusal to walk, secondary to symmetric bilateral lower extremity pain that resolves quickly, usually within 3 days. In general, no investigation is needed except in severe cases for which screening bloodwork and a urine myoglobin test can confirm the diagnosis and rule out complications. Myoglobinuria and highly elevated creatine phosphokinase levels are rare but should be a consideration for admission to hospital. Prognosis is excellent and management might include rest and analgesia. Copyright© the College of Family Physicians of Canada.

  6. Focal myositis: A review.

    Science.gov (United States)

    Devic, P; Gallay, L; Streichenberger, N; Petiot, P

    2016-11-01

    Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of focal myositis is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern. MRI reveals a contrast enhanced enlarged muscle appearing hyper-intense on FAT-SAT T2 weighted images. Adjacent structures are spared and there are no calcifications. Serum creatine kinase (CK) levels are usually moderately augmented and biological markers of systemic inflammation are absent in most cases. Pathological histological features include marked variation in fibre size, inflammatory infiltrates mostly composed of T CD4+ lymphocytes and macrophages, degenerating/regenerating fibres and interstitial fibrosis. Differential diagnoses are numerous and include myositis of other origin with focal onset. Steroid treatment should be reserved for patients who present with major pain, nerve lesions, associated autoimmune disease, or elevated C reactive protein or CK. Copyright © 2016 Elsevier B.V. All rights reserved.

  7. Inclusion body myositis.

    Science.gov (United States)

    Dimachkie, Mazen M; Barohn, Richard J

    2014-08-01

    The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare disorders that share many similarities. In addition to sporadic inclusion body myositis (IBM), these include dermatomyositis, polymyositis, and autoimmune necrotizing myopathy. IBM is the most common IIM after age 50 years. Muscle histopathology shows endomysial inflammatory exudates surrounding and invading nonnecrotic muscle fibers often accompanied by rimmed vacuoles and protein deposits. It is likely that IBM is has a prominent degenerative component. This article reviews the evolution of knowledge in IBM, with emphasis on recent developments in the field, and discusses ongoing clinical trials. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Computed tomography of orbital myositis

    International Nuclear Information System (INIS)

    Dresner, S.C.; Rothfus, W.E.; Slamovits, T.L.; Kennerdell, J.S.; Curtin, H.D.

    1984-01-01

    The computerized tomographic (CT) scans of 11 consecutive patients with orbital myositis were reviewed to better characterize the CT appearance of this condition. The findings in this series differed from those of previous reports in several ways. Multiple muscle involvement predominated. Bilateral involvement was more frequent than previously reported. Enlargement of the tendon as well as the muscle was a frequent finding, but a normal tendinous insertion did not preclude the diagnosis of orbital myositis. Although the CT appearance of orbital myositis is often helpful, the findings are not pathognomonic; correlation with history, clinical findings, and therapeutic response must be considered in making the diagnosis

  9. The EuroMyositis registry

    DEFF Research Database (Denmark)

    Lilleker, James B; Vencovsky, Jiri; Wang, Guochun

    2018-01-01

    AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtyp...

  10. INCLUSION BODY MYOSITIS

    Directory of Open Access Journals (Sweden)

    Luh Yeni Laksmini

    2014-09-01

    Full Text Available Inclusion body myositis (IBM merupakan penyakit inflamasi pada otot yang bersifat progresif dengan penyebab yang tidak diketahui dan tidak menunjukkan respon yang baik terhadap berbagai terapi. Gambaran histopatologi IBM ditandai dengan infiltrat sel-sel limfosit diantara ruangan endomisial, di dalam otot dan di sekitar otot dengan fokus-fokus inklusi di dalam miosit (rimmed vacuole serta beberapa serat otot terlihat atrofi dan nekrosis. Dilaporkan wanita, usia 46 tahun dengan IBM. Keluhan utama pasien berupa kelemahan pada kedua tangan, kaki kanan terasa berat jika diangkat sehingga susah berjalan. Pemeriksaan saraf sensorik ekstremitas dekstra dan sinistra dalam batas normal. Pemeriksaan enzim cretinine kinase meningkat secara dramatik. Pemeriksaan histopatologi dari biospi otot gastrocnemius menunjukkan gambaran yang sesuai untuk IBM dan telah dilakukan penanganan dengan pemberian oral methilprednisolon 3x32 mg dan mecobalmin 1x500ìg intravena, namun tidak menunjukkan respon yang baik terhadap terapi dan akhirnya pasien meninggal. [MEDICINA 2013;44:118-123].

  11. Iliopsoas myositis mimicking appendicitis: MRI diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Wysoki, M.G. [Department of Radiology, Medical College of Pennsylvania Hospital and Hahnemann University, 3300 Henry Avenue, Philadelphia PA 19129 (United States); Angeid-Backman, E. [Department of Radiology, Medical College of Pennsylvania Hospital and Hahnemann University, 3300 Henry Avenue, Philadelphia PA 19129 (United States); Izes, B.A. [Department of Radiology, Medical College of Pennsylvania Hospital and Hahnemann University, 3300 Henry Avenue, Philadelphia PA 19129 (United States)

    1997-05-01

    Myositis of the truncal muscles can closely mimic acute appendicitis. Myositis is the early stage of muscular infection. It is characterized by diffuse muscular pain and swelling without a distinct mass. Early diagnosis of myositis improves the outcome and surgical debridement is usually avoided. Pyomyositis, the advanced stage of the disease, can be diagnosed by MRI examination. We present a case of early bacterial myositis that was diagnosed by MRI. (orig.). With 3 figs.

  12. Iliopsoas myositis mimicking appendicitis: MRI diagnosis

    International Nuclear Information System (INIS)

    Wysoki, M.G.; Angeid-Backman, E.; Izes, B.A.

    1997-01-01

    Myositis of the truncal muscles can closely mimic acute appendicitis. Myositis is the early stage of muscular infection. It is characterized by diffuse muscular pain and swelling without a distinct mass. Early diagnosis of myositis improves the outcome and surgical debridement is usually avoided. Pyomyositis, the advanced stage of the disease, can be diagnosed by MRI examination. We present a case of early bacterial myositis that was diagnosed by MRI. (orig.). With 3 figs

  13. Fibrodysplasia ossificans progressive: a case report and radiographic findings

    International Nuclear Information System (INIS)

    Araujo Junior, Cyrillo Rodrigues de; Carvalho, Tarcisio Nunes; Costa, Marlos Augusto Bittencourt; Lobo, Leonardo Valadares; Fonseca, Cristiano Rezio; Teixiera, Kim-Ir-Sem Santos

    2001-01-01

    Fibrodysplasia ossificans progressive is a rare hereditary connective tissue disease characterized by disseminated soft tissue ossification and congenital abnormality of the extremities. It is genetically inherited as a dominant trait with complete penetrance but variable expression. The onset takes place during childhood and the progressive involvement of the spine and proximal extremities leads to immobilization and articular deformity. We report a case of a 22-year-old male patient with typical symptoms of fibrodysplasia ossificans progressive and discuss the new advances in the diagnosis and pathophysiology. (author)

  14. Myositis specific autoantibodies; specificity and clinical applications.

    NARCIS (Netherlands)

    Hengstman, G.J.D.

    2005-01-01

    The sera of about half of the patients with myositis contain autoantibodies that are specific for this group of diseases compared to other inflammatory connective tissue disorders. In a recent study we showed that these myositis specific autoantibodies (MSAs) are also specific for myositis as

  15. Morphoea with Myositis: A Rare Association

    Directory of Open Access Journals (Sweden)

    Mary Sommerlad

    2011-01-01

    Full Text Available In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy, who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea and myositis has not been described.

  16. Characteristic calcaneal ossification: an additional early radiographic finding in infants with fibrodysplasia ossificans progressiva

    Energy Technology Data Exchange (ETDEWEB)

    Hasegawa, Sachi [Nagoya University Graduate School of Medicine, Department of Orthopaedic Surgery, Nagoya, Aichi (Japan); Victoria, Teresa [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Kayserili, Huelya [Koc University School of Medicine (KUSOM), Medical Genetics Department, Istanbul (Turkey); Zackai, Elaine [Children' s Hospital of Philadelphia, Department of Medical Genetics, Philadelphia, PA (United States); Nishimura, Gen; Haga, Nobuhiko; Nakashima, Yasuharu; Miyazaki, Osamu [The Research Committee on Fibrodysplasia Ossificans Progressiva, Tokyo (Japan); Kitoh, Hiroshi [Nagoya University Graduate School of Medicine, Department of Orthopaedic Surgery, Nagoya, Aichi (Japan); The Research Committee on Fibrodysplasia Ossificans Progressiva, Tokyo (Japan)

    2016-10-15

    We have clinically encountered children with fibrodysplasia ossificans progressiva who had abnormal calcaneal ossification. To evaluate whether calcaneal ossification variants are significant radiographic findings in children with fibrodysplasia ossificans progressiva. Lateral feet radiographs in nine children who fulfilled the diagnostic criteria of fibrodysplasia ossificans progressiva were reviewed. The studies were obtained during infancy or early childhood. Fourteen lateral foot radiographs of fibrodysplasia ossificans progressiva were available for this study (ages at examination: 1-104 months). Four children ages 2 months to 11 months showed double calcaneal ossification centers; 7 children had plantar calcaneal spurs that decreased in size with age. Overall, eight of nine children with fibrodysplasia ossificans progressiva demonstrated double calcaneal ossifications and/or plantar calcaneal spurs in infancy or childhood. Double calcaneal ossification centers in early infancy and plantar calcaneal spurs in childhood are frequently seen in children with fibrodysplasia ossificans progressiva and may be a useful radiologic indicator for early diagnosis. (orig.)

  17. Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations.

    NARCIS (Netherlands)

    Hengstman, G.J.D.; Engelen, B.G.M. van; Venrooij, W.J.W. van

    2004-01-01

    PURPOSE OF REVIEW: Defined autoantibodies are found in about half of the patients with myositis. Traditionally, these autoantibodies have been divided into myositis specific autoantibodies (MSAs) and myositis associated autoantibodies. Several studies have shown that MSAs are associated with

  18. Potential Environmental Triggers of Myositis

    Science.gov (United States)

    2012-10-01

    expression disturbances in myositis patient samples at the whole genome level. With our collaborators at the Karolinska Institute and Genpathway, Inc. we... Karolinska Institute in Stockholm, Sweden. Aim 1 Methods: A total of 500 ng of genomic DNA was used for bisulfate conversion using a Bisulfite

  19. Inclusion body myositis. Clinical aspects

    NARCIS (Netherlands)

    Cox, Fieke Maria Elisabeth

    2014-01-01

    Sporadische inclusion body myositis (IBM) is een van de meest voor voorkomende verworven spierziekte die ontstaat na het 50e levensjaar. In dit proefschrift worden de klinische aspecten van sporadische IBM beschreven. Uit de studie met betrekking tot het natuurlijk beloop blijkt dat de ziekte niet

  20. Fibrodysplasia Ossificans Progressiva: Clinical and Genetic Aspects

    Directory of Open Access Journals (Sweden)

    Pignolo Robert J

    2011-12-01

    Full Text Available Abstract Fibrodysplasia ossificans progressiva (FOP is a severely disabling heritable disorder of connective tissue characterized by congenital malformations of the great toes and progressive heterotopic ossification that forms qualitatively normal bone in characteristic extraskeletal sites. The worldwide prevalence is approximately 1/2,000,000. There is no ethnic, racial, gender, or geographic predilection to FOP. Children who have FOP appear normal at birth except for congenital malformations of the great toes. During the first decade of life, sporadic episodes of painful soft tissue swellings (flare-ups occur which are often precipitated by soft tissue injury, intramuscular injections, viral infection, muscular stretching, falls or fatigue. These flare-ups transform skeletal muscles, tendons, ligaments, fascia, and aponeuroses into heterotopic bone, rendering movement impossible. Patients with atypical forms of FOP have been described. They either present with the classic features of FOP plus one or more atypical features [FOP plus], or present with major variations in one or both of the two classic defining features of FOP [FOP variants]. Classic FOP is caused by a recurrent activating mutation (617G>A; R206H in the gene ACVR1/ALK2 encoding Activin A receptor type I/Activin-like kinase 2, a bone morphogenetic protein (BMP type I receptor. Atypical FOP patients also have heterozygous ACVR1 missense mutations in conserved amino acids. The diagnosis of FOP is made by clinical evaluation. Confirmatory genetic testing is available. Differential diagnosis includes progressive osseous heteroplasia, osteosarcoma, lymphedema, soft tissue sarcoma, desmoid tumors, aggressive juvenile fibromatosis, and non-hereditary (acquired heterotopic ossification. Although most cases of FOP are sporadic (noninherited mutations, a small number of inherited FOP cases show germline transmission in an autosomal dominant pattern. At present, there is no definitive

  1. Ultrasonography and radiography to identify early post traumatic myosistis ossificans in an 18-year-old male: a case report.

    Science.gov (United States)

    Yochum, Alicia M; Reckelhoff, Kenneth; Kaeser, Martha; Kettner, Norman W

    2014-06-01

    The purpose of this case report is to describe a patient with post traumatic myositis ossificans (PTMO) of the anterior thigh following blunt trauma and discuss the incidence, clinical presentation, management, and imaging findings. An 18-year-old male presented to a chiropractic clinic with a chief complaint of left knee pain and reduced range of motion after an impact injury to his left anterior thigh during hurdling 6 weeks earlier. Immediately after the injury, he presented to the emergency department where radiography of the left knee was negative and he was diagnosed with a muscle sprain. Follow-up radiography and ultrasonography of the left knee in a chiropractic radiology department revealed ossification consistent with PTMO within his vastus intermedius. The patient underwent a course of rehabilitation for 2 months including ice, class IV cold laser and vibration applied to his anterior thigh, and myofascial release of his quadriceps musculature with targeted and progressive rehabilitative exercises. His left knee pain resolved within 2 weeks of care. He resumed sports participation (American football) pain-free, while wearing protective padding over the affected thigh, 1 month after presentation, which was approximately 2 1/2 months following his injury. This case demonstrates that ultrasonography may have the capability to detect early phases of PTMO approximately 2 weeks prior to radiographic evidence and to monitor progression throughout its course.

  2. Bacterial, Fungal, Parasitic, and Viral Myositis

    OpenAIRE

    Crum-Cianflone, Nancy F.

    2008-01-01

    Infectious myositis may be caused by a broad range of bacterial, fungal, parasitic, and viral agents. Infectious myositis is overall uncommon given the relative resistance of the musculature to infection. For example, inciting events, including trauma, surgery, or the presence of foreign bodies or devitalized tissue, are often present in cases of bacterial myositis. Bacterial causes are categorized by clinical presentation, anatomic location, and causative organisms into the categories of pyo...

  3. Myositis-specific and myositis-associated autoantibodies in Indian patients with inflammatory myositis.

    Science.gov (United States)

    Srivastava, Puja; Dwivedi, Sanjay; Misra, Ramnath

    2016-07-01

    We aimed to study the prevalence and clinical associations of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in a large cohort of Indian patients with idiopathic inflammatory myositis (IIM). Clinical details and serum samples were collected from patients with IIM (satisfying Bohan and Peter Criteria, 1975) and CTD-associated myositis. Sera were analysed for antibodies against SRP, Mi2, Jo1, PL7, PL12, EJ, OJ, Ro52, Ku, Pm-Scl 75 and PM-Scl 100, using immunoblot assay. The cohort comprised 124 patients with IIM (M:F = 1:3.6). Fifty-five of them had dermatomyositis (DM), 22 had juvenile dermatomyositis (JDM), 25 had polymyositis (PM) and 22 had connective tissue disease-associated myositis (CTD myositis). Mean disease duration was 10.9 months. ANA was positive in 84 (68.9 %), and MSAs in 61 (49.2 %) patients. Among MSAs, autoantibodies to Mi2, synthetase (Jo1, PL7, PL12, EJ) and SRP were present in 26 (20.9 %), 29 (23.4 %) and 6 (4.8 %) patients, respectively. Prevalence of MAAs was as follows: antibodies to Ro52 in 45 (36.3 %), Ku and PM-Scl 75 in 13 (10.5 %) and PM-Scl 100 in 5 (4 %) patients. Anti-Mi2 antibodies were positively associated with DM (21/55, 38.2 %; p < 0.0001) and pharyngeal weakness (13/34, 38.2 %; p = 0.004) and negatively associated with ILD (0/28; p = 0.001). ILD and mechanics' hands were significantly more in patients with anti-synthetase antibodies (16/28, 57 % and 14/22, 63.6 %; p < 0.0001). Four of six patients with anti-SRP antibody showed poor response to multiple drugs. Higher prevalence of anti-Mi2 is probably related to higher proportion of patients with DM. Absence of ILD in patients with anti-Mi2 antibody suggests that it may protect against ILD. In Indian population also, anti-synthetase antibodies are associated with ILD, and anti-SRP antibodies with poor response to treatment.

  4. Proliferative myositis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Sook; Jeon, Ho Jong [Chosun University College of Medicine, Gwangju (Korea, Republic of)

    2002-09-01

    We report a case of proliferative myositis arising in the pectoralis major muscle of a 59-year-old man who presented with palpable mass. The initial clinical impression was a malignant tumor. Ultrasonography revealed the lesion as a spindle-shaped hypoechoic mass, and MR imaging of the left pectoralis major muscle showed hypointensity at T1-weighted imaging, hyperintensity at T2-weighted imaging, and strong enhancement at contrast-enhanced T1-weighted imaging.

  5. Proliferative myositis: a case report

    International Nuclear Information System (INIS)

    Kim, Young Sook; Jeon, Ho Jong

    2002-01-01

    We report a case of proliferative myositis arising in the pectoralis major muscle of a 59-year-old man who presented with palpable mass. The initial clinical impression was a malignant tumor. Ultrasonography revealed the lesion as a spindle-shaped hypoechoic mass, and MR imaging of the left pectoralis major muscle showed hypointensity at T1-weighted imaging, hyperintensity at T2-weighted imaging, and strong enhancement at contrast-enhanced T1-weighted imaging

  6. Eosinophilic myositis: an updated review.

    Science.gov (United States)

    Selva-O'Callaghan, A; Trallero-Araguás, E; Grau, J M

    2014-01-01

    Eosinophilia-associated myopathies are clinically and pathologically heterogeneous conditions characterized by the presence of peripheral and/or muscle eosinophilia. There are at least three distinct subtypes: focal eosinophilic myositis, eosinophilic polymyositis, and eosinophilic perimyositis. Infiltrating eosinophils are not always identified in conventional muscle histologic examination, but the eosinophil major basic protein, whose extracellular diffusion is considered a hallmark of eosinophilic cytotoxicity, is usually detected by immunostaining in muscle biopsy. Whereas focal eosinophilic myositis seems to be a benign and isolated condition, and perimyositis is usually related with the inflammatory infiltrate due to fasciitis, eosinophilic polymyositis can be associated with muscular dystrophy or be a feature of multiorgan hypereosinophilic syndrome. Muscle biopsy remains the cornerstone for the diagnosis. Parasitic infections, connective tissue disorders, hematologic and non-hematologic malignancies, drugs, and toxic substances are the main etiologic agents of eosinophilia-associated myopathy. However, in some cases, no known etiologic factor is identified, and these are considered idiopathic. Glucocorticoids are the mainstay therapy in idiopathic forms. Imatinib and mepolizumab, a humanized anti-interleukin 5 monoclonal antibody, may be useful in patients with eosinophilic myositis as part of a hypereosinophilic syndrome. Copyright © 2014 Elsevier B.V. All rights reserved.

  7. Multimodality imaging of Candida tropicalis myositis

    Energy Technology Data Exchange (ETDEWEB)

    Schwartz, Daniel M. [Children' s Memorial Hospital, Department of Medical Imaging, 2300 Children' s Plaza, Box 9, Chicago, IL (United States); Morgan, Elaine R. [Children' s Memorial Hospital, Department of Hematology and Oncology, Chicago, IL (United States)

    2008-04-15

    Fungal myositis is a rare entity that has been described in immunocompromised patients. We present a boy with biopsy proven fungal myositis who was examined with multiple imaging modalities. MR imaging proved to be very effective for diagnostic purposes, while US imaging was able to provide guidance for biopsy. (orig.)

  8. Multimodality imaging of Candida tropicalis myositis

    International Nuclear Information System (INIS)

    Schwartz, Daniel M.; Morgan, Elaine R.

    2008-01-01

    Fungal myositis is a rare entity that has been described in immunocompromised patients. We present a boy with biopsy proven fungal myositis who was examined with multiple imaging modalities. MR imaging proved to be very effective for diagnostic purposes, while US imaging was able to provide guidance for biopsy. (orig.)

  9. Magnetic resonance imaging of infectious myositis

    Energy Technology Data Exchange (ETDEWEB)

    Yun, Ji Young; Kim, Jee Young; Kim, Sang Heum; Jung, Youn Ju; Cha, Eun Suk; Park, Joung Mi; Park, Young Ha [The Catholic Univ., College of Medicine, Suwon (Korea, Republic of)

    1998-09-01

    To describe the findings of magnetic resonance imaging in infectious myositis and to determine their value for differentiation between ruberculous and bacterial myositis. Magnetic resonance images of ten proven cases of infectious myositis (five tuberculous and five bacterial) were retrospectively reviewed in the light of clinical and laboratory findings. On the basis of magnetic resonance images, signal intensity of the mass, the presence or absence of an abscess, signal intensity of the peripheral wall, patterns of contrast enhancement, and associated findings were evaluated. Compared with those of bacterial myositis, the symptoms of tuberculous myositis lasted longer but there were no difinite local inflammatory signs. In three of five cases of bacterial myositis there were specific medical records;trauma in two cases and systemic lupus erythematosus in one. All tuberculous myositis cases involved a single muscle, but bacterial myositis affected multipe muscles in three cases(60%). All but one case showed a mass in the involved muscles. In one bacterial case, there was diffuse swelling in the involved muscle. On T1-weighted images, eight infectious cases showed low signal intensity;two, of the bactrerial type, showed subtle increased signal intensity. all cases demonstrated high signal intensity on t2-weighted images. The signal intensity of peripheral wall was slightly increased on T1-weighted images, but low on T2-weighted. In four cases there was associated cellulitis, and in one case each, adjacent joint effusion and deep vein thrombosis were seen. After gadolinium infusion, peripheral rim enhancement was noted in nine cases and heterogeneous enhancement in one. After magnetic resonance imaging of infectious myositis, the characteristic finding was an abscessed lesion, with the peripheral wall showing high signal intensity on T1-weighted images and low signal intensity on T2 weighted. Although we found it difficult to differentiate bacterial from tuberculous

  10. Magnetic resonance imaging of infectious myositis

    International Nuclear Information System (INIS)

    Yun, Ji Young; Kim, Jee Young; Kim, Sang Heum; Jung, Youn Ju; Cha, Eun Suk; Park, Joung Mi; Park, Young Ha

    1998-01-01

    To describe the findings of magnetic resonance imaging in infectious myositis and to determine their value for differentiation between ruberculous and bacterial myositis. Magnetic resonance images of ten proven cases of infectious myositis (five tuberculous and five bacterial) were retrospectively reviewed in the light of clinical and laboratory findings. On the basis of magnetic resonance images, signal intensity of the mass, the presence or absence of an abscess, signal intensity of the peripheral wall, patterns of contrast enhancement, and associated findings were evaluated. Compared with those of bacterial myositis, the symptoms of tuberculous myositis lasted longer but there were no difinite local inflammatory signs. In three of five cases of bacterial myositis there were specific medical records;trauma in two cases and systemic lupus erythematosus in one. All tuberculous myositis cases involved a single muscle, but bacterial myositis affected multipe muscles in three cases(60%). All but one case showed a mass in the involved muscles. In one bacterial case, there was diffuse swelling in the involved muscle. On T1-weighted images, eight infectious cases showed low signal intensity;two, of the bactrerial type, showed subtle increased signal intensity. all cases demonstrated high signal intensity on t2-weighted images. The signal intensity of peripheral wall was slightly increased on T1-weighted images, but low on T2-weighted. In four cases there was associated cellulitis, and in one case each, adjacent joint effusion and deep vein thrombosis were seen. After gadolinium infusion, peripheral rim enhancement was noted in nine cases and heterogeneous enhancement in one. After magnetic resonance imaging of infectious myositis, the characteristic finding was an abscessed lesion, with the peripheral wall showing high signal intensity on T1-weighted images and low signal intensity on T2 weighted. Although we found it difficult to differentiate bacterial from tuberculous

  11. Corticosteroids in Myositis and Scleroderma.

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2016-02-01

    Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. Published by Elsevier Inc.

  12. Antibodies in juvenile-onset myositis.

    Science.gov (United States)

    Tansley, Sarah L

    2016-11-01

    Juvenile-onset myositis is a highly heterogeneous disease. Myositis-specific and associated autoantibodies provide a potential means of subdividing patients into clinically homogenous subgroups. Given the increasing availability of autoantibody testing, this review explores the phenotypes associated with different autoantibodies in juvenile-onset myositis and the potential clinical utility of autoantibody testing. Autoantibodies can be identified in 60-70% of children with myositis and the recent discovery of novel myositis-associated autoantibodies in adult patients suggests this may increase in the near future. Detailed phenotype descriptions are now known for several autoantibodies commonly identified in juvenile-onset disease. Whilst there is insufficient evidence to recommend a differential treatment approach based on autoantibody status, it is becoming increasingly clear that some autoantibody subgroups are often treatment resistant and may benefit from a more aggressive approach. The validation of nonspecialised methods for myositis-specific autoantibody detection should lead to more widely available testing. In juvenile-onset disease, this will provide detailed prognostic information and in the future may also influence approach.

  13. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis.

    Science.gov (United States)

    Betteridge, Z; McHugh, N

    2016-07-01

    The idiopathic inflammatory myopathies are characterized by muscle weakness, skin disease and internal organ involvement. Autoimmunity is known to have a role in myositis pathogenesis, and myositis-specific autoantibodies, targeting important intracellular proteins, are regarded as key biomarkers aiding in the diagnosis of patients. In recent years, a number of novel myositis autoantibodies including anti-TIF1, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR and anti-cN1A have been identified in both adult and juvenile patients. These autoantibodies correlate with distinct clinical manifestations and importantly are found in inclusion body, statin-induced, clinically amyopathic and juvenile groups of myositis patients, previously believed to be mainly autoantibody negative. In this review, we will describe the main myositis-specific and myositis-associated autoantibodies and their frequencies and clinical associations across different ages and ethnic groups. We will also discuss preliminary studies investigating correlations between specific myositis autoantibody titres and clinical markers of disease course, collectively demonstrating the utility of myositis autoantibodies as both diagnostic and prognostic markers of disease. © 2015 The Association for the Publication of the Journal of Internal Medicine.

  14. Imaging of nontraumatic benign splenic lesions

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jin Young; Kim, Eun Kyung; Chung, Jae Joon; Kim, Myeong Jin; Lee, Jong Tae; Yoo, Hyung Sik; Kim, Seong Joon [Yonsei Univ. College of Medicine Research Institute of Radiological Science, Seoul (Korea, Republic of); Kim, Lu Ci A [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-04-01

    The spleen is one of the largest organ in the reticuloendothelial system and plays an important role in the activation of immune response. It is the organ most commonly injured after blunt abdominal trauma, and malignant lesions such as lymphoma, or these due to metastasis, occur not infrequently. Even so, it is ignored even in abdominal ultrasonography. Some benign splenic lesions, however can cause severe symptoms and result in high mortality, and their accurate diagnosis is therefore essential. This study describes the imaging findings and histopathologic features of various nontraumatic benign splenic lesions.

  15. Imaging of nontraumatic benign splenic lesions

    International Nuclear Information System (INIS)

    Choi, Jin Young; Kim, Eun Kyung; Chung, Jae Joon; Kim, Myeong Jin; Lee, Jong Tae; Yoo, Hyung Sik; Kim, Seong Joon; Kim, Lu Ci A

    1999-01-01

    The spleen is one of the largest organ in the reticuloendothelial system and plays an important role in the activation of immune response. It is the organ most commonly injured after blunt abdominal trauma, and malignant lesions such as lymphoma, or these due to metastasis, occur not infrequently. Even so, it is ignored even in abdominal ultrasonography. Some benign splenic lesions, however can cause severe symptoms and result in high mortality, and their accurate diagnosis is therefore essential. This study describes the imaging findings and histopathologic features of various nontraumatic benign splenic lesions

  16. Eosinophilic Fasciitis Associated with Myositis

    Directory of Open Access Journals (Sweden)

    Yuko Adachi

    2015-04-01

    Full Text Available Eosinophilic fasciitis is clinically characterized by symmetrical scleroderma-like indurations of the skin with pain. The histological features are fascial inflammation with lymphocytes and eosinophils as well as thickened and fibrotic fascia. Lymphocytic infiltration and degeneration of the underlying muscle are rarely observed. We report a 69-year-old Japanese woman who presented with multiple areas of glossy induration and painful peau d'orange-like lesions on the chest and four extremities. T2-weighted magnetic resonance imaging showed significant hyperintense thickening of the fascia of the lower extremities. Histopathological examination of a biopsy specimen from the induration showed marked fibrinoid degeneration of the fascia and the neighboring muscle with mixed cellular infiltration of lymphocytes and eosinophils. The predominant CD8+ lymphocytic infiltrates were observed by immunohistological study. A diagnosis of eosinophilic fasciitis with myositis was made. Oral administration of prednisolone and discontinuation of exercise significantly improved the lesions and pain.

  17. [Focal myositis: An unknown disease].

    Science.gov (United States)

    Gallay, L; Streichenberger, N; Benveniste, O; Allenbach, Y

    2017-10-01

    Focal myositis are inflammatory muscle diseases of unknown origin. At the opposite from the other idiopathic inflammatory myopathies, they are restricted to a single muscle or to a muscle group. They are not associated with extramuscular manifestations, and they have a good prognosis without any treatment. They are characterized by a localized swelling affecting mostly lower limbs. The pseudo-tumor can be painful, but is not associated with a muscle weakness. Creatine kinase level is normal. Muscle MRI shows an inflammation restricted to a muscle or a muscle group. Muscle biopsy and pathological analysis remain necessary for the diagnosis, showing inflammatory infiltrates composed by macrophages and lymphocytes without any specific distribution within the muscle. Focal overexpression of HLA-1 by the muscle fibers is frequently observed. The muscle biopsy permits to rule out differential diagnosis such a malignancy (sarcoma). Spontaneous remission occurs within weeks or months after the first symptoms, relapse is unusual. Copyright © 2017. Published by Elsevier SAS.

  18. Corticosteroids in Myositis and Scleroderma

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2017-01-01

    Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. PMID:26611554

  19. Postoperative Bowel Perforation due to Heterotopic Ossification (Myositis Ossificans Traumatica: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Victoria Valinluck Lao

    2011-01-01

    Full Text Available Heterotopic ossification (HO is the ectopic development of normal bone within soft tissue that can occur after traumatic injury. It is uncommon and may be missed or misdiagnosed, which can lead to complications. We report the case of an 84-year-old male with a previous history of a laparotomy who underwent resection of an intra-abdominal tumor through a midline incision. On postoperative day six, the patient was taken to the operating room, as succus was draining from the incision. Upon re-exploration, sharp bone-like material was found in the wound directly adjacent to an enterotomy. Pathology confirmed mature lamellar bone and the diagnosis of HO. This is the first report of postoperative intestinal perforation secondary to HO in a midline wound. We report this case to encourage accurate reporting of HO and its morbidity and complications for the benefit of appropriate surgical planning and epidemiologic tracking of outcomes.

  20. Nontraumatic spinal epidural hematomas. MR features

    International Nuclear Information System (INIS)

    Loevblad, K.O.; Baumgartner, R.W.; Zambaz, B.D.; Remonda, L.; Ozdoba, C.; Schroth, G.

    1997-01-01

    Purpose: Spinal epidural hematoma (SEH) is a rare clinical entity with a bleak outcome. The aim of our study was to establish the value of MR findings in the diagnosis of nontraumatic SEH. Material and Methods: Seven patients with nontraumatic SEH were examined by MR at 1.5 T. Two patients were under anticoagulation therapy with heparin, and 2 others were taking salicylic acid. One patient had lupus erythematodes with a marked thrombocytopenia. One patient had a spinal arteriovenous malformation. Results: MR imaging permitted the accurate localization of extradural intraspinal expansive lesions which exhibited the characteristic signal intensities of blood. Five patients underwent laminectomy and evacuation of the hematoma. In the acute phase, the hematomas appeared isointense when compared with the spinal cord on T1-weighted images and hyperintense on T2-weighted images. Later the hematomas were hyperintense on T1-weighted images and showed signals identical to those of cerebrospinal fluid (CSF) on T2-weighted images. Conclusion: MR imaging established the exact diagnosis and localization of SEH in all cases. MR also can provide useful information about the age of the hematomas. (orig.)

  1. Hepatitis E-induced severe myositis.

    Science.gov (United States)

    Mengel, Annerose M; Stenzel, Werner; Meisel, Andreas; Büning, Carsten

    2016-02-01

    Hepatitis E virus (HEV) is endemic in Asian and African countries but is rarely reported in Western countries. Although there are some prominent neurological manifestations, HEV is rarely recognized by neurologists. This is a case report of myositis induced by HEV. We report the life-threatening case of a 57-year-old man with flaccid tetraparesis due to myositis, acute hepatitis, and renal failure caused by HEV infection. Muscle biopsy revealed scattered myofiber necrosis with a diffuse, mild lymphomonocytic infiltrate in the endomysium and perimysium. Because the patient suffered from an acute HEV infection with a rapidly progressive course of severe myopathy, we started ribavirin treatment. He recovered partially within 3 weeks and recovered fully within 6 months. This case highlights a neurological manifestation of endemic HEV infection with severe myositis in a patient with alcoholic chronic liver disease. Ribavirin treatment is effective in severe HEV infection and may also lead to rapid neurological recovery. © 2015 Wiley Periodicals, Inc.

  2. Necrotising Myositis, the Deadly Impersonator

    Directory of Open Access Journals (Sweden)

    A. Rahman

    2014-01-01

    Full Text Available We report two cases of patients with necrotising myositis who presented initially with limb pain and swelling on a background of respiratory complaints. Patient 1, a previously well 38-year-old female, underwent various investigations in the emergency department for excessive lower limb pain and a skin rash. Patient 2, a 61-year-old female with a background of rheumatoid arthritis and hypertension, presented to accident and emergency feeling generally unwell and was treated for presumed respiratory sepsis. Both deteriorated rapidly and were referred to the plastic surgery team with soft tissue necrosis, impending multiorgan failure and toxaemia. Large areas of necrotic muscle and skin were debrided, which grew group A streptococci, Streptococcus pyogenes. Patient 1 had a high above knee amputation of the left leg with extensive debridement of the right. Despite aggressive surgical intervention and microbiological input with intensive care support, patient 2 died. These two cases highlight the importance of early diagnosis and prompt surgical and pharmacological intervention in managing this life-threatening disease. Pain is the primary symptom with skin changes being a late and subtle sign in a septic patient. The Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC may be of use if there is concern to aid diagnosis of this life-threatening disease.

  3. Radiologic Findings in Gabapentin-Induced Myositis.

    Science.gov (United States)

    Coupal, Tyler Michael; Chang, David Ross; Pennycooke, Kevin; Ouellette, Hugue Alcide; Munk, Peter Loren

    2017-04-01

    Throughout recent years, Gabapentin has become increasingly used for the treatment of neuropathic pain. We report on a case of a 31 year old female who presented to the emergency department with unilateral leg pain, weakness, and swelling after increasingly titrating her Gabapentin dosage over three weeks. Magnetic resonance imaging confirmed the presence of myositis confined to the left thigh and the patient's symptoms and laboratory abnormalities resolved following Gabapentin cessation. While Gabapentin-induced myositis and rhabdomyolysis is a rare entity, it should be a diagnostic consideration for radiologists, particularly in the absence of infection or trauma.

  4. Case of bilateral non-traumatic subperiosteal orbital haematomas

    International Nuclear Information System (INIS)

    Lee, Daniel K.; Tran, Phillip V.; Lau, Kenneth K.

    2013-01-01

    Subperiosteal orbital haematoma is an uncommon entity, usually unilateral and caused by trauma. We present a unique case of bilateral non-traumatic subperiosteal orbital haematomas caused by thrombolysis and anticoagulation.

  5. Labyrinthitis ossificans in a child with sickle cell disease: CT and MRI findings

    International Nuclear Information System (INIS)

    Liu, Benjamin P.; Saito, Naoko; Wang, Jimmy J.; Mian, Asim Z.; Sakai, Osamu

    2009-01-01

    The association between sensorineural hearing loss and sickle cell disease has been described, and labyrinthine hemorrhage has been reported with sickle cell disease. We report the CT and MRI findings of labyrinthitis ossificans in a child with sickle cell disease who presented with sensorineural hearing loss. Labyrinthitis ossificans is associated with an infectious, inflammatory, or destructive insult to the membranous labyrinth; however, it has not been specifically described with sickle cell disease. Recognition of this condition is important because it affects both management and prognosis of this disease. (orig.)

  6. Ultrasound and MR imaging of acute myositis

    International Nuclear Information System (INIS)

    Schedel, H.; Reimers, C.D.; Vogl, T.; Lissner, J.

    1992-01-01

    Ultrasound and MR imaging are both methods suitable for imaging neuromuscular diseases; however, contrast media like Gd-DTPA are, to our knowledge, not used so far. In this article we report about our experience of the use of Gd-DTPA in imaging myositis. (orig.)

  7. Myositis is meer dan een spierziekte

    NARCIS (Netherlands)

    Lim, J.; van Royen-Kerkhof, A.; Jonkers, R. E.; Starink, M. V.; Voskuyl, A. E.; van der Kooi, A. J.

    2018-01-01

    Idiopathic inflammatory myopathy (IIM), commonly referred to as "myositis", is a rare but treatable auto-immune disease that is often misdiagnosed or diagnosed after significant delay. Using three clinical case reports as introductory examples, an overview is given - and pitfalls are discussed - of

  8. Periostitis and localised myositis in polyarteritis nodosa

    International Nuclear Information System (INIS)

    Macdonald, W.B.G.; Blake, M.P.

    2003-01-01

    Full text: A 20-year-old man with previously diagnosed polyarteritis nodosa was referred for a bone scan to investigate longstanding knee and lower leg pain. The patient's symptoms worsened with any reduction of steroid dose and his physician was concerned about avascular necrosis of the hips. Plain x-rays of the lower limbs were normal. The blood pool images showed bilaterally increased activity in the anterior muscle compartments of the lower legs, suggestive of localised myositis. Three-hour delayed images showed widespread, increased subperiosteal activity with no evidence of avascular necrosis. Subsequent MRI scanning showed patchy muscle enhancement in both lower legs, also typical of myositis. A muscle biopsy was performed which demonstrated features of both myositis and vasculitis. The patient remains dependent on high-dose steroids for symptom relief. Localised myositis has previously been reported in polyarteritis nodosa and is a recognised, albeit rare, complication of the disorder, the basis of which is not well understood. Diagnostic muscle biopsy should be directed at involved muscle groups, which are best detected with MRI. Lower limb periostitis is well described in polyarteritis nodosa and may result in gross deformity. Several cases have previously been reported in the literature based on radiographic abnormalities, which were not present in our patient. Bone scanning demonstrated the sub-periosteal activity well in our subject and is suggested as a useful investigation in patients with polyarteritis nodosa who complain of lower limb pain. Copyright (2003) The Australian and New Zealand Society of Nuclear Medicine Inc

  9. Mobility challenges and solutions for fibrodysplasia ossificans progressiva.

    Science.gov (United States)

    Levy, C; Berner, T F; Sandhu, P S; McCarty, B; Denniston, N L

    1999-10-01

    Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive soft tissue ossification. Although signs may be present at birth, the first appearance of ectopic bone typically occurs in early childhood. The primary target is the axial musculature. Eventually ectopic bone also occurs in ligaments, fascia, aponeurosis, tendons, and joint capsules of the appendicular skeleton with a proximal to distal predilection. As the disease advances, mobility becomes restricted, and affected individuals are typically limited to bed or chair by their early 30s. This report describes a 30-year-old woman with advanced FOP. She had a fused spine and a fixed pelvis, with hips and knees locked in flexion and feet in plantarflexion. Her upper limb mobility was similarly restricted. She was not able to stand upright or sit independently. The modification of a commercially available power wheelchair that allowed the patient to maintain her employment as a preschool teacher and custom shoes are described. Creative physiatric intervention is essential to liberate human potential for people with FOP.

  10. Value of MRI in diagnostics and evaluation of myositis.

    Science.gov (United States)

    Pipitone, Nicolò

    2016-11-01

    This review aims at covering the role of muscle MRI in supporting the diagnosis of myositis, in aiding to differentiate it from other muscle disorders, and in monitoring myositis patients over time by assessing response to treatment and by discriminating between muscle inflammation and chronic damage. MRI can assist in 'pattern recognition' of muscle involvement across numerous myopathies, including myositis. Novel applications of magnetic resonance such as cardiac MRI, MR elastography and blood oxigenation level-dependent magnetic resonance can shed light on different aspects of myositis and usefully complement conventional MRI in assessing patients with myositis. MRI can guide therapy by determining whether muscle weakness is related to edema (active inflammation) or muscle atrophy/fat replacement (chronic damage). There is a need to better standardize the assessment of MRI findings in myositis to provide defined outcome measures for use in clinical trials. VIDEO ABSTRACT.

  11. Nontraumatic femur fracture in an oligomenorrheic athlete.

    Science.gov (United States)

    Dugowson, C E; Drinkwater, B L; Clark, J M

    1991-12-01

    Exercise-associated amenorrhea is the cessation of menses in a woman following onset of training or an increase in training intensity. Its physiologic basis is characterized by consistently low levels of gonadotropin and ovarian hormones, but the underlying cause of this phenomenon is unknown. Although osteopenia has been described in amenorrheic women athletes, it has been primarily a laboratory diagnosis. Several recent studies have described a significantly lower bone mineral density (BMD) in the lumbar spine of amenorrheic athletes. Marcus et al. also reported an increased number of metatarsal and tibial stress fractures in a group of amenorrheic women. We report here the first case of a nontraumatic femur fracture in an amenorrheic athlete. A 32-yr-old white female, with four prior fibular stress fractures, suffered a left femoral shaft fracture during the 13th mile of a half-marathon. The fracture was successfully internally fixed. Biochemical studies showed no metabolic abnormality. Bone mineral density of the lumbar spine, femoral neck, tibia, and fibula were below the mean for both eumenorrheic and amenorrheic female athletes. Exercise-associated amenorrhea is a medical problem that may have serious implications for both competitive and high-intensity recreational female athletes.

  12. MYOSITIS ASSOCIATED WITH MALIGNANT TUMORS

    Directory of Open Access Journals (Sweden)

    O. A. Antelava

    2016-01-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of acquired systemic diseases mainly involving skeletal muscles. The main representatives of IIM are polymyositis (PM and dermatomyositis (DM. Epidemiological surveys demonstrate that there is a relationship between PM/DM and malignant neoplasms (MNs, the detection risk of which is higher than that in the population of respective age groups. The rate of MNs in PM/DM ranges from 9 to 50%. The relationship to MNs is described in each subtype of IIM; however, these are most common in DM. The patients suffering from PM/DM associated with MNs have a worse prognosis than those without MNs. The early detection of MNs could improve the prognosis in these patients. The investigations published identify demographic, clinical, and laboratory factors increasing MN detection risks in patients with PM/DM. Just the same, they all cover small patient groups; the findings are heterogeneous and not well convincing, which calls for a further larger-scale study of this problem.Objective: to reveal and identify the specific features of paraneoplastic myositis (PnM.Subjects and methods. The investigation included 320 patients with a valid diagnosis of IIM, who had been followed up in the period of 1996 to 2016. The patients underwent laboratory tests, manual proximal muscle strength testing using a 10-point scale and electromyographic examination with needle electrodes.Results and discussion. PnM was detected in 32 (10% of the 320 patients with IIM. Among the patients with PnM, there were 6 (19% men and 26 (81% women. The mean age at the onset of PnM was 55.4 years. PnM manifested with characteristic musculocutaneous syndrome in 19 (59% patients; 18 (41% of them were found to have MNs within the first year after disease onset. The manifestation of MNs was preliminary to the picture of PM/DM in 13 (41% patients. The most commonly detected conditions were ovarian cancer (37.5%, MNs of the lung and breast

  13. Pure White Cell Aplasia and Necrotizing Myositis

    Directory of Open Access Journals (Sweden)

    Peter Geon Kim

    2016-01-01

    Full Text Available Pure white cell aplasia (PWCA is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months.

  14. Proliferative myositis in a patient with AIDS

    Energy Technology Data Exchange (ETDEWEB)

    Wlachovska, B.; Deux, J.F.; Marsault, C.; Le Breton, C. [Department of Radiology, Hopital Tenon, 4 rue de la Chine, 75020, Paris (France); Abraham, B. [Department of Tropical and Infectious Diseases, Hopital Tenon, Paris (France); Sibony, M. [Department of Anatomy, Hopital Tenon, Paris (France)

    2004-04-01

    We report a case of proliferative myositis in the right biceps of a 56-year-old man with acquired immune deficiency syndrome (AIDS). Imaging methods included sonography, computed tomography and magnetic resonance imaging. The diagnosis was made by a core-cut biopsy and fine needle aspiration biopsy with immunohistochemical analysis. The lesion disappeared after 2 months without treatment. It is particularly important to determine whether intramuscular masses arising in patients with AIDS are due to an infectious or malignant process. (orig.)

  15. Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Mohammad Bagher Owlia

    2012-01-01

    Full Text Available Dermatomyositis (DM is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

  16. Proliferative myositis in a patient with AIDS

    International Nuclear Information System (INIS)

    Wlachovska, B.; Deux, J.F.; Marsault, C.; Le Breton, C.; Abraham, B.; Sibony, M.

    2004-01-01

    We report a case of proliferative myositis in the right biceps of a 56-year-old man with acquired immune deficiency syndrome (AIDS). Imaging methods included sonography, computed tomography and magnetic resonance imaging. The diagnosis was made by a core-cut biopsy and fine needle aspiration biopsy with immunohistochemical analysis. The lesion disappeared after 2 months without treatment. It is particularly important to determine whether intramuscular masses arising in patients with AIDS are due to an infectious or malignant process. (orig.)

  17. Fungal myositis in children: serial ultrasonographic findings

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Jung Hwa; Lee, Hee Jung; Choi, Jin Soo [Keimyung University School of Medicine, Daegu (Korea, Republic of)

    2003-08-01

    To evaluate serial ultrasonographic findings of fungal myositis in children. Eleven lesions caused by fungal myositis and occurring in six children were included in this study. Eight lesions in five children were histopathologically proven and the other three were clinically diagnosed. Serial ultrasonographic findings were retrospectively evaluated in terms of size, location, margin, internal echotexture and adjacent cortical change occurring during the follow-up period ranging from five days to two months. Three patients (50%) had multiple lesions. The sites of involvment were the thigh (n=4), calf (n=3), chest wall (n=2), abdominal wall (n=1) and forearm (n=1). Initially, diffuse muscular swelling was revealed, with ill-defined hypoechoic lesions confined to the muscle layer (n=8). Follow-up examination of eight lesions over a period of 5-10 days showed that round central echogenic lesions were surrounded by previous slightly echogenic lesions (n=6, 75%). Long-term follow-up of five lesions over a two-month period revealed periosteal thickening in one case (20%), and the peristence of echogenic solid nodules in four (80%). Pathologic examination showed that the central lesions correlated with a fungus ball and the peripheral slightly echogenic lesions corresponded to hematoma and necrosis. Serial ultrasonographic findings of fungal myositis in children revealed relatively constant features in each case. In particular, the findings of muscular necrosis and a fungus ball over a period of 5-14 days were thought to be characteristic.

  18. Fungal myositis in children: serial ultrasonographic findings

    International Nuclear Information System (INIS)

    Kwon, Jung Hwa; Lee, Hee Jung; Choi, Jin Soo

    2003-01-01

    To evaluate serial ultrasonographic findings of fungal myositis in children. Eleven lesions caused by fungal myositis and occurring in six children were included in this study. Eight lesions in five children were histopathologically proven and the other three were clinically diagnosed. Serial ultrasonographic findings were retrospectively evaluated in terms of size, location, margin, internal echotexture and adjacent cortical change occurring during the follow-up period ranging from five days to two months. Three patients (50%) had multiple lesions. The sites of involvment were the thigh (n=4), calf (n=3), chest wall (n=2), abdominal wall (n=1) and forearm (n=1). Initially, diffuse muscular swelling was revealed, with ill-defined hypoechoic lesions confined to the muscle layer (n=8). Follow-up examination of eight lesions over a period of 5-10 days showed that round central echogenic lesions were surrounded by previous slightly echogenic lesions (n=6, 75%). Long-term follow-up of five lesions over a two-month period revealed periosteal thickening in one case (20%), and the peristence of echogenic solid nodules in four (80%). Pathologic examination showed that the central lesions correlated with a fungus ball and the peripheral slightly echogenic lesions corresponded to hematoma and necrosis. Serial ultrasonographic findings of fungal myositis in children revealed relatively constant features in each case. In particular, the findings of muscular necrosis and a fungus ball over a period of 5-14 days were thought to be characteristic

  19. Acute Renal Failure due to Non-Traumatic Rhabdomyolysis

    Directory of Open Access Journals (Sweden)

    Nagehan Aslan

    2016-04-01

    Full Text Available Rhabdomyolysis is a musculoskeletal clinical and biochemical syndrome which is seen associated with traumatic and non-traumatic causes and is known as muscular dystrophy. Rhabdomyolysis which develops following crush-type trauma (Crush syndrome is rarely seen but is a well-known clinical event in the etiology of acute renal failure. Non-traumatic rhabdomyolysis is rare. The case is here presented of a patient who was diagnosed with rhabdomyolysis on presentation with acute renal failure and to whom repeated dialysis was applied.

  20. Benign acute childhood myositis: an unusual cause of calf pain

    Energy Technology Data Exchange (ETDEWEB)

    Panghaal, Vikash; Levin, Terry L. [Montefiore Medical Center, Department of Radiology, Bronx, NY (United States); Ortiz-Romero, Sara [Albert Einstein College of Medicine, Bronx, NY (United States); Lovinsky, Stephanie [Montefiore Medical Center, Department of Pediatrics, Bronx, NY (United States)

    2008-06-15

    We present a 17-year-old boy with benign acute childhood myositis (BACM) who presented with acute onset of right calf pain, swelling, and difficulty walking. The MR findings are reviewed. MR may be useful in diagnosing BACM and in differentiating it from other causes of myositis. (orig.)

  1. Benign acute childhood myositis: an unusual cause of calf pain

    International Nuclear Information System (INIS)

    Panghaal, Vikash; Levin, Terry L.; Ortiz-Romero, Sara; Lovinsky, Stephanie

    2008-01-01

    We present a 17-year-old boy with benign acute childhood myositis (BACM) who presented with acute onset of right calf pain, swelling, and difficulty walking. The MR findings are reviewed. MR may be useful in diagnosing BACM and in differentiating it from other causes of myositis. (orig.)

  2. Diagnosis, pathogenesis and treatment of myositis: recent advances.

    Science.gov (United States)

    Carstens, P-O; Schmidt, J

    2014-03-01

    Dermatomyositis (DM), polymyositis (PM), necrotizing myopathy (NM) and inclusion body myositis (IBM) are four distinct subtypes of idiopathic inflammatory myopathies - in short myositis. Recent studies have shed some light on the unique pathogenesis of each entity. Some of the clinical features are distinct, but muscle biopsy is indispensable for making a reliable diagnosis. The use of magnetic resonance imaging of skeletal muscles and detection of myositis-specific autoantibodies have become useful additions to our diagnostic repertoire. Only few controlled trials are available to substantiate current treatment approaches for myositis and hopes are high that novel modalities will become available within the next few years. In this review we provide an up-to-date overview of the pathogenesis and diagnostic approach of myositis. We aim to present a guide towards therapeutic and general management. © 2013 British Society for Immunology.

  3. Myositis with endomysial cell invasion indicates inclusion body myositis even if other criteria are not fulfilled

    NARCIS (Netherlands)

    Van de Vlekkert, J.; Hoogendijk, J. E.; de Visser, M.

    2015-01-01

    The objective of this study was to investigate if patients with endomysial mononuclear cell infiltrates invading non-necrotic fibers have a disease course consistent with inclusion body myositis (IBM), irrespective of other histopathological and clinical characteristics. All patients with a muscle

  4. Imaging of acquired non-traumatic cochlear lesions: iconographic essay

    International Nuclear Information System (INIS)

    Garcia, Marcelo de Mattos; Gonzaga, Juliana Gontijo

    2006-01-01

    Different non-traumatic acquired cochlear lesions are shown in this article with imaging methods. They may be responsible for neuro sensorial hearing loss or vertigo. The method of choice is computed tomography when evaluating the osseous labyrinth whereas magnetic resonance imaging has superior resolution in the studies of the membranaceous labyrinth. (author)

  5. Non-traumatic spinal cord compression at Parirenyatwa Hospital in ...

    African Journals Online (AJOL)

    Compression of the spinal cord by encroachment on its space is of major importance as a cause of injury to its tissues, with serious neurological consequences. Patients with non-traumatic spinal cord compression represent a significant proportion of paraplegic/paretic individuals attended to in the neurosurgical units in ...

  6. Non-traumatic injury profile of amateur cyclists

    African Journals Online (AJOL)

    in respondents who experienced neck, back, hand/wrist, buttock/perineum and foot/ankle problems. Conclusion. Non-traumatic injuries in amateur cyclists are common, with back, hand/wrist and buttock/perineal symptoms the most frequent problems. Knee problems caused the greatest need to stop training and seek ...

  7. NON-TRAUMATIC COMA- INCIDENCE, AETIOLOGY AND OUTCOME

    Directory of Open Access Journals (Sweden)

    Mallikarjun R. Patil

    2017-10-01

    Full Text Available BACKGROUND Acute non-traumatic coma is one of the most common paediatric emergencies, which arouses much anxiety and apprehension in both parents and physicians. Due to heterogeneity of causes in these patients, prediction of outcome is difficult and unfortunately no single clinical, laboratory or electrophysiological parameters singly predict their outcome. Aetiology of nontraumatic coma varies depending on different geographical area. We have attempted to find the incidence, aetiology and outcome and delineate neurological signs to predict the prognosis in this study. The aim of this study is to study the incidence, aetiology and outcome of non-traumatic coma in children. MATERIALS AND METHODS 100 consecutive cases of non-traumatic coma between 5months and 15 years of age were selected for the study. Clinical signs and findings were recorded at admission (‘0’ Hr and after ‘48’ Hrs. of hospital stay. Aetiology of coma is determined on the basis of clinical history, examination and relevant laboratory investigations by the treating physician. These children were followed up till the death in the hospital or discharged from the hospital. Discharged patients were asked for followup after 4 weeks. During this period, all of them were evaluated by formal neurological examination and for special sensory involvement. The neurological outcomes were categorised into 6 groups (I-VI based on the severity of neurological involvement. Chisquare test was applied to determine the predictors of outcome. RESULTS 1. The incidence of non-traumatic coma in our hospital based study was 8.02% of all paediatric admissions and 21.64% of all PICU admissions. 2. CNS infections contributed the majority (58% of cases. (Dengue encephalitis-28%, viral encephalitis-12%, TB meningitis-8%, pyogenic meningitis- 6%, Shigella encephalopathy-3% and cerebral malaria-1%. 3. Other non-infectious aetiologies were toxic and metabolic group- 21%, post status epilepticus- 9

  8. Streptococcal necrotizing myositis: a case report and clinical review.

    Science.gov (United States)

    Hourmozdi, Justin J; Hawley, Dean A; Hadi, Christiane M; Tahir, Bilal; Seupaul, Rawle A

    2014-03-01

    Streptococcal necrotizing myositis, also known as gangrenous myositis, is a very rare and severe soft tissue infection that predominately involves skeletal muscle and, eventually, superficial fascia and surrounding tissues. The presentation is often nonspecific until the rapidly progressing clinical course becomes apparent. A high morbidity and mortality rate has been reported in the small number of cases since 1900. Despite several attempts to better define the different entities causing necrotizing myositis, no single definitive causal relationship has been defined. A review of the literature is presented here to help clinicians distinguish those with necrotizing myositis from those with nonnecrotizing myositis when the clinician is at all confronted with the suspicion for such an infection. The case presented is that of a 48-year-old woman who had streptococcal necrotizing myositis. She died roughly 72 h after admission. After the patient's death, the clinical team sought consent for autopsy. Hospital staff made contact with family, and information was obtained from the family that the onset of the patient's symptoms was allegedly temporally related to her acquisition of a new tattoo on the right back, where the tattoo process allegedly included injection of cremated ashes of a pet dog. A high level of suspicion for necrotizing myositis must be maintained for a patient with unexplained severe muscle pain and soft tissue swelling accompanied by systemic inflammatory response syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Fibrodisplasia ossificante progressiva: relato de caso Fibrodysplasia ossificans progressiva: a case report

    Directory of Open Access Journals (Sweden)

    Daiana Martins de Campos

    2005-09-01

    Full Text Available Os autores descrevem um caso de fibrodisplasia ossificante progressiva, doença hereditária caracterizada por calcificações heterotópicas do tecido conectivo, geralmente induzida por trauma, gerando imobilidade permanente das articulações. Hálux valgo, clinodactilia e polegares curtos são as principais malformações congênitas associadas. Manifesta-se na infância, sendo o diagnóstico clínico-radiológico importante, pois procedimentos invasivos exacerbam a doença. Tratamentos disponíveis são apenas paliativos, tendo a prevenção relevância nesse contexto.The authors describe a case of fibrodysplasia ossificans progressiva, a hereditary disease characterized by heterotopic ossification of the connective tissues, usually triggered by trauma, resulting in permanent immobility of the joints. Hallux valgus, clinodactyly and short thumbs are the main associated congenital anomalies. Fibrodysplasia ossificans progressiva usually develops during early childhood. Clinical and radiological diagnosis is essential, since invasive procedures exacerbate the disease. Only palliative treatments are available and prevention plays an important role in patients with fibrodysplasia ossificans progressiva.

  10. New developments in genetics of myositis.

    Science.gov (United States)

    Rothwell, Simon; Lamb, Janine A; Chinoy, Hector

    2016-11-01

    This article reviews the advances that have been made in our understanding of the genetics of the idiopathic inflammatory myopathies (IIM) in the past 2 years, with a particular focus on polymyositis, dermatomyositis and inclusion body myositis. Two large human leukocyte antigen (HLA) imputation studies have confirmed a strong association with the 8.1 ancestral haplotype in clinical subgroups of myositis and suggest multiple independent associations on this haplotype. Risk in these genes may be due to specific amino acid positions within the peptide-binding grooves of HLA molecules. A large genetic study in 2566 IIM patients revealed associations such as PTPN22, STAT4, UBE2L3 and BLK, which overlap with risk variants reported in other seropositive autoimmune diseases. There is also evidence of different genetic architectures in clinical subgroups of IIM. Candidate gene studies in the Japanese and Chinese populations have replicated previous IIM associations which suggest common aetiology between ethnicities. International collaborations have facilitated large genetic studies in IIM that have revealed much about the genetics of this rare complex disease both within the HLA region and genome-wide. Future approaches, such as sequencing and trans-ethnic meta-analyses, will advance our knowledge of IIM genetics.

  11. Emerging therapeutic options for sporadic inclusion body myositis

    Directory of Open Access Journals (Sweden)

    Alfano LN

    2015-09-01

    Full Text Available Lindsay N Alfano, Linda P Lowes Nationwide Children’s Hospital, Center for Gene Therapy, Columbus, OH, USA Abstract: Sporadic inclusion body myositis is the most common inflammatory muscle disorder preferentially affecting males over the age of 40 years. Progressive muscle weakness of the finger flexors and quadriceps muscles results in loss of independence with activities of daily living and eventual wheelchair dependence. Initial signs of disease are often overlooked and can lead to mis- or delayed diagnosis. The underlying cause of disease is unknown, and disease progression appears refractory to available treatment options. This review discusses the clinical presentation of inclusion body myositis and the current efforts in diagnosis, and focuses on the current state of research for both nonpharmacological and pharmacological treatment options for this patient group. Keywords: myositis, inclusion body myositis, inflammatory myopathy, treatment, function, outcomes

  12. Tubuloreticular structures in different types of myositis: implications for pathogenesis

    NARCIS (Netherlands)

    Bronner, Irene M.; Hoogendijk, Jessica E.; Veldman, Henk; Ramkema, Marja; van den Bergh Weerman, Marius A.; Rozemuller, Annemieke J. M.; de Visser, Marianne

    2008-01-01

    In dermatomyositis (DM) there is strong histopathological evidence of a microvascular pathogenesis, including endothelial microtubular inclusions. In nonspecific myositis, perimysial and perivascular infiltrates in the muscle biopsy similar to DM are found. Microtubular inclusions in endothelial

  13. Focal myositis of lower extremity responsive to botulinum A toxin.

    Science.gov (United States)

    Mitrovic, Josko; Prka, Zeljko; Zic, Rado; Marusic, Srecko; Morovic-Vergles, Jadranka

    2014-01-01

    Focal myositis is a rare, mostly benign disease (pseudotumor) of skeletal muscle, histopathologically characterized by interstitial myositis and tumorous enlargement of a single muscle. The etiology of focal myositis remains unknown; however, localized myopathy has been postulated to be caused by denervation lesions. This case report describes a patient that presented with clinical, laboratory, electromyoneurography, and magnetic resonance imaging features of focal myositis complicated with intervertebral disk protrusion in the lumbosacral spine affected with radicular distress. In most cases, focal myositic lesions show spontaneous regression, relapses are rare, and long-term prognosis is good. There is a wide spectrum of therapeutic options, from no therapy at all through nonsteroidal antirheumatics and glucocorticoids to radiotherapy, surgical excision, and immunosuppressants. In the patient presented, treatment with glucocorticoids, methotrexate, and surgical excision failed to produce satisfactory results. Clinical improvement, pain relief, and reduction in lower leg volume were only achieved by local infiltration of botulinum A toxin.

  14. Tubuloreticular structures in different types of myositis: Implications for pathogenesis

    NARCIS (Netherlands)

    Bronner, I.M.; Hoogendijk, J.E.; Veldman, H.; Ramkema, M; Weerman, M.A.V.; Rozemuller, A.J.M.; Visser, M.

    2008-01-01

    In dermatomyositis (DM) there is strong histopathological evidence of a microvascular pathogenesis, including endothelial microtubular inclusions. In nonspecific myositis, perimysial and perivascular infiltrates in the muscle biopsy similar to DM are found. Microtubular inclusions in endothelial

  15. Disseminated tuberculous myositis in a child with acute myelogenous leukemia.

    Science.gov (United States)

    Chen, Yu-Chieh; Sheen, Jiunn-Ming; Huang, Li-Tung; Wu, Kuan-Sheng; Hsiao, Chih-Cheng

    2009-04-01

    Tuberculous myositis is extremely rare, even in immunocompromised hosts. We present a case of disseminated tuberculous myositis in a girl with secondary acute myelogenous Leukemia following successful chemotherapy for undifferentiated sarcoma of the maxillary sinus. The diagnosis was established by direct visualization of acid-fast bacilli in the biopsied nodule and by typical pathologic findings. Three weeks after initiation of antituberculosis treatment, the patient experienced both clinical and radiologic improvement.

  16. Disseminated Tuberculous Myositis in a Child with Acute Myelogenous Leukemia

    Directory of Open Access Journals (Sweden)

    Yu-Chieh Chen

    2009-04-01

    Full Text Available Tuberculous myositis is extremely rare, even in immunocompromised hosts. We present a case of disseminated tuberculous myositis in a girl with secondary acute myelogenous leukemia following successful chemotherapy for undifferentiated sarcoma of the maxillary sinus. The diagnosis was established by direct visualization of acid-fast bacilli in the biopsied nodule and by typical pathologic findings. Three weeks after initiation of antituberculosis treatment, the patient experienced both clinical and radiologic improvement.

  17. Non-traumatic Thickening of the Anterior Cruciate Ligament

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Hyun Jun; Park, Jin Gyoon; Song, Sang Gook [Chonnam National University Hospital, Gwangju (Korea, Republic of)

    2009-08-15

    To describe the magnetic resonance (MR) imaging findings of non-traumatic thickening of the anterior cruciate ligament (ACL) and to evaluate the associated lesions. Between January 2003 and August 2005, 44 knees of 44 patients who had thickened ACLs on MR images and had no history of knee trauma were analyzed retrospectively. The normal thickness of the ACL was measured on axial T2-weighted images of 40 healthy adult knees. The MR imaging findings of the thickened ACLs and associated lesions were analyzed. In 40 cases of healthy knees, the thickness of the proximal ACL was 3-6 mm. In 44 cases of non-traumatic thickening of the ACL, the thickness of the proximal ACL was 8-14 mm. There was an increased signal intensity and ill-defined border in all cases of thickened ACLs, linear low-signal intensity fibers parallel to the long axis of the thickened ACL (celery stalk appearance) in 24 cases, and entrapment in 10 cases. With respect to associated lesions, there was osteoarthritis in 40 cases, meniscal tears in 42 cases, and degeneration of the posterior cruciate ligament in 7 cases. Non-traumatic thickening of the ACL was associated with osteoarthritis and meniscal tears in almost all cases and showed increased signal intensity and ill-defined borders simulating acute ligamentous tears

  18. Inflammatory muscle diseases (myositis); Entzuendliche Muskelerkrankungen (Myositiden)

    Energy Technology Data Exchange (ETDEWEB)

    Vahlensieck, M. [Praxisnetz Radiologie und Nuklearmedizin Bonn Bad Godesberg Rhein Sieg, Bonn (Germany)

    2017-12-15

    Inflammatory muscle diseases are quite heterogeneous and frequently a specific diagnosis is not easy. Magnetic resonance imaging (MRI) is best suited to demonstrate pattern and size of inflamed muscles. Sensitivity of MRI in showing inflamed muscles is nearly 100%. Specificity, however, is much lower. In cases of suspected bacterial myositis, sonography may be used to exclude abscess formation prior to MRI for more precise information about inflamed muscles. (orig.) [German] Entzuendliche Muskelerkrankungen stellen eine heterogene Gruppe dar und sind mitunter nur schwer exakt zu diagnostizieren. Die Magnetresonanztomographie (MRT) stellt Ausdehnung und Befallsmuster am besten dar. Der Nachweis entzuendlicher Oedeme oder von Einschmelzungen gelingt mit der MRT in annaehernd 100 % der Faelle. Die Spezifitaet der Methode ist allerdings deutlich geringer. Bei Verdacht auf bakterielle Myositiden kommt erst die Sonographie zum Ausschluss von Einschmelzungen und dann die MRT zum Einsatz. (orig.)

  19. Statin-induced autoimmune necrotizing myositis

    Directory of Open Access Journals (Sweden)

    Katarzyna Ząber

    2016-02-01

    Full Text Available Myositides comprise a large group of disorders involving limb muscle weakness. In differential diagnosis we have to consider idiopathic myositides, myositides associated with other diseases, and those induced by external factors, e.g. drug-induced. Statins are commonly used drugs, but many patients experience a broad spectrum of adverse effects including symptoms from skeletal muscle. Physicians should pay special attention to patients reporting muscle weakness lasting longer than 12 weeks, despite statin withdrawal, as well as other symptoms: dysphagia, disturbed grip function, elevated creatinine kinase (CK levels and abnormal electromyography. The reported case deals with the problem of differential diagnosis of drug-induced muscle injury, polymyositis with a recently reported myopathy – statin-induced autoimmune necrotizing myositis, related to anti-HMGCR antibodies.

  20. Extraocular myositis in a female puppy

    Directory of Open Access Journals (Sweden)

    O. Adegboye

    2015-04-01

    Full Text Available Extraocular myositis (EOM is not commonly encountered in dogs. It is generally diagnosed based on clinical features of exophthalmos without third eyelid protrusion, pain or vision loss. The traditional treatment of choice is prednisolone. This report describes a case of a mixed-breed puppy with clinical signs consistent with EOM, the use of ascorbic acid as an adjuvant to traditional corticosteroid therapy and rapid resolution of the condition without recurrence. It also shows that prolapse of the third eyelid and ptosis of the lower eyelids are possible signs of EOM during recovery. This is the first report of this sort from Africa and therefore the report is of epidemiological significance.

  1. Genomic signatures characterize leukocyte infiltration in myositis muscles

    Science.gov (United States)

    2012-01-01

    Background Leukocyte infiltration plays an important role in the pathogenesis and progression of myositis, and is highly associated with disease severity. Currently, there is a lack of: efficacious therapies for myositis; understanding of the molecular features important for disease pathogenesis; and potential molecular biomarkers for characterizing inflammatory myopathies to aid in clinical development. Methods In this study, we developed a simple model and predicted that 1) leukocyte-specific transcripts (including both protein-coding transcripts and microRNAs) should be coherently overexpressed in myositis muscle and 2) the level of over-expression of these transcripts should be correlated with leukocyte infiltration. We applied this model to assess immune cell infiltration in myositis by examining mRNA and microRNA (miRNA) expression profiles in muscle biopsies from 31 myositis patients and 5 normal controls. Results Several gene signatures, including a leukocyte index, type 1 interferon (IFN), MHC class I, and immunoglobulin signature, were developed to characterize myositis patients at the molecular level. The leukocyte index, consisting of genes predominantly associated with immune function, displayed strong concordance with pathological assessment of immune cell infiltration. This leukocyte index was subsequently utilized to differentiate transcriptional changes due to leukocyte infiltration from other alterations in myositis muscle. Results from this differentiation revealed biologically relevant differences in the relationship between the type 1 IFN pathway, miR-146a, and leukocyte infiltration within various myositis subtypes. Conclusions Results indicate that a likely interaction between miR-146a expression and the type 1 IFN pathway is confounded by the level of leukocyte infiltration into muscle tissue. Although the role of miR-146a in myositis remains uncertain, our results highlight the potential benefit of deconvoluting the source of

  2. Testing for myositis specific autoantibodies: Comparison between line blot and immunoprecipitation assays in 57 myositis sera.

    Science.gov (United States)

    Cavazzana, Ilaria; Fredi, Micaela; Ceribelli, Angela; Mordenti, Cristina; Ferrari, Fabio; Carabellese, Nice; Tincani, Angela; Satoh, Minoru; Franceschini, Franco

    2016-06-01

    To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. 66 sera of patients with myositis (23 polymyositis, 8 anti-synthetase syndromes, 29 dermatomyositis and 6 overlap syndromes) were tested by commercial LB (Euroimmun, Lubeck, Germany); 57 sera were analyzed also by IP of K562 cell extract radiolabeled with (35)S-methionine. Inter-rater agreement was calculated with Cohen's k coefficient. Myositis-specific antibodies (MSA) were detected in 36/57 sera (63%) by IP and in 39/66 sera (59%) by LB. The most frequent MSA found by LB were anti-Jo1 and anti-Mi2 found in 15% (10/66) of sera, followed by anti-NXP2 and anti-SRP detected in 106% (7/66) of sera. Anti-TIF1gamma and anti-MDA5 were found in 6 (9%) and 5 sera (7.6%), respectively. A good agreement between methods was found only for anti-TIF1γ, anti-MDA5 and anti-NXP-2 antibodies, while a moderate agreement was estimated for anti-Mi2 and anti-EJ. By contrast, a high discordance rate for the detection of anti-Jo1 antibodies was evident (k: 0.3). Multiple positivity for MSA were found in 11/66 (17%) by LB and 0/57 by IP (p: 0001). Comparing the clinical features of these 11 sera, we found total discrepancies between assays in 3 sera (27.3%), a relative discrepancy due to the occurrence of one discordant autoantibody (not confirmed by IP) in 5 cases (45.5%) and a total discrepancy between LB and IP results, but with a relative concordance with clinical features were found in other 3 sera (27.3%). The semiquantitative results do not support the interpretation of the data. The use of LB assay allowed the detection of new MSA, such as anti-MDA5, anti-MJ and anti-TIF1gamma antibodies, previously not found with routine methods. However, the high prevalence of multiple positivities and the high discondant rate of anti-Jo1 antibodies could create some misinterpretation of the results from the

  3. Nontraumatic osteonecrosis of the distal pole of the scaphoid

    Directory of Open Access Journals (Sweden)

    Bhavuk Garg

    2011-01-01

    Full Text Available Post traumatic osteonecrosis of distal pole of scaphoid is very rare. We present a case of 34 years old male, drill operator by occupation with nontraumatic osteonecrosis of distal pole of the scaphoid. The patient was managed conservatively and was kept under regular follow-up every three months. The patient was also asked to change his profession. Two years later, the patient had no pain and had mild restriction of wrist movements (less than 15 degrees in either direction. The radiographs revealed normal density of the scaphoid suggesting revascularization.

  4. Necrotizing streptococcal myositis of the upper extremity: a case report.

    Science.gov (United States)

    Reichert, Johannes C; Habild, Götz; Simon, Paul; Nöth, Ulrich; Krümpelmann, Jan B

    2017-08-15

    Necrotizing myositis is a rare but life-threatening soft-tissue infection characterized by rapidly spreading inflammation and subsequent necrosis of the affected tissue. The myositis is often caused by toxin-producing, virulent bacteria such as group A β-hemolytic streptococcus and associated with severe systemic toxicity. It is rapidly fatal unless diagnosed promptly and treated aggressively. However, necrotizing myositis is often initially misdiagnosed as a more benign soft-tissue infection as such fulminant, invasive muscle infections are rare with no more than 30 cases reported over the last century. We illustrate the case of a 74-year-old male Caucasian initially presenting with a progressing swelling and gradually oncoming pain of the upper right extremity. Rapidly, livid discolorations of the skin, blisters, hypoesthesia and severe pain resistant to analgesics treatment developed accompanied by disruption of the arterial blood flow. Due to a manifest compartment syndrome the patient was admitted to theater for fasciotomy of the arm. After multiple revision surgeries wound closure was achieved using a pedicled, fasciocutaneous parascapular flap and a free, ipsilateral anterolateral thigh flap. Microbiological analysis revealed group A β-hemolytic streptococcus, histology a bacterial interstitial myositis with necrotic muscular fibers. A high degree of clinical suspicion is necessary to avert potentially disastrous consequences of necrotizing myositis. Timely diagnosis, broad-spectrum antibiotic therapy, and aggressive surgical debridement of affected tissue are keys to the treatment of this serious, often life-threatening infection.

  5. MASTICATORY MUSCLE MYOSITIS IN A GRAY WOLF (CANIS LUPUS).

    Science.gov (United States)

    Kent, Marc; Glass, Eric N; Castro, Fernando A; Miller, Andrew D; de Lahunta, Alexander

    2017-03-01

    A 10-yr-old male, neutered gray wolf ( Canis lupus ) was presented for atrophy of the temporalis and masseter muscles. Clinical signs and magnetic resonance imaging were consistent with a myopathy. Positive serology for antibody titers directed against Type 2M myofibers, and the observation of a mixed mononuclear inflammatory cell infiltrate along with eosinophils and neutrophils within the temporalis muscle, were diagnostic for masticatory muscle myositis. Importantly, protozoal myositis was excluded based on other clinicopathologic data. The case highlights the potential for immune-mediated polymyositis in canids other than the domesticated dog ( Canis lupus familaris). Additionally, awareness of a diet in which raw meat is used should prompt a thorough investigation for an underlying infectious myositis in the gray wolf.

  6. Myositis as the initial presentation of panarteritis nodosa.

    Science.gov (United States)

    Calvo, Romina; Negri, Melina; Ortiz, Alberto; Roverano, Susana; Paira, Sergio

    2017-07-26

    A 47-year-old man presented with weight loss, bilateral calf pain, fever, hypertension, orchitis and oligoarthritis. Lab tests: anemia and elevated muscle enzymes. Resonance magnetic imaging: hyperintensity in gastrocnemius muscles (myositis). Histologic exam of the muscles: inflammatory infiltrate with atrophy and perifascicular regeneration. methylprednisone (bolus) and cyclophosphamide. Muscle pain and swelling and difficulty in walking are common in panarteritis nodosa (PAN), whereas histologically demonstrated myositis is not. Even more rare is myositis as the initial presentation of this vasculitis. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  7. Non-traumatic spinal extradural haematoma: magnetic resonance findings

    Energy Technology Data Exchange (ETDEWEB)

    Law, E.M.; Smith, P.J.; Fitt, G.; Hennessy, O.F. [St. Vincent`s Hospital, Fitzroy, VIC (Australia). University of Melbourne, Department of Medical Imaging

    1999-05-01

    Non-traumatic extradural spinal haematoma is an uncommon condition that is usually associated with a poor outcome. It may present acutely with signs and symptoms of major neurological dysfunction secondary to cord compression, or subacutely over a number of days or weeks with fluctuating symptoms. The exact aetiology of this condition is incompletely understood, but it is believed that the blood is venous in origin, as distinct from the arterial origin of intracranial extradural haematomas. Causes of non-traumatic extradural spinal haematoma include anticoagulation, vasculitis such as systemic lupus erythematosus (SLE), and spinal arteriovenous malformations. Conditions that may mimic an acute spinal haematoma include extradural abscess and extradural metastatic infiltration. It is important to make a diagnosis of extradural compression because surgery may offer the best hope in restoring neurological function in these patients. Imaging modalities used for the investigation of extradural haematomas include myelography, CT myelography (CTM) and MRI with or without gadolinium enhancement. The MR appearances of acute extradural abscess and extradural tumour can mimic an extradural haematoma. In subacute haematoma, owing to the magnetic properties of blood degradation products, MR is more specific in diagnosing and ageing of the haematoma. Copyright (1999) Blackwell Science Pty Ltd 11 refs., 3 figs.

  8. Usefulness of emergency ultrasound in nontraumatic cardiac arrest.

    Science.gov (United States)

    Volpicelli, Giovanni

    2011-02-01

    Treatment of nontraumatic cardiac arrest in the hospital setting depends on the recognition of heart rhythm and differential diagnosis of the underlying condition while maintaining a constant oxygenated blood flow by ventilation and chest compression. Diagnostic process relies only on patient's history, physical findings, and active electrocardiography. Ultrasound is not currently scheduled in the resuscitation guidelines. Nevertheless, the use of real-time ultrasonography during resuscitation has the potential to improve diagnostic accuracy and allows the physician a greater confidence in deciding aggressive life-saving therapeutic procedures. This article reviews the current opinions and literature about the use of emergency ultrasound during resuscitation of nontraumatic cardiac arrest. Cardiac and lung ultrasound have a great potential in identifying the reversible mechanical causes of pulseless electrical activity or asystole. Brief examination of the heart can even detect a real cardiac standstill regardless of electrical activity displayed on the monitor, which is a crucial prognostic indicator. Moreover, ultrasound can be useful to verify and monitor the tracheal tube placement. Limitation to the use of ultrasound is the need to minimize the no-flow intervals during mechanical cardiopulmonary resuscitation. However, real-time ultrasound can be successfully applied during brief pausing of chest compression and first pulse-check. Finally, lung sonographic examination targeted to the detection of signs of pulmonary congestion has the potential to allow hemodynamic noninvasive monitoring before and after mechanical cardiopulmonary maneuvers. Copyright © 2011 Elsevier Inc. All rights reserved.

  9. Focal myositis of the thigh: unusual MR pattern

    International Nuclear Information System (INIS)

    Llauger, Jaume; Palmer, Jaume; San Roman, Luis; Bague, Silvia; Matias-Guiu, Xavier; Doncel, Antonio

    2002-01-01

    Focal myositis is a commonly referenced, infrequently reported and poorly documented benign inflammatory pseudotumor which may be misdiagnosed clinically as a malignant tumor. We report the clinicopathologic features and magnetic resonance imaging findings in a case of focal myositis in the thigh of a 55-year-old woman. A different radiologic presentation of this disorder is described. The gross appearance of the lesion, previously undescribed, appears to be rather specific for such a pseudoneoplastic disorder, and correlates very well with the magnetic resonance imaging features. (orig.)

  10. Focal myositis of the thigh: unusual MR pattern

    Energy Technology Data Exchange (ETDEWEB)

    Llauger, Jaume; Palmer, Jaume; San Roman, Luis [Department of Radiology, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain); Bague, Silvia; Matias-Guiu, Xavier [Department of Pathology, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain); Doncel, Antonio [Department of Orthopedic Surgery, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain)

    2002-05-01

    Focal myositis is a commonly referenced, infrequently reported and poorly documented benign inflammatory pseudotumor which may be misdiagnosed clinically as a malignant tumor. We report the clinicopathologic features and magnetic resonance imaging findings in a case of focal myositis in the thigh of a 55-year-old woman. A different radiologic presentation of this disorder is described. The gross appearance of the lesion, previously undescribed, appears to be rather specific for such a pseudoneoplastic disorder, and correlates very well with the magnetic resonance imaging features. (orig.)

  11. Sodium and chloride channelopathies with myositis: coincidence or connection?

    Science.gov (United States)

    Matthews, E.; Miller, J.A.L.; Macleod, M.R.; Ironside, J.; Ambler, G.; Labrum, R.; Sud, R.; Holton, J.L.; Hanna, M.G.

    2011-01-01

    Introduction A proximal myopathy develops in some patients with muscle channelopathies, but the causative molecular mechanisms are unknown. Methods We reviewed retrospectively all clinical and muscle biopsy findings of three patients with channelopathy and additional myositis. Direct DNA sequencing was performed. Results Pathogenic mutations were identified in each case. Biopsies illustrated inflammatory infiltrates. Conclusions Clinicians should consider muscle biopsy in channelopathy patients with severe myalgia and/or subacute weakness and accompanying elevated CK. Chance association of myositis and channelopathy is statistically unlikely. An alternative hypothesis suggests that inflammatory insults could contribute to myopathy in some patients. PMID:21698652

  12. Associated Variables of Myositis in Systemic Lupus Erythematosus: A Cross-Sectional Study.

    Science.gov (United States)

    Liang, Yan; Leng, Rui-Xue; Pan, Hai-Feng; Ye, Dong-Qing

    2017-05-26

    BACKGROUND This study aimed to estimate the point prevalence of myositis and identify associated variables of myositis in systemic lupus erythematosus (SLE). MATERIAL AND METHODS Clinical date of patients hospitalized with lupus at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital were collected. Patients were defined as having myositis if they reported the presence of persistent invalidating muscular weakness combined with increased levels of creatine phosphokinase (CPK) and abnormal electromyography (EMG). RESULTS The study sample comprised 1701 lupus patients, of which 44 had myositis. Patients with SLE-associated myositis are more likely to have skin rash, alopecia, pericarditis, vasculitis, anti-Sm, anti-RNP, anti-dsDNA, thrombocytopenia, leukopenia, low C3, low C4, high erythrocyte sedimentation rate (ESR), high D-dimer, and active disease. Multivariate logistic regression found positive associations between leukopenia, alopecia, and active disease with myositis. Negative associations between myositis with the use of corticosteroids or immunosuppressive drugs were revealed in univariate and multivariate analysis. CONCLUSIONS The point prevalence of myositis was 2.6% in SLE patients. The significant association of alopecia, leukopenia, and active disease with myositis suggests that organ damage, hematological abnormality, and high disease activity promote the progression of myositis in lupus patients.

  13. Brucellosis presenting as piriformis myositis: a case report

    Directory of Open Access Journals (Sweden)

    Romanos Odysseas

    2011-03-01

    Full Text Available Abstract Introduction Myositis is a rare bacterial muscle infection. Involvement of the piriformis muscle has been rarely reported in the literature. In this report we describe a case of piriformis myositis due to Brucella melitensis, which to the best of our knowledge is the first such case presented in the literature. Case presentation We report the case of a 19-year-old Caucasian man who presented to our institution with fever and right hip pain. Brucellosis was suspected, but the clinical suspicion was for spondylodiscitis. A pelvic magnetic resonance imaging scan allowed prompt diagnosis of inflammatory involvement of the right piriformis muscle. Blood culture results were positive for B. melitensis. Our patient was treated with antibiotics, and follow-up magnetic resonance imaging scans showed resolution of the inflammation. Conclusion Brucellosis can present as piriformis myositis. The clinical diagnosis of piriformis myositis is difficult, as it can mimic other common entities such as referred back pain from spondylodiscitis. Magnetic resonance imaging is the method of choice for establishing the diagnosis in the early stages of the disease, as late diagnosis can lead to abscess formation and the need for drainage.

  14. Oval pulsed high-dose dexamethasone for myositis

    NARCIS (Netherlands)

    Hoogendijk, JE; Wokke, JHJ; de Visser, M

    To study the short-term effect of oral pulsed high-dose dexamethasone for myositis we treated eight newly diagnosed patients with three 28-day cycles of oral dexamethasone. Primary outcome measures were muscle strength, pain, and serum creatine kinase activity. Sis patients responded. Side effects

  15. Management of a femoral fracture complicated by clostridial myositis

    International Nuclear Information System (INIS)

    Thomson, M.J.; Eger, C.E.

    1997-01-01

    A clinical case of clostridial myositis secondary to a comminuted femoral fracture is described. This case is unusual because, despite the severe degree of obvious muscle necrosis and gas production, the dog had minimal signs of systemic toxicity. Union of the fracture was achieved but six months postoperatively muscular contracture had resulted in permanent stifle extension

  16. Physical function and muscle strength in sporadic inclusion body myositis

    DEFF Research Database (Denmark)

    Jørgensen, Anders N; Aagaard, Per; Nielsen, Jakob L

    2017-01-01

    INTRODUCTION: In this study, self-reported physical function, functional capacity, and isolated muscle function were investigated in sporadic inclusion body myositis (sIBM) patients. METHODS: The 36-item Short Form (SF-36) Health Survey and 2-min walk test (2MWT), timed up & go test (TUG), and 30-s...

  17. Oral pulsed high-dose dexamethasone for myositis

    NARCIS (Netherlands)

    van der Meulen, M. F.; Hoogendijk, J. E.; Wokke, J. H.; de Visser, M.

    2000-01-01

    To study the short-term effect of oral pulsed high-dose dexamethasone for myositis we treated eight newly diagnosed patients with three 28-day cycles of oral dexamethasone. Primary outcome measures were muscle strength, pain, and serum creatine kinase activity. Six patients responded. Side effects

  18. Update on the pharmacological treatment of adult myositis.

    Science.gov (United States)

    Oddis, C V

    2016-07-01

    The management of patients with idiopathic inflammatory myopathy (IIM) remains a challenge given the systemic features beyond active myositis. That is, recognizing the inflammatory arthropathy, varying dermatomyositis rashes, and overt and occult features of interstitial lung disease in addition to myositis adds to the complexity of diagnosis and treatment of IIM. However, clinicians now have available many more immunosuppressive drugs as well as biologic agents for use in patients with myositis and other autoimmune diseases. Here, the use of these agents is reviewed and support based on available published literature is provided even though many studies have been small and results somewhat anecdotal. Glucocorticoids remain the initial treatment of choice in most instances and methotrexate and azathioprine are often used early in the treatment course. These agents are followed by other immunosuppressive drugs, for example mycophenolate mofetil, tacrolimus, cyclosporine and cyclophosphamide, some of which are used alone while combinations of these agents also provide an effective option. There is more rationale for the use of biologic agents such as rituximab from a mechanistic perspective and, given the incorporation of validated core set measures in assessing myositis patients, we can look forward to better designed clinical trials in the future. © 2016 The Association for the Publication of the Journal of Internal Medicine.

  19. [The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'

    NARCIS (Netherlands)

    Hoogendijk, J.E.; Bijlsma, J.W.J.; Engelen, B.G.M. van; Lindeman, E.J.M.; Royen-Kerkhof, A. van; Rie, M.A. de; Visser, M. de; Jennekens, F.G.I.

    2005-01-01

    This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

  20. Richtlijn 'dermatomyositis, polymyositis en sporadische "inclusion body"-myositis'

    NARCIS (Netherlands)

    Hoogendijk, J. E.; Bijlsma, J. W. J.; van Engelen, B. G. M.; Lindeman, E.; van Royen-Kerkhof, A.; de Rie, M. A.; de Visser, M.; Jennekens, F. G. I.

    2005-01-01

    This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

  1. Plasma Exchange for Refractory MDA5 Myositis and ILD

    Science.gov (United States)

    2017-04-26

    plasma exchange, the patient had improvement of hypocarbic respiratory alkalosis and reversal of myositis with resolution of dysphagia/odynophagia and...symptoms with respiratory distress. A five day course of plasmapheresis was initiated on hospital day eight. Subsequently his respiratory acid-base

  2. Genetic transmission of fibrodysplasia ossificans progressiva : report of two cases in a family

    Energy Technology Data Exchange (ETDEWEB)

    Pyo, Hyun Soon; Hwang, Ho Kyeung; Park, Byung Moon [Kwangmyungsungae General Hospital, Kwangmyung (Korea, Republic of)

    2001-08-01

    Fibrodysplasia ossificans progressiva (FOP) is a rare connective tissue disorder characterized by congenital malformation of the great toes and by progressive heterotopic ossification of the tendons, ligaments, fasciae and skeletal muscles. We document the radiologic manifestation of FOP passed from a sporadically affected father to each of his two children (a son and a daughter). Previous consideration of a genetic etiology was based on the fact that the disease has been reported in several sets of monozygotic twins and that increased paternal age has been associated with sporadic occurrence of the disorder. Although autosomal-dominant transmission has long been suspected, the findings in this family provide confirmation for such inheritance and a basis for the diagnosis and counseling of patients with FOP.

  3. [Is there a place in primary care for rare diseases? The case of fibrodysplasia ossificans progressiva].

    Science.gov (United States)

    Morales-Piga, Antonio; García Ribes, Miguel; Arribas Álvaro, Pilar; Casado Álvaro, Carlos; Posada de La Paz, Manuel; Bachiller-Corral, Javier

    2013-01-01

    Fibrodysplasia ossificans progressiva is one of the most devastating constitutional diseases of the bone, and may be a valid example to establish the role of Primary Care in the care of rare diseases. Although rare diseases usually present with marked anomalies, they can mimic signs and symptoms of common disorders, with the risk of going unnoticed. For this reason, all health professionals should proceed with a reasonable suspicion when confronted with a patient with an apparently common disease with atypical symptoms and a non-conventional progress. The care given by the Primary Care team along with other health care services are fundamental in the integrated and individualised follow-up. The quality of care in rare diseases must not be inferior to that provided to the other chronic diseases, since, besides being a requirement of justice and fairness, these patients are, in essence, the "paradigm of chronicity". Copyright © 2012 Elsevier España, S.L. All rights reserved.

  4. Genetic transmission of fibrodysplasia ossificans progressiva : report of two cases in a family

    International Nuclear Information System (INIS)

    Pyo, Hyun Soon; Hwang, Ho Kyeung; Park, Byung Moon

    2001-01-01

    Fibrodysplasia ossificans progressiva (FOP) is a rare connective tissue disorder characterized by congenital malformation of the great toes and by progressive heterotopic ossification of the tendons, ligaments, fasciae and skeletal muscles. We document the radiologic manifestation of FOP passed from a sporadically affected father to each of his two children (a son and a daughter). Previous consideration of a genetic etiology was based on the fact that the disease has been reported in several sets of monozygotic twins and that increased paternal age has been associated with sporadic occurrence of the disorder. Although autosomal-dominant transmission has long been suspected, the findings in this family provide confirmation for such inheritance and a basis for the diagnosis and counseling of patients with FOP

  5. Radiographic and CT findings in a case of fibro-dysplasia ossificans progressiva

    International Nuclear Information System (INIS)

    Haddad, S.; Menassa, L.; Slaba, S.; Atallah, N.; Nasnas, R.; Abadjian, G.

    1997-01-01

    Fibro-dysplasia ossificans progressiva is a rare congenital disease that affects children under the age of five years. Soft tissue swelling of the cervical and dorsal regions with local pain, warmth and low grade fever are the early clinical manifestations, usually associated with hallux valgus and microdactyly of the fingers and toes. Calcifications of the fascias and muscles cause muscular contractures leading to progressive disability and restrictive lung disease. In the early stage, CT shows edema of the soft tissues and later on, calcifications of muscular fascia. The association of these radiographic and CT findings is specific and should avoid muscular biopsies which are known to be an aggravating factor in this disease. (author)

  6. Mast cell inhibition as a therapeutic approach in fibrodysplasia ossificans progressiva (FOP).

    Science.gov (United States)

    Brennan, Tracy A; Lindborg, Carter M; Bergbauer, Christian R; Wang, Haitao; Kaplan, Frederick S; Pignolo, Robert J

    2018-04-01

    Episodic flare-ups of fibrodysplasia ossificans progressiva (FOP) are characterized clinically by severe, often posttraumatic, connective tissue swelling and intramuscular edema, followed histologically by an intense and highly angiogenic fibroproliferative reaction. This early inflammatory and angiogenic fibroproliferative response is accompanied by the presence of abundant mast cells far in excess of other reported myopathies. Using an injury-induced, constitutively-active transgenic mouse model of FOP we show that mast cell inhibition by cromolyn, but not aprepitant, results in a dramatic reduction of heterotopic ossification. Cromolyn, but not aprepitant, significantly decreases the total number of mast cells in FOP lesions. Furthermore, cromolyn specifically diminishes the number of degranulating and resting degranulated mast cells in pre-osseous lesions. This work demonstrates that consideration of FOP as a type of localized mastocytosis may offer new therapeutic interventions for treatment of this devastating condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Purulent myositis of the thigh as a presentation of perforated low rectal cancer.

    Science.gov (United States)

    Jenkins, V; Steinke, J; Rajendran, N; Kumar, D

    2018-03-01

    Purulent myositis is an acute, intramuscular bacterial infection involving abscess formation most commonly affecting the quadriceps, hamstring and gluteal muscles. We present a case of extensive purulent myositis of the thigh and lower leg caused by bowel perforation below the peritoneal reflection secondary to rectal cancer. Cases of lower limb and perineal purulent myositis should raise suspicion of rectal perforation and should prompt investigations to exclude rectal malignancy.

  8. Adductor muscle pyo-myositis simulating appendicitis: CT and MR imaging findings

    International Nuclear Information System (INIS)

    Coumbaras, M.; Le Hir, P.; Jomaah, N.; Arrive, L.; Tubiana, J.M.

    2001-01-01

    Pyo-myositis is a primary bacterial infection of skeletal muscle. This infection tends to occur in the large muscles of the lower extremity. Pyo-myositis of the proximal muscles of the thigh can simulate acute abdominal disease. Early diagnosis improves the outcome. Delayed diagnosis may lead to septicemia and shock. We report the CT and MRI findings in a patient with pyo-myositis of the proximal muscles of the thigh. (authors)

  9. Lateral rectus myositis mimicking an abducens nerve palsy in a pregnant woman.

    Science.gov (United States)

    Haslinda, Abd-Rahim; Shatriah, Ismail; Azhany, Yaakub; Nik-Ahmad-Zuky, Nik-Lah; Yunus, Rohaizan

    2014-01-01

    Myositis is a rare unknown inflammatory disorder of the skeletal muscle tissue. Generalized inflammatory myopathies, polymyositis, and dermatomyositis have been reported during pregnancy. Isolated orbital myositis in pregnancy has not been previously described in the literature. The authors report a case of left isolated orbital myositis in a primigravida at 38 weeks gestation affecting the patient's left lateral rectus muscle. MRI of the orbit was consistent with the diagnosis. She showed remarkable clinical improvement with oral corticosteroids therapy.

  10. Unilateral temporal myositis heralding polymyositis: ultrasonographic and elastographic findings. Case report.

    Science.gov (United States)

    Damian, Laura; Botar Jid, Carolina; Rogojan, Liliana; Dinu, Cristian; Maniu, Alma; Fodor, Daniela; Rednic, Simona; Simon, Siao-Pin

    2016-03-01

    Temporal myositis is a rare inflammatory disease of the temporal muscle. We report a case of unilateral temporal myositis, in which a polymyositis was diagnosed two years thereafter. Although focal myositis may rarely herald polymyositis, isolated temporal myositis preceding inflammatory myopathies has not been described, to our knowledge. In the setting of a temporal pain and swelling, ultrasonography may help in diagnosis, biopsy guidance, disease extension, and progression assessment. Further studies are necessary to establish the role of elastography in differentiating between muscle inflammation and hypertrophy.

  11. The EuroMyositis registry: an international collaborative tool to facilitate myositis research.

    Science.gov (United States)

    Lilleker, James B; Vencovsky, Jiri; Wang, Guochun; Wedderburn, Lucy R; Diederichsen, Louise Pyndt; Schmidt, Jens; Oakley, Paula; Benveniste, Olivier; Danieli, Maria Giovanna; Danko, Katalin; Thuy, Nguyen Thi Phuong; Vazquez-Del Mercado, Monica; Andersson, Helena; De Paepe, Boel; deBleecker, Jan L; Maurer, Britta; McCann, Liza J; Pipitone, Nicolo; McHugh, Neil; Betteridge, Zoe E; New, Paul; Cooper, Robert G; Ollier, William E; Lamb, Janine A; Krogh, Niels Steen; Lundberg, Ingrid E; Chinoy, Hector

    2018-01-01

    The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%). Smoking was more frequent in connective tissue disease overlap cases (45%, OR 1.44, 95% CI 1.09 to 1.90, p=0.012). Smoking was associated with interstitial lung disease (ILD) (OR 1.32, 95% CI 1.06 to 1.65, p=0.013), dysphagia (OR 1.43, 95% CI 1.16 to 1.77, p=0.001), malignancy ever (OR 1.78, 95% CI 1.36 to 2.33, p<0.001) and cardiac involvement (OR 2.40, 95% CI 1.60 to 3.60, p<0.001).Dysphagia occurred in 39% and cardiac involvement in 9%; either occurrence was associated with higher Health Assessment Questionnaire (HAQ) scores (adjusted OR 1.79, 95% CI 1.43 to 2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95% CI 2.52 to 5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, OR 2.06, 95% CI 1.65 to 2.57, p<0.001).ILD occurred in 30%, most frequently in antisynthetase syndrome (71%, OR 10.7, 95% CI 8.6 to 13.4, p<0.001). Rash characteristics differed between adult-onset and juvenile-onset DM cases ('V' sign: 56% DM vs 16% juvenile-DM, OR 0.16, 95% CI 0.07 to 0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71% and azathioprine in 51%. This large multicentre cohort demonstrates the importance of extramuscular involvement in patients with IIM, its association with smoking and its influence on disease severity. Our findings emphasise that IIM is a multisystem inflammatory disease and will help inform prognosis and clinical management of patients. © Article author(s) (or their employer(s) unless otherwise stated

  12. Management of Myositis-Related Interstitial Lung Disease.

    Science.gov (United States)

    Morisset, Julie; Johnson, Cheilonda; Rich, Eric; Collard, Harold R; Lee, Joyce S

    2016-11-01

    Interstitial lung disease (ILD) is a frequent pulmonary manifestation and an important cause of morbidity and mortality in patients with idiopathic inflammatory myopathy. Myositis-related ILD presents a therapeutic challenge for clinicians, as there are no available guidelines to help with management decisions. This review covers the existing evidence on the pharmacologic and nonpharmacologic management of myositis-related ILD, highlighting the lack of randomized controlled data to guide treatment. Given the absence of existing guidelines to inform treatment decisions, we provide a comprehensive summary, including dosing, side effects, and suggested monitoring of the commonly used immunosuppressive agents and a proposed treatment algorithm based on the existing literature. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  13. TEST performance of a myositis panel in a clinical immunology laboratory in New South Wales, Australia.

    Science.gov (United States)

    Tan, Teck Choon; Wienholt, Louise; Adelstein, Stephen

    2016-10-01

    There is increasing recognition of a clinico-serological correlation between the idiopathic inflammatory myopathies and myositis-specific autoantibodies (MSA). We review the use of a line immunoassay-based myositis panel incorporating both MSA and myositis-associated autoantibodies (MAA) in a selected population of patients. A retrospective analysis of patients with myositis panel assays performed in 2013 were reviewed and compared against clinical diagnoses. A total of 96 patient samples were evaluated, the clinical indications include 60 patients with suspected idiopathic inflammatory myositis (IIM), 24 patients with suspected interstitial lung disease (ILD) and 12 patients with suspected systemic autoimmune disease (SAD). In the myositis group, there were 21 patients diagnosed with IIM and 18 patients diagnosed with IIM had a positive myositis panel. Of the 39 patients without IIM, nine of these patients had a positive myositis panel. In the ILD group, 10 of 24 patients had a positive myositis panel; of these, two were diagnosed anti-synthetase syndrome (ASS) and five patients with ILD. In the suspected SAD group, three had positive myositis panel and all did not appear associated with their final diagnoses. In patients with a clinical diagnosis of IIM or ILD-associated SAD, four patients with anti-PL-12 were detected, three patients with anti-signal recognition protein, two patients with anti-Jo-1, and two patients with anti-Mi2. The myositis panel is an objective investigative modality with a sensitivity of 80.00% and a specificity of 75.76% in a setting of high pretest clinical suspicion. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  14. Diagnostic Evaluation of Nontraumatic Chest Pain in Athletes.

    Science.gov (United States)

    Moran, Byron; Bryan, Sean; Farrar, Ted; Salud, Chris; Visser, Gary; Decuba, Raymond; Renelus, Deborah; Buckley, Tyler; Dressing, Michael; Peterkin, Nicholas; Coris, Eric

    This article is a clinically relevant review of the existing medical literature relating to the assessment and diagnostic evaluation for athletes complaining of nontraumatic chest pain. The literature was searched using the following databases for the years 1975 forward: Cochrane Database of Systematic Reviews; CINAHL; PubMed (MEDLINE); and SportDiscus. The general search used the keywords chest pain and athletes. The search was revised to include subject headings and subheadings, including chest pain and prevalence and athletes. Cross-referencing published articles from the databases searched discovered additional articles. No dissertations, theses, or meeting proceedings were reviewed. The authors discuss the scope of this complex problem and the diagnostic dilemma chest pain in athletes can provide. Next, the authors delve into the vast differential and attempt to simplify this process for the sports medicine physician by dividing potential etiologies into cardiac and noncardiac conditions. Life-threatening causes of chest pain in athletes may be cardiac or noncardiac in origin, which highlights the need for the sports medicine physician to consider pathology in multiple organ systems simultaneously. This article emphasizes the importance of ruling out immediately life threatening diagnoses, while acknowledging the most common causes of noncardiac chest pain in young athletes are benign. The authors propose a practical algorithm the sports medicine physician can use as a guide for the assessment and diagnostic work-up of the athlete with chest pain designed to help the physician arrive at the correct diagnosis in a clinically efficient and cost-effective manner.

  15. Endoscopic optic nerve decompression for nontraumatic compressive optic neuropathy

    Directory of Open Access Journals (Sweden)

    Cheng-long REN

    2015-11-01

    Full Text Available Objective To describe the preliminary experience with endoscopic optic nerve decompression (EOND for nontraumatic compressive optic neuropathies (NCONs. Methods The clinical data of 10 patients, male 5 and female 5, with a mean age of 44.3±5.1 years, who underwent EOND for visual loss (n=5 or visual deterioration (n=5 due to tumor compression in General Hospital of Armed Police Forces of China in the period from April 2013 to April 2014 were analyzed retrospectively. Preoperative and 6-month-postoperative clinical and imaging data of these patients were reviewed and analyzed. Results Among 5 patients who lost light perception (including 2 patients with bilateral optic nerve compression before operation, 4 of them showed visual improvement to different degrees on the 7th day after operation (with improvement of bilateral visual acuity. The other 5 patients with visual impairment before operation recovered their visual acuity to different extent after the operation. All of the patients had no obvious post-operative complications. Conclusion EOND is a safe, effective, and minimally invasive surgical technique affording recovery of visual function to NCON patients. DOI: 10.11855/j.issn.0577-7402.2015.11.12

  16. Nontraumatic spontaneous rupture of the kidney : etiology and CT findings

    International Nuclear Information System (INIS)

    Heo, Tae Haeng; Jeon, Hae Jeong; Shin, Hyun Joon; Kim, Bo Hyun; Cho, Kyoung Sik; Kim, Young Hwa; Kim, Seung Hyup; Park, Churl Min

    1997-01-01

    To evaluate the usefulness of CT scanning in determining the etiology of spontaneous rupture of the kidney We retrospectively analyzed the CT findings of spontaneous rupture of the kidney in eleven patients, Four were male and seven were female, and they were aged between 20 and 71 (mean, 46.6) years. Both pre- and post-contrast enhanced CT scanning was performed in all patients. Spontaneous renal rupture was induced in seven cases by neoplasms (three angiomyolipomas, three renal cell carcinomas, and one metastatic choriocarcinoma), in three cases by infection or inflammation (acute and chronic pyelonephritis, and renal abscess), and in one, by renal cyst. Common CT findings of rupture of the kidney were the accumulation of high density fluid in the perirenal and anterior pararenal space, and inhomogeneous irregular low density of renal parenchyma and the rupture site. Angiomyolipoma showed fat and an angiomatous component in the lesion, while acute and chronic pyelonephrities revealed thinning of the renal parenchyma and an irregular renal outline. Renal cell carcinoma showed a dense soft tissue mass in the parenchyma. Well-defined, round low-density lesions were noted in the case of renal cyst and renal abscess. CT is very useful in diagnosing and determining the etiology of non-traumatic spontaneous rupture of the kidney and plays an important role in the evaluation of emergency cases

  17. The Clinical Features of Myositis-Associated Autoantibodies: a Review.

    Science.gov (United States)

    Gunawardena, Harsha

    2017-02-01

    The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

  18. Tubercular myositis of infraspinatus: a rare clinical entity

    OpenAIRE

    Vikas Verma; Yogesh Kumar Yadav; Anuj Rastogi; Farid Mohammed

    2016-01-01

    Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the sc...

  19. CT in idiopathic pyogenic myositis of the iliopsoas muscle

    International Nuclear Information System (INIS)

    Kvernebo, K.; Stiris, G.; Haaland, M.; Aker Sykehus, Oslo; Buskerud Country Hospital

    1983-01-01

    Pyogenic myositis of the iliopsoas muscle may occur as a primary clinical entity of an idiopathie nature, or more commonly secondarily to an adjacent disease process. We report 2 cases of idiopathic pyogenic infection caused by Staphylococcus aureus. This disease entity is rare in temperate climates. CT combined with clinical and biochemical information enabled the correct diagnosis, and appropriate treatment could thus be started. (orig.)

  20. Patients' Experience of Myositis and Further Validation of a Myositis-specific Patient Reported Outcome Measure - Establishing Core Domains and Expanding Patient Input on Clinical Assessment in Myositis. Report from OMERACT 12.

    Science.gov (United States)

    Regardt, Malin; Basharat, Pari; Christopher-Stine, Lisa; Sarver, Catherine; Björn, Anita; Lundberg, Ingrid E; Wook Song, Yeong; Bingham, Clifton O; Alexanderson, Helene

    2015-12-01

    The Outcome Measures in Rheumatology (OMERACT) myositis working group was established to examine patient-reported outcomes (PRO) as well as to validate patient-reported outcome measures (PROM) in myositis. Qualitative studies using focus group interviews and cognitive debriefing of the myositis-specific Myositis Activities Profile (MAP) were used to explore the experience of adults living with polymyositis (PM) and dermatomyositis (DM). Preliminary results underscore the importance of patient input in the development of PROM to ensure content validity. Results from multicenter focus groups indicate the range of symptoms experienced including pain, fatigue, and impaired cognitive function, which are not currently assessed in myositis. Preliminary cognitive debriefing of the MAP indicated that while content was deemed relevant and important, several activities were not included; and that questionnaire construction and wording may benefit from revision. A research agenda was developed to continue work toward optimizing PRO assessment in myositis with 2 work streams. The first would continue to conduct and analyze focus groups until saturation in the thematic analysis was achieved to develop a framework that encompassed the patient-relevant aspects of myositis. The second would continue cognitive debriefing of the MAP to identify potential areas for revision. There was agreement that further work would be needed for inclusion body myositis and juvenile dermatomyositis, and that the inclusion of additional contributors such as caregivers and individuals from the pharmaceutical/regulatory spheres would be desirable. The currently used PROM do not assess symptoms or the effects of disease that are most important to patients; this emphasizes the necessity of patient involvement. Our work provides concrete examples for PRO identification.

  1. Brief Report: Association of Myositis Autoantibodies, Clinical Features, and Environmental Exposures at Illness Onset With Disease Course in Juvenile Myositis.

    Science.gov (United States)

    Habers, G Esther A; Huber, Adam M; Mamyrova, Gulnara; Targoff, Ira N; O'Hanlon, Terrance P; Adams, Sharon; Pandey, Janardan P; Boonacker, Chantal; van Brussel, Marco; Miller, Frederick W; van Royen-Kerkhof, Annet; Rider, Lisa G

    2016-03-01

    To identify early factors associated with disease course in patients with juvenile idiopathic inflammatory myopathies (IIMs). Univariable and multivariable multinomial logistic regression analyses were performed in a large juvenile IIM registry (n = 365) and included demographic characteristics, early clinical features, serum muscle enzyme levels, myositis autoantibodies, environmental exposures, and immunogenetic polymorphisms. Multivariable associations with chronic or polycyclic courses compared to a monocyclic course included myositis-specific autoantibodies (multinomial odds ratio [OR] 4.2 and 2.8, respectively), myositis-associated autoantibodies (multinomial OR 4.8 and 3.5), and a documented infection within 6 months of illness onset (multinomial OR 2.5 and 4.7). A higher overall clinical symptom score at diagnosis was associated with chronic or monocyclic courses compared to a polycyclic course. Furthermore, severe illness onset was associated with a chronic course compared to monocyclic or polycyclic courses (multinomial OR 2.1 and 2.6, respectively), while anti-p155/140 autoantibodies were associated with chronic or polycyclic courses compared to a monocyclic course (multinomial OR 3.9 and 2.3, respectively). Additional univariable associations of a chronic course compared to a monocyclic course included photosensitivity, V-sign or shawl sign rashes, and cuticular overgrowth (OR 2.2-3.2). The mean ultraviolet index and highest ultraviolet index in the month before diagnosis were associated with a chronic course compared to a polycyclic course in boys (OR 1.5 and 1.3), while residing in the Northwest was less frequently associated with a chronic course (OR 0.2). Our findings indicate that myositis autoantibodies, in particular anti-p155/140, and a number of early clinical features and environmental exposures are associated with a chronic course in patients with juvenile IIM. These findings suggest that early factors, which are associated with poorer

  2. Scintigraphic evaluation of Lyme disease: Gallium-67 imaging of Lyme myositis

    International Nuclear Information System (INIS)

    Kengen, R.A.; Linde, M. von der; Sprenger, H.G.; Piers, D.A.

    1989-01-01

    A patient suffering from Lyme disease had cardiac conduction abnormalities, symptoms of arthritis, and myalgia. A Ga-67 image showed evidence of endomyocarditis, but intense skeletal muscle uptake pointed to Lyme myositis. Reference is made to two other case reports of Lyme myositis

  3. Recurrent Focal Myositis in Childhood: A Case Report and Systematic Review of the Literature.

    Science.gov (United States)

    Milani, Gregorio P; Mazzoni, Marta B M; Gatti, Helga; Bertolozzi, Giuseppe; Fossali, Emilio F

    2017-06-01

    Recurrent focal myositis in adulthood has been documented in case reports and case series. Existing textbooks and reviews do not mention or mention only in passing this entity in childhood. We present a patient with recurrent focal myositis and summarize available clinical, laboratory, management, and outcome data on this entity in the pediatric ages. We describe a nine-year-old patient with recurrent myositis of the left biceps. The terms "myositis" and "relapsing" or "recurrent" or "recurrence" were searched using the United States National Library of Medicine and the Excerpta Medica Database. Pertinent secondary references were also screened. Another seven pediatric patients (five males and two females, median age ten years, interquartile range 7-14 years) with recurrent focal myositis were identified. In children, the calf was the most frequently involved muscle. Unlike adults, the myositis in children was usually painful. Episodes could be associated with normal or elevated erythrocyte sedimentation rate and blood levels of C-reactive protein, creatine kinase, and aspartate aminotransferase. Abnormalities of the creatine kinase value did not seem to be associated with a higher risk of recurrences. Focal myositis has a favorable outcome in children. Recurrent focal myositis is rare and usually benign in childhood. More data are needed to improve the understanding of this condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

    Science.gov (United States)

    Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

    2013-07-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

  5. Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients

    Science.gov (United States)

    Lloyd, Thomas E.; Pinal-Fernandez, Iago; Michelle, E. Harlan; Christopher-Stine, Lisa; Pak, Katherine; Sacktor, Ned

    2017-01-01

    Objective: To characterize patients with myositis with HIV infection. Methods: All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti–nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed. Results: Eleven of 1,562 (0.7%) patients with myositis were HIV-positive. Myositis was the presenting feature of HIV infection in 3 patients. Eight of 11 patients had weakness onset at age 45 years or less. The mean time from the onset of weakness to the diagnosis of myositis was 3.6 years (SD 3.2 years). The mean of the highest measured CK levels was 2,796 IU/L (SD 1,592 IU/L). On muscle biopsy, 9 of 10 (90%) had endomysial inflammation, 7 of 10 (70%) had rimmed vacuoles, and none had perifascicular atrophy. Seven of 11 (64%) patients were anti-NT5C1A-positive. Upon presentation, all had proximal and distal weakness. Five of 6 (83%) patients followed 1 year or longer on immunosuppressive therapy had improved proximal muscle strength. However, each eventually developed weakness primarily affecting wrist flexors, finger flexors, knee extensors, or ankle dorsiflexors. Conclusions: HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies. PMID:28283597

  6. Application of human induced pluripotent stem cells to model fibrodysplasia ossificans progressiva.

    Science.gov (United States)

    Barruet, Emilie; Hsiao, Edward C

    2018-04-01

    Fibrodysplasia ossificans progressiva (FOP) is a genetic condition characterized by massive heterotopic ossification. FOP patients have mutations in the Activin A type I receptor (ACVR1), a bone morphogenetic protein (BMP) receptor. FOP is a progressive and debilitating disease characterized by bone formation flares that often occur after trauma. Since it is often difficult or impossible to obtain large amounts of tissue from human donors due to the risks of inciting more heterotopic bone formation, human induced pluripotent stem cells (hiPSCs) provide an attractive source for establishing in vitro disease models and for applications in drug screening. hiPSCs have the ability to self-renew, allowing researchers to obtain large amounts of starting material. hiPSCs also have the potential to differentiate into any cell type in the body. In this review, we discuss how the application of hiPSC technology to studying FOP has changed our perspectives on FOP disease pathogenesis. We also consider ongoing challenges and emerging opportunities for the use of human iPSCs in drug discovery and regenerative medicine. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Clinical aspects and conservative dental management of a patient with fibrodysplasia ossificans progressiva.

    Science.gov (United States)

    Oliveira, Francisco Artur Forte; Fernandes, Clarissa Pessoa; Araujo, Kenia Samara Barbosa; Alves, Ana Paula Negreiros Nunes; Sousa, Fabrício Bitu; Mota, Mário Rogério Lima

    2014-01-01

    T o present the clinical findings of a patient with fibrodysplasia ossificans progressiva (FOP), highlighting peculiarities of dental treatment in patients with this condition. FOP is a rare genetic disease characterized by skeletal malformations and ectopic ossifications in skeletal muscles, tendons, ligaments and aponeurosis. Exacerbation of these ossifications can be caused by dental treatment, resulting in disease progression. A 26-year-old male patient with a diagnosis of FOP was referred to our service for dental treatment. The patient presented decreased mobility in peripheral joints (knees and elbows), postural disability (ankylosis of the vertebral column), lateral deviation and shortness of the hallux, as well as heterotopic ossifications on the hands and back. The implementation of conservative dental procedures, such as oral hygiene instructions and recurrent topical fluoride applications, were performed in addition to endodontic and restorative treatments. Brief dental appointments were conducted without using regional anesthesia or dental dam clamps. The dental chair was positioned at 45° to provide more comfort and to avoid exacerbating the disease. The patient has now completed 6 months of follow-up and is free of heterotopic ossifications resulting from dental treatment. The dental treatment modifications implemented for the present case were sufficient to establish good oral health and to prevent the formation of heterotopic ossifications in the maxillofacial region. FOP is a rare disease dentists must familiarize themselves with to provide adequate, personalized treatment, which minimizes traumas that may exacerbate the disease.

  8. Nontraumatic head and neck emergencies: a clinical approach. Part 1: cervicofacial swelling, dysphagia, and dyspnea.

    Science.gov (United States)

    Brea Álvarez, B; Tuñón Gómez, M; Esteban García, L; García Hidalgo, C Y; Ruiz Peralbo, R M

    2016-01-01

    Nontraumatic emergencies of the head and neck represent a challenge in the field of neuroradiology for two reasons: first, they affect an area where the thorax joins the cranial cavity and can thus compromise both structures; second, they are uncommon, so they are not well known. Various publications focus on nontraumatic emergencies of the head and neck from the viewpoints of anatomic location or of particular diseases. However, these are not the most helpful viewpoints for dealing with patients in the emergency department, who present with particular signs and symptoms. We propose an analysis starting from the four most common clinical presentations of patients who come to the emergency department for nontraumatic head and neck emergencies: cervical swelling, dysphagia, dyspnea, and loss of vision. Starting from these entities, we develop an approach to the radiologic management and diagnosis of these patients. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Usefulness of MR angiography in patients with non-traumatic intracranial hemorrhagic diseases

    International Nuclear Information System (INIS)

    Chung, Hyun Ung; Seo, Jeong Jin; Kim, Yun Hyeon; Moon, Woong Jae; Kim, Jae Kyu; Kang, Heoung Keun

    1994-01-01

    We assessed the usefulness of magnetic resonance angiography(MRA) and its techniques for differential diagnosis of hemorrhagic causes in patients with non-traumatic intracranial hemorrhagic diseases. We retrospectively reviewed 74 patients with non-traumatic intracranial hemorrhagic diseases, which were confirmed by radiological examinations(36 cases) and operations(38 cases). We compared the usefulness of magnetic resonance imaging(MRI) alone from MRI with MRA in evaluation of hemorrhagic causes. MRA was obtained by Time-of-Flight(TOF) and Phase Contrast(PC) technique. We investigated the usefulness of TOF and PC technique. MRI with MRA for detection of hemorrhagic causes(89%, 66 cases) was better than MRI only (64%, 47 cases). PC was better than TOF for evaluation of arteriovenous malformation and aneurysm due to subtraction of background noise(hemorrhage). MRI with MRA is more useful than MRI alone for evaluation of non-traumatic intracranial hemorrhagic causes

  10. Diagnostic performance of a commercial immunoblot assay for myositis antibody testing.

    Science.gov (United States)

    Bundell, Chris; Rojana-Udomsart, Arada; Mastaglia, Frank; Hollingsworth, Peter; McLean-Tooke, Andrew

    2016-06-01

    The objective of this study was to establish a population based reference range for a commercial immunoblot assay detecting myositis specific autoantibodies (MSAs) and myositis associated autoantibodies (MAAs), and to assess the diagnostic performance of this reference range against the manufacturer's recommended ranges in a myositis patient cohort. A total of 124 patients from a myositis cohort and 197 healthy controls were serologically assessed using a commercial immunoblot containing eleven autoantigens (Jo-1, EJ, OJ, PL7, PL12, Mi-2, SRP, Ku, PMScl75, PMScl100 and Ro52) according to the manufacturer's instructions. Use of the manufacturer's reference ranges resulted in detection of MSAs in 19.4% of myositis patients and 9.1% of controls; MAAs were detected in 41.1% of myositis patients and 14.2% of controls. Reference values derived from the healthy control population resulted in significant differences in cut-off values for some autoantibodies, particularly Ro52 and PMScl75. Use of local reference ranges reduced detection of MSAs to 16.9% of myositis patients and 3% of healthy controls, with MAAs 23.4% of patients and 2% of healthy controls. Application of population based reference ranges resulted in significant differences in detection of MSAs and MAAs compared to the manufacturer's recommended ranges. Cut-off levels should be assessed to ensure suitability for the population tested. Copyright © 2016. Published by Elsevier B.V.

  11. Significance of myositis autoantibody in patients with idiopathic interstitial lung disease.

    Science.gov (United States)

    Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo; Kang, Eun-Suk

    2015-05-01

    Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category.

  12. International collaboration including patients is essential to develop new therapies for patients with myositis.

    Science.gov (United States)

    Lundberg, Ingrid E; Vencovsky, Jiri

    2017-05-01

    To discuss the needs for international collaborations between investigators in different disciplines working with myositis and with patients with myositis. Recent advances in detection of several myositis-specific autoantibodies that are associated with distinct clinical phenotypes, will enable studies in new well defined clinically homogenous subgroups of myositis This is likely to lead to development of new information on molecular pathogenesis that might be different in different myositis subgroups. Subgrouping patients according to autoantibody profile may also be important to assess outcome, to identify prognostic biomarkers and in clinical trials. As these are rare disorders international collaboration is essential to enrol large enough cohorts of the subgroups. To facilitate such collaboration we have developed a web-based international myositis register, www.euromyositis.eu, which includes validated outcome measures and patient reported outcome measures. This register is to support research but also to support decision-making in the clinic. We welcome investigators to join the Euromyositis register. Myositis is a heterogeneous disorder with varying treatment response and outcome. There is a high unmet need for new therapies which can only be achieved by increased knowledge on molecular disease mechanisms. Subgrouping patients according to autoantibody profile may be a new way forward to get a better understanding on disease mechanisms and to develop novel therapies.

  13. Idiopathic Inflammatory Myopathies; Association with Overlap Myositis and Syndromes: Classification, Clinical Characteristics, and Associated Autoantibodies

    Directory of Open Access Journals (Sweden)

    Pari Basharat

    2016-07-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are traditionally identified as a group of disorders that target skeletal muscle due to autoimmune dysfunction. The IIM can be divided into subtypes based on certain clinical characteristics, and several classification schemes have been proposed. The predominant diagnostic criteria for IIM is the Bohan and Peter criteria, which subdivides IIM into primary polymyositis (PM, primary dermatomyositis (DM, myositis with another connective tissue disease, and myositis associated with cancer. However, this measure has been criticised for several reasons including lack of specific criteria to help distinguish between muscle biopsy findings of PM, DM, and immune-mediated necrotising myopathy, as well as the lack of identification of cases of overlap myositis (OM. Because of this issue, other classification criteria for IIM have been proposed, which include utilising myositis-associated antibodies and myositis-specific antibodies, as well as overlap features such as Raynaud’s phenomenon, polyarthritis, oesophageal abnormalities, interstitial lung disease, small bowel abnormalities such as hypomotility and malabsorption, and renal crises, amongst others. Indeed, the identification of autoantibodies associated with certain clinical phenotypes of myositis, in particular connective tissue disease-myositis overlap, has further helped divide IIM into distinct clinical subsets, which include OM and overlap syndromes (OS. This paper reviews the concepts of OM and OS as they pertain to IIM, including definitions in the literature, clinical characteristics, and overlap autoantibodies.

  14. Increased visfatin levels are associated with higher disease activity in anti-Jo-1-positive myositis patients.

    Science.gov (United States)

    Hulejová, Hana; Kryštůfková, Olga; Mann, Heřman; Klein, Martin; Pavlíčková, Klára; Zámečník, Josef; Vencovský, Jiří; Šenolt, Ladislav

    2016-01-01

    The aim of this study was to evaluate serum levels of visfatin in anti-Jo-1-positive myositis patients, its expression in muscle tissue and to investigate potential relationships between visfatin, B-cell activating factor of the TNF family (BAFF), disease activity and anti-Jo-1 autoantibody levels. Serum levels of visfatin and BAFF were measured in 38 anti-Jo-1 positive myositis patients and 35 healthy subjects. Disease activity was evaluated by myositis disease activity assessment tool (MYOACT) using visual analogue scales (VAS) and by serum muscle enzymes. Visfatin expression was evaluated by immunohistochemistry in muscle tissue of myositis patients (n=10) and compared with non-inflammatory control muscle tissue samples from patients with myasthenia gravis (n=5). Serum visfatin and BAFF levels were significantly higher in myositis patients compared to healthy subjects and were associated with clinical muscle activity assessed by VAS. Only serum BAFF levels, but not visfatin levels, positively correlated with muscle enzyme concentrations and anti-Jo1 antibody levels. There was a positive correlation between visfatin and BAFF serum levels in myositis patients but a negative correlation was observed in healthy subjects. Visfatin expression was up-regulated in endomysial and perimysial inflammatory infiltrates of muscle tissue from myositis patients. Up-regulation of visfatin in myositis muscle tissue and an association between increased visfatin levels and muscle disease activity evaluated by MYOACT in anti-Jo-1 positive myositis patients could support possible role of visfatin in the pathogenesis of myositis.

  15. Crura sign: differentiation between traumatic rupture of the diaphragm and nontraumatic diaphragmatic elevation on CT

    International Nuclear Information System (INIS)

    Kim, Sung Jin; Han, Hae Ja; Kim, Wang Jung; Youk, Yong Soo; Han, Gi Seok; Cha, Sang Hoon; Park, Kil Sun; Kim, Dae Young

    1997-01-01

    To evaluate whether in patients with diffuse evaluation of a hemidiaphragm on chest radiographs, the apparence of the crura on CT might be helpful in differentiating between traumatic rupture of the diaphragm (TRD) and nontraumatic causes such as diaphragmatic palsy or diffuse diaphragmatic eventration. Among patients with diffuse elevations of a hemidiaphragm on chest radiograph, 27 who had patients undergone CT scans were retrospectively reviewed. Twelve patients had surgically proven TRD, and 15 had nontraumatic elevation of a hemidiaphragm such as diaphragmatic palsy or diffuse diaphragmatic eventration. Under the hypothesis that the affected crus is markedly thinner than the normal side in nontraumatic elevation but is normal in TRD ('crura sign', we optically assessed without measurement the thickness of both crura. In all patients with TRD, the thickness of the affected crus was similar to that of the contralateral side. In all patients with nontraumatic causes, however, the crus of the elevated hemidiaphragm was markedly thinner than that of the normal side. The 'crura sign' may be useful additional CT finding of traumatic rupture the diaphragm

  16. Non-traumatic perforation of the small bowel | Eid | African Health ...

    African Journals Online (AJOL)

    ... medical records of four patients who have presented with non-traumatic perforation of the small bowel and were treated at Al-Ain Hospital during the last 5 years were studied retrospectively. Results: The presenting symptoms of all patients were similar. Erect chest X-ray has shown free air under diaphragm in 3 patients.

  17. Acute pure motor quadriplegia: is it dengue myositis?

    Science.gov (United States)

    Kalita, J; Misra, U K; Mahadevan, A; Shankar, S K

    2005-01-01

    In view of paucity of comprehensive evaluation about dengue infection producing quadriplegia, we report the clinical, laboratory and neurophysiological studies in these patients. Seven out of 16 patients with dengue infection presented with quadriplegia and they were subjected to a detailed clinical history and examination. Diagnosis of dengue was based on characteristic clinical and positive serum IgM ELISA. Blood counts, serum chemistry, CSF analysis and nerve conduction and electromyographic (EMG) studies were performed in all. Outcome was defined at the end of 1 month into complete, partial and poor on the basis of activities of daily living The age of the patients ranged between 9 and 42 years and 2 were females. Fever was present in all and myalgia in 5 patients. Weakness developed within 3-5 days of illness, which was severe in 4 and moderate in 3 patients. Hypotonia and hyporeflexia were present in 5 patients. Nerve conduction and EMG studies were normal in all except one whose EMG was myopathic. Serum CPK and SGPT were raised in all and serum bilirubin in 3 patients. All the patients had coagulopathy and 6 had thrombocytopenia. Muscle biopsy in 1 patient was suggestive of myositis. Six patients improved completely and one had poor recovery who needed ventilatory support. Dengue virus infection may result in acute pure motor quadriplegia due to myositis. In an endemic area it should be considered in the differential diagnosis of acute flaccid paralysis.

  18. Temporal relationship between cancer and myositis identifies two distinctive subgroups of cancers: impact on cancer risk and survival in patients with myositis.

    Science.gov (United States)

    Kang, Eun Ha; Lee, Sang Jin; Ascherman, Dana P; Lee, Yun Jong; Lee, Eun Young; Lee, Eun Bong; Song, Yeong Wook

    2016-09-01

    The aim was to compare standardized incidence ratios (SIRs) of cancers temporally related and unrelated to active myositis in patients with myositis. Fifty-two cancer cases were identified in 281 myositis patients. SIRs of cancers having temporal overlap with the active phase of myositis [cancers concurrent with active myositis (CAM), n = 30] and cancers not having such temporal overlap [cancers non-concurrent with active myositis (CNM), n = 22] were compared in 281 patients. Patients with CAM were older at diagnosis of myositis, had a greater tendency to be male, more frequent dysphagia and less frequent interstitial lung disease than patients with CNM. CAM SIR (95% CI) was 1.78 (1.19, 2.56) and CNM SIR 1.23 (0.75, 1.90). The peak SIR was observed in the seventh decade of life for CAM and in the third decade for CNM. When stratified by myositis-cancer intervals, CAM SIR was 9.94 (6.43, 14.67) within 1 year of myositis diagnosis, whereas no temporal relationship was found for CNM. Elevated SIRs were observed for oesophageal cancer [57.77 (11.91, 168.82)], non-Hodgkin's lymphoma [41.43 (13.45, 96.69)], adenocarcinoma of unknown primary origin [67.6 (18.42, 173.07]), lung cancer [7.27 (1.98, 18.61)] and ovarian cancer [19.15 (2.32, 69.17)] within 3 years of CAM diagnosis. The cancer stage at the time of diagnosis was more advanced in CAM than CNM (P < 0.001), with a correspondingly increased hazard ratio of mortality [4.3 (1.5, 12.7)] in patients with CAM vs CNM. A significantly elevated SIR was found for CAM, whereas there was a comparable SIR for CNM relative to the general population. Multiple types of cancers showed elevated SIRs among CAM, but none among CNM. Given that cancer stages in CAM were far advanced at diagnosis, mortality risk was greater in patients with CAM. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  19. Development of detection method of early stage nontraumatic osteo-necrosis of the femoral head by dynamic MRI

    International Nuclear Information System (INIS)

    Morita, Fuminori; Ikehira, Hiroo; Kitahara, Hiroshi; Terada, Tomoko; Nakano, Yoshitada; Ishii, Teruyuki; Iida, Tetsu; Ikenouchi, Sumio; Moriya, Hideshige

    1999-01-01

    The early detection methods of nontraumatic osteo-necrosis of the femoral head were demonstrated with the dynamic or static enhancement MR imaging method using gadolinium-DTPA (Gd-DTPA). Even if with these methods we could not detect stage 0 of nontraumatic osteo-necrosis, but these pathological change should be induced at 0 stage in the patients who were medicated high-dose corticosteroids. The authors designed the big ROI (region of interest) dynamic MR imaging method to brake this diagnostic difficulty for 0 stage of femoral, and evaluated the efficacy of this technology with normal and femoral nontraumatic osteo-necrosis patients volunteer. (author)

  20. INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

    NARCIS (Netherlands)

    GROEN, H; POSTMA, DS; KALLENBERG, CGM

    1993-01-01

    A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

  1. Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

    Directory of Open Access Journals (Sweden)

    Joshua R. Francis

    2016-01-01

    Full Text Available A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described.

  2. MR findings of infectious myositis caused by vibrio vulnificus: case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Joon Ho; Na, Jae Boem [Gyeongsang National University College of Medicine, Jinju (Korea, Republic of)

    2003-03-01

    Vibrio vulnificus infection is a fatal disease occurring after the consumption of seafood in patients with underlying liver disease. Inflammation of the skin, subcutanous fat and fascia disseminates from the lower extremity to the trunk and upper extremity. Infection myositis caused by vibrio vulnificus is rare, and its MR findings have not been reported. We report these in a case of infectious myositis caused by vibrio vulnificus involving both lower extremities.

  3. MR findings of infectious myositis caused by vibrio vulnificus: case report

    International Nuclear Information System (INIS)

    Lee, Joon Ho; Na, Jae Boem

    2003-01-01

    Vibrio vulnificus infection is a fatal disease occurring after the consumption of seafood in patients with underlying liver disease. Inflammation of the skin, subcutanous fat and fascia disseminates from the lower extremity to the trunk and upper extremity. Infection myositis caused by vibrio vulnificus is rare, and its MR findings have not been reported. We report these in a case of infectious myositis caused by vibrio vulnificus involving both lower extremities

  4. Atypical proliferative myositis: original MR description with pathologic correlation: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Jarraya, Mohamed; Guermazi, Ali [Boston University School of Medicine, Department of Radiology, Musculoskeletal Section, Boston, MA (United States); Parva, Pedram [VA Boston Healthcare System, Boston, MA (United States); Stone, Michael [Stamford Hospital, Department of Surgery, Stamford, CT (United States); Klein, Michael J. [Hospital for Special Surgery, Department of Pathology and Laboratory Medicine, New York, NY (United States)

    2014-08-15

    Proliferative myositis (PM) along with proliferative fasciitis and nodular fasciitis are a group of pseudosarcomatous myofibroblastic proliferations. Although the histologic presentation of each is almost identical, the magnetic resonance imaging (MRI) appearance of proliferative myositis is closer to that of inflammatory myopathies. We report a case of PM in which the imaging and histologic features combine typical findings of PM with unusual imaging features, suggesting of reactive (or nodular) fasciitis. (orig.)

  5. Atypical proliferative myositis: original MR description with pathologic correlation: Case report

    International Nuclear Information System (INIS)

    Jarraya, Mohamed; Guermazi, Ali; Parva, Pedram; Stone, Michael; Klein, Michael J.

    2014-01-01

    Proliferative myositis (PM) along with proliferative fasciitis and nodular fasciitis are a group of pseudosarcomatous myofibroblastic proliferations. Although the histologic presentation of each is almost identical, the magnetic resonance imaging (MRI) appearance of proliferative myositis is closer to that of inflammatory myopathies. We report a case of PM in which the imaging and histologic features combine typical findings of PM with unusual imaging features, suggesting of reactive (or nodular) fasciitis. (orig.)

  6. Core biopsy as a simple and effective diagnostic tool in head and neck focal myositis.

    Science.gov (United States)

    Tan, Chun Yee; Chong, Sheldon; Shaw, Chi-Kee Leslie

    2015-12-01

    Most unilateral head and neck masses are benign, although malignancy is a possibility in some cases. However, there are other rare causes of unilateral neck masses, such as focal myositis, which is a rare, benign condition belonging to the family of inflammatory pseudotumors of the skeletal muscles, with rare presentations in the head and neck region. Focal myositis presents as a rapidly enlarging neck mass that can be misdiagnosed by fine-needle aspiration biopsy and/or radiologic imaging as either an infective or a neoplastic process. To date, there are only 5 reported cases of adult focal myositis of the sternocleidomastoid muscle in the medical literature. In this article, the authors present 2 cases involving patients with focal myositis of the sternocleidomastoid muscle that were successfully diagnosed with core-needle biopsy and managed conservatively. The pros and cons of fine-needle aspiration biopsy and core-needle biopsy are discussed. Based on the authors' results, fine-needle aspiration biopsy universally fails to provide the diagnosis of focal myositis. In contrast, core-needle biopsy successfully diagnosed focal myositis in both of our patients. Both of them had complete resolution with conservative management.

  7. Myositis registries and biorepositories: powerful tools to advance clinical, epidemiologic and pathogenic research.

    Science.gov (United States)

    Rider, Lisa G; Dankó, Katalin; Miller, Frederick W

    2014-11-01

    Clinical registries and biorepositories have proven extremely useful in many studies of diseases, especially rare diseases. Given their rarity and diversity, the idiopathic inflammatory myopathies, or myositis syndromes, have benefited from individual researchers' collections of cohorts of patients. Major efforts are being made to establish large registries and biorepositories that will allow many additional studies to be performed that were not possible before. Here, we describe the registries developed by investigators and patient support groups that are currently available for collaborative research purposes. We have identified 46 myositis research registries, including many with biorepositories, which have been developed for a wide variety of purposes and have resulted in great advances in understanding the range of phenotypes, clinical presentations, risk factors, pathogenic mechanisms, outcome assessment, therapeutic responses, and prognoses. These are now available for collaborative use to undertake additional studies. Two myositis patient registries have been developed for research, and myositis patient support groups maintain demographic registries with large numbers of patients available to be contacted for potential research participation. Investigator-initiated myositis research registries and biorepositories have proven extremely useful in understanding many aspects of these rare and diverse autoimmune diseases. These registries and biorepositories, in addition to those developed by myositis patient support groups, deserve continued support to maintain the momentum in this field as they offer major opportunities to improve understanding of the pathogenesis and treatment of these diseases in cost-effective ways.

  8. The Co-Existence of Myasthenia Gravis in Patients with Myositis: A Case Series

    Science.gov (United States)

    Paik, Julie J.; Corse, Andrea M.; Mammen, Andrew L.

    2014-01-01

    Objective Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients. Methods We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features. Results All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%). High dose steroids were associated with worsening weakness in 2/6 (33%) patients. Conclusions Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis-MG overlap, high dose steroids may exacerbate symptoms and pryidostigmine may play an important therapeutic role. PMID:24412588

  9. Autoantibody levels in myositis patients correlate with clinical response during B cell depletion with rituximab.

    Science.gov (United States)

    Aggarwal, Rohit; Oddis, Chester V; Goudeau, Danielle; Koontz, Diane; Qi, Zengbiao; Reed, Ann M; Ascherman, Dana P; Levesque, Marc C

    2016-06-01

    To determine the longitudinal trends in serum levels of four myositis-associated autoantibodies: anti-Jo-1, -transcription intermediary factor 1 γ (TIF1-γ), -signal recognition particle (SRP) and -Mi-2, after B cell depletion with rituximab, and to determine the longitudinal association of these autoantibody levels with disease activity as measured by myositis core-set measures (CSMs). Treatment-resistant adult and pediatric myositis subjects (n = 200) received rituximab in the 44-week Rituximab in Myositis Trial. CSMs [muscle enzymes, manual muscle testing (MMT), physician and patient global disease activity, HAQ, and extramuscular disease activity] were evaluated monthly and anti-Jo-1 (n = 28), -TIF1-γ (n = 23), -SRP (n = 25) and -Mi-2 (n = 26) serum levels were measured using validated quantitative ELISAs. Temporal trends and the longitudinal relationship between myositis-associated autoantibodies levels and CSM were estimated using linear mixed models. Following rituximab, anti-Jo-1 levels decreased over time (P myositis subjects decreased after B cell depletion and were correlated with changes in disease activity, whereas anti-SRP levels were only associated with longitudinal muscle enzyme levels. The strong association of anti-Jo-1 levels with clinical outcomes suggests that anti-Jo-1 autoantibodies may be a good biomarker for disease activity. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  10. [Clinical and histopathological features of myositis associated with anti-mitochondrial antibodies].

    Science.gov (United States)

    Shimizu, Jun

    2013-01-01

    Anti-mitochondrial antibodies (AMA) are known to be characteristic markers of primary biliary cirrhosis (PBC). The association of PBC with myositis has been reported mainly as case reports, and comprehensive studies of the clinical and histopathological features of patients with myositis and AMAs or PBC have not been conducted thus far. We retrospectively reviewed 212 patients with inflammatory myopathies in our laboratory and found 24 patients with AMA-positive myositis (11%) (seven patients with PBC and 17 patients without PBC). The analysis of clinical and histopathological features revealed that myositis associated with AMAs frequently include patients with a clinically chronic disease course, muscle atrophy, cardiopulmonary involvement and granulomatous inflammation, regardless of the presence or absence of PBC. We also reviewed and analyzed the clinical features of previously reported patients. The analysis of 75 patients, which have been described in previous case reports including the ones of meeting abstracts, also showed the similar results about clinical features of myositis associated with AMAs and supported our findings. Our study suggests that myositis associated with AMAs form a characteristic subgroup.

  11. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting as diffuse myositis.

    Science.gov (United States)

    Parent, Marc-Etienne; Larue, Sandrine; Ellezam, Benjamin

    2014-11-21

    Eosinophilic granulomatosis with polyangiitis is a complex multisystemic syndrome with heterogeneous presentation. Most often, there is a clinical history of asthma or other atopic conditions, and current presentation generally includes signs of cutaneous or pulmonary involvement. Very few reports described myalgia or weakness as the chief complaint. Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis. We believe this report is the first to demonstrate true myositis in the setting of early eosinophilic granulomatosis with polyangiitis. This report describes a 74 year old Caucasian man, with no known allergies, presenting severe myalgia, muscle weakness, jaw claudication, and fever. Blood work showed marked eosinophilia and high creatine kinase levels. Biceps brachialis muscle biopsy revealed eosinophilic necrotizing vasculitis and true myositis with myophagocytosis of non-necrotic fibers and strong sarcolemmal MHC-1 overexpression by immunohistochemistry. This patient was successfully treated with prednisone and azathioprine. Our finding of true myositis in a case of eosinophilic granulomatosis with polyangiitis suggests that primary auto-immunity against muscle fibers, distinct from the secondary effects of vasculitis, can occur in this entity and may represent an overlap syndrome. Early recognition of eosinophilic granulomatosis with polyangiitis in patients presenting with myositis may provide an opportunity to treat the vasculitis before onset of severe multisystemic disease. We recommend the use of muscle biopsy with immunohistochemistry for MHC-1 to confirm the diagnosis of myositis in the setting of eosinophilic granulomatosis with polyangiitis.

  12. The Natural History of Flare-Ups in Fibrodysplasia Ossificans Progressiva (FOP): A Comprehensive Global Assessment.

    Science.gov (United States)

    Pignolo, Robert J; Bedford-Gay, Christopher; Liljesthröm, Moira; Durbin-Johnson, Blythe P; Shore, Eileen M; Rocke, David M; Kaplan, Frederick S

    2016-03-01

    Fibrodysplasia ossificans progressiva (FOP) leads to disabling heterotopic ossification (HO) from episodic flare-ups. However, the natural history of FOP flare-ups is poorly understood. A 78-question survey on FOP flare-ups, translated into 15 languages, was sent to 685 classically-affected patients in 45 countries (six continents). Five hundred patients or knowledgeable informants responded (73%; 44% males, 56% females; ages: 1 to 71 years; median: 23 years). The most common presenting symptoms of flare-ups were swelling (93%), pain (86%), or decreased mobility (79%). Seventy-one percent experienced a flare-up within the preceding 12 months (52% spontaneous; 48% trauma-related). Twenty-five percent of those who had received an intramuscular injection reported an immediate flare-up at the injection site, 84% of whom developed HO. Axial flare-ups most frequently involved the back (41.6%), neck (26.4%), or jaw (19.4%). Flare-ups occurred more frequently in the upper limbs before 8 years of age, but more frequently in the lower limbs thereafter. Appendicular flare-ups occurred more frequently at proximal than at distal sites without preferential sidedness. Seventy percent of patients reported functional loss from a flare-up. Thirty-two percent reported complete resolution of at least one flare-up and 12% without any functional loss (mostly in the head or back). The most disabling flare-ups occurred at the shoulders or hips. Surprisingly, 47% reported progression of FOP without obvious flare-ups. Worldwide, 198 treatments were reported; anti-inflammatory agents were most common. Seventy-five percent used short-term glucocorticoids as a treatment for flare-ups at appendicular sites. Fifty-five percent reported that glucocorticoids improved symptoms occasionally whereas 31% reported that they always did. Only 12% reported complete resolution of a flare-up with glucocorticoids. Forty-three percent reported rebound symptoms within 1 to 7 days after completing a course of

  13. Fibrodysplasia ossificans progressiva: case report Fibrodisplasia ossificante progressiva: relato de caso

    Directory of Open Access Journals (Sweden)

    Andre Leite Gonçalves

    2005-12-01

    Full Text Available Fibrodysplasia ossificans progressiva (FOP is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. We report on a nine-year-old girl with clinical and radiological features of FOP. She was born with bilateral hallux valgus and at the age of nine presented an indurate mass in the left cervical region that was painful. A significant decreased range of motion in all levels of the spine and shoulder girdle was found. The radiographs showed heterotopic ossification in the thoracic region. The patient had two outbreaks of the disease ("flare-ups" that were treated with prednisone 2 mg/kg/day for four days. After the "flare-ups", she had a continuous therapy with a Cox-2 inhibitor (25 mg/day and a leukotriene inhibitor, montelukast (10 mg/day.A fibrodisplasia ossificante progressiva (FOP é doença rara, autossômica dominante, caracterizada por ossificação heterotópica progressiva pós-natal do tecido conjuntivo e malformação congênita dos háluces. Relatamos o caso de menina de nove anos com o quadro clínico-radiológico típico de FOP, nascida com hálux valgo bilateral e que aos 9 anos de idade apresentou massa dolorosa, de consistência endurecida, sem sinais inflamatórios, situada na região cervical. Adicionalmente, era possível observar diminuição importante da movimentação em todos os níveis da coluna vertebral e da cintura escapular. A avaliação radiológica revelou a presença de ossificações heterotópicas na região torácica e malformação bilateral dos háluces. A paciente teve outros dois surtos da doença, que foram tratados com corticosteróide oral por quatro dias, (2 mg/kg/dia seguido por tratamento prolongado com inibidores da Cox-2 (25 mg/dia e com inibidor de leucotrienos (10 mg/dia.

  14. Group G streptococcal myositis in a patient with myeloproliferative neoplasm

    Directory of Open Access Journals (Sweden)

    Monica Midha, MD MBS

    2016-01-01

    Full Text Available While many cases of streptococcal infection are due to Lancefield groups A and B, there has been a rise in reported cases of infections due to group G streptococcus. We present a case of an individual with a hematologic malignancy who developed myositis secondary to group G streptococcus, with no clearly identifiable source of infection. The patient was managed with antibiotic therapy rather than surgical intervention due to high surgical risk related to severe thrombocytopenia. Targeted antibiotics initiated early in the course of disease may prevent the need for surgical intervention. Early diagnosis and treatment are critical to avoid the high morbidity and mortality of life-threatening infections caused by group G streptococcus.

  15. Sporadic inclusion body myositis: the genetic contributions to the pathogenesis

    Science.gov (United States)

    2014-01-01

    Sporadic inclusion body myositis (sIBM) is the commonest idiopathic inflammatory muscle disease in people over 50 years old. It is characterized by slowly progressive muscle weakness and atrophy, with typical pathological changes of inflammation, degeneration and mitochondrial abnormality in affected muscle fibres. The cause(s) of sIBM are still unknown, but are considered complex, with the contribution of multiple factors such as environmental triggers, ageing and genetic susceptibility. This review summarizes the current understanding of the genetic contributions to sIBM and provides some insights for future research in this mysterious disease with the advantage of the rapid development of advanced genetic technology. An international sIBM genetic study is ongoing and whole-exome sequencing will be applied in a large cohort of sIBM patients with the aim of unravelling important genetic risk factors for sIBM. PMID:24948216

  16. Functional impairment in patients with sporadic Inclusion Body Myositis.

    Science.gov (United States)

    Dunlap, Heather V; Macneil, Lauren G; Tarnopolsky, Mark A

    2014-03-01

    We conducted a retrospective chart review of 53 patients diagnosed with sporadic Inclusion Body Myositis (sIBM) who have been followed at the McMaster Neuromuscular Clinic since 1996. We reviewed patient medical histories in order to compare our findings with similar cohorts, and analyzed quantitative strength data to determine functionality in guiding decisions related to gait assistive devices. Patient information was acquired through retrospective clinic chart review. Our study found knee extension strength decreased significantly as patients transitioned to using more supportive gait assistive devices (P cane)(P Falls and fear of falling poses a significant threat to patient physical well-being. The prevalence of dysphagia increased as patients required more supportive gait devices, and finally a significant negative correlation was found between time after onset and creatine kinase (CK) levels (P falling would be beneficial in preventing future falls and improving long-term patient outcomes.

  17. Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

    Directory of Open Access Journals (Sweden)

    Stéphane Mathis

    2016-01-01

    Full Text Available Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies. For each patient, we carried out a pathological analysis (nerve and muscle and an electrophysiological study (and follow-up. To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders.

  18. Rivaroxaban-Induced Nontraumatic Spinal Subdural Hematoma: An Uncommon Yet Life-Threatening Complication

    Directory of Open Access Journals (Sweden)

    Mazen Zaarour

    2015-01-01

    Full Text Available In the last decade, the desire for safer oral anticoagulants (OACs led to the emergence of newer drugs. Available clinical trials demonstrated a lower risk of OACs-associated life-threatening bleeding events, including intracranial hemorrhage, compared to warfarin. Nontraumatic spinal hematoma is an uncommon yet life-threatening neurosurgical emergency that can be associated with the use of these agents. Rivaroxaban, one of the newly approved OACs, is a direct factor Xa inhibitor. To the best of our knowledge, to date, only two published cases report the incidence of rivaroxaban-induced nontraumatic spinal subdural hematoma (SSDH. Our case is the third one described and the first one to involve the cervicothoracic spine.

  19. Nontraumatic tibial polyethylene insert cone fracture in mobile-bearing posterior-stabilized total knee arthroplasty

    OpenAIRE

    Tanikake, Yohei; Hayashi, Koji; Ogawa, Munehiro; Inagaki, Yusuke; Kawate, Kenji; Tomita, Tetsuya; Tanaka, Yasuhito

    2016-01-01

    A 72-year-old male patient underwent mobile-bearing posterior-stabilized total knee arthroplasty for osteoarthritis. He experienced a nontraumatic polyethylene tibial insert cone fracture 27 months after surgery. Scanning electron microscopy of the fracture surface of the tibial insert cone suggested progress of ductile breaking from the posterior toward the anterior of the cone due to repeated longitudinal bending stress, leading to fatigue breaking at the anterior side of the cone, followed...

  20. Subchondral insufficiency fracture of the knee: a non-traumatic injury with prolonged recovery time

    OpenAIRE

    Gourlay, Margaret L; Renner, Jordan B; Spang, Jeffrey T; Rubin, Janet E

    2015-01-01

    Subchondral insufficiency fractures are non-traumatic fractures that occur immediately below the cartilage of a joint. Although low bone density may be present concurrently, it is not the underlying cause of subchondral insufficiency fractures in the majority of patients. Patients with subchondral insufficiency fracture characteristically have unremarkable plain radiographs, while MRI examination may reveal extensive bone marrow oedema and subchondral bone collapse. This article presents a 51...

  1. Non-traumatic limping in Paediatric Emergencies: Epidemiology, evaluation and results.

    Science.gov (United States)

    Lázaro Carreño, M I; Fraile Currius, R; García Clemente, A

    Non-traumatic limping is a common reason for consultation in paediatric emergencies. Although transient synovitis of the hip (TS) is the most frequent diagnosis, there are cases of limping secondary to serious pathologies. The aim of this review is to describe the variables related to non-traumatic limp that come to the paediatric emergency department to establish the best management protocol, making the most of resources and speeding up emergency care. A prospective study was conducted, selecting all children less than 15 years old who consulted aspaediatric emergencies for non-traumatic limping during the 2014. Clinical variables, complementary examinations and diagnoses were collected in the emergency room consultation and 6 months after the consultation RESULTS: During 2014, 146 patients (0.69% of the emergencies) were included in the non-traumatic limping study. Four cases of severe limping were diagnosed: 2 leukaemias, 1 septic arthritis and 1 acetabular bone tumour. The most frequent diagnosis was TS (53.16%). At 6 months, 135 children (92.4%) had resolved lameness. The diagnosis was changed in 9 children (6.1%). Children with TS had fewer days of evolution, and 77% were between 3 and 10 years old. Children with a final diagnosis of severe pathology had a limp for longer, fever and did not weight bear on ambulation. In limping of probable hip origin, at the ages of between 3 and 10, without fever or systemic symptoms and of less than one week's onset, it is possible to make a clinical diagnosis of TS limiting the use of complementary examinations. Copyright © 2017 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. US Emergency Department Trends in Imaging for Pediatric Nontraumatic Abdominal Pain.

    Science.gov (United States)

    Niles, Lauren M; Goyal, Monika K; Badolato, Gia M; Chamberlain, James M; Cohen, Joanna S

    2017-10-01

    To describe national emergency department (ED) trends in computed tomography (CT) and ultrasound imaging for the evaluation of pediatric nontraumatic abdominal pain from 2007 through 2014. We used data from the National Hospital Ambulatory Medical Care Survey to measure trends in CT and ultrasound use among children with nontraumatic abdominal pain. We performed multivariable logistic regression to measure the strength of the association of ED type (pediatric versus general ED) with CT and ultrasound use adjusting for potential confounding variables. Of an estimated 21.1 million ED visits for nontraumatic abdominal pain, 14.6% (95% confidence interval [CI], 13.2%-16.0%) had CT imaging only, 10.9% (95% CI, 9.7%-12.1%) had ultrasound imaging only, and 1.9% (95% CI, 1.4%-2.4%) received both CT and ultrasound. The overall use of CT and ultrasound did not significantly change over the study period ( P trend .63 and .90, respectively). CT use was lower among children treated in pediatric EDs compared with general EDs (adjusted odds ratio 0.34; 95% CI, 0.17-0.69). Conversely, ultrasound use was higher among children treated in pediatric EDs compared with general EDs (adjusted odds ratio 2.14; 95% CI, 1.29-3.55). CT imaging for pediatric patients with nontraumatic abdominal pain has plateaued since 2007 after the steady increase seen in the preceding 9 years. Among this population, an increased likelihood of CT imaging was demonstrated in general EDs compared with pediatric EDs, in which there was a higher likelihood of ultrasound imaging. Dissemination of pediatric-focused radiology protocols to general EDs may help optimize radiation exposure in children. Copyright © 2017 by the American Academy of Pediatrics.

  3. Identification of a novel myositis-associated antibody directed against cortactin.

    Science.gov (United States)

    Labrador-Horrillo, Moisés; Martínez, Maria Angeles; Selva-O'Callaghan, Albert; Trallero-Araguás, Ernesto; Grau-Junyent, Josep M; Vilardell-Tarrés, Miquel; Juarez, Candido

    2014-10-01

    The aim of this study is to describe a novel myositis-associated autoantibody (anti-cortactin antibody) and assess related clinical and immunological manifestations and its clinical significance. Adult patients with myositis (dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis), as well as patients with other autoimmune diseases and non-inflammatory myopathies were analyzed for the presence of anti-cortactin antibody using in-house developed ELISA and immunoblotting techniques with a commercial source of purified cortactin. The cut-off for positive status was determined in a group of healthy volunteers. Antibody against cortactin was positive in 7/34 (20%) polymyositis patients, 9/117 (7.6%) dermatomyositis, 2/7 (26%) immune-mediated necrotizing myopathy, and none of the 4 patients with inclusion body myositis. The antibody also tested positive in 3/101 patients with other autoimmune diseases (2 systemic sclerosis and 1 systemic lupus erythematosus), and in 1/29 patients with non-inflammatory myopathy. No relevant association with specific clinical features was found in patients with these antibodies. Anti-cortactin antibody was more frequently positive in patients with polymyositis and immune-mediated necrotizing myopathy than in the remaining myositis patients, and was the only myositis autoantibody found in sera of 3 patients from these groups. Our data indicate that cortactin is a novel target antigen in patients with autoimmune diseases, especially patients with polymyositis or immune-mediated necrotizing myopathy. Anti-cortactin can be considered a new myositis-associated antibody. Copyright © 2014 Elsevier B.V. All rights reserved.

  4. An Autoimmune Myositis-Overlap Syndrome Associated With Autoantibodies to Nuclear Pore Complexes

    Science.gov (United States)

    Senécal, Jean-Luc; Isabelle, Catherine; Fritzler, Marvin J.; Targoff, Ira N.; Goldstein, Rose; Gagné, Michel; Raynauld, Jean-Pierre; Joyal, France; Troyanov, Yves; Dabauvalle, Marie-Christine

    2014-01-01

    Abstract Autoimmune myositis encompasses various myositis-overlap syndromes, each being identified by the presence of serum marker autoantibodies. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. There was no clinical or laboratory evidence for liver disease. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr). By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes. Reactivity with nuclear pore complexes was confirmed by immunoelectron microscopy. In a cohort of 100 French Canadian patients with autoimmune myositis, the nuclear pore complex fluorescent ANA pattern was restricted to these 4 patients (4%). It was not observed in sera from 393 adult patients with systemic sclerosis (n = 112), mixed connective tissue disease (n = 35), systemic lupus (n = 94), rheumatoid arthritis (n = 45), or other rheumatic diseases (n = 107), nor was it observed in 62 normal adults. Autoantibodies to nuclear pore complexes were predominantly of IgG isotype. No other IgG autoantibody markers for defined connective tissue diseases or overlap syndromes were present, indicating a selective and highly focused immune response. In 3 patients, anti-nuclear pore complex autoantibody titers varied in parallel with myositis activity, suggesting a pathogenic

  5. Rare myositis-specific autoantibody associations among Hungarian patients with idiopathic inflammatory myopathy.

    Science.gov (United States)

    Bodoki, L; Nagy-Vincze, M; Griger, Z; Betteridge, Z; Szöllősi, L; Jobanputra, R; Dankó, K

    2015-01-01

    Idiopathic inflammatory myopathies are systemic, chronic autoimmune diseases characterized by symmetrical, proximal muscle weakness. Homogeneous groups present with similar symptoms. The response to therapy and prognosis could be facilitated by myositis-specific autoantibodies, and in this way, give rise to immunoserological classification. The myositis-specific autoantibodies are directed against specific proteins found in the cytoplasm or in the nucleus of the cells. To date, literature suggests the rarity of the co-existence of two myositis-specific autoantibodies. In this study the authors highlight rare associations of myositis-specific autoantibodies. Three hundred and thirty-seven Hungarian patients with polymyositis or dermatomyositis were studied. Their clinical findings were noted retrospectively. Specific blood tests identified six patients with the rare co-existence of myositis-specific autoantibodies, anti-Jo-1 and anti-SRP, anti-Jo-1 and anti-Mi-2, anti-Mi-2 and anti-PL-12, anti-Mi-2 and anti-SRP, and anti-SRP and anti-PL-7, respectively. This case review aims to identify the clinical importance of these rare associations and their place within the immunoserological classification.

  6. Eosinophilic myositis as first manifestation in a patient with type 2 myotonic dystrophy CCTG expansion mutation and rheumatoid arthritis.

    Science.gov (United States)

    Meyer, Alain; Lannes, Béatrice; Carapito, Raphaël; Bahram, Seiamak; Echaniz-Laguna, Andoni; Geny, Bernard; Sibilia, Jean; Gottenberg, Jacques Eric

    2015-02-01

    Eosinophilic myositis is characterized by eosinophilic infiltration of skeletal muscles. In the absence of an identifiable causative factor or source (including parasitic infection, intake of drugs or L-tryptophan, certain systemic disorders as well as malignant diseases), the diagnosis of idiopathic eosinophilic myositis is usually retained. However, some muscular dystrophies have been recently identified in this subset of eosinophilic myositis. Here, we report a patient with an 8 kb CCTG expansion in intron 1 of the CNBP gene, a mutation characteristic of myotonic dystrophy type 2 (DM2), whose first manifestation was "idiopathic" eosinophilic myositis. This report suggests that in "idiopathic" eosinophilic myositis, clinicians should consider muscular dystrophies, including DM2. Copyright © 2014 Elsevier B.V. All rights reserved.

  7. Beneficial role of rapamycin in experimental autoimmune myositis.

    Directory of Open Access Journals (Sweden)

    Nicolas Prevel

    Full Text Available We developed an experimental autoimmune myositis (EAM mouse model of polymyositis where we outlined the role of regulatory T (Treg cells. Rapamycin, this immunosuppressant drug used to prevent rejection in organ transplantation, is known to spare Treg. Our aim was to test the efficacy of rapamycin in vivo in this EAM model and to investigate the effects of the drug on different immune cell sub-populations.EAM is induced by 3 injections of myosin emulsified in CFA. Mice received rapamycin during 25 days starting one day before myosin immunization (preventive treatment, or during 10 days following the last myosin immunization (curative treatment.Under preventive or curative treatment, an increase of muscle strength was observed with a parallel decrease of muscle inflammation, both being well correlated (R(2 = -0.645, p<0.0001. Rapamycin induced a general decrease in muscle of CD4 and CD8 T cells in lymphoid tissues, but spared B cells. Among T cells, the frequency of Treg was increased in rapamycin treated mice in draining lymph nodes (16.9 ± 2.2% vs. 9.3 ± 1.4%, p<0.001, which were mostly activated regulatory T cells (CD62L(lowCD44(high: 58.1 ± 5.78% vs. 33.1 ± 7%, treated vs. untreated, p<0.001. In rapamycin treated mice, inhibition of proliferation (Ki-67(+ is more important in effector T cells compared to Tregs cells (p<0.05. Furthermore, during preventive treatment, rapamycin increased the levels of KLF2 transcript in CD44(low CD62L(high naive T cell and in CD62L(low CD44(high activated T cell.Rapamycin showed efficacy both as curative and preventive treatment in our murine model of experimental myositis, in which it induced an increase of muscle strength with a parallel decrease in muscle inflammation. Rapamycin administration was also associated with a decrease in the frequency of effector T cells, an increase in Tregs, and, when administered as preventive treatment, an upregulation of KFL2 in naive and activated T cells.

  8. Association of inclusion body myositis with T cell large granular lymphocytic leukaemia

    DEFF Research Database (Denmark)

    Greenberg, Steven A; Pinkus, Jack L; Amato, Anthony A

    2016-01-01

    SEE HOHLFELD AND SCHULZE-KOOPS DOI101093/BRAIN/AWW053 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Inclusion body myositis and T cell large granular lymphocytic leukaemia are rare diseases involving pathogenic cytotoxic CD8+ T cells. After encountering four patients with both disorders, we...... prospectively screened 38 patients with inclusion body myositis for the presence of expanded large granular lymphocyte populations by standard clinical laboratory methods (flow cytometry, examination of blood smears, and T cell receptor gene rearrangements), and performed muscle immunohistochemistry for CD8, CD......57, and TIA1. Most (22/38; 58%) patients with inclusion body myositis had aberrant populations of large granular lymphocytes in their blood meeting standard diagnostic criteria for T cell large granular lymphocytic leukaemia. These T cell populations were clonal in 20/20 patients and stably present...

  9. Subacute sarcoid myositis with ocular muscle involvement; a case report and review of the literature.

    Science.gov (United States)

    Hayashi, Y; Ishii, Yoshiki; Nagasawa, J; Arai, S; Okada, H; Ohmi, F; Umetsu, T; Machida, Y; Kurasawa, K; Takemasa, A; Suzuki, S; Senoh, T; Sada, T; Hirata, K

    2016-10-07

    Sarcoidosis is a chronic granulomatous disease that can affect multiple organs. The lungs, eyes, and skin are known to be highly affected organs in sarcoidosis. There have been reports based on random muscle biopsy that 32-80% of systemic sarcoidosis comprises noncaseating granulomas; however, muscle involvement in sarcoidosis is generally asymptomatic and has an unknown frequency. We describe a case of acute to subacute sarcoid myositis of the skeletal and extraocular muscles. Typical ophthalmic involvement (manifested by infiltration of the ocular adnexa, intraocular inflammation, or infiltration of the retrobulbar visual pathways) and extraocular sarcoid myositis (as with the present case) is infrequently reported. It is important to keep in mind the rare yet perhaps underestimated entity of sarcoid myositis, and to utilize muscle biopsy and imaging tests for appropriate diagnosis and management of patients with sarcoidosis.

  10. Parvovirus B19 infection as a cause of acute myositis in an adult.

    Science.gov (United States)

    Cakirca, Mustafa; Karatoprak, Cumali; Ugurlu, Serdal; Zorlu, Mehmet; Kıskaç, Muharrem; Çetin, Güven

    2015-01-01

    Parvovirus B19 infection is often asymptomatic, but clinical expressions may include transient aplastic crisis, erythema infectiosum, non-immune hydrops fetalis, and chronic red cell aplasia. This virus has also been associated with rheumatoid arthritis and other autoimmune connective tissue diseases; however, we could not identify any acute adult myositis case developed after a Parvovirus B19 infection in the literature. For this reason, we would like to present a rare case of acute myositis developed after Parvovirus B19 infection. In patients presenting with symptoms of fever, rash on the legs and myositis, viral infections such as Parvovirus B19 should be kept in mind. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  11. Sjögren's syndrome-associated myositis with germinal centre-like structures.

    Science.gov (United States)

    Espitia-Thibault, Alexandra; Masseau, Agathe; Néel, Antoine; Espitia, Olivier; Toquet, Claire; Mussini, Jean-Marie; Hamidou, Mohamed

    2017-02-01

    Muscular impairment is a rare systemic manifestation of SS that is rarely described in the literature and classically non-specific, both clinically and histologically. We reviewed the cases of 4 patients with primary SS presenting with myositis and a common histologic pattern on muscular biopsy with germinal centre-like structures resembling that which occurs in salivary glands. We analysed the data files of patients with SS who had muscular manifestations and underwent a muscular biopsy. Among 23 patients with SS who had muscle biopsies, 13 had non-specific myositis and 10 (4 primary and 6 secondary SS) had a common histologic pattern consisting of germinal centre-like structures. We analysed the data files of the 4 patients with primary SS presenting with myositis with muscular germinal-centre like structures. The 4 patients had an unspecific clinical presentation, with myalgias, muscular weakness and normal or elevated values of CPK. In the four patients, SS-associated myositis had common histologic characteristics, with endomysial and perimysial inflammatory infiltrate. The cellular infiltrate was composed predominantly of CD4+ T lymphocytes and B lymphocytes. The B and T CD4+ cells infiltrates may gather into masses, even forming lymphoid follicles. Three patients were treated with corticosteroids and/or hydroxychloroquine with improvement of myositis and 1 patient was lost to follow-up. We describe four patients with a common histologic appearance of myositis with lymphoid follicles associated with primary SS. The clinical presentation was non-specific and non-severe, with favorable outcome with corticosteroids and/or hydroxycholoroquine. The discovery of this particular histologic appearance in a muscle biopsy independent of the final diagnosis should indicate the possibility of SS. Copyright © 2016 Elsevier B.V. All rights reserved.

  12. Cytosolic 5′-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis

    Science.gov (United States)

    Rietveld, A; Pye, S R; Mariampillai, K; Benveniste, O; Peeters, M T J; Miller, J A L; Hanna, M G; Machado, P M; Parton, M J; Gheorghe, K R; Badrising, U A; Lundberg, I E; Sacconi, S; Herbert, M K; McHugh, N J; Lecky, B R F; Brierley, C; Hilton-Jones, D; Lamb, J A; Roberts, M E; Cooper, R G; Saris, C G J; Pruijn, G J M; Chinoy, H; van Engelen, B G M

    2017-01-01

    Objectives Autoantibodies directed against cytosolic 5′-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5′-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis. Materials and methods Data from various European inclusion body myositis registries were pooled. Anticytosolic 5′-nucleotidase 1A status was determined by an established ELISA technique. Cases were stratified according to antibody status and comparisons made. Survival and mobility aid requirement analyses were performed using Kaplan-Meier curves and Cox proportional hazards regression. Results Data from 311 patients were available for analysis; 102 (33%) had anticytosolic 5′-nucleotidase 1A antibodies. Antibody-positive patients had a higher adjusted mortality risk (HR 1.89, 95% CI 1.11 to 3.21, p=0.019), lower frequency of proximal upper limb weakness at disease onset (8% vs 23%, adjusted OR 0.29, 95% CI 0.12 to 0.68, p=0.005) and an increased prevalence of excess of cytochrome oxidase deficient fibres on muscle biopsy analysis (87% vs 72%, adjusted OR 2.80, 95% CI 1.17 to 6.66, p=0.020), compared with antibody-negative patients. Interpretation Differences were observed in clinical and histopathological features between anticytosolic 5′-nucleotidase 1A antibody positive and negative patients with inclusion body myositis, and antibody-positive patients had a higher adjusted mortality risk. Stratification of inclusion body myositis by anticytosolic 5′-nucleotidase 1A antibody status may be useful, potentially highlighting a distinct inclusion body myositis subtype with a more severe phenotype. PMID:28122761

  13. Cytosolic 5'-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis.

    Science.gov (United States)

    Lilleker, J B; Rietveld, A; Pye, S R; Mariampillai, K; Benveniste, O; Peeters, M T J; Miller, J A L; Hanna, M G; Machado, P M; Parton, M J; Gheorghe, K R; Badrising, U A; Lundberg, I E; Sacconi, S; Herbert, M K; McHugh, N J; Lecky, B R F; Brierley, C; Hilton-Jones, D; Lamb, J A; Roberts, M E; Cooper, R G; Saris, C G J; Pruijn, G J M; Chinoy, H; van Engelen, B G M

    2017-05-01

    Autoantibodies directed against cytosolic 5'-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5'-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis. Data from various European inclusion body myositis registries were pooled. Anticytosolic 5'-nucleotidase 1A status was determined by an established ELISA technique. Cases were stratified according to antibody status and comparisons made. Survival and mobility aid requirement analyses were performed using Kaplan-Meier curves and Cox proportional hazards regression. Data from 311 patients were available for analysis; 102 (33%) had anticytosolic 5'-nucleotidase 1A antibodies. Antibody-positive patients had a higher adjusted mortality risk (HR 1.89, 95% CI 1.11 to 3.21, p=0.019), lower frequency of proximal upper limb weakness at disease onset (8% vs 23%, adjusted OR 0.29, 95% CI 0.12 to 0.68, p=0.005) and an increased prevalence of excess of cytochrome oxidase deficient fibres on muscle biopsy analysis (87% vs 72%, adjusted OR 2.80, 95% CI 1.17 to 6.66, p=0.020), compared with antibody-negative patients. Differences were observed in clinical and histopathological features between anticytosolic 5'-nucleotidase 1A antibody positive and negative patients with inclusion body myositis, and antibody-positive patients had a higher adjusted mortality risk. Stratification of inclusion body myositis by anticytosolic 5'-nucleotidase 1A antibody status may be useful, potentially highlighting a distinct inclusion body myositis subtype with a more severe phenotype. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  14. International consensus on preliminary definitions of improvement in adult and juvenile myositis.

    Science.gov (United States)

    Rider, Lisa G; Giannini, Edward H; Brunner, Hermine I; Ruperto, Nicola; James-Newton, Laura; Reed, Ann M; Lachenbruch, Peter A; Miller, Frederick W

    2004-07-01

    To use a core set of outcome measures to develop preliminary definitions of improvement for adult and juvenile myositis as composite end points for therapeutic trials. Twenty-nine experts in the assessment of myositis achieved consensus on 102 adult and 102 juvenile paper patient profiles as clinically improved or not improved. Two hundred twenty-seven candidate definitions of improvement were developed using the experts' consensus ratings as a gold standard and their judgment of clinically meaningful change in the core set of measures. Seventeen additional candidate definitions of improvement were developed from classification and regression tree analysis, a data-mining decision tree tool analysis. Six candidate definitions specifying percentage change or raw change in the core set of measures were developed using logistic regression analysis. Adult and pediatric working groups ranked the 13 top-performing candidate definitions for face validity, clinical sensibility, and ease of use, in which the sensitivity and specificity were >/=75% in adult, pediatric, and combined data sets. Nominal group technique was used to facilitate consensus formation. The definition of improvement (common to the adult and pediatric working groups) that ranked highest was 3 of any 6 of the core set measures improved by >/=20%, with no more than 2 worse by >/=25% (which could not include manual muscle testing to assess strength). Five and 4 additional preliminary definitions of improvement for adult and juvenile myositis, respectively, were also developed, with several definitions common to both groups. Participants also agreed to prospectively test 6 logistic regression definitions of improvement in clinical trials. Consensus preliminary definitions of improvement were developed for adult and juvenile myositis, and these incorporate clinically meaningful change in all myositis core set measures in a composite end point. These definitions require prospective validation, but they are now

  15. Botulinum toxin alleviates dysphagia of patients with inclusion body myositis.

    Science.gov (United States)

    Schrey, Aleksi; Airas, Laura; Jokela, Manu; Pulkkinen, Jaakko

    2017-09-15

    Oropharyngeal dysphagia is a disabling and undertreated symptom that often occurs in patients with sporadic inclusion body myositis (s-IBM). In this study, we examined the effect of botulinum neurotoxin A (BoNT-A) injections to the cricopharyngeus muscle (CPM) of patients with s-IBM and dysphagia. A single-center retrospective study involving 40 biopsy-proven s-IBM-patients treated in the District of Southwest Finland from 2000 to 2013. The incidence of dysphagia, rate of aspirations, rate of aspiration pneumonias and treatment results of dysphagia were analyzed. Patients treated for dysphagia were evaluated before and after surgery by video-fluoroscopy and/or using a questionnaire. Twenty-five of the 40 s-IBM patients (62.5%) experienced dysphagia. BoNT-A was injected a median of 2 times (range 1-7) in 12 patients with dysphagia. Before the injections 7 patients reported aspiration, none afterwards. The corresponding figures for aspiration pneumonia were 3 and 0. All of these patients had normal swallowing function 12months (median, range 2-60) after the last injection. BoNT-A injections to the CPM alleviate the dysphagia of s-IBM patients reversibly and appear to reduce the rate of aspiration effectively. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Evaluation and construction of diagnostic criteria for inclusion body myositis

    Science.gov (United States)

    Mammen, Andrew L.; Amato, Anthony A.; Weiss, Michael D.; Needham, Merrilee

    2014-01-01

    Objective: To use patient data to evaluate and construct diagnostic criteria for inclusion body myositis (IBM), a progressive disease of skeletal muscle. Methods: The literature was reviewed to identify all previously proposed IBM diagnostic criteria. These criteria were applied through medical records review to 200 patients diagnosed as having IBM and 171 patients diagnosed as having a muscle disease other than IBM by neuromuscular specialists at 2 institutions, and to a validating set of 66 additional patients with IBM from 2 other institutions. Machine learning techniques were used for unbiased construction of diagnostic criteria. Results: Twenty-four previously proposed IBM diagnostic categories were identified. Twelve categories all performed with high (≥97%) specificity but varied substantially in their sensitivities (11%–84%). The best performing category was European Neuromuscular Centre 2013 probable (sensitivity of 84%). Specialized pathologic features and newly introduced strength criteria (comparative knee extension/hip flexion strength) performed poorly. Unbiased data-directed analysis of 20 features in 371 patients resulted in construction of higher-performing data-derived diagnostic criteria (90% sensitivity and 96% specificity). Conclusions: Published expert consensus–derived IBM diagnostic categories have uniformly high specificity but wide-ranging sensitivities. High-performing IBM diagnostic category criteria can be developed directly from principled unbiased analysis of patient data. Classification of evidence: This study provides Class II evidence that published expert consensus–derived IBM diagnostic categories accurately distinguish IBM from other muscle disease with high specificity but wide-ranging sensitivities. PMID:24975859

  17. Proliferating Myositis: An Inflammatory Lesion often Misdiagnosed as A Malignant Tumor.

    Science.gov (United States)

    Binesh, Fariba; Sobhanardekani, Mohammad; Zabihi, Somayeh; Behniafard, Nasim

    2016-12-01

    Proliferative myositis (PM) is a rare inflammatory disease. Most commonly, the lesion occurs in the extremities. Regarding its fast growth and bizarre shape of the cellular components this entity commonly misdiagnosed and the patients undergo improper therapeutic approaches. In other words, it is often misdiagnosed as sarcoma. The diagnosis can only be made by the microscopic examination, so biopsy is mandatory. Here the authors report a patient with PM who was initially misdiagnosed as pleomorphic sarcoma of the lower extremity and explain this rare entity. Proliferative myositis should be taken into account if a fast growing, intramuscular mass occurs in the extremities.

  18. Blood-flow restricted resistance training in patients with sporadic inclusion body myositis

    DEFF Research Database (Denmark)

    Jørgensen, A.; Aagaard, P.; Frandsen, U.

    2018-01-01

    Objectives: To investigate the effect of 12 weeks of low-load blood-flow restricted resistance (BFR) training on self-reported and objective physical function, and maximal muscle strength in patients with sporadic inclusion body myositis (sIBM). Method: Twenty-two patients with sIBM were randomized......), which was used to measure self-reported physical function. All patients performed physical function tests (2-Minute Walk Test, Timed Up and Go, and 30-Second Chair Stand), completed the Inclusion Body Myositis Functional Rating Scale (IBMFRS), and were tested for isolated knee extensor muscle strength...

  19. Fatal Tuberculous Myositis in an Immunocompromised Adult With Primary Sjögren's Syndrome

    Directory of Open Access Journals (Sweden)

    Chi-Chang Huang

    2010-09-01

    Full Text Available Tuberculous myositis, which mimics rheumatic symptoms, is an extremely rare disease. Clinical ambiguity easily leads to misdiagnosis and delayed initial treatment. We present the case of a 55-year-old man who had primary Sjögren's syndrome and active cutaneous vasculitis treated with steroid and immunosuppressive drugs. He presented with a swollen, painful, hot left thigh. Although anti-tuberculosis medications were administered soon after a positive acid-fast stain of incisional muscular tissue, he died of rapidly progressive tuberculous myositis and multiorgan failure following 18 days of hospitalization. This case is presented to increase the awareness of this rare entity in clinical practice.

  20. Condylar Joint Fusion and Stabilization (by Screws and Plates) in Nontraumatic Atlanto-Occipital Dislocation: Technical Report of 2 Cases.

    Science.gov (United States)

    Chowdhury, Forhad H; Haque, Mohammod Raziul; Alam, Sarwar Murshed; Khaled Chowdhury, S M Noman; Khan, Shamsul Islam; Goel, Atul

    2017-11-01

    Nontraumatic spontaneous atlanto-occipital dislocation (AOD) is rare. In this report, we discuss the technical steps of condylar joint fusion and stabilization (by screws and plates) in nontraumatic AOD. To the best of our knowledge, it is the first report of such techniques. A young girl and a young man with progressive quadriparesis due to nontraumatic spontaneous atlanto-occipital dislocation were managed by microsurgical reduction, fusion, and stabilization of the joint by occipital condylar and C1 lateral mass screw and plate fixation after mobilization of vertebral artery. In both cases, condylar joints fixation and fusion were done successfully. Condylar joint stabilization and fusion may be a good or alternative option for AOD. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Chronic nontraumatic spinal epidural hematoma of the lumbar spine: MRI diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Vazquez-Barquero, A.; Pinto, J.I. [Univ. Hospital ' ' Marques de Valdecilla' ' , Santander (Spain). Dept. of Neurosurgery; Abascal, F.; Garcia-Valtuille, R.; Cerezal, L. [Hospital Mompia, Cantabria, (Spain). Dept. of Radiology; Figols, F.J. [Univ. Hospital Marques de Valdecilla, Santander (Spain). Dept. of Pathology

    2000-10-01

    An uncommon case of chronic nontraumatic spinal epidural hematoma of the lumbar spine in a 75-year-old woman is reported. The patient presented with a 7-month history of low back pain and bilateral sciatica. Magnetic resonance imaging enabled a correct preoperative diagnosis revealing a nodular, well-circumscribed epidural mass with peripheral enhancement and signal intensities consistent with chronic hematoma, which extended from L2 to L3. Laminectomy of L2-L3 was performed and the hematoma was totally resected. Histological examination of the surgical specimen demonstrated a chronic encapsulated hematoma. No evidence of vascular malformation was found. The patient recovered fully after surgical treatment. (orig.)

  2. Chronic nontraumatic spinal epidural hematoma of the lumbar spine: MRI diagnosis

    International Nuclear Information System (INIS)

    Vazquez-Barquero, A.; Pinto, J.I.; Abascal, F.; Garcia-Valtuille, R.; Cerezal, L.; Figols, F.J.

    2000-01-01

    An uncommon case of chronic nontraumatic spinal epidural hematoma of the lumbar spine in a 75-year-old woman is reported. The patient presented with a 7-month history of low back pain and bilateral sciatica. Magnetic resonance imaging enabled a correct preoperative diagnosis revealing a nodular, well-circumscribed epidural mass with peripheral enhancement and signal intensities consistent with chronic hematoma, which extended from L2 to L3. Laminectomy of L2-L3 was performed and the hematoma was totally resected. Histological examination of the surgical specimen demonstrated a chronic encapsulated hematoma. No evidence of vascular malformation was found. The patient recovered fully after surgical treatment. (orig.)

  3. Technetium-99m pyrophosphate imaging in acute renal failure associated with nontraumatic rhabdomyolysis

    International Nuclear Information System (INIS)

    Patel, R.; Mishkin, F.S.

    1986-01-01

    Technetium-99m pyrophosphate (Tc-PYP) imaging was performed in five patients with acute renal failure associated with nontraumatic rhabdomyolysis. Four patients had phencyclidine intoxication and one had viral pneumonia. During the acute phase, marked uptake of pyrophosphate was seen in all patients in several muscle groups, but always in the thigh adductors. The results show that phencyclidine intoxication can result in diffuse muscle uptake of Tc-PYP without overt evidence of muscle injury. Tc-PYP imaging may provide a clue to the cause of acute renal failure in patients with suspected rhabdomyolysis in whom elevations of serum creatine phosphokinase concentrations are equivocal

  4. Acute nontraumatic hemoabdomen in the dog: a retrospective analysis of 39 cases (1987-2001).

    Science.gov (United States)

    Pintar, Jason; Breitschwerdt, Edward B; Hardie, Elizabeth M; Spaulding, Kathy A

    2003-01-01

    The medical records of 39 dogs with acute nontraumatic hemoabdomen were identified and reviewed. Anemia and hypoalbuminemia were identified in 36/37 (97%) and 25/33 (76%) dogs, respectively. Coagulopathies were identified in 26/31 (84%) dogs. When a definitive diagnosis was obtained, malignant neoplasia was diagnosed most frequently and occurred in 24/30 (80%) dogs. Hemangiosarcoma accounted for 21/30 (70%) diagnoses. Sixteen dogs underwent exploratory laparotomy, of which seven (44%) survived the perioperative period. Of the dogs that did not undergo surgery, 9/23 (39%) survived to be discharged from the hospital.

  5. Technetium-99m pyrophosphate imaging in acute renal failure associated with nontraumatic rhabdomyolysis

    Energy Technology Data Exchange (ETDEWEB)

    Patel, R.; Mishkin, F.S.

    1986-10-01

    Technetium-99m pyrophosphate (Tc-PYP) imaging was performed in five patients with acute renal failure associated with nontraumatic rhabdomyolysis. Four patients had phencyclidine intoxication and one had viral pneumonia. During the acute phase, marked uptake of pyrophosphate was seen in all patients in several muscle groups, but always in the thigh adductors. The results show that phencyclidine intoxication can result in diffuse muscle uptake of Tc-PYP without overt evidence of muscle injury. Tc-PYP imaging may provide a clue to the cause of acute renal failure in patients with suspected rhabdomyolysis in whom elevations of serum creatine phosphokinase concentrations are equivocal.

  6. Acute myositis associated with concurrent infection of rotavirus and norovirus in a 2-year-old girl

    Directory of Open Access Journals (Sweden)

    Kei Yamamoto

    2015-09-01

    Full Text Available Rotavirus and norovirus are common pathogens associated with gastroenteritis in children. Although rotavirus occasionally induces central nervous system disease, only 3 cases with rotavirus-induced acute myositis have been reported in the English literature. We recently treated a female patient with acute myositis associated with gastroenteritis induced by concurrent infection with rotavirus and norovirus. Having suffered from gastroenteritis for 3 days, she suddenly developed myositis affecting her lower extremities with concomitant creatine kinase elevation. Herein, we present our patient and review the previous cases including those reported in the Japanese literature.

  7. Association of Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients.

    Science.gov (United States)

    Kishi, Takayuki; Rider, Lisa G; Pak, Katherine; Barillas-Arias, Lilliana; Henrickson, Michael; McCarthy, Paul L; Shaham, Bracha; Weiss, Pamela F; Horkayne-Szakaly, Iren; Targoff, Ira N; Miller, Frederick W; Mammen, Andrew L

    2017-07-01

    Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of juvenile anti-HMGCR-positive myositis patients. The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). Five of 440 patients (1.1%) were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and 2 patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/liter. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications, and their disease often took a chronic course. The DRB1*07:01 allele was present in all 5 patients, compared to 26.25% of healthy controls (corrected P = 0.01); none of the 5 juvenile patients had DRB1*11:01. Compared to children with other MSAs, muscle disease appears to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, in anti-HMGCR-positive children, there is a strong association with HLA-DRB1*07:01. © 2017, American College of Rheumatology.

  8. ACVR1, a Therapeutic Target of Fibrodysplasia Ossificans Progressiva, Is Negatively Regulated by miR-148a

    Directory of Open Access Journals (Sweden)

    Jun Cheng

    2012-02-01

    Full Text Available Fibrodysplasia ossificans progressiva (FOP is a rare congenital disorder of skeletal malformations and progressive extraskeletal ossification. There is still no effective treatment for FOP. All FOP individuals harbor conserved point mutations in ACVR1 gene that are thought to cause ACVR1 constitutive activation and activate BMP signal pathway. The constitutively active ACVR1 is also found to be able to cause endothelial-to-mesenchymal transition (EndMT in endothelial cells, which may cause the formation of FOP lesions. MicroRNAs (miRNAs play an essential role in regulating cell differentiation. Here, we verified that miR-148a directly targeted the 3' UTR of ACVR1 mRNA by reporter gene assays and mutational analysis at the miRNA binding sites, and inhibited ACVR1 both at the protein level and mRNA level. Further, we verified that miR-148a could inhibit the mRNA expression of the Inhibitor of DNA binding (Id gene family thereby suppressing the BMP signaling pathway. This study suggests miR-148a is an important mediator of ACVR1, thus offering a new potential target for the development of therapeutic agents against FOP.

  9. An Activin Receptor IA/Activin-Like Kinase-2 (R206H Mutation in Fibrodysplasia Ossificans Progressiva

    Directory of Open Access Journals (Sweden)

    Rafael Herrera-Esparza

    2013-01-01

    Full Text Available Fibrodysplasia ossificans progressiva (FOP is an exceptionally rare genetic disease that is characterised by congenital malformations of the great toes and progressive heterotopic ossification (HO in specific anatomical areas. This disease is caused by a mutation in activin receptor IA/activin-like kinase-2 (ACVR1/ALK2. A Mexican family with one member affected by FOP was studied. The patient is a 19-year-old female who first presented with symptoms of FOP at 8 years old; she developed spontaneous and painful swelling of the right scapular area accompanied by functional limitation of movement. Mutation analysis was performed in which genomic DNA as PCR amplified using primers flanking exons 4 and 6, and PCR products were digested with Cac8I and HphI restriction enzymes. The most informative results were obtained with the exon 4 flanking primers and the Cac8I restriction enzyme, which generated a 253 bp product that carries the ACVR1 617G>A mutation, which causes an amino acid substitution of histidine for arginine at position 206 of the glycine-serine (GS domain, and its mutation results in the dysregulation of bone morphogenetic protein (BMP signalling that causes FOP.

  10. Non-traumatic causes and the management of the open abdomen.

    Science.gov (United States)

    Mentula, P

    2011-04-01

    The open abdomen is increasingly used for the treatment and prevention of abdominal compartment syndrome. The leading non-traumatic conditions that may cause abdominal compartment syndrome requiring surgical decompression include secondary peritonitis, ruptured abdominal aortic aneurysm and severe acute pancreatitis. Patients may also end up with the open abdomen when the laparotomy wound cannot be closed without tension because of excessive visceral swelling. Also, surgical complications such as laparotomy wound dehiscence, may require temporary abdominal closure techniques. In critically ill surgical patients and in situations when second-look laparotomy is mandatory the open abdomen can be utilized in a preventive manner like in damage control trauma surgery. Underlying disease and the indication for the open abdomen significantly contributes to outcome of patient with open abdomen. Non-traumatic aetiology of the open abdomen is associated with lower likelihood of primary fascial closure and higher rate of open abdomen related complications compared with traumatic aetiology. A number of temporal abdominal closure techniques have been described. Ideally, temporal abdominal closure technique should prevent the development of recurrent abdominal compartment syndrome and facilitate later primary fascia closure with low complication rate. Although fascial closure rate varies between techniques, there are few evidence-based data to support one technique over another. However, recent evolution of temporary abdominal closure techniques have decreased the number of patients with frozen abdomen and reduced the need for planned hernia management. Highest fascial closure rates have been achieved with vacuum-assisted closure systems and systems that provide continuous fascial traction.

  11. Management of nontraumatic corneal perforation with tectonic drape patch and cyanoacrylate glue.

    Science.gov (United States)

    Khalifa, Yousuf M; Bailony, M Rami; Bloomer, Michele M; Killingsworth, Daniel; Jeng, Bennie H

    2010-10-01

    To report a case of nontraumatic corneal perforation managed with a tectonic drape patch. Interventional case report. A 60-year-old patient with a corneal scar in his left eye likely secondary to herpes simplex virus interstitial keratitis underwent laser peripheral iridotomy for narrow angles. He developed progressive thinning of the cornea overlying the scar that led to a descemetocele and then ultimately a 1.2- × 1.7-mm perforation. Intraoperatively, several attempts were made to seal the perforation with cyanoacrylate glue, but the wound continued to leak. Sterile plastic drape that was on the surgical field was fashioned into a 2-mm-diameter patch, and the peripheral edge of the tectonic drape patch was glued over the perforation, successfully sealing the cornea. One week later, the drape patch was intact without leak, and a penetrating keratoplasty was carried out without complication. Tectonic drape patch technique for nontraumatic corneal perforations in which there is tissue loss is a viable temporizing option when cyanoacrylate glue alone fails and when there is no corneal tissue or amniotic membrane available to close the wound.

  12. Frequency of non-traumatic anterior knee pain in secondary school students

    International Nuclear Information System (INIS)

    Shafiq, W.; Ajmad, F.; Ahmed, A.; Fatima, M.

    2017-01-01

    To find out the frequency of non-traumatic anterior knee pain in secondary school students of Lahore, Pakistan. Methodology: This observational study was conducted among 350 students of secondary school of Lahore during a 3 months period. Both males and females of age 11-17 years were included in the study. A questionnaire was completed which included demographic data, Numeric Rating scale (NRS) and Kujala Scale. The data were being analyzed using SPSS version 21.0. Results: Out of 350 secondary school students, 145 (41%) had anterior knee pain (AKP). Twenty seven (19%) had pain in the left knee only, 54 (37%) had pain in right knee only while 64 (44%) had pain in both knees. The incidence of AKP was highest among 11 to 15 years old students. Moreover, the girls reported high frequency of AKP then the boys. The activities that were affected because of AKP included running, walking, stair climbing and jumping. Conclusion: Non-traumatic AKP is common among students of age 11-17 years, with a peak during adolescence (11-15). This pain not only affects the sports activities but also affects the activity of daily livings. (author)

  13. Non-traumatic brachial plexopathies, clinical, radiological and neurophysiological findings from a tertiary centre.

    LENUS (Irish Health Repository)

    Mullins, G M

    2012-02-03

    OBJECTIVE: To establish the clinical characteristics, aetiology, neuro-physiological characteristics, imaging findings and other investigations in a cohort of patients with non-traumatic brachial plexopathy (BP). METHODS: A 3-year retrospective study of patients with non-traumatic BP identified by electromyography (EMG) and nerve conduction studies (NCS). Clinical information was retrieved from patients\\' medical charts. RESULTS: Twenty-five patients were identified. Causes of BP included neuralgic amyotrophy (NA) (48%), neoplastic (16%), radiation (8%), post infectious (12%), obstetric (4%), rucksack injury (4%), thoracic outlet syndrome (4%) and iatrogenic (4%). Patients with NA presented acutely in 50%. The onset was subacute in all others. Outcome was better for patients with NA. All patients with neoplastic disease had a previous history of cancer. MRI was abnormal in 3\\/16 patients (18.8%). PET scanning diagnosed metastatic plexopathy in two cases. CONCLUSIONS: NA was the most common cause of BP in our cohort and was associated with a more favourable outcome. The authors note potentially discriminating clinical characteristics in our population that aid in the assessment of patients with brachial plexopathies. We advise NCS and EMG be performed in all patients with suspected plexopathy. Imaging studies are useful in selected patients.

  14. Sarcoidosis presenting as granulomatous myositis in a 16-year-old adolescent.

    Science.gov (United States)

    Orandi, Amir B; Eutsler, Eric; Ferguson, Cole; White, Andrew J; Kitcharoensakkul, Maleewan

    2016-11-10

    Sarcoidosis is a multi-system disease characterized by the presence of non-caseating epithelioid granulomas in affected tissues, including skeletal muscle. These organized collections of immune cells have important pathophysiologic action including cytokine production leading to inflammation as well as enzymatic conversion of cholecalciferol to calcitriol via 1-α hydroxylase. There are limited reports of isolated granulomatous myositis causing hypercalcemia in pediatric patients. Our patient uniquely presented with symptoms from hypercalcemia and renal insufficiency caused by an overwhelming burden of granulomatous myositis in her lower extremities, but was otherwise asymptomatic. A 16 year old Caucasian female presented with protracted symptoms of fatigue, nausea and prominent weight loss with laboratory evidence of hypercalcemia and renal insufficiency. She lacked clinical and physical findings of arthritis, weakness, rash, uveitis, fever, lymphadenopathy or respiratory symptoms. After extensive negative investigations, re-examination yielded subtle soft tissue changes in her lower extremities, with striking MRI findings of extensive myositis without correlative weakness or serum enzyme elevation. Biopsy showed the presence of non-caseating epithelioid granulomas and calcium oxalate crystals. The patient responded well to prednisone and methotrexate but relapsed with weaning of steroids. She reachieved remission with addition of adalimumab. Sarcoidosis should be considered in patients presenting with symptomatic hypercalcemia with no apparent causes and negative routine workup. The absences of decreased muscle strength or elevated muscle enzymes do not preclude the diagnosis of granulomatous myositis.

  15. Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo

    NARCIS (Netherlands)

    Badrising, UA; Maat-Schieman, MLC; Ferrari, MD; Zwinderman, AH; Wessels, JAM; Breedveld, FC; van Doorn, PA; van Engelen, BGM; Hoogendijk, JE; Howeler, CJ; de Jager, AE; Jennekens, FGI; Koehler, PJ; de Visser, M; Viddeleer, A; Verschuuren, JJ; Wintzen, AR

    We investigated whether 5 to 20mg per week oral methotrexate could slow down disease progression in 44 patients with inclusion body myositis in a randomized double-blind placebo-controlled study over 48 weeks. Mean change of quantitative muscle strength testing sum scores was the primary study

  16. Topical action of Buriti oil (Mauritia flexuosa L.) in myositis induced in rats.

    Science.gov (United States)

    Barbosa, Marília Ursulino; Silva, Marcello de Alencar; Barros, Esmeralda Maria Lustosa; Barbosa, Margarida Ursulino; Sousa, Rayssilane Cardoso de; Lopes, Mateus Aguiar da Costa; Coelho, Nayana Pinheiro Machado de Freitas

    2017-11-01

    To analyze the topical effects of Buriti oil (Mauritia flexuosa L.) in induced myositis in rats. Thirty six male rats divided into three groups: Control group (C), induced myositis group (MI) and induced myositis group reated with Mauritia flexuosa L. (MT). After inducing myositis with 1% acetic acid, was topically applied 0.5 ml of Mauritia flexuosa L.extract on the posterior region of the right gastrocnemius muscle in animals belonging to group MT, for 7 and 14 days. The neutrophil number there was statistically significant difference, after 7 and 14 days, between groups C and MI (p <0.001) (p<0.01). The group MT there was a significant difference in relation to MI group in both experimental times with (p<0.001). The number of fibroblasts in the 14 days showed that when comparing the groups M and MT the differences were also significant (p<0.001). As for the DLL, in 7 days, there was a significant difference between group C and MI group (p <0.001). When considering the MT group, there was a significant difference in relation to the MI group (p <0.001). The extract of Mauritia flexuosa L. leaves lessened acute and chronic inflammation, increased fibroblast proliferation and reduced macroscopically edema.

  17. Myositis in the head and neck: challenges in diagnosis and management.

    Science.gov (United States)

    Ratansi, R; Fabbroni, G; Kanatas, A

    2017-09-01

    Myositis in the head and neck may present with non-specific symptoms, and radiographically may mimic malignancy. Multidisciplinary management is often essential, and we describe the challenges in an effort to raise awareness of the condition. Copyright © 2017 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  18. Epidemiology of inclusion body myositis in the Netherlands : A nationwide study

    NARCIS (Netherlands)

    Badrising, UA; Maat-Schieman, M; van Duinen, SG; Breedveld, F; van Doorn, P; van Engelen, B; van den Hoogen, F; Hoogendijk, J; Howeler, C; de Jager, A; Jennekens, F; Koehler, P; van der Leeuw, H; de Visser, M; Verschuuren, JJ; Wintzen, AR

    2000-01-01

    Epidemiologic data on inclusion body myositis (IBM) are scarce, and possibly biased, because they are derived from larger neuromuscular centers. The present nationwide collaborative cross-sectional study, which culminated on July 1, 1999, resulted in identification of 76 patients with IBM and the

  19. Radiation-recall myositis presenting as low-back pain (2010: 4b)

    International Nuclear Information System (INIS)

    Heirwegh, Geert; Bruyeer, Eveline; Demaerel, Philippe; Renard, Marleen; Uyttebroeck, Anne

    2010-01-01

    We report on a patient with a history of Ewing sarcoma who underwent surgery and subsequent adjuvant chemotherapy and radiotherapy. He developed low-back pain 6 months after the end of the radiotherapy and during consolidation chemotherapy. Magnetic resonance imaging showed evidence of myositis corresponding to the 'radiation-recall phenomenon', an inflammatory reaction of irradiated tissue. (orig.)

  20. Statin-induced focal myositis of the upper extremity. A report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Wagner, M., E-mail: wagner.radiologie@herzchirurgie.de [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany); Muehldorfer-Fodor, M.; Prommersberger, K.J. [Department of Handsurgery, Herz- und Gefaessklinik GmbH, Bad Neustadt an der Saale (Germany); Schmitt, R. [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany)

    2011-02-15

    Statins are widely used to lower increased cholesterol levels with the aim to prevent major cardiovascular events. However, they bare the risk of myotoxic side effects. We report on two patients with focal weakness and pain in the upper extremities. In both patients, abnormal MRI signal heights in the muscle groups involved were indicative of the final diagnosis of focal myositis during statin therapy.

  1. Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report.

    Science.gov (United States)

    Chinniah, Keith J; Mody, Girish M

    2017-06-01

    Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. A 56-year old African Black woman initially presented with systemic sclerosis (SSC) - myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia. Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal and she remained well 20 months after hospital discharge. Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge.

  2. Medical and Surgical Treatment in Pediatric Orbital Myositis Associated with Coxsackie Virus

    Directory of Open Access Journals (Sweden)

    Pedro Gil

    2015-01-01

    Full Text Available Purpose. To report a case of orbital myositis associated with Coxsackie virus and its medical and surgical approach. Methods. Complete ophthalmological examination and imaging and analytical investigation were performed. Results. A 6-year-old male presented with subacute painless binocular horizontal diplopia. Examination revealed bilateral best-corrected visual acuity (BCVA of 20/20 and right eye 45-prism-dioptre (PD esotropia in near and distance fixations, with no motility restrictions. Serologic screening was positive for Coxsackie virus acute infection and computerized tomography (CT suggested right eye medial rectus orbital myositis. An oral corticosteroid 1.0 mg/kg/day regimen was started. A new CT after two months showed symmetrical lesions in both medial rectus muscles. Corticosteroids were increased to 1.5 mg/kg/day. After imagiological resolution on the 4th month, alternating 45 PD esotropia persisted. Bilateral 7 mm medial rectus recession was performed after 1 year without spontaneous recovery. At 1-year follow-up, the patient is orthophoric with 200′′ stereopsis and bilateral 20/20 BCVA. Conclusions. To our knowledge, this is the first reported case of orbital myositis associated with Coxsackie virus. This is also the first reported case of isolated strabismus surgery after orbital myositis in pediatric age, highlighting the favourable aesthetic and functional outcomes even in cases of late ocular motility disorders.

  3. Radiation-recall myositis presenting as low-back pain (2010: 4b)

    Energy Technology Data Exchange (ETDEWEB)

    Heirwegh, Geert [AZ Damiaanziekenhuis, Department of Radiology, Oostende (Belgium); University Hospital K.U. Leuven, Department of Radiology, Leuven (Belgium); Bruyeer, Eveline; Demaerel, Philippe [University Hospital K.U. Leuven, Department of Radiology, Leuven (Belgium); Renard, Marleen; Uyttebroeck, Anne [University Hospital K.U. Leuven, Department of Paediatrics, Leuven (Belgium)

    2010-07-15

    We report on a patient with a history of Ewing sarcoma who underwent surgery and subsequent adjuvant chemotherapy and radiotherapy. He developed low-back pain 6 months after the end of the radiotherapy and during consolidation chemotherapy. Magnetic resonance imaging showed evidence of myositis corresponding to the 'radiation-recall phenomenon', an inflammatory reaction of irradiated tissue. (orig.)

  4. ''Dropped-head'' syndrome due to isolated myositis of neck extensor muscles: MRI findings

    International Nuclear Information System (INIS)

    Gaeta, Michele; Mazziotti, Silvio; Blandino, Alfredo; Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna

    2006-01-01

    MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings. (orig.)

  5. Statin-induced focal myositis of the upper extremity. A report of two cases

    International Nuclear Information System (INIS)

    Wagner, M.; Muehldorfer-Fodor, M.; Prommersberger, K.J.; Schmitt, R.

    2011-01-01

    Statins are widely used to lower increased cholesterol levels with the aim to prevent major cardiovascular events. However, they bare the risk of myotoxic side effects. We report on two patients with focal weakness and pain in the upper extremities. In both patients, abnormal MRI signal heights in the muscle groups involved were indicative of the final diagnosis of focal myositis during statin therapy.

  6. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

    Energy Technology Data Exchange (ETDEWEB)

    Berko, Netanel S.; Levin, Terry L. [Montefiore Medical Center, Department of Radiology, Bronx, NY (United States); Hay, Arielle [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States); Miami Children' s Hospital, Department of Pediatrics, Miami, FL (United States); Sterba, Yonit; Wahezi, Dawn [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States)

    2015-09-15

    Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

  7. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

    Science.gov (United States)

    Berko, Netanel S; Hay, Arielle; Sterba, Yonit; Wahezi, Dawn; Levin, Terry L

    2015-09-01

    Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P myositis were 75% and 31%, respectively, with a sensitivity of 40% and specificity of 67%. Compression-strain US

  8. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

    International Nuclear Information System (INIS)

    Berko, Netanel S.; Levin, Terry L.; Hay, Arielle; Sterba, Yonit; Wahezi, Dawn

    2015-01-01

    Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

  9. Associations between apparent diffusion coefficient and electromyography parameters in myositis-A preliminary study.

    Science.gov (United States)

    Meyer, Hans-Jonas; Emmer, Alexander; Kornhuber, Malte; Surov, Alexey

    2018-05-01

    MRI is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an emergent imaging modality sensitive to microstructural alterations in tissue. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules. Electromyography (EMG) is a clinically used diagnostic tool in myositis. The aim of this study was to elucidate possible associations between ADC values and EMG findings in myositis patients. Seven patients (eight investigated muscles) with myositis (mean age 51.43 ± 19 years) were included in this study. The diagnosis was confirmed by histopathology in every case. DWI was obtained with a 1.5-T scanner using two b-values 0 and 1000 s/mm². In all patients, a needle electromyography (EMG) was performed within 3 days to the MRI. The following EMG parameters were studied: motor unit action potential (MUAP) amplitudes and durations, as well as pathological spontaneous activity. Spearman's correlation coefficient was used to analyze associations between investigated parameters. The estimated mean ADC mean value was 1.51 ± 0.29 × 10 -3  mm²/s, mean ADC min was 1.28 ± 0.27 × 10 -3  mm²/s, and mean ADC max was 1.73 ± 0.28 × 10 -3  mm²/s. Correlation analysis identified significant associations between ADC mean and duration of the MUAP (p   = .78 P = .0279) and between ADC min and duration of the MUAP (p = .85, P = .01). There were no significant differences according to pathological spontaneous activity. ADC mean and ADC min showed strong positive correlations with the duration of the MUAP in myositis patients. Both modalities might similarly reflect muscle fiber loss in myositis patients.

  10. A case of late-onset, thymoma-associated myasthenia gravis with ryanodine receptor and titin antibodies and concomitant granulomatous myositis.

    Science.gov (United States)

    Stefanou, M I; Komorowski, L; Kade, S; Bornemann, A; Ziemann, U; Synofzik, M

    2016-09-13

    Myasthenia gravis is an autoimmune neuromuscular disorder, which has only rarely been reported to co-manifest with myositis. The diagnosis of concomitant myositis in patients with myasthenia gravis is clinically challenging, and requires targeted investigations for the differential diagnosis, including EMG, autoantibody assays, muscle biopsy and, importantly, imaging of the mediastinum for thymoma screening. This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies. The diagnosis of concurrent myositis and myasthenia gravis, especially in the presence of ryanodine receptor and titin antibodies, should lead neurologists to adopt different treatment strategies compared to those applied in myasthenia or myositis alone. Moreover, further evidence is warranted that titin and, particularly, ryanodine receptor antibodies may co-occur or be pathophysiologically involved in myasthenia-myositis cases.

  11. Adductor muscle pyo-myositis simulating appendicitis: CT and MR imaging findings; Pyomyosite des mulcles adducteurs mimant une appendicite aigue: aspects tomodensitometriques et IRM

    Energy Technology Data Exchange (ETDEWEB)

    Coumbaras, M.; Le Hir, P.; Jomaah, N.; Arrive, L.; Tubiana, J.M. [Hopital Saint Antoine, 75 - Paris (France)

    2001-04-01

    Pyo-myositis is a primary bacterial infection of skeletal muscle. This infection tends to occur in the large muscles of the lower extremity. Pyo-myositis of the proximal muscles of the thigh can simulate acute abdominal disease. Early diagnosis improves the outcome. Delayed diagnosis may lead to septicemia and shock. We report the CT and MRI findings in a patient with pyo-myositis of the proximal muscles of the thigh. (authors)

  12. Factors predictive of survival and estimated years of life lost in the decade following nontraumatic and traumatic spinal cord injury.

    Science.gov (United States)

    Hatch, B B; Wood-Wentz, C M; Therneau, T M; Walker, M G; Payne, J M; Reeves, R K

    2017-06-01

    Retrospective chart review. To identify factors predictive of survival after spinal cord injury (SCI). Tertiary care institution. Multiple-variable Cox proportional hazards regression analysis for 759 patients with SCI (535 nontraumatic and 221 traumatic) included age, sex, completeness of injury, level of injury, functional independence measure (FIM) scores, rehabilitation length of stay and SCI cause. Estimated years of life lost in the decade after injury was calculated for patients vs uninjured controls. Median follow-up was 11.4 years. Population characteristics included paraplegia, 58%; complete injury, 11%; male sex, 64%; and median rehabilitation length of stay, 16 days. Factors independently predictive of decreased survival were increased age (+10 years; hazard ratio (HR (95% CI)), 1.6 (1.4-1.7)), male sex (1.3 (1.0-1.6)), lower dismissal FIM score (-10 points; 1.3 (1.2-1.3)) and all nontraumatic causes. Metastatic cancer had the largest decrease in survival (HR (95% CI), 13.3 (8.7-20.2)). Primary tumors (HR (95% CI), 2.5 (1.7-3.8)), vascular (2.5 (1.6-3.8)), musculoskeletal/stenosis (1.7 (1.2-2.5)) and other nontraumatic SCI (2.3 (1.5-3.6)) were associated with decreased survival. Ten-year survival was decreased in nontraumatic SCI (mean (s.d.), 1.8 (0.3) years lost), with largest decreases in survival for metastatic cancer and spinal cord ischemia. Age, male sex and lower dismissal FIM score were associated with decreased survival, but neither injury severity nor level was associated with it. Survival after SCI varies depending on SCI cause, with survival better after traumatic SCI than after nontraumatic SCI. Metastatic cancer and vascular ischemia were associated with the greatest survival reduction.

  13. High-energy extracorporeal shock wave therapy for nontraumatic osteonecrosis of the femoral head.

    Science.gov (United States)

    Xie, Kai; Mao, Yuanqing; Qu, Xinhua; Dai, Kerong; Jia, Qingwei; Zhu, Zhenan; Yan, Mengning

    2018-02-02

    Nontraumatic osteonecrosis of the femoral head (ONFH) is treated with a series of methods. High-energy extracorporeal shock wave therapy (ESWT) is an option with promising mid-term outcomes. The objective of this study was to determine the long-term outcomes of ESWT for ONFH. Fifty-three hips in 39 consecutive patients were treated with ESWT in our hospital between January 2005 and July 2006. Forty-four hips in 31 patients with stage I-III nontraumatic ONFH, according to the Association Research Circulation Osseous (ARCO) system, were reviewed in the current retrospective study. The visual analog pain scale (VAS), Harris hip score, radiography, and magnetic resonance imaging were used to estimate treatment results. The progression of ONFH was evaluated by imaging examination and clinical outcomes. The results were classified as clinical success (no progression of hip symptoms) and imaging success (no progression of stage or substage on radiography and MRI). The mean follow-up duration was 130.6 months (range, 121 to 138 months). The mean VAS decreased from 3.8 before ESWT to 2.2 points at the 10-year follow-up (p < 0.001). The mean Harris hip score improved from 77.4 before ESWT to 86.9 points at the 10-year follow-up. The clinical success rates were 87.5% in ARCO stage I patients, 71.4% in ARCO stage II patients, and 75.0% in ARCO stage III patients. Imaging success was observed in all stage I hips, 64.3% of stage II hips, and 12.5% of stage III hips. Seventeen hips showed progression of the ARCO stage/substage on imaging examination. Eight hips showed femoral head collapse at the 10-year follow-up. Four hips in ARCO stage III and one hip in ARCO stage II were treated with total hip arthroplasty during the follow-up. Three were performed 1 year after ESWT, one at 2 years, and one at 5 years. The results of the current study indicated that ESWT is an effective treatment method for nontraumatic ONFH, resulting in pain relief and function restoration

  14. Gemfibrozil-induced myositis in a patient with normal renal function.

    Science.gov (United States)

    Hahn, Martin; Sriharan, Kalavally; McFarland, M Shawn

    2010-01-01

    To describe a case of gemfibrozil monotherapy-induced myositis in a patient with normal renal function A 68-year-old white man presented to his primary care clinic complaining of a 6-month history of total body pain. His past medical history was significant for hypertension, diabetes mellitus, hyperlipidemia, gastroesophageal reflux disease, benign prostatic hypertrophy, arthritis, impotence, and pancreatic cancer that required excision of part of his pancreas. His home drug regimen included bupropion 75 mg twice daily, gemfibrozil 600 mg twice daily for the past 8 months, glimiperide 1 mg daily, insulin glargine 5 units at bedtime, insulin aspart 5 units in the evening, lisinopril 10 mg daily, omeprazole 40 mg daily, pregabalin 100 mg daily, and sildenafil 100 mg as needed. Laboratory test results were significant for elevated aspartate aminotransferase (AST) 78 U/L (reference range 15-46 U/L), alanine aminotransferase (ALT) 83 U/L (13-69 U/L), and creatine kinase (CK) 3495 U/L (55-170 U/L). Serum creatinine was normal at 1.19 mg/dL. The physician determined that the elevated CK indicated myositis secondary to gemfibrozil use, and gemfibrozil was subsequently discontinued. The patient returned 1 week later to repeat the laboratory tests. Results were CK 220 U/L, AST 26 U/L, ALT 43 U/L, and serum creatinine 1.28 mg/dL. The patient was asked to return in 3 weeks to repeat the laboratory tests. At that time, CK had continued to decrease to 142 U/L, and the AST and ALT had returned to normal, at 22 and 29 U/L, respectively. The patient reported complete resolution of total body pain 3 weeks after discontinuation of gemfibrozil. Follow-up 5 weeks after discontinuation revealed no change compared to the 3-week follow-up. Myositis most often produces weakness and elevated CK levels more than 10 times the upper limit of normal. The risk of developing myositis, myopathy, or rhabdomyolysis is low (1%) when fibrates such as gemfibrozil are used as monotherapy. Evaluation of

  15. Nontraumatic tibial polyethylene insert cone fracture in mobile-bearing posterior-stabilized total knee arthroplasty

    Directory of Open Access Journals (Sweden)

    Yohei Tanikake

    2016-12-01

    Full Text Available A 72-year-old male patient underwent mobile-bearing posterior-stabilized total knee arthroplasty for osteoarthritis. He experienced a nontraumatic polyethylene tibial insert cone fracture 27 months after surgery. Scanning electron microscopy of the fracture surface of the tibial insert cone suggested progress of ductile breaking from the posterior toward the anterior of the cone due to repeated longitudinal bending stress, leading to fatigue breaking at the anterior side of the cone, followed by the tibial insert cone fracture at the anterior side of the cone, resulting in fracture at the base of the cone. This analysis shows the risk of tibial insert cone fracture due to longitudinal stress in mobile-bearing posterior-stabilized total knee arthroplasty in which an insert is designed to highly conform to the femoral component.

  16. Subchondral insufficiency fracture of the knee: a non-traumatic injury with prolonged recovery time.

    Science.gov (United States)

    Gourlay, Margaret L; Renner, Jordan B; Spang, Jeffrey T; Rubin, Janet E

    2015-06-08

    Subchondral insufficiency fractures are non-traumatic fractures that occur immediately below the cartilage of a joint. Although low bone density may be present concurrently, it is not the underlying cause of subchondral insufficiency fractures in the majority of patients. Patients with subchondral insufficiency fracture characteristically have unremarkable plain radiographs, while MRI examination may reveal extensive bone marrow oedema and subchondral bone collapse. This article presents a 51-year-old postmenopausal woman, a physician, who had subchondral insufficiency fractures of the knee associated with prolonged standing during clinical work. She was treated with partial weight bearing on crutches until 14 months after the injury, viscosupplementation at 4 months to treat osteoarthritis and teriparatide treatment to improve bone healing at 7 months. By 26 months after the injury, she tolerated independent walking with a fabric knee support but still experienced mild posterolateral knee pain and numbness on prolonged standing. 2015 BMJ Publishing Group Ltd.

  17. Nontraumatic Fracture of the Femoral Condylar Prosthesis in a Total Knee Arthroplasty Leading to Mechanical Failure

    Directory of Open Access Journals (Sweden)

    Girish N. Swamy

    2014-01-01

    Full Text Available This paper reports a case of fatigue fracture of the femoral component in a cruciate-retaining cemented total knee arthroplasty (TKA. A 64-year-old man had undergone a primary TKA for osteoarthritis 10 years previously at another institution using the PFC-Sigma prosthesis. The patient recovered fully and was back to his regular activities. He presented with a history of sudden onset pain and locking of the left knee since the preceding three months. There was no history of trauma, and the patient was mobilizing with difficulty using crutches. Radiographs revealed fracture of the posterior condyle of the femoral prosthesis. Revision surgery was performed as an elective procedure revealing the broken prosthesis. The TC3RP-PFC revision prosthesis was used with a medial parapatellar approach. The patient recovered fully without any squeal. Mechanical failure of the knee arthroplasty prosthesis is rare, and nontraumatic fracture of the femoral metallic component has not been reported before.

  18. Nontraumatic fracture of the femoral condylar prosthesis in a total knee arthroplasty leading to mechanical failure.

    Science.gov (United States)

    Swamy, Girish N; Quah, Conal; Bagouri, Elmunzar; Badhe, Nitin P

    2014-01-01

    This paper reports a case of fatigue fracture of the femoral component in a cruciate-retaining cemented total knee arthroplasty (TKA). A 64-year-old man had undergone a primary TKA for osteoarthritis 10 years previously at another institution using the PFC-Sigma prosthesis. The patient recovered fully and was back to his regular activities. He presented with a history of sudden onset pain and locking of the left knee since the preceding three months. There was no history of trauma, and the patient was mobilizing with difficulty using crutches. Radiographs revealed fracture of the posterior condyle of the femoral prosthesis. Revision surgery was performed as an elective procedure revealing the broken prosthesis. The TC3RP-PFC revision prosthesis was used with a medial parapatellar approach. The patient recovered fully without any squeal. Mechanical failure of the knee arthroplasty prosthesis is rare, and nontraumatic fracture of the femoral metallic component has not been reported before.

  19. Imaging of non-cardiac, non-traumatic causes of acute chest pain

    Energy Technology Data Exchange (ETDEWEB)

    Kienzl, Daniela, E-mail: daniela.kienzl@meduniwien.ac.at [Department of Radiology, Medical University of Vienna (Austria); Prosch, Helmut; Töpker, Michael; Herold, Christian [Department of Radiology, Medical University of Vienna (Austria)

    2012-12-15

    Non-traumatic chest pain is a common symptom in patients who present in the emergency department. From a clinical point of view, it is important to differentiate cardiac chest pain from non-cardiac chest pain (NCCP). Among the plethora of potential causes of NCCP, life-threatening diseases, such as aortic dissection, pulmonary embolism, tension pneumothorax, and esophageal rupture, must be differentiated from non-life threatening causes. The majority of NCCP, however, is reported to be benign in nature. The presentation of pain plays an important role in narrowing the differential diagnosis and initiating further diagnostic management and treatment. As the benign causes tend to recur, and may lead to patient anxiety and great costs, a meticulous evaluation of the patient is necessary to diagnose the underlying disorder or disease.

  20. Non-traumatic acute abdomen in the adult: a critical review of imaging modalities

    International Nuclear Information System (INIS)

    Buitrago-Tellez, C.; Boos, S.; Heinemann, F.; Wenz, W.

    1992-01-01

    Two different series collected at the University Hospital of Freiburg are presented as a basis for the critical appraisal of available imaging tests and their correlation with anatomic pain sites. The first series comprises 284 patients of non-traumatic acute abdomen admitted to the emergency department during 1990. The radiological approach revealed an increased use of immediate ultrasound (58%) followed by plain film radiography (53%), contrast studies (15%), and computed tomography (9.8%). Five groups of entities were identified according to the diagnostic imaging procedure prior to surgery. In a second series, the clinical presentation, the distribution and the efficacy of imaging studies for the confirmation of acute mesenteric ischemia were evaluated in 55 patients over a period of 14 years. A reduced mortality rate emphasizes the value of early and agressive diagnosis and angiographic management. (orig.)

  1. MR imaging of recent non-traumatic intracranial hemorrhage: early experience at 3 T

    International Nuclear Information System (INIS)

    Griffiths, Paul D.; Wilkinson, Iain D.

    2006-01-01

    Magnetic resonance imaging (MRI) using 3.0 T scanners in the clinical environment is in its infancy and is only available at a limited number of sites worldwide. There is great interest amongst radiologists about the perceived benefits of clinical imaging at 3.0 T; however, it remains to be seen whether the theoretical advantages will bring real gains. MRI in patients with non-traumatic intracranial hemorrhage (ICH) is difficult, yet, these patients benefit from non-invasive angiography. Conventional catheter angiography (CCA) remains the reference standard for excluding/confirming the presence of intracranial vascular abnormalities, but MR angiography at 3.0 T may offer opportunities for significant changes in patient management. We present our experiences of using 3.0 T MR angiography in 27 patients with acute or early subacute ICH. (orig.)

  2. Chronic neck pain patients with traumatic or non-traumatic onset

    DEFF Research Database (Denmark)

    Ris Hansen, Inge; Juul-Kristensen, Birgit; Boyle, Eleanor

    2017-01-01

    . The purpose of this study was to investigate the clinical presentation of chronic neck pain patients with and without traumatic onset by examining cervical mobility, sensorimotor function, cervical muscle performance and pressure pain threshold in addition to the following self-reported characteristics...... tests of cervical range of motion, gaze stability, eye movement, cranio-cervical flexion, cervical extensors, and pressure pain threshold. The participants completed the following questionnaires: physical and mental component summary of the Short Form Health Survey, EuroQol-5D, Neck Disability Index...... in the traumatic group showed worse results on all measures compared with those in the non-traumatic group, significantly on neck muscle function (cervical extension mobility p = 0.005, cranio-cervical flexion test p = 0.007, cervical extensor test p = 0.006) and cervical pressure pain threshold bilateral (p = 0...

  3. Tumour TIF1 mutations and loss of heterozygosity related to cancer-associated myositis.

    Science.gov (United States)

    Pinal-Fernandez, Iago; Ferrer-Fabregas, Berta; Trallero-Araguas, Ernesto; Balada, Eva; Martínez, Maria Angeles; Milisenda, Jose César; Aparicio-Español, Gloria; Labrador-Horrillo, Moises; Garcia-Patos, Vicente; Grau-Junyent, Josep M; Selva-O'Callaghan, Albert

    2018-02-01

    To analyse the influence of genetic alterations and differential expression of transcription intermediary factor 1 (TIF1) genes in the pathophysiology of cancer-associated myositis (CAM). Paired blood and tumour DNA samples from patients with anti-TIF1γ-positive CAM and from controls were analysed by whole-exome sequencing for the presence of somatic mutations and loss of heterozygosity (LOH) in their TIF1 genes. The genesis and maintenance of the autoimmune process were investigated immunohistochemically by studying TIF1γ expression in the different tissues involved in CAM (skin, muscle and tumour) based on the immunohistochemical H-score. From seven patients with anti-TIF1γ-positive CAM, we detected one somatic mutation and five cases of LOH in one or more of the four TIF1 genes compared with just one case of LOH in tumours from TIF1γ-negative myositis patients (86% vs 17%; P = 0.03). Compared with type-matched control tumours from non-myositis patients, TIF1γ staining was more intense in tumours from anti-TIF1γ-positive patients (H-score 255 vs 196; P = 0.01). Also, TIF1γ staining in muscle was slightly more intense in anti-TIF1γ-positive than in anti-TIF1γ-negative myositis (H-score 22 vs 5; P = 0.03). In contrast, intense TIF1γ staining was detected in the skin of both myositis and control patients. Tumours from paraneoplastic anti-TIF1γ-positive patients showed an increased number of genetic alterations, such as mutations and LOH, in TIF1 genes. These genetic alterations, in the context of a high expression of TIF1γ in the tumour, muscle and skin of these patients may be key to understanding the genesis of paraneoplastic myositis. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  4. Non-traumatic rhabdomyolysis: Background, laboratory features, and acute clinical management.

    Science.gov (United States)

    Cervellin, Gianfranco; Comelli, Ivan; Benatti, Mario; Sanchis-Gomar, Fabian; Bassi, Antonella; Lippi, Giuseppe

    2017-08-01

    Rhabdomyolysis is a relatively rare condition, but its clinical consequences are frequently dramatic in terms of both morbidity and mortality. Although no consensus has been reached so far about the precise definition of this condition, the term rhabdomyolysis describes a rapid breakdown of striated, or skeletal, muscle. It is hence characterized by the rupture and necrosis of muscle fibers, resulting in release of cell degradation products and intracellular elements within the bloodstream and extracellular space. Notably, the percentage of patients with rhabdomyolysis who develop acute kidney injury, the most dramatic consequence, varies from 13% to over 50% according to both the cause and the clinical and organizational setting where they are diagnosed. Despite direct muscle injury (i.e., traumatic rhabdomyolysis) remains the most common cause, additional causes, frequently overlapping, include hypoxic, physical, chemical or biological factors. The conventional triad of symptoms includes muscle pain, weakness and dark urine. The laboratory diagnosis is essentially based on the measurement of biomarkers of muscle injury, being creatine kinase (CK) the biochemical "gold standard" for diagnosis, and myoglobin the "gold standard" for prognostication, especially in patients with non-traumatic rhabdomyolysis. The essential clinical management in the emergency department is based on a targeted intervention to manage the underlying cause, combined with infusion of fluids and eventually sodium bicarbonate. We will present and discuss in this article the pathophysiological and clinical features of non-traumatic rhabdomyolysis, focusing specifically on Emergency Department (ED) management. Copyright © 2017 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

  5. High Prevalence of Nontraumatic Shoulder Pain in a Regional Sample of Female High School Volleyball Athletes

    Science.gov (United States)

    Frisch, Kayt E.; Clark, Jacob; Hanson, Chad; Fagerness, Chris; Conway, Adam; Hoogendoorn, Lindsay

    2017-01-01

    Background: Shoulder pain is becoming increasingly problematic in young players as volleyball gains popularity. Associations between repetitive motion and pain and overuse injury have been observed in other overhand sports (most notably baseball). Studies of adult athletes suggest that there is a shoulder pain and overuse problem present in volleyball players, but minimal research has been done to establish rates and causes in juvenile participants. Purpose: To establish rates of shoulder pain, regardless of whether it resulted in a loss of playing time, in female high school volleyball players. A secondary goal was to determine whether high repetition volumes correlated with an increased likelihood of experiencing pain. Study Design: Descriptive epidemiology study. Methods: A self-report survey focusing on the prevalence of pain not associated with a traumatic event in female high school youth volleyball players was developed. Survey questions were formulated by certified athletic trainers, experienced volleyball coaches, and biomechanics experts. Surveys were received from 175 healthy, active high school volleyball players in Iowa, South Dakota, and Minnesota. Results: Forty percent (70/175) of active high school volleyball players remembered experiencing shoulder pain not related to traumatic injury, but only 33% (23/70) reported taking time off to recover from the pain. Based on these self-reported data, activities associated with significantly increased risk of nontraumatic shoulder pain included number of years playing competitive volleyball (P = .01) and lifting weights out of season (P = .001). Players who reported multiple risk factors were more likely to experience nontraumatic shoulder pain. Conclusion: When using time off for recovery as the primary injury criterion, we found that the incidence of shoulder pain is more than twice as high as the incidence of injury reported by previous studies. Findings also indicated that the incidence of shoulder pain

  6. High Prevalence of Nontraumatic Shoulder Pain in a Regional Sample of Female High School Volleyball Athletes.

    Science.gov (United States)

    Frisch, Kayt E; Clark, Jacob; Hanson, Chad; Fagerness, Chris; Conway, Adam; Hoogendoorn, Lindsay

    2017-06-01

    Shoulder pain is becoming increasingly problematic in young players as volleyball gains popularity. Associations between repetitive motion and pain and overuse injury have been observed in other overhand sports (most notably baseball). Studies of adult athletes suggest that there is a shoulder pain and overuse problem present in volleyball players, but minimal research has been done to establish rates and causes in juvenile participants. To establish rates of shoulder pain, regardless of whether it resulted in a loss of playing time, in female high school volleyball players. A secondary goal was to determine whether high repetition volumes correlated with an increased likelihood of experiencing pain. Descriptive epidemiology study. A self-report survey focusing on the prevalence of pain not associated with a traumatic event in female high school youth volleyball players was developed. Survey questions were formulated by certified athletic trainers, experienced volleyball coaches, and biomechanics experts. Surveys were received from 175 healthy, active high school volleyball players in Iowa, South Dakota, and Minnesota. Forty percent (70/175) of active high school volleyball players remembered experiencing shoulder pain not related to traumatic injury, but only 33% (23/70) reported taking time off to recover from the pain. Based on these self-reported data, activities associated with significantly increased risk of nontraumatic shoulder pain included number of years playing competitive volleyball ( P = .01) and lifting weights out of season ( P = .001). Players who reported multiple risk factors were more likely to experience nontraumatic shoulder pain. When using time off for recovery as the primary injury criterion, we found that the incidence of shoulder pain is more than twice as high as the incidence of injury reported by previous studies. Findings also indicated that the incidence of shoulder pain may be correlated with volume of previous volleyball experience.

  7. Fibrodisplasia ossificante progressiva: relato de caso e achados radiográficos Fibrodysplasia ossificans progressiva: a case report and radiographic findings

    Directory of Open Access Journals (Sweden)

    Cyrillo Rodrigues de Araújo Júnior

    2005-02-01

    Full Text Available A fibrodisplasia ossificante progressiva é uma doença genética rara do tecido conjuntivo, caracterizada por ossificação disseminada em tecidos moles e alterações congênitas das extremidades. Sua transmissão é autossômica dominante, com penetrância completa, mas expressão variável. O início ocorre na infância e o envolvimento progressivo axial e da região proximal dos membros leva a uma conseqüente imobilização e deformação articular. Apresentamos um caso de um paciente de 22 anos de idade, do sexo masculino, com quadro clínico característico de fibrodisplasia ossificante progressiva e discutimos os últimos avanços no diagnóstico e na fisiopatogenia desta entidade.Fibrodysplasia ossificans progressiva is a rare hereditary connective tissue disease characterized by disseminated soft tissue ossification and congenital abnormality of the extremities. It is genetically inherited as a dominant trait with complete penetrance but variable expression. The onset takes place during childhood and the progressive involvement of the spine and proximal extremities leads to immobilization and articular deformity. We report a case of a 22-year-old male patient with typical symptoms of fibrodysplasia ossificans progressiva and discuss the new advances in the diagnosis and pathophysiology.

  8. New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter.

    Science.gov (United States)

    Leclair, Valérie; Lundberg, Ingrid E

    2018-03-17

    Idiopathic inflammatory myopathy (IIM) classification criteria have been a subject of debate for many decades. Despite several limitations, the Bohan and Peter criteria are still widely used. The aim of this review is to discuss the evolution of IIM classification criteria. New IIM classification criteria are periodically proposed. The discovery of myositis-specific and myositis-associated autoantibodies led to the development of clinico-serological criteria, while in-depth description of IIM morphological features improved histopathology-based criteria. The long-awaited European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) IIM classification criteria were recently published. The Bohan and Peter criteria are outdated and validated classification criteria are necessary to improve research in IIM. The new EULAR/ACR IIM classification criteria are thus a definite improvement and an important step forward in the field.

  9. [Ocular myositis as a rare cause of vision loss].

    Science.gov (United States)

    Rollnik, J D; Requadt, H

    2017-04-01

    Ocular myositis is a rare disease characterized by painful diplopia but loss of vision rarely occurs. The article reviews the literature focusing on the differential diagnostics. We report the case of an 80-year-old women suffering from slowly progressive loss of vision in the left eye. Diplopia was only present at the beginning and there was only moderate pain. Computed tomography and magnetic resonance imaging revealed a swelling of the left medial, lateral and inferior rectus muscles of the orbit leading to compression of the optic nerve in the orbital cone. An intravenous prednisolone stoss therapy (1000 mg per day for 3 consecutive days) was initiated, followed by oral medication of 100 mg per day then tapering over 10 weeks. Vision improved and no relapses were observed. Physicians should be aware of this rare disease to ensure quick diagnosis and treatment of ocular myositis.

  10. A case of acute orbital myositis which was difficult to diagnose at first

    International Nuclear Information System (INIS)

    Yamamoto, Kiyoshi; Terabayashi, Tadasu; Mori, Hiroshi; Niida, Hirohito; Sugiyama, Yoshiaki; Nakagawa, Masato

    1988-01-01

    We report a case of acute orbital myositis. A 61-year-old woman exhibited acute orbital pain, diplopia, and left proptosis. Examination revealed a 5-mm left proptosis, left chemosis, and limitations in all directions of the movement of the left eye. Visual acuity was unimpaired, however, and the neurological examination was otherwise normal. CT demonstrated a left inferior orbital mass. We suspected an acute orbital pseudotumor based on the rapid onset and the clinical symptoms. We treated her with systemic corticosteroids. Four weeks later CT documented a reduced left orbital mass; there seemed to be left only an inferior rectus muscle enlargement. We diagnosed acute orbital myositis, a subgroup of orbital pseudotumors, based upon the rapid clinical presentation, the CT features, and the resolution after treatment with systemic corticosteroids. (author)

  11. Case of acute orbital myositis which was difficult to diagnose at first

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Kiyoshi; Terabayashi, Tadasu; Mori, Hiroshi; Niida, Hirohito; Sugiyama, Yoshiaki; Nakagawa, Masato

    1988-02-01

    We report a case of acute orbital myositis. A 61-year-old woman exhibited acute orbital pain, diplopia, and left proptosis. Examination revealed a 5-mm left proptosis, left chemosis, and limitations in all directions of the movement of the left eye. Visual acuity was unimpaired, however, and the neurological examination was otherwise normal. CT demonstrated a left inferior orbital mass. We suspected an acute orbital pseudotumor based on the rapid onset and the clinical symptoms. We treated her with systemic corticosteroids. Four weeks later CT documented a reduced left orbital mass; there seemed to be left only an inferior rectus muscle enlargement. We diagnosed acute orbital myositis, a subgroup of orbital pseudotumors, based upon the rapid clinical presentation, the CT features, and the resolution after treatment with systemic corticosteroids.

  12. Acute myositis: an unusual and severe side effect of docetaxel: a case report and literature review.

    Science.gov (United States)

    Rochigneux, Philippe; Schleinitz, Nicolas; Ebbo, Mikael; Aymonier, Marie; Pourroy, Bertrand; Boissier, Romain; Salas, Sébastien; Deville, Jean-Laurent

    2018-06-01

    Docetaxel is an antimicrotubules cytotoxic agent prescribed widely by medical oncologists in multiple tumor types (breast, lung, prostate, stomach, head, and neck). However, the side effects of docetaxel are numerous (cytopenia, peripheral edema, myalgia, arthralgia, alopecia, and sensitive neuropathy) and recent concerns have been raised about neutropenic enterocolitis in France. Here, we report the case of a 57-year-old patient with metastatic prostatic cancer, who developed a severe myositis and fasciitis grade IV 1 week after his second docetaxel infusion. We reviewed the five cases of docetaxel-related myositis described in the literature, and found that most of them occurred in patients with diabetes (n=5/5) or hypertension (n=4/5). A vascular toxicity may explain this severe complication, and patients with diabetes or hypertension should be monitored closely in the context of a docetaxel chemotherapy.

  13. [Clinical features and management of acute myositis in idiopathic orbital inflammation].

    Science.gov (United States)

    Halimi, E; Rosenberg, R; Wavreille, O; Bouckehove, S; Franquet, N; Labalette, P

    2013-09-01

    Acute myositis is the second most common component of non-specific orbital inflammation. We will describe its clinical features and natural history. This is a retrospective study of 10 cases. The diagnosis of acute myositis was based on clinical and imaging criteria. Our study includes five men and five women. The average age was 35.8 years (17-59 years). Clinical symptoms were: pain increased on eye movement (10/10), diplopia (4/10), proptosis (6/10), visual loss (3/10), lid edema (6/10), conjunctival hyperemia (7/10), anterior scleritis (2/10), episcleritis (2/10), chemosis (4/10), upper lid retraction (1/10), limitation of eye movement (3/10), fundus abnormalities (2/10). Imaging showed thickening of one or more extraocular muscles (10/10). Recovery was complete with anti-inflammatory therapy in six patients. Three patients experienced recurrence, and one patient had a clinical rebound upon tapering the treatment. Acute myositis can be defined by pain on eye movement, signs of inflammation, and extraocular muscle thickening on imaging. If the clinical presentation is typical, histopathological analysis can be deferred but remains necessary in cases of poor response to treatment, chronic duration or suspicion of tumor infiltration. The diagnosis of acute myositis may be suspected in the presence of consistent, well-defined clinical signs. Contiguous inflammation is often associated. Treatment is based on steroids or non-steroidal treatment anti-inflammatory therapy, administered alone or consecutively. Recurrences are frequent but do not alter the final prognosis. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  14. Vaccine-induced myositis with intramuscular sterile abscess formation: MRI and ultrasound findings

    Energy Technology Data Exchange (ETDEWEB)

    Polat, Ahmet Veysel; Bekci, Tumay; Selcuk, Mustafa Bekir [Ondokuz Mayis University, Department of Radiology, Faculty of Medicine, Samsun (Turkey); Dabak, Nevzat [Ondokuz Mayis University, Department of Orthopaedics and Traumatology, Faculty of Medicine, Samsun (Turkey); Ulu, Esra Meltem Kayahan [Samsun Medical Park Hospital, Department of Radiology, Samsun (Turkey)

    2015-12-15

    Although limb swelling is a well-known complication of vaccination, its rarity and wide band of differential diagnosis of limb swelling make it a diagnostic challenge. In this case report, we describe three cases of vaccine-induced myositis with intramuscular sterile abscess formation in patients with limb swelling and their magnetic resonance imaging and ultrasonography findings. Both radiologists and clinicians should be familiar with this rare entity, its clinical and imaging spectrum, and follow-up strategies. (orig.)

  15. Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

    Science.gov (United States)

    Colafrancesco, Serena; Priori, Roberta; Gattamelata, Angelica; Picarelli, Giovanna; Minniti, Antonina; Brancatisano, Filippo; D'Amati, Giulia; Giordano, Carla; Cerbelli, Bruna; Maset, Marta; Quartuccio, Luca; Bartoloni, Elena; Carubbi, Francesco; Cipriani, Paola; Baldini, Chiara; Luciano, Nicoletta; De Vita, Salvatore; Gerli, Roberto; Giacomelli, Roberto; Bombardieri, Stefano; Valesini, Guido

    2015-01-01

    In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested "negative", four showed "non-specific" findings. All patients were treated with corticosteroids followed by different DMARDs. Our retrospective analysis shows a prevalence of myositis in pSS lower than previously reported, mainly appearing as an overlapping syndrome. Histological findings confirm the possible presence of an IBM or of a myopathy more similar to PM with a decreased COX activity. Classical immunosuppressants are effective although in most difficult cases IVIg or RTX may be used with benefit.

  16. Clinicopathologic features of myositis patients with CD8-MHC-1 complex pathology.

    Science.gov (United States)

    Ikenaga, Chiseko; Kubota, Akatsuki; Kadoya, Masato; Taira, Kenichiro; Uchio, Naohiro; Hida, Ayumi; Maeda, Meiko Hashimoto; Nagashima, Yu; Ishiura, Hiroyuki; Kaida, Kenichi; Goto, Jun; Tsuji, Shoji; Shimizu, Jun

    2017-09-05

    To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers. All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed. The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy. CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis. © 2017 American Academy of Neurology.

  17. Vaccine-induced myositis with intramuscular sterile abscess formation: MRI and ultrasound findings

    International Nuclear Information System (INIS)

    Polat, Ahmet Veysel; Bekci, Tumay; Selcuk, Mustafa Bekir; Dabak, Nevzat; Ulu, Esra Meltem Kayahan

    2015-01-01

    Although limb swelling is a well-known complication of vaccination, its rarity and wide band of differential diagnosis of limb swelling make it a diagnostic challenge. In this case report, we describe three cases of vaccine-induced myositis with intramuscular sterile abscess formation in patients with limb swelling and their magnetic resonance imaging and ultrasonography findings. Both radiologists and clinicians should be familiar with this rare entity, its clinical and imaging spectrum, and follow-up strategies. (orig.)

  18. A rare case of Enterococcus faecalis-induced orbital cellulitis and myositis

    Directory of Open Access Journals (Sweden)

    Piyush Kohli

    2016-01-01

    Full Text Available Orbital cellulitis is an infection of soft tissue behind the orbital septum. Common pathogens isolated include Staphylococcus aureus, Staphylococcus epidermidis, and Streptococcus pneumoniae. It is a straightforward diagnosis and usually responds to empirical treatment without any sequela. We report a case of orbital cellulitis caused by Enterococcus faecalis, which was complicated by myositis of levator palpebrae superioris. To the best of our knowledge, only one case report exists dating way back to 1986.

  19. Apparent diffusion coefficient (ADC) does not correlate with different serological parameters in myositis and myopathy.

    Science.gov (United States)

    Meyer, Hans-Jonas; Ziemann, Oliver; Kornhuber, Malte; Emmer, Alexander; Quäschling, Ulf; Schob, Stefan; Surov, Alexey

    2018-06-01

    Background Magnetic resonance imaging (MRI) is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an imaging modality, which can reflect microstructural tissue composition. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules in tissue. Purpose To investigate ADC values in patients with myositis and non-inflammatory myopathy and to analyze possible associations between ADC and laboratory parameters in these patients. Material and Methods Overall, 17 patients with several myositis entities, eight patients with non-inflammatory myopathies, and nine patients without muscle disorder as a control group were included in the study (mean age = 55.3 ± 14.3 years). The diagnosis was confirmed by histopathology in every case. DWI was obtained in a 1.5-T scanner using two b-values: 0 and 1000 s/mm 2 . In all patients, the blood sample was acquired within three days to the MRI. The following serological parameters were estimated: C-reactive protein, lactate dehydrogenase, alanine aminotransferase, aspartate aminotransferase, creatine kinase, and myoglobine. Results The estimated mean ADC value for the myositis group was 1.89 ± 0.37 × 10 -3  mm 2 /s and for the non-inflammatory myopathy group was 1.79 ± 0.33 × 10 -3  mm 2 /s, respectively. The mean ADC values (1.15 ± 0.37 × 10 -3  mm 2 /s) were significantly higher to unaffected muscles (vs. myositis P = 0.0002 and vs. myopathy P = 0.0021). There were no significant correlations between serological parameters and ADC values. Conclusion Affected muscles showed statistically significantly higher ADC values than normal muscles. No linear correlations between ADC and serological parameters were identified.

  20. Adult bacterial myositis: report of a single-center series of 26 cases

    Directory of Open Access Journals (Sweden)

    Fernando Gallucci

    2016-09-01

    Full Text Available Bacterial infections involving muscle are quite uncommon and generally require specific predisposing factors. Bacterial myositis is more rarely described in the typical kind of patients observed in Internal Medicine (presence of multiple co-morbidities, partial/limited immune-deficiency, advanced age. Twenty-six patients suffering from bacterial myositis (8 women and 18 men; mean age 58.5 years, range 27-82 observed in a single Internal Medicine Unit were reported. Muscles involved were ileopsoas, thigh, paravertebral, gluteus, calf, forearm and rectus abdomen. Simultaneous presence of arthritis was registered in 17 patients and all patients presented relevant comorbidity. Main cultured bacteria were Staphylococcus aureus, Escherichia coli, other Gram-negative bacteria, Streptococcus spp. Multi-drug-resistance was observed in 14 out 26 (53.8%. Computed tomography, ultrasound and magnetic resonance imaging were utilized for diagnostic purposes. Antibiotic treatment was administered to all patients. Surgical debridement and drainage were performed in 12 patients; 7 patients were treated with percutaneous aspiration and drainage. At discharge, relevant functional impairment was present in 17 patients (65.3%. Four patients died (in-hospital mortality 7.6%, global mortality at three months 15.3%. Management of bacterial myositis is difficult and its prognosis is poor. In the near future, this demanding infection will be more frequently observed in Internal Medicine setting as comorbidity, which is very often the main characteristic of these patients.

  1. Dynamic exophthalmos and lateral strabismus in a dog caused by masticatory muscle myositis.

    Science.gov (United States)

    Czerwinski, Sarah L; Plummer, Caryn E; Greenberg, Shari M; Craft, William F; Conway, Julia A; Perez, Mayrim L; Cooke, Kirsten L; Winter, Matthew D

    2015-11-01

    A 5.5-year-old neutered male mixed-breed dog was presented for evaluation of a 5-month history of deviation of the right globe upon opening the mouth and a 1-month history of conjunctivitis in the same eye. Clinical findings, diagnostic imaging results, treatment and follow-up are described. When the mouth was opened, the right globe deviated rostrally and laterally. There was no pain or resistance to opening the mouth, or resistance to retropulsion of the globe. No other abnormalities were observed. Computed tomography was performed, revealing a contrast-enhancing region associated with the right masseter muscle impinging into the right orbit; this was more pronounced with the mouth open. Cytology revealed a small number of mildly to moderately atypical mesenchymal cells. Histopathology was consistent with polyphasic myositis, with a predominance of lymphocytes and plasma cells. No infectious agents were identified. Serum antibody titers for Toxoplasma gondii and Neospora caninum were negative. Serum titers for 2 M antibody were positive at 1:500, consistent with a diagnosis of masticatory muscle myositis. Therapy with prednisone was initiated. During a follow-up period of 5 months, there was no recurrence of clinical signs, and the dose of prednisone was reduced by 25%. To the authors' knowledge, this is the first reported case of masticatory muscle myositis causing dynamic exophthalmos and strabismus in a dog. © 2015 American College of Veterinary Ophthalmologists.

  2. The Immune Response and the Pathogenesis of Idiopathic Inflammatory Myositis: a Critical Review.

    Science.gov (United States)

    Ceribelli, Angela; De Santis, Maria; Isailovic, Natasa; Gershwin, M Eric; Selmi, Carlo

    2017-02-01

    The pathogenesis of idiopathic inflammatory myositis (IIMs, including polymyositis and dermatomyositis) remains largely enigmatic, despite advances in the study of the role played by innate immunity, adaptive immunity, genetic predisposition, and environmental factors in an orchestrated response. Several factors are involved in the inflammatory state that characterizes the different forms of IIMs which share features and mechanisms but are clearly different with respect to the involved sites and characteristics of the inflammation. Cellular and non-cellular mechanisms of both the immune and non-immune systems have been identified as key regulators of inflammation in polymyositis/dermatomyositis, particularly at different stages of disease, leading to the fibrotic state that characterizes the end stage. Among these, a special role is played by an interferon signature and complement cascade with different mechanisms in polymyositis and dermatomyositis; these differences can be identified also histologically in muscle biopsies. Numerous cellular components of the adaptive and innate immune response are present in the site of tissue inflammation, and the complexity of idiopathic inflammatory myositis is further supported by the involvement of non-immune mechanisms such as hypoxia and autophagy. The aim of this comprehensive review is to describe the major pathogenic mechanisms involved in the onset of idiopathic inflammatory myositis and to report on the major working hypothesis with therapeutic implications.

  3. Secreted histidyl-tRNA synthetase splice variants elaborate major epitopes for autoantibodies in inflammatory myositis.

    Science.gov (United States)

    Zhou, Jie J; Wang, Feng; Xu, Zhiwen; Lo, Wing-Sze; Lau, Ching-Fun; Chiang, Kyle P; Nangle, Leslie A; Ashlock, Melissa A; Mendlein, John D; Yang, Xiang-Lei; Zhang, Mingjie; Schimmel, Paul

    2014-07-11

    Inflammatory and debilitating myositis and interstitial lung disease are commonly associated with autoantibodies (anti-Jo-1 antibodies) to cytoplasmic histidyl-tRNA synthetase (HisRS). Anti-Jo-1 antibodies from different disease-afflicted patients react mostly with spatially separated epitopes in the three-dimensional structure of human HisRS. We noted that two HisRS splice variants (SVs) include these spatially separated regions, but each SV lacks the HisRS catalytic domain. Despite the large deletions, the two SVs cross-react with a substantial population of anti-Jo-l antibodies from myositis patients. Moreover, expression of at least one of the SVs is up-regulated in dermatomyositis patients, and cell-based experiments show that both SVs and HisRS can be secreted. We suggest that, in patients with inflammatory myositis, anti-Jo-1 antibodies may have extracellular activity. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.

  4. Rheumatoid myositis leading to acute lower extremity compartment syndrome: a case-based review.

    Science.gov (United States)

    Jo, Daniel; Pompa, Tiffany; Khalil, Ambreen; Kong, Frank; Wetz, Robert; Goldstein, Mark

    2015-10-01

    Muscle pain and weakness in a rheumatoid arthritis (RA) patient has a broad differential, and myositis should be considered early in the disease course as serious limb and life-threatening sequelae may occur. A 55-year-old woman with a past medical history of methotrexate-controlled RA presented with right leg pain for 4 days. The patient suffered sensory loss in the right foot and decreased strength in the toes. Lab tests revealed elevated creatine kinase, ESR, and anti-rheumatoid factor antibody titers. CT scan revealed myositis of posterior compartment muscles. Progressive edema, pain, and neuromuscular deficits persisted despite steroid and antibiotic therapy, so the patient was taken for urgent fasciotomy for acute compartment syndrome. The muscle biopsy showed diffuse mononuclear cell infiltration as well as perivascular and perineural involvement consistent with rheumatoid myositis (RM). The patient did well post-op on a prednisone taper. This case underlines the systemic nature of RA and exemplifies the severity of inflammation that may lead to grave consequences such as compartment syndrome. The histopathology is diagnostic when there is evidence of mononuclear cell infiltration; however, this is not entirely specific. Early, aggressive therapy with immunosuppressives is warranted in such patients. RM has not, to our knowledge, been recorded to cause acute compartment syndrome. Clinicians should be aware of this uncommon manifestation of RA keeping the various presentations of rheumatoid disease in mind when faced with these patients.

  5. Cervicocephalic kinesthetic sensibility and postural balance in patients with nontraumatic chronic neck pain – a pilot study

    Science.gov (United States)

    Palmgren, Per J; Andreasson, Daniel; Eriksson, Magnus; Hägglund, Andreas

    2009-01-01

    Background Although cervical pain is widespread, most victims are only mildly and occasionally affected. A minority, however, suffer chronic pain and/or functional impairments. Although there is abundant literature regarding nontraumatic neck pain, little focuses on diagnostic criteria. During the last decade, research on neck pain has been designed to evaluate underlying pathophysiological mechanisms, without noteworthy success. Independent researchers have investigated postural balance and cervicocephalic kinesthetic sensibility among patients with chronic neck pain, and have (in most cases) concluded the source of the problem is a reduced ability in the neck's proprioceptive system. Here, we investigated cervicocephalic kinesthetic sensibility and postural balance among patients with nontraumatic chronic neck pain. Methods Ours was a two-group, observational pilot study of patients with complaints of continuous neck pain during the 3 months prior to recruitment. Thirteen patients with chronic neck pain of nontraumatic origin were recruited from an institutional outpatient clinic. Sixteen healthy persons were recruited as a control group. Cervicocephalic kinesthetic sensibility was assessed by exploring head repositioning accuracy and postural balance was measured with computerized static posturography. Results Parameters of cervicocephalic kinesthetic sensibility were not reduced. However, in one of six test movements (flexion), global repositioning errors were significantly larger in the experimental group than in the control group (p < .05). Measurements did not demonstrate any general impaired postural balance, and varied substantially among participants in both groups. Conclusion In patients with nontraumatic chronic neck pain, we found statistically significant global repositioning errors in only one of six test movements. In this cohort, we found no evidence of impaired postural balance. Head repositioning accuracy and computerized static posturography are

  6. Cervicocephalic kinesthetic sensibility and postural balance in patients with nontraumatic chronic neck pain--a pilot study.

    Science.gov (United States)

    Palmgren, Per J; Andreasson, Daniel; Eriksson, Magnus; Hägglund, Andreas

    2009-06-30

    Although cervical pain is widespread, most victims are only mildly and occasionally affected. A minority, however, suffer chronic pain and/or functional impairments. Although there is abundant literature regarding nontraumatic neck pain, little focuses on diagnostic criteria. During the last decade, research on neck pain has been designed to evaluate underlying pathophysiological mechanisms, without noteworthy success. Independent researchers have investigated postural balance and cervicocephalic kinesthetic sensibility among patients with chronic neck pain, and have (in most cases) concluded the source of the problem is a reduced ability in the neck's proprioceptive system. Here, we investigated cervicocephalic kinesthetic sensibility and postural balance among patients with nontraumatic chronic neck pain. Ours was a two-group, observational pilot study of patients with complaints of continuous neck pain during the 3 months prior to recruitment. Thirteen patients with chronic neck pain of nontraumatic origin were recruited from an institutional outpatient clinic. Sixteen healthy persons were recruited as a control group. Cervicocephalic kinesthetic sensibility was assessed by exploring head repositioning accuracy and postural balance was measured with computerized static posturography. Parameters of cervicocephalic kinesthetic sensibility were not reduced. However, in one of six test movements (flexion), global repositioning errors were significantly larger in the experimental group than in the control group (p < .05). Measurements did not demonstrate any general impaired postural balance, and varied substantially among participants in both groups. In patients with nontraumatic chronic neck pain, we found statistically significant global repositioning errors in only one of six test movements. In this cohort, we found no evidence of impaired postural balance.Head repositioning accuracy and computerized static posturography are imperfect measures of functional

  7. Association of non-traumatic complex regional pain syndrome with adenocarcinoma lung on 99mTc-MDP bone scan

    International Nuclear Information System (INIS)

    Damle, Nishikant A.; Tripathi, Madhavi; Singhal, Abhinav; Bal, Chandrasekhar; Praveen Kumar; Kandasamy, Devasenathipathi; Jana, Manisha

    2012-01-01

    Complex regional pain syndrome (CRPS) is usually associated with trauma. Rarely, it may be seen in association with malignancies. We present here the bone scan and X-ray findings in the case of a 56-year-male-patient with adenocarcinoma lung who also had non-traumatic CRPS without involvement of the stellate ganglion. The case highlights the fact that spontaneous development of reflex sympathetic dystrophy may be associated with a neoplastic etiology. (author)

  8. A cementless, proximally fixed anatomic femoral stem induces high micromotion with nontraumatic femoral avascular necrosis: A finite element study

    Directory of Open Access Journals (Sweden)

    Wen-Chuan Chen

    2014-07-01

    Full Text Available Decrease in bone mineral density of metaphysis in patients with nontraumatic avascular necrosis of the femoral head (AVN is considered the main factor leading to aseptic loosening of the femoral component. Researchers have hypothesized that a cementless, anatomic stem fixed proximally to the metaphysis has a higher risk for aseptic loosening than a straight stem that is fixed at the diaphysis in patients with nontraumatic AVN. The purpose of the current study was to evaluate the effects of cancellous bone stiffness at the metaphysis and stem geometry on the micromotion of the femoral stem relative to the femur. The VerSys (straight and ABG (anatomic femoral stems were enrolled in this finite element study to determine the performance of prosthetic micromotion. The simulated load to the hip joint during heel strike was assigned. Results showed that the VerSys model represented better resistance in micromotion between the bone/stem interface than the ABG model in either normal or poor cancellous bone stiffness at the metaphysis. The bone quality at the metaphysis of patients with nontraumatic AVN should be considered prior to selecting a femoral stem. In consideration of initial stability, acementless, straight stem that fits the isthmus is more favourable than an anatomic stem that is fixed to the proximal area of the canal.

  9. Utilization of Hospital Emergency Departments for non-traumatic dental care in New Hampshire, 2001-2008.

    Science.gov (United States)

    Anderson, Ludmila; Cherala, Sai; Traore, Elizabeth; Martin, Nancy R

    2011-08-01

    Hospital Emergency Departments (ED) provide a variety of medical care, some of which is for non-urgent, chronic conditions. We describe the statewide use of hospital ED for selected non-traumatic dental conditions that occurred during 2001-2008 in New Hampshire. Using the administrative hospital discharge dataset for 2001-2007, and provisional 2008 data, we identified all visits for selected dental conditions and calculated age-adjusted rates per 10,000 New Hampshire residents by several socio-demographic characteristics. The Spearman correlation coefficient was used to assess the statistical significance for trend over time. Emergency department visits for non-traumatic dental conditions increased significantly from 11,067 in 2001 to 16,238 visits in 2007 (P dental care users. The most frequent dental complains (46%) were diseases of the teeth and supporting structures, diagnostic code ICD-9-CM-525. Dental care associated ED visits have increased in New Hampshire. Individuals seeking dental treatment in ED are not receiving definitive treatment, and they misuse limited resources. Future studies need to determine the specific barriers to timely and effective dental care in dental offices. Ongoing consistent monitoring of ED use for non-traumatic dental conditions is essential.

  10. Experimental myositis inducible with transfer of dendritic cells presenting a skeletal muscle C protein-derived CD8 epitope peptide.

    Science.gov (United States)

    Okiyama, Naoko; Hasegawa, Hisanori; Oida, Takatoku; Hirata, Shinya; Yokozeki, Hiroo; Fujimoto, Manabu; Miyasaka, Nobuyuki; Kohsaka, Hitoshi

    2015-07-01

    It is suggested that polymyositis, an autoimmune inflammatory myopathy, is mediated by autoaggressive CD8 T cells. Skeletal muscle C protein is a self-antigen that induces C protein-induced myositis, a murine model of polymyositis. To establish a new murine model of myositis inducible with a single CD8 T-cell epitope peptide that derives from the C protein, three internet-based prediction systems were employed to identify 24 candidate peptides of the immunogenic fragment of the C protein and bind theoretically to major histocompatibility complex class I molecules of C57BL/6 (B6) mice. RMA-S cell assay revealed that a HILIYSDV peptide, amino acid position 399-406 of the C protein, had the highest affinity to the H2-K(b) molecules. Transfer of mature bone marrow-derived dendritic cells pulsed with HILIYSDV induced myositis in naive B6 mice. This myositis was suppressed by anti-CD8-depleting antibodies but not by anti-CD4-depleting antibodies. Because this myositis model is mediated by CD8 T cells independently of CD4 T cells, it should be a useful tool to investigate pathology of polymyositis and develop therapies targeting CD8 T cells. © The Japanese Society for Immunology. 2015. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  11. Resilience and vulnerability among refugee children of traumatized and non-traumatized parents

    Directory of Open Access Journals (Sweden)

    Daud Atia

    2008-03-01

    Full Text Available Abstract Background The aim of the study was to explore resilience among refugee children whose parents had been traumatized and were suffering from Post-Traumatic Stress Disorder (PTSD. Methods The study comprised 80 refugee children (40 boys and 40 girls, age range 6–17 yrs, divided into two groups. The test group consisted of 40 refugee children whose parents had been tortured in Iraq before coming to Sweden. In accordance with DSM-IV criteria, these children were further divided in two sub-groups, those who were assessed as having PTSD-related symptoms (n = 31 and those who did not have PTSD-related symptoms (n = 9. The comparison group consisted of 40 children from Egypt, Syria and Morocco whose parents had not been tortured. Wechsler Intelligence Scale for Children, 3rd edn. (WISC-III, Diagnostic Interview for Children and Adolescents- Revised (DICA-R, Post-Traumatic Stress Symptoms checklist (PTSS, "I Think I am" (ITIA and Strengths and Difficulties Questionnaire (SDQ were used to assess IQ; PTSD-related symptoms; self-esteem; possible resilience and vulnerability. Results Children without PTSD/PTSS in the traumatized parents group had more favorable values (ITIA and SDQ with respect to total scores, emotionality, relation to family, peer relations and prosocial behavior than the children in the same group with PTSD/PTSS and these values were similar to those the children in the comparison group (the non-traumatized parents group. The children in the non-traumatized parents group scored significantly higher on the IQ test than the children with traumatized parents, both the children with PTSD-related symptoms and those without PTSD-related symptoms. Conclusion Adequate emotional expression, supportive family relations, good peer relations, and prosociality constituted the main indicators of resilience. Further investigation is needed to explore the possible effects of these factors and the effects of IQ. The findings of this study are

  12. Radiographic and CT findings in a case of fibro-dysplasia ossificans progressiva; Aspect en imagerie d`un cas de fibrodysplasie ossifiante progressive

    Energy Technology Data Exchange (ETDEWEB)

    Haddad, S.; Menassa, L.; Slaba, S.; Atallah, N. [Service de Radiologie, Hotel-Dieu de France, Beyrouth, (Lebanon); Nasnas, R.; Abadjian, G. [Hotel-Dieu de France (France)

    1997-06-01

    Fibro-dysplasia ossificans progressiva is a rare congenital disease that affects children under the age of five years. Soft tissue swelling of the cervical and dorsal regions with local pain, warmth and low grade fever are the early clinical manifestations, usually associated with hallux valgus and microdactyly of the fingers and toes. Calcifications of the fascias and muscles cause muscular contractures leading to progressive disability and restrictive lung disease. In the early stage, CT shows edema of the soft tissues and later on, calcifications of muscular fascia. The association of these radiographic and CT findings is specific and should avoid muscular biopsies which are known to be an aggravating factor in this disease. (author) 11 refs.

  13. Non-traumatic cortical subarachnoid haemorrhage: diagnostic work-up and aetiological background

    Energy Technology Data Exchange (ETDEWEB)

    Spitzer, C.; Kosinski, C.M. [University Hospital of RWTH Aachen, Department of Neurology, Aachen (Germany); Mull, M. [University Hospital of RWTH Aachen, Department of Neuroradiology, Aachen (Germany); Rohde, V. [University Hospital of RWTH Aachen, Department of Neurosurgery, Aachen (Germany)

    2005-07-01

    Only 15% of all subarachnoid haemorrhages (SAHs) are not of aneurysmal origin. Among those, circumscribed SAHs along the cortical convexity are rare and have only been described in singular case reports so far. Here, we present a collection of 12 cases of SAH along the convexity, of non-traumatic origin. Over a period of 10 years, 12 cases of circumscribed SAH along the convexity were identified at our clinic. The clinical presentations, neuroradiological SAH characteristics, further diagnostic work-up to identify the underlying aetiologies, the therapy and clinical outcome were analysed. The patients' chief complaints were unspecific cephalgia, focal or generalised seizures and focal neurological deficits. Typical signs of basal SAH, such as nuchal rigidity, thunderclap-headache or alteration of consciousness, were rare. Magnetic resonance imaging (MRI) and digital subtraction angiography (DSA) revealed different aetiologies, namely postpartal posterior encephalopathy (three), cerebral vasculitis (two), dural sinus thrombosis (two), cortical venous thrombosis (one), intracerebral abscesses (one) and cerebral cavernoma (one). Two cases remained unresolved. Treatment of the underlying disease and symptomatic medication led to good clinical outcome in almost all cases. On the basis of these findings, we demonstrate that the clinical presentation, localisation and aetiology of cortical SAH differ clearly from other SAHs. A diagnostic work-up with MRI and eventually DSA is essential. Mostly, the causative disease can be identified, and specific treatment allows a favourable outcome. (orig.)

  14. Challenging non-traumatic posterior urethral strictures treated with urethroplasty: a preliminary report

    Directory of Open Access Journals (Sweden)

    Nicolaas Lumen

    2009-08-01

    Full Text Available Introduction: Posterior urethral strictures after prostatic radiotherapy or surgery for benign prostatic hyperplasia (BPH refractory to minimal invasive procedures (dilation and/or endoscopic urethrotomy are challenging to treat. Published reports of alternative curative management are extremely rare. This is a preliminary report on the treatment of these difficult strictures by urethroplasty. Materials and Methods: Seven cases were treated: 4 cases occurred after open prostatectomy or transurethral resection of the prostate for BPH, one case after external beam irradiation and 2 after brachytherapy. The 4 cases after BPH-related surgery were in fact complete obstructions at the bladder neck and the membranous urethra with the prostatic urethra still partially patent. Anastomotic repair by perineal route was done in all cases with bladder neck incision in the BPH-cases and prostatic apex resection in the radiotherapy cases. Results: Mean follow-up was 31 months (range: 12-72 months. The operation was successful, with preserved continence, in 3 of the 4 BPH-cases and in 2 of the 3 radiotherapy cases. An endoscopic incision was able to treat a short re-stricture in the BPH-patient and a longer stricture at the bulbar urethra could be managed with a perineostomy in the radiotherapy-patient. Conclusion: Posterior non-traumatic strictures refractory to minimal invasive procedures (dilation/endoscopic urethrotomy can be treated by urethroplasty using an anastomotic repair with a bladder neck incision if necessary.

  15. A Rare Case of Spontaneous Pneumocephalus Associated with Nontraumatic Cerebrospinal Fluid Leak

    Directory of Open Access Journals (Sweden)

    Murad Baba

    2016-01-01

    Full Text Available Introduction. Spontaneous nontraumatic pneumocephalus (PNC and cerebrospinal fluid (CSF leaks are both very uncommon conditions. We report a rare case of spontaneous pneumocephalus associated with CSF leak secondary to right sphenoid sinus bony defect without history of trauma. Case Description. 51-year-old Hispanic female with past medical history of hypertension and idiopathic intracranial hypertension (Pseudotumor Cerebri presented to the emergency room complaining of headache and clear discharge from the right nostril. Physical examination was significant for right frontal sinus tenderness and clear discharge from right nostril. Computed Tomography (CT scan of the brain showed moderate amount of extra-axial air within the right cerebral hemisphere indicative of pneumocephalus. CT scan of facial bones showed bony defect along the right sphenoid sinus with abnormal CSF collection. The patient was started on intravenous antibiotics for meningitis prophylaxis and subsequently underwent transsphenoidal repair of cerebrospinal fluid leak with abdominal fat graft. CSF rhinorrhea stopped completely after the surgery with near complete resolution of pneumocephalus before discharge. Conclusions. Early identification of pneumocephalus and surgical intervention can help decrease the morbidity and avoid possible complications. Idiopathic intracranial hypertension, although rare, can lead to CSF leak and pneumocepahlus.

  16. Very low survival rates after non-traumatic lower limb amputation in a consecutive series

    DEFF Research Database (Denmark)

    Kristensen, Morten Tange; Holm, Gitte; Kirketerp-Møller, Klaus

    2012-01-01

    The aim of this retrospective study was to evaluate factors potentially influencing short- and long-term mortality in patients who had a non-traumatic lower limb amputation in a university hospital. A consecutive series of 93 amputations (16% toe/foot, 33% trans-tibial, 9% through knee and 42......% trans-femoral) were studied. Their mean age was 75.8 years; 21 (23%) were admitted from a nursing home and 87 (92%) were amputated due to a vascular disease and/or diabetes. Thirty days and 1-year mortality were 30 and 54%, respectively. Cox regression analysis demonstrated that the 30-day mortality...... was associated with older age (P = 0.01), and the number of co-morbidities (P = 0.04), when adjusted for gender, previous amputations, cause of and amputation level, and residential status. Thus, a patient with 4 or 5 co-morbidities (n = 20) was seven times more likely to die within 30 days, compared...

  17. [A case of non-traumatic tension pneumocephalus 12 years after initial ventriculo-peritoneal shunt].

    Science.gov (United States)

    Idei, Masaru; Yamane, Kanji; Okita, Shinji; Kumano, Kiyoshi; Nakae, Ryuta; Nishizawa, Shigeru

    2009-10-01

    The authors presented a patient with non-traumatic tension pneumocephalus 12 years after initial ventriculo-peritoneal shunt. A 60-year-old man had a past history of subarachnoid hemorrhage. At ÿtime, he underwent clipping surgery and emplacement of a ventriculoperitoneal shunt with a low pressure valve, and was discharged from hospital in a bedridden condition. After 12 years, he was transferred to our hospital because of dysarthria and right hemiparesis. On admission, he had multiple dimples of the scalp in the left frontal area where craniotomy had been carried out 12 years before. Head CT scan revealed air located mainly in bilateral subdural space and collapsed ventricles. We suspected that he had a tension pneumocephalus through a scalp-subdural fistula induced by long-term low intracranial pressure. Operation for closure of the fistula was intended. During the operation a subdural abscess was found so, artificial bone and the shunt system was removed. We were able to detect the fistula from the defect of the scalp to the subdural space and closed it by repairing a scalp defect, using dural plasty. Postoperative clinical course was uneventful and the patient was discharged. About 6 month after the operation, cranioplasty and lumbo-peritoneal shunt was performed. We must recognize that appropriate management of intracranial pressure after installation of a ventriculo-peritoneal shunt is necessary for prevention of tension pneumocephalus.

  18. Increased energy expenditure and glucose oxidation during acute nontraumatic skin pain in humans.

    Science.gov (United States)

    Holland-Fischer, Peter; Greisen, Jacob; Grøfte, Thorbjørn; Jensen, Troels S; Hansen, Peter O; Vilstrup, Hendrik

    2009-04-01

    Tissue injury is accompanied by pain and results in increased energy expenditure, which may promote catabolism. The extent to which pain contributes to this sequence of events is not known. In a cross-over design, 10 healthy volunteers were examined on three occasions; first, during self-controlled nontraumatic electrical painful stimulus to the abdominal skin, maintaining an intensity of 8 on the visual analogue scale (0-10). Next, the electrical stimulus was reproduced during local analgesia and, finally, there was a control session without stimulus. Indirect calorimetry and blood and urine sampling was done in order to calculate energy expenditure and substrate utilization. During pain stimulus, energy expenditure increased acutely and reversibly by 62% (95% confidence interval, 43-83), which was abolished by local analgesia. Energy expenditure paralleled both heart rate and blood catecholamine levels. The energy expenditure increase was fuelled by all energy sources, with the largest increase in glucose utilization. The pain-related increase in energy expenditure was possibly mediated by adrenergic activity and was probably to a large extent due to increased muscle tone. These effects may be enhanced by cortical events related to the pain. The increase in glucose consumption favours catabolism. Our findings emphasize the clinical importance of pain management.

  19. Non-traumatic acute epidural spinal hematomas diagnosed by magnetic resonance

    International Nuclear Information System (INIS)

    Rovira, A.; Grive, E.; Pedraza, S.; Capellades, J.; Nos, C.; Alarcon, M.; Rovira, A.

    2000-01-01

    The non-traumatic spinal epidural hematoma (NTSEH) is a rare entity that can be the cause of an acute spinal compression syndrome. the objective of this review is to identify the characteristics by MRI and NTSEH and to analyze the factors that influence in its prognosis. In the years 1994 and 1999, 12 patients with NTSEH have been diagnosed in our hospital, and a MRI was performed during the acute phase. the characteristics of the lesions have been analyzed by MRI, with special emphasis on the topographic data and resonance signal and the factors that can influence in the clinical prognosis of the patients. Initially, all of the patients presented pain in the cervical dorsal or interscapular site, followed by a sensitive-motor deficit picture. The MRI showed a lesion of expansive character and posterior epidural location in every case that would produce varying degrees of compression on the spinal cord. The NTSEH should be considered as one of the causes of acute spinal cord compression. The clinical association of intense cervical, dorsal or interscapular pain followed by a sensomotor deficit picture should lead to the suspicion of this entity, that would require an immediate examination with MRI to verify its diagnosis. Both the clinical manifestations as well as the characteristics observed by MRI of the NTSEH have a prognostic value and determine the therapeutic decision. (Author) 34 refs

  20. A case of non-specific interstitial pneumonia with recurrent gastric carcinoma and anti-Jo-1 antibody positive myositis.

    Science.gov (United States)

    Ebisutani, Chikara; Ito, Isao; Kitaichi, Masanori; Tanabe, Naoya; Mishima, Michiaki; Kadowaki, Seizo

    2016-07-01

    We report the first case of non-specific interstitial pneumonia (NSIP) in a patient with cancer-associated myositis (CAM) that emerged along with the recurrence of the cancer. A 60-year-old woman, with a history of partial gastrectomy for gastric cancer 11 years ago, presented with exertional dyspnea with anti-Jo-1 antibody-positive myositis. Surgical lung biopsy showed NSIP with metastatic gastric cancer. Accordingly, her condition was diagnosed as CAM with cancer recurrence. In patients with a history of cancer, development of myositis may indicate cancer recurrence; therefore, careful observation would be necessary. Copyright © 2016 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  1. Outcome and prognostic factors in a French cohort of patients with myositis-associated interstitial lung disease.

    Science.gov (United States)

    Obert, Julie; Freynet, Olivia; Nunes, Hilario; Brillet, Pierre-Yves; Miyara, Makoto; Dhote, Robin; Valeyre, Dominique; Naccache, Jean-Marc

    2016-12-01

    Interstitial lung disease (ILD) is a common form of extramuscular involvement in patients with polymyositis/dermatomyositis and is associated with poor prognosis. This study was designed to describe the long-term outcome of myositis-associated ILD. This retrospective observational study was conducted in 48 consecutive patients. Two groups defined according to outcome were compared to determine prognostic factors: a "severe" group (vital capacity [VC] myositis-associated ILD had severe initial PFT results but a low mortality rate. Independent prognostic factors at presentation were initial VC and myopathic changes on electromyography. This study highlights the need for studies focusing on the correlation between muscle and lung pathogenic mechanisms.

  2. A case of all-trans retinoic acid-induced myositis in the treatment of acute promyelocytic leukaemia.

    Science.gov (United States)

    Chan, K H; Yuen, S L S; Joshua, D

    2005-12-01

    The use of all-trans retinoic acid (ATRA) is now standard therapy for the treatment of acute promyelocytic leukaemia (APML). There have been increasing reports of ATRA-induced myositis, with its frequent association with retinoic acid syndrome and Sweet's syndrome. We report a case of a young man with APML who developed ATRA-induced myositis characterized by unexplained fevers, bilateral leg swelling and a non-painful purpuric, petechial rash, with prompt resolution of symptoms and signs with high-dose steroids and cessation of ATRA. Rapid recognition of this adverse reaction and prompt institution of steroids is of prime importance given its potentially fatal course.

  3. Myositis-specific autoantibodies and their association with malignancy in Italian patients with polymyositis and dermatomyositis.

    Science.gov (United States)

    Ceribelli, Angela; Isailovic, Natasa; De Santis, Maria; Generali, Elena; Fredi, Micaela; Cavazzana, Ilaria; Franceschini, Franco; Cantarini, Luca; Satoh, Minoru; Selmi, Carlo

    2017-02-01

    This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis. In six dermatomyositis cases, we found five solid forms of cancer and one Hodgkin's lymphoma in long-term remission. Among patients with cancer-associated dermatomyositis, three were positive for anti-TIF1γ/α, two for anti-Mi-2, and one for anti-MJ/NXP-2. The strongest positivity of anti-TIF1γ was seen in two active forms of cancer, and this antibody was either negative or positive at low titers in the absence of cancer or in the 7-year remission Hodgkin's lymphoma. Four out of twenty (20 %) patients with polymyositis had solid cancer, but no specific association with autoantibodies was identified; further, none of the four cases of antisynthetase syndrome had a history of cancer. No serum myositis-associated autoantibody was observed in control sera, resulting in positive predictive value 75 %, negative predictive value 78.5 %, sensitivity 50 %, specificity 92 %, and area under the ROC curve 0.7083 for the risk of paraneoplastic DM in anti-TIF1γ/α (+) patients. Myositis-specific autoantibodies can be identified thanks to the use of immunoprecipitation, and their association with cancer is particularly clear for anti-TIF1γ/α in dermatomyositis. This association should be evaluated in a prospective study by immunoprecipitation in clinical practice.

  4. Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

    Science.gov (United States)

    Hida, Ayumi; Yamashita, Takenari; Hosono, Yuji; Inoue, Manami; Kaida, Kenichi; Kadoya, Masato; Miwa, Yusuke; Yajima, Nobuyuki; Maezawa, Reika; Arai, Satoko; Kurasawa, Kazuhiro; Ito, Kazuhiro; Shimada, Hiroyuki; Iwanami, Tomoko; Sonoo, Masahiro; Hatanaka, Yuki; Murayama, Shigeo; Uchibori, Ayumi; Chiba, Atsuro; Aizawa, Hitoshi; Momoo, Takayuki; Nakae, Yoshiharu; Sakurai, Yasuhisa; Shiio, Yasushi; Hashida, Hideji; Yoshizawa, Toshihiro; Sakiyama, Yoshio; Oda, Aya; Inoue, Kiyoharu; Takeuchi, Sousuke; Iwata, Nobue K; Date, Hidetoshi; Masuda, Naoki; Mikata, Takashi; Motoyoshi, Yasufumi; Uesaka, Yoshikazu; Maeda, Meiko Hashimoto; Nakashima, Ran; Tsuji, Shoji; Kwak, Shin; Mimori, Tsuneyo; Shimizu, Jun

    2016-07-19

    We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association. We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis. Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs. CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM. © 2016 American Academy of Neurology.

  5. A Unique Case of Muscle Invasive Metastatic Breast Cancer Mimicking Myositis

    Science.gov (United States)

    2017-06-28

    TYPE 08/ 03/20 17 Publ ication/Journal 4. TITLE AND SUBTITLE A unique case of muscle-invasive metastatic breast cancer mimicking myositis 6...Rev. 8/98) Prescnbed by ANSI Std Z39. 18 Adobe Profes11on11 7.0 Title: A Unique Case of M uscle-Invasive Metastatic Breast Cancer M imicking...an 84-year-old female who presented with neck swelling and upper airway obstruction due to metastatic breast cancer invading the sternocleidomastoid

  6. Proliferative myositis of the latissimus dorsi presenting in a 20-year-old male athlete

    LENUS (Irish Health Repository)

    Mc Hugh, N

    2017-08-01

    We describe the case of a 20-year-old rower presenting with an uncommon condition of Proliferative Myositis (PM) affecting the Latissimus Dorsi (LD). PM is a rare, benign tumour infrequently developing in the upper back. Its rapid growth and firm consistency may mistake it for sarcoma at presentation. Therefore, careful multidisciplinary work-up is crucial, and should involve appropriate radiological and histopathological investigations. Here, we propose the aetiology of LD PM to be persistent myotrauma induced by repetitive rowing motions. Symptoms and rate of progression ultimately determine the management which includes surveillance and\\/or conservative resection. There have been no documented cases of recurrence or malignant transformation.

  7. International Spinal Cord Injury Data Sets for non-traumatic spinal cord injury.

    Science.gov (United States)

    New, P W; Marshall, R

    2014-02-01

    Multifaceted: extensive discussions at workshop and conference presentations, survey of experts and feedback. Present the background, purpose and development of the International Spinal Cord Injury (SCI) Data Sets for Non-Traumatic SCI (NTSCI), including a hierarchical classification of aetiology. International. Consultation via e-mail, presentations and discussions at ISCoS conferences (2006-2009), and workshop (1 September 2008). The consultation processes aimed to: (1) clarify aspects of the classification structure, (2) determine placement of certain aetiologies and identify important missing causes of NTSCI and (3) resolve coding issues and refine definitions. Every effort was made to consider feedback and suggestions from participants. The International Data Sets for NTSCI includes basic and an extended versions. The extended data set includes a two-axis classification system for the causes of NTSCI. Axis 1 consists of a five-level, two-tier (congenital-genetic and acquired) hierarchy that allows for increasing detail to specify the aetiology. Axis 2 uses the International Statistical Classification of Diseases (ICD) and Related Health Problems for coding the initiating diseases(s) that may have triggered the events that resulted in the axis 1 diagnosis, where appropriate. Additional items cover the timeframe of onset of NTSCI symptoms and presence of iatrogenicity. Complete instructions for data collection, data sheet and training cases are available at the websites of ISCoS (http://www.iscos.org.uk) and ASIA (http://www.asia-spinalinjury.org). The data sets should facilitate comparative research involving NTSCI participants, especially epidemiological studies and prevention projects. Further work is anticipated to refine the data sets, particularly regarding iatrogenicity.

  8. The etiology and outcome of non-traumatic coma in critical care: a systematic review.

    Science.gov (United States)

    Horsting, Marlene Wb B; Franken, Mira D; Meulenbelt, Jan; van Klei, Wilton A; de Lange, Dylan W

    2015-04-29

    Non-traumatic coma (NTC) is a serious condition requiring swift medical or surgical decision making upon arrival at the emergency department. Knowledge of the most frequent etiologies of NTC and associated mortality might improve the management of these patients. Here, we present the results of a systematic literature search on the etiologies and prognosis of NTC. Two reviewers independently performed a systematic literature search in the Pubmed, Embase and Cochrane databases with subsequent reference and citation checking. Inclusion criteria were retrospective or prospective observational studies on NTC, which reported on etiologies and prognostic information of patients admitted to the emergency department or intensive care unit. Eventually, 14 studies with enough data on NTC, were selected for this systematic literature review. The most common causes of NTC were stroke (6-54%), post-anoxic coma (3-42%), poisoning (coma (54-89%) and lowest for poisoning (0-39%) and epilepsy (0-10%). NTC represents a challenge to the emergency and the critical care physicians with an important mortality and moderate-severe disability rate. Even though, included studies were very heterogeneous, the most common causes of NTC are stroke, post anoxic, poisoning and various metabolic etiologies. The best outcome is achieved for patients with poisoning and epilepsy, while the worst outcome was seen in patients with stroke and post-anoxic coma. Adequate knowledge of the most common causes of NTC and prioritizing the causes by mortality ensures a swift and adequate work-up in diagnosis of NTC and may improve outcome.

  9. Angiography in non-traumatic brain haematoma. An analysis of 100 cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Academic Dept. of Radiology; Beveridge, C.J.; Gholkar, A. [Newcastle General Hospital, Newcastle upon Tyne (United Kingdom). Dept. of Neuroradiology

    1997-09-01

    Purpose: The primary purpose of this project was to study the anatomical characteristics of intracerebral haematoma (ICH) in order to determine features that may negate the need for angiography in some patients. Material and Methods: The study was prospective and designed to investigate the underlying cause of non-traumatic ICH in 100 cases assessed by conventional angiography. Patients were excluded if there was a history of trauma or known pre-existing brain abnormality. All patients were examined with CT and angiography within 4 days of the ictus. Results: Ruptured aneurysms or arteriovenous malformations (AVMs) were diagnosed on the initial angiogram in 49% of cases: 27 AVMs and 22 aneurysms. One case of superior sagittal sinus thrombosis was also detected. Vascular abnormalities were found most frequently in the under-40 age group and in cases in which subarachnoid haemorrhage, intraventricular haemorrhage or extracerebral haematoma accompanied the ICH. The temporal lobe was the most frequent anatomical location (37%). When a temporal lobe haematoma extended into the Sylvian fissure from the inferior pole of the temporal lobe or when it was associated with subarachnoid haemorrhage, structural abnormalities were found in over 90% of cases. Conclusions: There are groups of patients with ICH in whom the CT features are highly suggestive of AVM or aneurysm rupture. If the initial angiography is negative in these cases, careful follow up by repeat angiography and/or MR imaging is essential. However, potentially treatable abnormalities cannot be excluded with certainty by the distribution of the haematoma on CT alone, even if there is a history of pre-existing hypertension. (orig.).

  10. Global maps of non-traumatic spinal cord injury epidemiology: towards a living data repository.

    Science.gov (United States)

    New, P W; Cripps, R A; Bonne Lee, B

    2014-02-01

    Literature review. Globally map non-traumatic spinal cord injury (NTSCI) incidence, prevalence, survival, level of injury and aetiology. Propose a research framework for NTSCI prevention and launch a repository of NTSCI data. Initiative of the International Spinal Cord Society Prevention Committee. Literature search of Medline and Embase (1959-June 2011). Relevant articles in any language regarding adults with NTSCI were included. Stratification of information about incidence and prevalence into green/yellow/orange/red data quality 'zones' and comparisons between World Health Organisation (WHO) regions and countries. Three hundred and seventy-seven abstracts reviewed--45 of these from 24 countries in 12 of the 21 WHO global regions had relevant information. Only one publication had survival data. Prevalence data for NTSCI existed for only two countries, India (prevalence of 2,310/million population, Kashmir region) and Canada (prevalence of 1,120/million population). The incidence rates for WHO regions were: Asia Pacific, high income 20/million population/year; Australasia (26/million population/year); Western Europe median of 6/million population/year; North America, high income median 76/million population/year (based on poor-quality studies); and Oceania 9/million population/year. Developed countries tended to have a higher proportion of cases with degenerative conditions and tumours. Developing countries, in comparison, tended to have a higher proportion of infections, particularly tuberculosis and HIV, although a number also reported tumours as a major cause. Insufficient survival, prevalence and incidence data are a predominant finding of this review. The piecemeal approach to epidemiological reporting of NTSCI, particularly failing to include sound regional population denominators, has exhausted its utility. Minimum data collection standards are required.

  11. Risk of contralateral avascular necrosis (AVN) after total hip arthroplasty (THA) for non-traumatic AVN.

    Science.gov (United States)

    Goker, Berna; Block, Joel A

    2006-01-01

    The risk of developing bilateral disease progressing to total hip arthroplasty (THA) among patients who undergo unilateral THA for non-traumatic avascular necrosis (AVN) remains poorly understood. An analysis of the time-course to contralateral THA, as well as the effects of underlying AVN risk factors, is presented. Forty-seven consecutive patients who underwent THA for AVN were evaluated. Peri-operative and annual post-operative antero-posterior pelvis radiographs were examined for evidence of contralateral involvement. Patient age, weight, height, underlying AVN risk factor(s), date of onset of contralateral hip pain if occurred, and date of contralateral THA if performed, were recorded. Bone scan, computerized tomography and magnetic resonance imaging data were utilized when available. Twenty-one patients (46.6%) underwent contralateral THA for AVN within a median of 9 months after the initial THA (range 0-93, interquartile range 28.5 months). The median follow-up for patients without contralateral THA was 75 months (range 3-109, interquartile range 69 months). Thirty-four patients had radiographic findings of contralateral AVN at study entry; 25 were symptomatic bilaterally at entry and 7 developed contralateral symptoms within a mean time of 12 months (median 10 months, interquartile range 12 months). None of the 13 patients who were free of radiographic evidence of contralateral AVN at study entry developed evidence of AVN during the follow-up. AVN associated with glucocorticoid use was more likely to manifest as bilateral disease than either idiopathic AVN or ethanol-associated AVN (P=0.02 and P=0.03 respectively). Radiographically-evident AVN in the contralateral hip at THA is unlikely to remain asymptomatic for a prolonged period of time. Conversely, asymptomatic contralateral hips without radiographic evidence of AVN are unlikely to develop clinically significant AVN.

  12. Angiography in non-traumatic brain haematoma. An analysis of 100 cases

    International Nuclear Information System (INIS)

    Griffiths, P.D.; Beveridge, C.J.; Gholkar, A.

    1997-01-01

    Purpose: The primary purpose of this project was to study the anatomical characteristics of intracerebral haematoma (ICH) in order to determine features that may negate the need for angiography in some patients. Material and Methods: The study was prospective and designed to investigate the underlying cause of non-traumatic ICH in 100 cases assessed by conventional angiography. Patients were excluded if there was a history of trauma or known pre-existing brain abnormality. All patients were examined with CT and angiography within 4 days of the ictus. Results: Ruptured aneurysms or arteriovenous malformations (AVMs) were diagnosed on the initial angiogram in 49% of cases: 27 AVMs and 22 aneurysms. One case of superior sagittal sinus thrombosis was also detected. Vascular abnormalities were found most frequently in the under-40 age group and in cases in which subarachnoid haemorrhage, intraventricular haemorrhage or extracerebral haematoma accompanied the ICH. The temporal lobe was the most frequent anatomical location (37%). When a temporal lobe haematoma extended into the Sylvian fissure from the inferior pole of the temporal lobe or when it was associated with subarachnoid haemorrhage, structural abnormalities were found in over 90% of cases. Conclusions: There are groups of patients with ICH in whom the CT features are highly suggestive of AVM or aneurysm rupture. If the initial angiography is negative in these cases, careful follow up by repeat angiography and/or MR imaging is essential. However, potentially treatable abnormalities cannot be excluded with certainty by the distribution of the haematoma on CT alone, even if there is a history of pre-existing hypertension. (orig.)

  13. Nontraumatic hypotension and shock in the emergency department and the prehospital setting, prevalence, etiology, and mortality: a systematic review.

    Directory of Open Access Journals (Sweden)

    Jon Gitz Holler

    Full Text Available Acute patients presenting with hypotension in the prehospital or emergency department (ED setting are in need of focused management and knowledge of the epidemiology characteristics might help the clinician. The aim of this review was to address prevalence, etiology and mortality of nontraumatic hypotension (SBP ≤ 90 mmHg with or without the presence of shock in the prehospital and ED setting.We performed a systematic literature search up to August 2013, using Medline, Embase, Cinahl, Dare and The Cochrane Library. The analysis and eligibility criteria were documented according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA-guidelines and The Cochrane Collaboration. No restrictions on language, publication date, or status were imposed. We used the Newcastle-Ottawa quality assessment scale (NOS-scale and the Strengthening the Reporting of Observational studies in Epidemiology (STROBE-statement to assess the quality.Six observational studies were considered eligible for analysis based on the evaluation of 11,880 identified papers. Prehospital prevalence of hypotension was 19.5/1000 emergency medicine service (EMS contacts, and the prevalence of hypotensive shock was 9.5-19/1000 EMS contacts with an inhospital mortality of shock between 33 to 52%. ED prevalence of hypotension was 4-13/1000 contacts with a mortality of 12%. Information on mortality, prevalence and etiology of shock in the ED was limited. A meta-analysis was not feasible due to substantial heterogeneity between studies.There is inadequate evidence to establish concise estimates of the characteristics of nontraumatic hypotension and shock in the ED or in the prehospital setting. The available studies suggest that 2% of EMS contacts present with nontraumatic hypotension while 1-2% present with shock. The inhospital mortality of prehospital shock is 33-52%. Prevalence of hypotension in the ED is 1% with an inhospital mortality of 12%. Prevalence

  14. Head posture and neck pain of chronic nontraumatic origin: a comparison between patients and pain-free persons.

    Science.gov (United States)

    Silva, Anabela G; Punt, T David; Sharples, Paul; Vilas-Boas, João P; Johnson, Mark I

    2009-04-01

    To compare standing head posture measurements between patients with nontraumatic neck pain (NP) and pain-free individuals. Single-blind (assessor) cross-sectional study. Hospital and general community. Consecutive patients (n=40) with chronic nontraumatic NP and age- and sex-matched pain-free participants (n=40). Not applicable. Three angular measurements: the angle between C7, the tragus of the ear, and the horizontal; the angle between the tragus of the ear, the eye, and the horizontal; and the angle between the inferior margins of the right and the left ear and the horizontal were calculated through the digitization of video images. NP patients were found to have a significantly smaller angle between C7, the tragus, and the horizontal, resulting in a more forward head posture than pain-free participants (NP, mean +/- SD, 45.4 degrees +/-6.8 degrees ; pain-free, mean +/- SD, 48.6 degrees +/-7.1 degrees ; P50y) revealed an interaction, with a statistically significant difference in head posture for younger participants with NP compared with younger pain-free participants (NP, mean +/- SD, 46.1 degrees +/-6.7 degrees ; pain-free, mean +/- SD, 51.8 degrees +/-5.9 degrees ; P.05; CI for the difference between groups, -4.9 degrees -4.2 degrees ). No other differences were found between patients and pain-free participants. Younger patients with chronic nontraumatic NP were shown to have a more forward head posture in standing than matched pain-free participants. However, the difference, although statistically significant, was perhaps too small to be clinically meaningful.

  15. The CT frequencies of various non-traumatic acute abdominal emergencies in hemodialysis, peritoneal dialysis patients and the general population

    International Nuclear Information System (INIS)

    Ergün, Tarkan; Lakadamyal, Hatice

    2012-01-01

    Purpose: To investigate the frequency of non-traumatic acute abdominal emergencies in end-stage renal disease (ESRD) (peritoneal dialysis (PD) and haemodialysis (HD)) patients and in the general population as diagnosed by computed tomography (CT) imaging. Methods: The abdominal CT findings of ESRD patients with non-traumatic acute abdominal pain during the years 2001–2010 have been retrospectively evaluated. Thirty-three HD (14 females, 19 males, mean age: 62 ± 10.5) and 22 PD patients (12 females, 10 males, mean age: 59 ± 9.4) with acute abdominal pathology based on their CT scans have been included into the study. In addition, 127 individuals (68 females, 59 males, mean age: 40.7 ± 12.8) with normal renal functions who presented with non-traumatic acute abdominal pain diagnosed with an acute abdominal pathology based on their CT scans have been prospectively evaluated during the years 2009–2010. Results: While the most frequent etiology in PD patients was peritonitis (45.4%), acute pancreatitis (13.6%) and perforation (18.1), and in HD patients it was nonocclusive mesenteric ischemia (18.1%) and spontaneous intraabdominal bleeding (21.2%). The basic causes of acute abdomen in the general population were ureteral stone (34.6%) and appendicitis (18.1%). Conclusions: The causes of acute abdominal pain in ESRD patients is significantly different when compared to the general population. And within this special patient population the etiology of acute abdomen differs depending on the renal replacement therapy modality they are receiving. Thus, the causes of acute abdomen in PD patients are mostly peritonitis, acute pancreatitis, and perforation, while being mostly nonocclusive mesenteric ischemia and spontaneous intraabdominal bleeding in patients receiving HD therapy.

  16. An enhanced treatment program with markedly reduced mortality after a transtibial or higher non-traumatic lower extremity amputation

    DEFF Research Database (Denmark)

    Kristensen, Morten T; Holm, Gitte; Krasheninnikoff, Michael

    2016-01-01

    Background and purpose - Historically, high 30-day and 1-year mortality post-amputation rates (> 30% and 50%, respectively) have been reported in patients with a transtibial or higher non-traumatic lower extremity amputation (LEA). We evaluated whether allocating experienced staff and implementing...... adjusted for age, sex, residential and health status, the disease that caused the amputation, and the index amputation level showed that 30-day and 1-year mortality risk was reduced by 52% (HR =0.48, 95% CI: 0.25-0.91) and by 46% (HR =0.54, 95% CI: 0.35-0.86), respectively, in the intervention group...

  17. ''Dropped-head'' syndrome due to isolated myositis of neck extensor muscles: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Gaeta, Michele; Mazziotti, Silvio; Blandino, Alfredo [University of Messina, Department of Radiological Sciences, Messina (Italy); Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna [University of Messina, Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

    2006-02-15

    MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings. (orig.)

  18. Developing standardised treatment for adults with myositis and different phenotypes: an international survey of current prescribing preferences.

    Science.gov (United States)

    Tansley, Sarah; Shaddick, Gavin; Christopher-Stine, Lisa; Sharp, Charlotte; Dourmishev, Lyubomir; Maurer, Britta; Chinoy, Hector; McHugh, Neil

    2016-01-01

    The evidence base for treatment of the idiopathic inflammatory myopathies is extremely limited. The rarity and heterogeneity of these diseases has hampered the development of good quality clinical trials and while a range of immunomodulatory treatments are commonly used in clinical practice, as yet there are no clear guidelines directing their use. We aimed to establish current prescribing regimens used to treat adults with myositis internationally. An electronic survey based on different clinical scenarios was distributed internationally to clinicians involved in the treatment of patients with myositis. Participants were asked to select their first-line treatment preferences in each situation. A multinomial regression analysis was used to assess the influence of clinical scenario, respondent expertise and country of origin on first-line treatment choice. 107 survey responses were received. 57% of respondents considered themselves an expert in myositis and the majority of respondents were rheumatologists although responses from other specialities were also received. Pharmacological treatment with steroids and additional immunotherapy was the preference in most scenarios. First-line immunosuppressant choice was significantly influenced by the clinical scenario, the expertise of the treating physician and country of practice. Azathioprine, methotrexate and mycophenolate mofetil were the most commonly chosen agents. In the absence of available evidence, clinical experience and expert consensus often forms the basis of treatment guidelines. These results suggest that an international consensus approach would be possible in myositis and would overcome an urgent, yet unmet need for patients suffering with this difficult disease.

  19. [Clinical features and therapeutic response of our anti-SRP positive patients with myositis].

    Science.gov (United States)

    Botos, Balázs; Nagy-Vincze, Melinda; Dankó, Katalin

    2017-09-01

    Idiopathic inflammatory myopathies are a group of clinically heterogeneous diseases, which have been classified by myositis specific antibodies recently. The anti-SRP positive subset of this group is characterized by more severe clinical prognosis than other myositis specific antibody positive types. Our goal was to compare 16 anti-SRP positive patients in the Division of Clinical Immunology, Department of Internal Medicine, University of Debrecen with 16 antibody negative ones. Muscle strength validated in both groups by the manual muscle test proved to be significantly decreased both before and after therapy (χ 2 = 0.006 and 0.019) in the anti-SRP positive group. Muscle-specific inflammatory laboratory parameters showed significant difference only in case of LDH-levels after therapy. Both groups showed good clinical response to first line steroid treatment, yet the significantly higher rate of second line administration suggests worse therapeutic response of the antibody positive group. Based on these facts we determined poor clinical prognosis and therapeutic response of the anti-SRP positive group. Orv Hetil. 2017; 158(35): 1382-1389.

  20. MR imaging findings of focal myositis: a pseudotumour that may mimic muscle neoplasm

    International Nuclear Information System (INIS)

    Gaeta, Michele; Mazziotti, Silvio; Minutoli, Fabio; Genitori, Antonino; Blandino, Alfredo; Toscano, Antonio; Rodolico, Carmelo

    2009-01-01

    The authors describe magnetic resonance (MR) findings in eight patients with histologically confirmed focal myositis. In each patient, axial TSE T1-weighted and fast short-tau inversion recovery (STIR) images were obtained using a 1.5-T MR scanner. Three patients also underwent dynamic contrast-enhanced MR examination using a GE T1-weighted sequence. The following features were evaluated: anatomical distribution, extent of the involvement, signal intensity characteristics, dynamic enhancement pattern and outcome at follow-up examinations. Seven of eight lesions were located in the lower extremities, one of eight in the arm; four of eight involved part of a muscle, two of eight diffusely involved a muscle and two of eight showed multifocal involvement of two or more muscles. All lesions were hyperintense on fast-STIR images: the hyperintensity was homogeneous in six of eight and inhomogeneous in two of eight. On T1-weighted unenhanced images, all lesions but two appeared isointense or slightly hypointense in comparison to normal muscles; two lesions showed a slight hyperintensity. Dynamic enhancement pattern corresponded to the type usually seen in benign soft tissue lesions. All lesions disappeared. Focal myositis is an uncommon pseudotumour which should be considered in the differential diagnosis of muscular masses and myopathies. (orig.)

  1. MR imaging findings of focal myositis: a pseudotumour that may mimic muscle neoplasm

    Energy Technology Data Exchange (ETDEWEB)

    Gaeta, Michele; Mazziotti, Silvio; Minutoli, Fabio; Genitori, Antonino; Blandino, Alfredo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Radiological Sciences, Messina (Italy); Toscano, Antonio; Rodolico, Carmelo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

    2009-06-15

    The authors describe magnetic resonance (MR) findings in eight patients with histologically confirmed focal myositis. In each patient, axial TSE T1-weighted and fast short-tau inversion recovery (STIR) images were obtained using a 1.5-T MR scanner. Three patients also underwent dynamic contrast-enhanced MR examination using a GE T1-weighted sequence. The following features were evaluated: anatomical distribution, extent of the involvement, signal intensity characteristics, dynamic enhancement pattern and outcome at follow-up examinations. Seven of eight lesions were located in the lower extremities, one of eight in the arm; four of eight involved part of a muscle, two of eight diffusely involved a muscle and two of eight showed multifocal involvement of two or more muscles. All lesions were hyperintense on fast-STIR images: the hyperintensity was homogeneous in six of eight and inhomogeneous in two of eight. On T1-weighted unenhanced images, all lesions but two appeared isointense or slightly hypointense in comparison to normal muscles; two lesions showed a slight hyperintensity. Dynamic enhancement pattern corresponded to the type usually seen in benign soft tissue lesions. All lesions disappeared. Focal myositis is an uncommon pseudotumour which should be considered in the differential diagnosis of muscular masses and myopathies. (orig.)

  2. HMGCR-associated myositis: a New Zealand case series and estimate of incidence.

    Science.gov (United States)

    Kennedy, N; Keating, P; O'Donnell, J

    2016-05-01

    Statins are one of the most commonly prescribed drugs in New Zealand, with 525 772 or 16.5% of the adult New Zealand population prescribed a statin between June 2013 and July 2014. While generally well-tolerated, statins are known to cause a range of muscle-related side effects, ranging from myalgia to life-threatening rhabdomyolysis. Recently, it has been recognised that in rare instances, statins can induce an immune-mediated necrotising myositis with antibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), the enzymatic target of statins. In 2014, anti-HMGCR antibody testing was introduced to Canterbury Health Laboratories (CHL), with this being the only laboratory in New Zealand performing this test during the period of this case series. This article describes an index case and characterises the clinical features of a subsequent 12-month series. From this series, we estimated the yearly incidence of HMGCR-associated myositis at 1.7/million/year or ~1/90 000 New Zealand statin users. © 2016 Royal Australasian College of Physicians.

  3. Decay-accelerating factor 1 deficiency exacerbates Trypanosoma cruzi-induced murine chronic myositis.

    Science.gov (United States)

    Solana, María E; Ferrer, María F; Novoa, María Mercedes; Song, Wen-Chao; Gómez, Ricardo M

    2012-10-01

    Murine infection with Trypanosoma cruzi (Tc) has been used to study the role of T-cells in the pathogenesis of human inflammatory idiopathic myositis. Absence of decay-accelerating factor 1 (Daf1) has been shown to enhance murine T-cell responses and autoimmunity. To determine whether Daf1 deficiency can exacerbate Tc-induced myositis, C57BL/6 DAF(+/+) and DAF(-/-) mice were inoculated with 5 × 10(4) trypomastigotes, and their morbidity, parasitemia, parasite burden, histopathology, and T-cell expansion were studied in the acute and chronic stages. DAF(-/-) mice had lower parasitemia and parasite burden but higher morbidity, muscle histopathology, and increased number of CD44(+) (activated/memory phenotype) splenic CD4(+) and CD8(+) T-cells. An enhanced CD8(+) T-cell immune-specific response may explain the lower parasitemia and parasite burden levels and the increase in histopathological lesions. We propose that Tc-inoculated DAF(-/-) mice are a useful model to study T-cell mediated immunity in skeletal muscle tissues. Copyright © 2012 Wiley Periodicals, Inc.

  4. Association of eosinophilic myositis with an unusual species of Sarcocystis in a beef cow.

    Science.gov (United States)

    Gajadhar, A A; Yates, W D; Allen, J R

    1987-01-01

    The carcass of a mature cow had numerous, disseminated lesions typical of eosinophilic myositis. To elucidate the nature and possible cause of the lesions, histological sections were examined by light microscopy and selected areas were removed and processed for electron microscopy. The lesions were granulomatous in nature. Each granuloma contained at its centre an intact or ruptured sarcocyst associated with degenerate muscle fibers. Surrounding this was a layer of epithelioid cells and an intense accumulation of inflammatory cells, most of which were eosinophils. The primary cyst wall of the sarcocysts in these granulomas consisted of hair-like protrusions that featured many unusual electron-dense bodies. Sarcocysts with ultrastructures characteristic of Sarcocystis cruzi and Sarcocystis hirsuta were also present in muscle from the same animal, but these sarcocysts lacked any associated cellular responses. The eosinophilic myositis in this case appeared to be associated with sarcocystosis of an unknown species. Possibly, the inflammatory reaction was due to the host-parasite interaction in an unusual host. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. Fig. 6. Fig. 7. Fig. 8. Fig. 9. Fig. 10. PMID:3115553

  5. Cancer-associated myositis associated with oesophageal adenocarcinoma arising in Barrett's oesophagus without serum myogenic enzymes elevation: an example suggesting the importance of MRI.

    Science.gov (United States)

    Sasaki, Yosuke; Shimizu, Hiroshige; Nemoto, Tetsuo; Urita, Yoshihisa

    2016-04-21

    The strong association between myositis and malignancy has been well recognised. Cancer-associated myositis (CAM) is thought to be a cross-reaction to regenerating muscle tissue similar to tumour antigen. We report a case of CAM due to oesophageal adenocarcinoma arising in Barrett's oesophagus without elevation of myogenic enzymes, diagnosed by MRI and repeated endoscopy. Elderly onset, prominent symptoms, lack of interstitial pneumonia, poorer response to immunosuppressive therapies, and the combination of negative conventional myositis-related antibodies and positive anti-p155/140 antibody may help to distinguish CAM from idiopathic inflammatory myopathy. As the prognosis of patients with CAM depends on the malignancy, aggressive diagnosis of CAM and the causative malignancy is required. Our experience underscores the importance of avoiding the over-reliance on serum myogenic enzymes for excluding CAM and recognising MRI as a useful diagnostic tool of myositis. 2016 BMJ Publishing Group Ltd.

  6. Validation and clinical significance of the childhood myositis assessment scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies

    NARCIS (Netherlands)

    Huber, AM; Feldman, BM; Rennebohm, RM; Hicks, JE; Lindsley, CB; Perez, MD; Zemel, LS; Wallace, CA; Ballinger, SH; Passo, MH; Reed, AM; Summers, RM; Katona, IM; Miller, FW; Lachenbruch, PA; Rider, LG; White, P.H.

    Objective. To examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores. Methods. One hundred eight children with

  7. Childhood acute non-traumatic coma: aetiology and challenges in management in resource-poor countries of Africa and Asia.

    Science.gov (United States)

    Gwer, Samson; Chacha, Clifford; Newton, Charles R; Idro, Richard

    2013-08-01

    This review examines the best available evidence on the aetiology of childhood acute non-traumatic coma in resource-poor countries (RPCs), discusses the challenges associated with management, and explores strategies to address them. Publications in English and French which reported on studies on the aetiology of childhood non-traumatic coma in RPCs are reviewed. Primarily, the MEDLINE database was searched using the keywords coma, unconsciousness, causality, aetiology, child, malaria cerebral, meningitis, encephalitis, Africa, Asia, and developing countries. 14 records were identified for inclusion in the review. Cerebral malaria (CM) was the commonest cause of childhood coma in most of the studies conducted in Africa. Acute bacterial meningitis (ABM) was the second most common known cause of coma in seven of the African studies. Of the studies in Asia, encephalitides were the commonest cause of coma in two studies in India, and ABM was the commonest cause of coma in Pakistan. Streptococcus pneumoniae was the most commonly isolated organism in ABM. Japanese encephalitis, dengue fever and enteroviruses were the viral agents most commonly isolated. Accurate diagnosis of the aetiology of childhood coma in RPCs is complicated by overlap in clinical presentation, limited diagnostic resources, disease endemicity and co-morbidity. For improved outcomes, studies are needed to further elucidate the aetiology of childhood coma in RPCs, explore simple and practical diagnostic tools, and investigate the most appropriate specific and supportive interventions to manage and prevent infectious encephalopathies.

  8. Bone scintigraphy screening for osteonecrosis of the shoulder in patients with non-traumatic osteonecrosis of the femoral head

    International Nuclear Information System (INIS)

    Sakai, Takashi; Sugano, Nobuhiko; Nishii, Takashi; Miki, Hidenobu; Yoshikawa, Hideki; Ohzono, Kenji

    2002-01-01

    In patients with non-traumatic osteonecrosis of the femoral head (ONFH), the shoulder is one of the major affected sites secondary to the proximal and distal femur in cases of multiple osteonecrosis. The present study attempted to investigate whether technetium bone scintigraphy is useful for screening of non-traumatic osteonecrosis of the shoulder (ONS).Design and patients. A total of 170 shoulder joints in 85 patients with ONFH were evaluated by bone scintigraphy and the findings compared with those of magnetic resonance imaging (MRI). The MR diagnosis was used as the gold standard.Results. Based on the diagnosis by MRI, ONS was detected in 43 shoulders of 27 patients (25%). All necrotic lesions were located in the humeral head. Sensitivity, specificity, accuracy, positive predictive value and negative predictive value of bone scintigraphy for ONS detection were 65%, 81%, 77%, 54% and 87%, respectively. When the necrotic angle of the lesions on the mid-coronal MRI was more than 40 , the sensitivity of bone scintigraphy for ONS detection increased to 88% (21/24 shoulders).Conclusion. Bone scintigraphy may be useful for demonstrating medium or large ONS lesions on screening of patients with ONFH. (orig.)

  9. Controlled release pharmaceutical composition useful for the treatment of diseases and conditions affecting metabolism and/or structural integrity of cartilage and/or bone in male comprises strontium salt

    DEFF Research Database (Denmark)

    2004-01-01

    , hyperparathyroidism, periarticular erosions in rheumatoid arthritis, osteodystrophy, myositis ossificans, Bechterew's disease, osteolytic lesions produced by bone metastasis, bone pain due to bone metastasis, bone loss due to sex steroid hormone deficiency, bone abnormalities due to steroid hormone treatment, bone...

  10. Patient characteristics and trends in nontraumatic dental condition visits to emergency departments in the United States

    Directory of Open Access Journals (Sweden)

    Xiang Q

    2012-01-01

    Full Text Available Christopher Okunseri1, Elaye Okunseri1, Joshua M Thorpe2, Qun Xiang3, Aniko Szabo31Department of Clinical Services, Marquette University School of Dentistry, Milwaukee, WI, 2Division of Social and Administrative Sciences, University of Wisconsin, Madison School of Pharmacy, Madison WI, 3Division of Biostatistics, Department of Population Health, Medical College of Wisconsin, Milwaukee, WI, USAObjective: We examined trends and patient characteristics for non-traumatic dental condition (NTDC visits to emergency departments (EDs, and compared them to other ED visit types, specifically non-dental ambulatory care sensitive conditions (non-dental ACSCs and non-ambulatory care sensitive conditions (non-ACSCs in the United States.Methods: We analyzed data from the National Hospital Ambulatory Medical Care survey (NHAMCS for 1997 to 2007. We performed descriptive statistics and used a multivariate multinomial logistic regression to examine the odds of one of the three visit types occurring at an ED. All analyses were adjusted for the survey design.Results: NTDC visits accounted for 1.4% of all ED visits with a 4% annual rate of increase (from 1.0% in 1997 to 1.7% in 2007. Self-pay patients (32% and Medicaid enrollees (27% were over-represented among NTDC visits compared to non-dental ACSC and non-ACSC visits (P < 0.0001. Females consistently accounted for over 50% of all types of ED visits examined. Compared to whites, Hispanics had significantly lower odds of an NDTC visit versus other visit types (P < 0.0001. Blacks had significantly lower odds of making NDTC visits when compared to non-dental ACSC visits only (P < 0.0001. Compared to private insurance enrollees, Medicaid and self-pay patients had 2–3 times the odds of making NTDC visits compared to other visit types.Conclusion: Nationally, NTDC visits to emergency departments increased over time. Medicaid and self-pay patients had significantly higher odds of making NDTC visits.Keywords: emergency

  11. [Risk factors for cancer in patients with myositis. Clinical, immunological characteristics and the role of the anti-p155/140 antibody].

    Science.gov (United States)

    Szankai, Zsuzsanna; Nagy-Vincze, Melinda; Bodoki, Levente; Jakab, András; Betteridge, Zoe; Dankó, Katalin

    2014-09-07

    Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by progressive proximal muscle weakness. Cancer-associated myositis represents the worst prognostic group within this heterogeneous disease. The aim of this study was to reveal factors which increase the risk factors for association of cancerous disease in patients with myositis. Furthermore, the authors explored the most common types of associated malignancies in their patients with myositis and characterize the clinical findings in a sub-group of anti-p155/140 positive patients. In this retrospective study, myositis patients with and without associated cancer were analysed (32 and 64 patients, respectively). In addition, anti-p155/140 positive and negative groups were compared, irrespective to the presence of associated malignancies. The risk for associated malignancy was higher in patients with severe muscle and skin symptoms and those with dermatomyositis. Furthermore, increased risk for malignancy was noted in the presence of particular skin symptoms and the absence of systemic symptoms. The anti-p155/140 antibody was proved to be a feasible marker of an independent clinical sub-group which overlapped clinical characteristics with cancer-associated myositis. These results may help the identification of patients with myositis with a higher risk for associated malignancy.

  12. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy

    Science.gov (United States)

    Satoh, Minoru; Tanaka, Shin; Ceribelli, Angela; Calise, S. John; Chan, Edward K. L.

    2018-01-01

    Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP). Anti-Jo-1 is the first autoantibodies to ARS detected in 15–25 % of patients. In addition to anti-Jo-1, antibodies to seven other aminoacyl tRNA synthetases (ARS) have been reported with prevalence, usually 1–5 % or lower. Patients with any antiARS antibodies are associated with anti-synthetase syndrome characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud’s phenomenon, and others. Several recent studies suggested heterogeneity in clinical features among different anti-ARS antibody-positive patients and anti-ARS may also be found in idiopathic ILD without myositis. Anti-Mi-2 is a classic marker for DM and associated with good response to steroid treatment and good prognosis. Anti-SRP is specific for PM and associated with treatment-resistant myopathy histologically characterized as necrotizing myopathy. In addition to classic MSAs, several new autoantibodies with strong clinical significance have been described in DM. Antibodies to transcription intermediary factor 1γ/α (TIF1γ/α, p155/140) are frequently found in DM associated with malignancy while anti-melanoma differentiation-associated gene 5 (MDA5; CADM140) are associated with clinically amyopathic DM (CADM) complicated by rapidly progressive ILD. Also, anti-MJ/nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modifier-1 (SUMO-1) activating enzyme (SAE) are recognized as new DM-specific autoantibodies. Addition of

  13. Expert consensus on facilitators and barriers to return-to-work following surgery for non-traumatic upper extremity conditions : A Delphi study

    NARCIS (Netherlands)

    Peters, S. E.; Johnston, V.; Ross, M.; Coppieters, M. W.

    2017-01-01

    This Delphi study aimed to reach consensus on important facilitators and barriers for return-to-work following surgery for non-traumatic upper extremity conditions. In Round 1, experts (n = 42) listed 134 factors, which were appraised in Rounds 2 and 3. Consensus (3/485% agreement) was achieved for

  14. Imaging of acquired non-traumatic cochlear lesions: iconographic essay; Avaliacao por imagem das lesoes cocleares adquiridas (nao-traumaticas): ensaio iconografico

    Energy Technology Data Exchange (ETDEWEB)

    Garcia, Marcelo de Mattos; Gonzaga, Juliana Gontijo [Clinica Axial - Centro de Imagem, Belo Horizonte, MG (Brazil)]. E-mail: cidbh@cidbh.com.br; marcelogarcia@superig.com.br

    2006-04-15

    Different non-traumatic acquired cochlear lesions are shown in this article with imaging methods. They may be responsible for neuro sensorial hearing loss or vertigo. The method of choice is computed tomography when evaluating the osseous labyrinth whereas magnetic resonance imaging has superior resolution in the studies of the membranaceous labyrinth. (author)

  15. Shoulder-specific outcomes 1 year after nontraumatic full-thickness rotator cuff repair: a systematic literature review and meta-analysis.

    Science.gov (United States)

    Gurnani, Navin; van Deurzen, Derek F P; van den Bekerom, Michel P J

    2017-10-01

    Nontraumatic full-thickness rotator cuff tears are commonly initially treated conservatively. If conservative treatment fails, rotator cuff repair is a viable subsequent option. The objective of the present meta-analysis is to evaluate the shoulder-specific outcomes one year after arthroscopic or mini-open rotator cuff repair of nontraumatic rotator cuff tears. A literature search was conducted in PubMed and EMBASE within the period January 2000 to January 2017. All studies measuring the clinical outcome at 12 months after nontraumatic rotator cuff repair of full-thickness rotator cuff tears were listed. We included 16 randomized controlled trials that met our inclusion criteria with a total of 1.221 shoulders. At 12 months after rotator cuff repair, the mean Constant score had increased 29.5 points; the mean American Shoulder and Elbow Score score increased by 38.6 points; mean Simple Shoulder Test score was 5.6 points; mean University of California Los Angeles score improved by 13.0 points; and finally, mean Visual Analogue Scale score decreased by 4.1 points. Based on this meta-analysis, significant improvements in the shoulder-specific indices are observed 12 months after nontraumatic arthroscopic or mini-open rotator cuff repair.

  16. The effectiveness of postmortem multidetector computed tomography in the detection of fatal findings related to cause of non-traumatic death in the emergency department

    International Nuclear Information System (INIS)

    Takahashi, Naoya; Higuchi, Takeshi; Shiotani, Motoi; Hirose, Yasuo; Shibuya, Hiroyuki; Hashidate, Hideki; Yamanouchi, Haruo; Funayama, Kazuhisa

    2012-01-01

    To investigate the diagnostic performance of postmortem multidetector computed tomography (PMMDCT) for the detection of fatal findings related to causes of non-traumatic death in the emergency department (ED). 494 consecutive cases of clinically diagnosed non-traumatic death in ED involving PMMDCT were enrolled. The fatal findings were detected on PMMDCT and classified as definite or possible findings. These findings were confirmed by autopsy in 20 cases. The fatal findings were detected in 188 subjects (38.1%) including 122 with definite (24.7%) and 66 with possible finding (13.4%). Definite findings included 21 cases of intracranial vascular lesions, 84 with intra-thoracic haemorrhage, 13 with retroperitoneal haemorrhage and one with oesophagogastric haemorrhage. In three patients who had initially been diagnosed with non-traumatic death, PMMDCT revealed fatal traumatic findings. Two definite findings (two haemopericardiums) and seven possible findings (two intestinal obstructions, one each of multiple liver tumours central pulmonary artery dilatation, pulmonary congestion, peritoneal haematoma, and brain oedema) were confirmed by autopsy. The causes of death were not determined in cases with possible findings without autopsy. PMMDCT is a feasible tool for detecting morphological fatal findings in non-traumatic death in ED. It is important to know the ability and limitation of PMMDCT. (orig.)

  17. Sporadic Fibrodysplasia Ossificans Progressiva in an Egyptian Infant with c.617G > A Mutation in ACVR1 Gene: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Mohammad Al-Haggar

    2013-01-01

    Full Text Available Fibrodysplasia ossificans progressiva (FOP is an autosomal dominant severe musculoskeletal disease characterized by extensive new bone formation within soft connective tissues and unique skeletal malformations of the big toes which represent a birth hallmark for the disease. Most of the isolated classic cases of FOP showed heterozygous mutation in the ACVR1 gene on chromosome 2q23 that encodes a bone morphogenetic protein BMP (ALK2. The most common mutation is (c.617G > A leading to the amino acid substitution of arginine by histidine (p.Arg206His. We currently report on an Egyptian infant with a sporadic classic FOP in whom c.617G > A mutation had been documented. The patient presented with the unique congenital malformation of big toe and radiological evidence of heterotopic ossification in the back muscles. The triggering trauma was related to the infant's head, however; neither neck region nor sites of routine intramuscular vaccination given during the first year showed any ossifications. Characterization of the big toe malformation is detailed to serve as an early diagnostic marker for this rare disabling disease.

  18. Eosinophilic myositis resulted from Sarcocystis infection in prime marbled beef of Japanese black cattle

    Directory of Open Access Journals (Sweden)

    Tohru Kimura

    Full Text Available Partial changes of color (greenish to brownish were found in prime marbled beef of Japanese black cattle. The disseminated lesions of the skeletal muscles were histopathologically examined in relation to Sarcocystis infection. The lesions in the muscles showed granulomas with inflammatory cell infiltration. The sarcocysts had a distinct wall, which was radically striated by palisading villar protrusions. The sarcocyst wall was surrounded by degenerative eosinophils and necrotic muscle fibers. In conclusion, eosinophilic myositis in prime marbled beef of Japanese black cattle resulted from Sarcocystis spp. infection. The muscular lesions were characterized by the presence of granulomas and capsulated sarcocysts surrounded by numerous eosinophils. [Vet. World 2011; 4(11.000: 500-502

  19. Simple diagnosis of benign acute childhood myositis: Lessons from a case report.

    Science.gov (United States)

    Terlizzi, Vito; Improta, Federica; Raia, Valeria

    2014-01-01

    Acute muscle pain and walking difficulty are symptoms compatible with both benign and severe degenerative diseases. As a consequence, in some cases invasive tests and hospitalizations are improperly scheduled. We report the case of a 7-year-old child suffering from acute calf pain and abnormal gait following flu-like symptoms. A review of the literature will be helpful to better define differential diagnosis in cases of muscle pain in children. Daily physical examination and urine dipstick are sufficient to confirm the diagnosis of benign acute childhood myositis (BACM) during the acute phase, to promptly detect severe complications and to rule out degenerative diseases. Children with BACM do not require hospitalization, medical interventions or long-term follow-up.

  20. Management of necrotizing myositis in a field hospital: a case report

    Directory of Open Access Journals (Sweden)

    Bharathi Ramanathan

    2009-04-01

    Full Text Available Abstract Necrotizing myositis is a rare and fatal disease of skeletal muscles caused by group A beta hemolytic streptococci (GABHS. Its early detection by advanced imaging forms the basis of current management strategy. Paucity of advanced imaging in field/rural hospitals necessitates adoption of management strategy excluding imaging as its basis. Such a protocol, based on our experience and literature, constitutes: i. Prompt recognition of the clinical triad: disproportionate pain; precipitous course; and early loss of power- in a swollen limb with/without preceding trauma. ii. Support of clinical suspicion by 2 ubiquitous laboratory tests: gram staining- of exudates from bullae/muscles to indicate GABHS infection; and CPK estimation- to indicate myonecrosis. iii. Replacement of empirical antibiotics with high intravenous doses of sodium penicillin and clindamycin iv. Exploratory fasciotomy: to confirm myonecrosis without suppuration- its hallmark v. Emergent radical debridement vi. Primary closure with viable flaps – unconventional, if need be.

  1. Asymptomatic hyper-creatine-kinase-emia as sole manifestation of inclusion body myositis

    Directory of Open Access Journals (Sweden)

    Josef Finsterer

    2013-06-01

    Full Text Available Sporadic inclusion body myositis (sIBM usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L, since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia.

  2. New insights into the benefits of exercise for muscle health in patients with idiopathic inflammatory myositis.

    Science.gov (United States)

    Alemo Munters, Li; Alexanderson, Helene; Crofford, Leslie J; Lundberg, Ingrid E

    2014-07-01

    With recommended treatment, a majority with idiopathic inflammatory myopathy (IIM) develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis and to some extent in active disease and inclusion body myositis (IBM). Importantly, randomized controlled trials (RCTs) indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking mechanisms underlying effects of exercise in skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes in aerobic phenotype and muscle growth programs, and down regulation in genes related to inflammation. Altogether, exercise contributes to both systemic and within-muscle adaptations demonstrating that exercise is fundamental to improve muscle performance and health in IIM. There is a need for RCTs to study effects of exercise in active disease and IBM.

  3. Calcium dysregulation, functional calpainopathy, and endoplasmic reticulum stress in sporadic inclusion body myositis.

    Science.gov (United States)

    Amici, David R; Pinal-Fernandez, Iago; Mázala, Davi A G; Lloyd, Thomas E; Corse, Andrea M; Christopher-Stine, Lisa; Mammen, Andrew L; Chin, Eva R

    2017-03-22

    Sporadic inclusion body myositis (IBM) is the most common primary myopathy in the elderly, but its pathoetiology is still unclear. Perturbed myocellular calcium (Ca 2+ ) homeostasis can exacerbate many of the factors proposed to mediate muscle degeneration in IBM, such as mitochondrial dysfunction, protein aggregation, and endoplasmic reticulum stress. Ca 2+ dysregulation may plausibly be initiated in IBM by immune-mediated membrane damage and/or abnormally accumulating proteins, but no studies to date have investigated Ca 2+ regulation in IBM patients. We first investigated protein expression via immunoblot in muscle biopsies from IBM, dermatomyositis, and non-myositis control patients, identifying several differentially expressed Ca 2+ -regulatory proteins in IBM. Next, we investigated the Ca 2+ -signaling transcriptome by RNA-seq, finding 54 of 183 (29.5%) genes from an unbiased list differentially expressed in IBM vs. controls. Using an established statistical approach to relate genes with causal transcription networks, Ca 2+ abundance was considered a significant upstream regulator of observed whole-transcriptome changes. Post-hoc analyses of Ca 2+ -regulatory mRNA and protein data indicated a lower protein to transcript ratio in IBM vs. controls, which we hypothesized may relate to increased Ca 2+ -dependent proteolysis and decreased protein translation. Supporting this hypothesis, we observed robust (4-fold) elevation in the autolytic activation of a Ca 2+ -activated protease, calpain-1, as well as increased signaling for translational attenuation (eIF2a phosphorylation) downstream of the unfolded protein response. Finally, in IBM samples we observed mRNA and protein under-expression of calpain-3, the skeletal muscle-specific calpain, which broadly supports proper Ca 2+ homeostasis. Together, these data provide novel insight into mechanisms by which intracellular Ca 2+ regulation is perturbed in IBM and offer evidence of pathological downstream effects.

  4. Muscle myeloid type I interferon gene expression may predict therapeutic responses to rituximab in myositis patients.

    Science.gov (United States)

    Nagaraju, Kanneboyina; Ghimbovschi, Svetlana; Rayavarapu, Sree; Phadke, Aditi; Rider, Lisa G; Hoffman, Eric P; Miller, Frederick W

    2016-09-01

    To identify muscle gene expression patterns that predict rituximab responses and assess the effects of rituximab on muscle gene expression in PM and DM. In an attempt to understand the molecular mechanism of response and non-response to rituximab therapy, we performed Affymetrix gene expression array analyses on muscle biopsy specimens taken before and after rituximab therapy from eight PM and two DM patients in the Rituximab in Myositis study. We also analysed selected muscle-infiltrating cell phenotypes in these biopsies by immunohistochemical staining. Partek and Ingenuity pathway analyses assessed the gene pathways and networks. Myeloid type I IFN signature genes were expressed at higher levels at baseline in the skeletal muscle of rituximab responders than in non-responders, whereas classic non-myeloid IFN signature genes were expressed at higher levels in non-responders at baseline. Also, rituximab responders have a greater reduction of the myeloid and non-myeloid type I IFN signatures than non-responders. The decrease in the type I IFN signature following administration of rituximab may be associated with the decreases in muscle-infiltrating CD19(+) B cells and CD68(+) macrophages in responders. Our findings suggest that high levels of myeloid type I IFN gene expression in skeletal muscle predict responses to rituximab in PM/DM and that rituximab responders also have a greater decrease in the expression of these genes. These data add further evidence to recent studies defining the type I IFN signature as both a predictor of therapeutic responses and a biomarker of myositis disease activity. Published by Oxford University Press on behalf British Society for Rheumatology 2016. This work is written by US Government employees and is in the public domain in the US.

  5. Anti-Jo-1 antibody-positive patients show a characteristic necrotizing perifascicular myositis.

    Science.gov (United States)

    Mescam-Mancini, Lénaig; Allenbach, Yves; Hervier, Baptiste; Devilliers, Hervé; Mariampillay, Kuberaka; Dubourg, Odile; Maisonobe, Thierry; Gherardi, Romain; Mezin, Paulette; Preusse, Corinna; Stenzel, Werner; Benveniste, Olivier

    2015-09-01

    Idiopathic inflammatory myopathies can be classified as polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, sporadic inclusion body myositis or non-specific myositis. Anti-Jo-1 antibody-positive patients are assigned to either polymyositis or dermatomyositis suggesting overlapping pathological features. We aimed to determine if anti-Jo-1 antibody-positive myopathy has a specific morphological phenotype. In a series of 53 muscle biopsies of anti-Jo-1 antibody-positive patients, relevant descriptive criteria defining a characteristic morphological pattern were identified. They were tested in a second series of anti-Jo-1 antibody-positive patients and compared to 63 biopsies from patients suffering from other idiopathic inflammatory myopathies. In anti-Jo-1 antibody-positive patients, necrotic fibres, which strongly clustered in perifascicular regions, were frequently observed. Sarcolemmal complement deposition was detected specifically in perifascicular areas. Inflammation was mainly located in the perimysium and around vessels in 90.6%. Perimysial fragmentation was observed in 90% of cases. Major histocompatibility complex class I staining was diffusely positive, with a perifascicular reinforcement. Multivariate analysis showed that criteria defining perifascicular pathology: perifascicular necrosis, atrophy, and perimysial fragmentation allow the distinction of anti-Jo-1 antibody-positive patients, among patients suffering from other idiopathic inflammatory myopathies. Anti-Jo-1 antibody-positive patients displayed perifascicular necrosis, whereas dermatomyositis patients exhibited perifascicular atrophy. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  6. Differences in response between traumatic and non-traumatic chronic neck pain patients in a multimodal intervention?

    DEFF Research Database (Denmark)

    Ris Hansen, Inge; Søgaard, Karen; Gram, Bibi

    , Pain Bothersomeness, Patient-Specific Functioning Scale, Tampa Scale of Kinesiophobia, Global Perceived Effect) and clinical tests (cervical Range of Motion, Pressure Pain Threshold at infraspinatus, tibialis anterior and cervical spine, Cranio-cervical Flexion, Cervical Extension muscle function......) showed statistically significant improvements in physical HR-QoL, mental HR-QoL, depression, cervical pressure pain threshold, cervical extension movement, muscle function, and oculomotion. Per protocol analyses confirmed these results with additional significant improvements in the exercise group......Abstract title: Do traumatic and non-traumatic chronic neck pain patients respond different on a combination of pain education, exercises and training? Authors: Inge Ris, Karen Søgaard, Bibi Gram, Karina Agerbo, Eleanor Boyle, Birgit Juul-Kristensen Background: Previous studies have investigated...

  7. Nivolumab-induced synchronous occurrence of myositis and hypothyroidism in a patient with squamous cell lung cancer.

    Science.gov (United States)

    Badovinac, Sonja; Korsic, Marta; Zarkovic, Kamelija; Mursic, Davorka; Roglic, Mihovil; Jakopovic, Marko; Samarzija, Miroslav

    2018-03-01

    Alongside the proven efficacy, immunotherapy in treatment of malignant diseases can cause immune-related adverse events different from commonly known chemotherapy-related toxicities. During nivolumab treatment of metastatic squamous cell lung cancer, the patient developed a symptomatic inflammatory myositis confirmed with muscle biopsy and primary hypothyroidism. After initiation of corticosteroids and thyroid hormone replacement, the clinical and laboratory improvement occurred. To the best of our knowledge, this is the first description of a case of nivolumab-induced synchronous manifestation of immune-related myositis and hypothyroidism. Immunotherapy can trigger a wide spectrum of immune-related adverse events that could occur simultaneously. If not detected and treated, these events could become severe or even fatal and require clinicians' awareness and routine check-ups.

  8. Clinical utility and cost-effectiveness of CT-angiography in the diagnosis of nontraumatic subarachnoid hemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Jabbarli, Ramazan; Shah, Mukesch; Hippchen, Beate; Velthoven, Vera van [University Hospital of Freiburg, Department of Neurosurgery, Freiburg/Breisgau (Germany); Taschner, Christian [University Hospital of Freiburg, Department of Neuroradiology, Freiburg (Germany); Kaier, Klaus [University Hospital of Freiburg, Institute for Medical Biometry and Medical Informatics, Freiburg (Germany)

    2014-10-15

    CT-angiography gains an increasing role in the initial diagnosis of patients with nontraumatic subarachnoid hemorrhage (SAH). However, the implementation of CT-angiography does not always exclude the necessity of conventional angiography. Our objective was to determine the practical utility and cost-effectiveness of CT-angiography. All patients with nontraumatic subarachnoid hemorrhage admitted to our university hospital after implementation of CT-angiography between June 1, 2011 and June 30, 2012 were retrospectively analyzed in regard to factors of treatment flow, radiation exposure, harms of contrast medium loading, and diagnostic costs. A control group of the same size was assembled from previously admitted SAH patients, who did not undergo pretreatment CT-angiography. Furthermore, cost-effectiveness analysis was performed. The final analysis consisted of 93 patients in each group. Of 93 patients with pretreatment CT-angiography, 74 had to undergo conventional angiography for diagnostic and/or therapeutic purposes. CT-angiography had significant impact on the reduction of collective effective radiation dose by 4.419 mSv per person (p = 0.0002) and was not associated with additional harms. Despite the significantly earlier detection of aneurysms with CT-angiography (p < 0.0001), there were no significant differences in the timing of aneurysm repair and duration of ICU and general hospital stay. There was an increase of diagnostic costs - the cost-effectiveness analysis showed, however, that benefits of CT-angiography in respect to radiation exposure and risk of conventional angiography-related complications justify the additional costs of CT-angiography. Although the implementation of CT-angiography in SAH diagnosis cannot completely replace conventional angiography, it can be approved in regard to radiation hygiene and cost-effectiveness. (orig.)

  9. Index to Predict In-hospital Mortality in Older Adults after Non-traumatic Emergency Department Intubations

    Directory of Open Access Journals (Sweden)

    Kei Ouchi

    2017-04-01

    Full Text Available Introduction: Our goal was to develop and validate an index to predict in-hospital mortality in older adults after non-traumatic emergency department (ED intubations. Methods: We used Vizient administrative data from hospitalizations of 22,374 adults ≥75 years who underwent non-traumatic ED intubation from 2008–2015 at nearly 300 U.S. hospitals to develop and validate an index to predict in-hospital mortality. We randomly selected one half of participants for the development cohort and one half for the validation cohort. Considering 25 potential predictors, we developed a multivariable logistic regression model using least absolute shrinkage and selection operator method to determine factors associated with in-hospital mortality. We calculated risk scores using points derived from the final model’s beta coefficients. To evaluate calibration and discrimination of the final model, we used Hosmer-Lemeshow chi-square test and receiver-operating characteristic analysis and compared mortality by risk groups in the development and validation cohorts. Results: Death during the index hospitalization occurred in 40% of cases. The final model included six variables: history of myocardial infarction, history of cerebrovascular disease, history of metastatic cancer, age, admission diagnosis of sepsis, and admission diagnosis of stroke/ intracranial hemorrhage. Those with low-risk scores (10 had 58% risk of in-hospital mortality. The Hosmer-Lemeshow chi-square of the model was 6.47 (p=0.09, and the c-statistic was 0.62 in the validation cohort. Conclusion: The model may be useful in identifying older adults at high risk of death after ED intubation.

  10. Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature.

    Science.gov (United States)

    Binns, E L; Moraitis, E; Maillard, S; Tansley, S; McHugh, N; Jacques, T S; Wedderburn, L R; Pilkington, C; Yasin, S A; Nistala, K

    2017-10-31

    Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation. Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash. Histology revealed a destructive myopathy characterized by scattered atrophic and necrotic fibres with little or no inflammatory infiltrate. All 3 patients responded to induction with cyclophosphamide, IVIG and rituximab, in conjunction with intensive physiotherapy and methotrexate as the maintenance agent. Our patients regained near-normal strength (MMT > 70/80), in contrast with the current literature where >50% of cases reported severe residual weakness. A literature search on paediatric anti-SRP myositis was performed to June 2016; PubMed was screened using a combination of the following terms: signal recognition particle, autoantibodies, antibodies, myositis, muscular diseases, skeletal muscle, childhood, paediatric, juvenile. Articles in a foreign language were excluded. Nine case studies were found. This paper supports the hypothesis that anti-SRP myositis is distinct from other JIIM. It is an important differential to JDM and should be considered where there is severe weakness without rash or if highly elevated muscle enzymes (CK > 10,000 U/l) are found. Early identification is essential to initiate aggressive medical and physical therapy. Greater international collaboration and long-term follow-up data is needed to establish the most effective treatment strategy for this rare group of patients.

  11. Aberrant Muscle Antigen Exposure in Mice Is Sufficient to Cause Myositis in a Treg Cell–Deficient Milieu

    Science.gov (United States)

    Young, Nicholas A; Sharma, Rahul; Friedman, Alexandra K; Kaffenberger, Benjamin H; Bolon, Brad; Jarjour, Wael N

    2013-01-01

    Objective Myositis is associated with muscle-targeted inflammation and is observed in some Treg cell–deficient mouse models. Because an autoimmune pathogenesis has been strongly implicated, the aim of this study was to investigate the hypothesis that abnormal exposure to muscle antigens, as observed in muscle injury, can induce autoimmune-mediated myositis in susceptible hosts. Methods FoxP3 mutant (scurfy) mice were mated to synaptotagmin VII (Syt VII) mutant mice, which resulted in a new mouse strain that combines impaired membrane resealing with Treg cell deficiency. Lymphocyte preparations from double-mutant mice were adoptively transferred intraperitoneally, with or without purified Treg cells, into recombination-activating gene 1 (RAG-1)–null recipients. Lymph node cells from mice with the FoxP3 mutation were transferred into RAG-1–null mice either 1) intraperitoneally in conjunction with muscle homogenate or purified myosin protein or 2) intramuscularly with or without cotransfer of purified Treg cells. Results FoxP3-deficient mouse lymph node cells transferred in conjunction with myosin protein or muscle homogenate induced robust skeletal muscle inflammation. The infiltrates consisted predominantly of CD4+ and CD8+ T cells, a limited number of macrophages, and no B cells. Significant inflammation was also seen in similar experiments using lymph node cells from FoxP3/Syt VII double-mutant mice but was absent in experiments using adoptive transfer of FoxP3 mutant mouse cells alone. The cotransfer of Treg cells completely suppressed myositis. Conclusion These data, derived from a new, reproducible model, demonstrate the critical roles of Treg cell deficiency and aberrant muscle antigen exposure in the priming of autoreactive cells to induce myositis. This mouse system has multifaceted potential for examining the interplay in vivo between tissue injury and autoimmunity. PMID:24022275

  12. An autoimmune myositis-overlap syndrome associated with autoantibodies to nuclear pore complexes: description and long-term follow-up of the anti-Nup syndrome.

    Science.gov (United States)

    Senécal, Jean-Luc; Isabelle, Catherine; Fritzler, Marvin J; Targoff, Ira N; Goldstein, Rose; Gagné, Michel; Raynauld, Jean-Pierre; Joyal, France; Troyanov, Yves; Dabauvalle, Marie-Christine

    2014-11-01

    Autoimmune myositis encompasses various myositis-overlap syndromes, each being identified by the presence of serum marker autoantibodies. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. There was no clinical or laboratory evidence for liver disease. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr).By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes. Reactivity with nuclear pore complexes was confirmed by immunoelectron microscopy. In a cohort of 100 French Canadian patients with autoimmune myositis, the nuclear pore complex fluorescent ANA pattern was restricted to these 4 patients (4%). It was not observed in sera from 393 adult patients with systemic sclerosis (n = 112), mixed connective tissue disease (n = 35), systemic lupus (n = 94), rheumatoid arthritis (n = 45), or other rheumatic diseases (n = 107), nor was it observed in 62 normal adults.Autoantibodies to nuclear pore complexes were predominantly of IgG isotype. No other IgG autoantibody markers for defined connective tissue diseases or overlap syndromes were present, indicating a selective and highly focused immune response. In 3 patients, anti-nuclear pore complex autoantibody titers varied in parallel with myositis activity, suggesting a pathogenic link to

  13. Restricted Mandibular Movement Attributed to Ossification of Mandibular Depressors and Medial Pterygoid Muscles in Patients With Fibrodysplasia Ossificans Progressiva: A Report of 3 Cases.

    Science.gov (United States)

    Okuno, Tetsuko; Suzuki, Hitoshi; Inoue, Akio; Kusukawa, Jingo

    2017-09-01

    Fibrodysplasia ossificans progressiva (FOP) is an extremely rare genetic condition characterized by congenital malformation and progressive heterotopic ossification (HO) caused by a recurrent single nucleotide substitution at position 617 in the ACVR1 gene. As the condition progresses, HO leads to joint ankylosis, breathing difficulties, and mouth-opening restriction, and it can shorten the patient's lifespan. This report describes 3 cases of FOP confirmed by genetic testing in patients with restricted mouth opening. Each patient presented a different onset and degree of jaw movement restriction. The anatomic ossification site of the mandibular joint was examined in each patient using reconstructed computed tomographic (CT) images and 3-dimensional reconstructed CT (3D-CT) images. A 29-year-old woman complained of jaw movement restriction since 13 years of age. 3D-CT image of the mandibular joint showed an osseous bridge, formed by the mandibular depressors that open the mouth, between the hyoid bone and the mentum of the mandible. A 39-year-old man presented with jaw movement restriction that developed at 3 years of age after a mouth injury. 3D-CT image of the jaw showed ankylosis of the jaw from ossification of the mandibular depressors that was worse than in patient 1. CT images showed no HO findings of the masticatory muscles. To the authors' knowledge, these are the first 2 case descriptions of the anatomic site of ankylosis involving HO of the mandibular depressors in the jaw resulting from FOP. In contrast, a 62-year-old bedridden woman with an interincisal distance longer than 10 mm (onset, 39 years of age) had no HO of the mandibular depressors and slight HO of the medial pterygoid muscle on the right and left sides. These findings suggest that restricted mouth opening varies according to the presence or absence of HO of the mandibular depressors. Copyright © 2017. Published by Elsevier Inc.

  14. Redefining dermatomyositis: a description of new diagnostic criteria that differentiate pure dermatomyositis from overlap myositis with dermatomyositis features.

    Science.gov (United States)

    Troyanov, Yves; Targoff, Ira N; Payette, Marie-Pier; Raynauld, Jean-Pierre; Chartier, Suzanne; Goulet, Jean-Richard; Bourré-Tessier, Josiane; Rich, Eric; Grodzicky, Tamara; Fritzler, Marvin J; Joyal, France; Koenig, Martial; Senécal, Jean-Luc

    2014-11-01

    Dermatomyositis (DM) is a major clinical subset of autoimmune myositis (AIM). The characteristic DM rash (Gottron papules, heliotrope rash) and perifascicular atrophy at skeletal muscle biopsy are regarded as specific features for this diagnosis. However, new concepts are challenging the current definition of DM. A modified Bohan and Peter classification of AIM was proposed in which the core concept was the inclusion of the diagnostic significance of overlap connective tissue disease features. In this clinical classification, a DM rash in association with myositis in the absence of overlap features indicates a diagnosis of pure DM. However, overlap features in association with myositis allow a diagnosis of overlap myositis (OM), irrespective of the presence or absence of the DM rash. Perifascicular atrophy may be present in both pure DM and OM. Recently, the presence of perifascicular atrophy in myositis without a DM rash was proposed as diagnostic of a novel entity, adermatopathic DM. We conducted the present study to evaluate these new concepts to further differentiate pure DM from OM.Using the modified Bohan and Peter classification, we performed a follow-up study of a longitudinal cohort of 100 consecutive adult French Canadian patients with AIM, including 44 patients with a DM phenotype, defined as a DM rash, and/or DM-type calcinosis, and/or the presence of perifascicular atrophy on muscle biopsy. A detailed evaluation was performed for overlap features, the extent and natural history of the DM rash, adermatopathic DM, DM-specific and overlap autoantibodies by protein A immunoprecipitation on coded serum samples, and associations with cancer and survival.Two distinct subsets were identified in patients with a DM phenotype: pure DM (n = 24) and OM with DM features, or OMDM (n = 20). In pure DM, the DM rash was a dominant finding. It was the first disease manifestation, was always present at the time of myositis diagnosis, and was associated with a high

  15. Resection of the lateral end of the clavicle following osteolysis, with emphasis on non-traumatic osteolysis of the acromial end of the clavicle in athletes.

    Science.gov (United States)

    Scavenius, M; Iversen, B F; Stürup, J

    1987-07-01

    Preoperative radiographs of 38 patients who had undergone resection of the lateral end of the clavicle were reviewed. Seven cases of osteolysis of the lateral end of the clavicle were found, of which four followed severe injury of the shoulder girdle. Three of the cases were young male athletes, with nontraumatic osteolysis. One additional patient with this disorder, in whom resection has not yet been performed, was also included. All four had practised weightlifting and benchpressing as part of their training. Hence, a feasible explanation for the osteolytic process seems to be repeated microfractures due to stresses imposed by these activities. Several conservative regimens provided only temporary relief. After resection, the symptoms ceased and the patients were able to return to competitive sport. With the increasing interest in bodybuilding, non-traumatic osteolysis of the acromial end of the clavicle should be borne in mind in cases of pain in the shoulder in athletes.

  16. Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Aggarwal, Rohit; McBurney, Christine; Schneider, Frank; Yousem, Samuel A; Gibson, Kevin F; Lindell, Kathleen; Fuhrman, Carl R; Oddis, Chester V

    2017-03-01

    To compare the survival outcomes between myositis-associated usual interstitial pneumonia (MA-UIP) and idiopathic pulmonary fibrosis (IPF-UIP). Adult MA-UIP and IPF-UIP patients were identified using CTD and IPF registries. The MA-UIP cohort included myositis or anti-synthetase syndrome patients with interstitial lung disease while manifesting UIP on high-resolution CT chest and/or a lung biopsy revealing UIP histology. IPF subjects met American Thoracic Society criteria and similarly had UIP histopathology. Kaplan-Meier survival curves compared cumulative and pulmonary event-free survival (event = transplant or death) between (i) all MA-UIP and IPF-UIP subjects, (ii) MA-UIP with biopsy proven UIP (n = 25) vs IPF-UIP subjects matched for age, gender and baseline forced vital capacity (±10%). Cox proportional hazards ratios compared the survival controlling for co-variates. Eighty-one IPF-UIP and 43 MA-UIP subjects were identified. The median cumulative and event-free survival time in IPF vs MA-UIP was 5.25/1.8 years vs 16.2/10.8 years, respectively. Cumulative and event-free survival was significantly worse in IPF-UIP vs MA-UIP [hazards ratio of IPF-UIP was 2.9 (95% CI: 1.5, 5.6) and 5.0 (95% CI: 2.8, 8.7) (P < 0.001), respectively]. IPF-UIP event-free survival (but not cumulative) remained significantly worse than MA-UIP with a hazards ratio of 6.4 (95% CI: 3.0, 13.8) after controlling for age at interstitial lung disease diagnosis, gender, ethnicity and baseline forced vital capacity%. Respiratory failure was the most common cause of death in both groups. A sub-analysis of 25 biopsy-proven MA-UIP subjects showed similar results. MA-UIP patients demonstrated a significant survival advantage over a matched IPF cohort, suggesting that despite similar histological and radiographic findings at presentation, the prognosis of MA-UIP is superior to that of IPF-UIP. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology

  17. Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis.

    Science.gov (United States)

    Malkan, Ashish; Cappelen-Smith, Cecilia; Beran, Roy; Griffith, Neil; Toong, Catherine; Wang, Min-Xia; Cordato, Dennis

    2015-02-01

    We report a 37-year-old woman with a 2 month history of proximal muscle weakness and extremely high creatine kinase (21,808 U/L) due to necrotizing auto-immune myositis (NAM) in association with anti-synthetase syndrome. Myositis-specific auto-immune antibody panel was positive for anti-Signal recognition particle and anti-PL-12. CT scan of the chest confirmed interstitial lung disease. Prednisolone, intravenous immunoglobulin and cyclophosphamide therapy was given with gradual improvement. This patient is notable for the unusual combination of NAM and anti-synthetase syndrome with the rare finding of two myositis-specific autoantibodies, which directed testing for associated extramuscular features and management with more aggressive immunotherapy. Copyright © 2014 Elsevier Ltd. All rights reserved.

  18. Pain originating from the sacroiliac joint is a common non-traumatic musculoskeletal complaint in elite inline-speedskaters - an observational study

    Directory of Open Access Journals (Sweden)

    Ruhe Alexander

    2012-03-01

    Full Text Available Abstract Study design Observational study Objectives To investigate common non-traumatic musculoskeletal complaints of the low back in elite inline-speedskaters of the German national team. Summary of background data Traumatic injuries associated with falls or collisions are well documented in speedskaters but so far no studies have investigated non-traumatic low back pain. Previously, the sacroiliac joint was suspected as a frequent origin of complaint, we aimed to investigate this assumption. Methods Two chiropractors examined elite inline-speedskaters of the German national team during three sports events between summer 2010 and 2011. A test cluster of five provocative tests for the sacroiliac joint was selected based on reliability and validity. Results A total of 37 examinations were conducted on 34 athletes with low back pain during the three sport events. The reported pain intensities ranged from mild to moderate pain (VAS 23.4 ± 13.4 to 35.1 ± 19.2. About 90% of cases showed involvement of the SI joint of which again 90% presented with left sided symptoms. Conclusions Non-traumatic complaints of the low back originating from the left sacroiliac joint frequently occur in competitive inline speedskaters.

  19. Diagnostic accuracy of low-dose CT compared with abdominal radiography in non-traumatic acute abdominal pain: prospective study and systematic review.

    Science.gov (United States)

    Alshamari, Muhammed; Norrman, Eva; Geijer, Mats; Jansson, Kjell; Geijer, Håkan

    2016-06-01

    Abdominal radiography is frequently used in acute abdominal non-traumatic pain despite the availability of more advanced diagnostic modalities. This study evaluates the diagnostic accuracy of low-dose CT compared with abdominal radiography, at similar radiation dose levels. Fifty-eight patients were imaged with both methods and were reviewed independently by three radiologists. The reference standard was obtained from the diagnosis in medical records. Sensitivity and specificity were calculated. A systematic review was performed after a literature search, finding a total of six relevant studies including the present. Overall sensitivity with 95 % CI for CT was 75 % (66-83 %) and 46 % (37-56 %) for radiography. Specificity was 87 % (77-94 %) for both methods. In the systematic review the overall sensitivity for CT varied between 75 and 96 % with specificity from 83 to 95 % while the overall sensitivity for abdominal radiography varied between 30 and 77 % with specificity 75 to 88 %. Based on the current study and available evidence, low-dose CT has higher diagnostic accuracy than abdominal radiography and it should, where logistically possible, replace abdominal radiography in the workup of adult patients with acute non-traumatic abdominal pain. • Low-dose CT has a higher diagnostic accuracy than radiography. • A systematic review shows that CT has better diagnostic accuracy than radiography. • Radiography has no place in the workup of acute non-traumatic abdominal pain.

  20. The Role of Bone Subtraction Computed Tomographic Angiography in Determining Intracranial Aneurysms in Non-Traumatic Subarachnoid Hemorrhage

    International Nuclear Information System (INIS)

    Kayhan, Aysegul; Koc, Osman; Keskin, Suat; Keskin, Fatih

    2014-01-01

    The presence of blood in the subarachnoid space is an acute pathology with a serious risk of death and complications. The most common etiology (approximately 80%) is intracranial aneurysm. The aim of this study was to assess the role of bone subtracted computed tomographic angiography (BSCTA), a novel and noninvasive method for determining and characterizing intracranial aneurysms. Sixty consecutive patients with clinically suspected non-traumatic subarachnoid hemorrhage (SAH) were considered to enter the study. The subtraction quality was inadequate in ten patients; thus, they were excluded, leaving 50 patients (84.4%) in the study. Bone subtracted and non-subtracted 3D images were obtained from the BSCTA raw data sets. All images obtained by digital subtraction angiography (DSA), BSCTA, and computed tomographic angiography (CTA) were evaluated for the presence or absence of an aneurysm and the location, minimal sac diameter, and neck size ratio of the aneurysm. DSA was considered as the gold standard during the evaluation of the data. Of the 50 patients who participated in this study, 11 had no aneurysms as determined by both CTA and DSA. Examination of the remaining 39 patients revealed the presence of 51 aneurysms. While 3D-CTA could not detect six aneurysms that were located in the base of the skull, 3D-BSCTA easily detected them. Moreover, five aneurysms were only partially detected by 3D-CTA. According to this data, the sensitivity of 3D-BSCTA and 3D-CTA was calculated as 98% and 86.3%, respectively; the specificity was calculated as 100% and 90.9%, respectively, per aneurysm; and the sensitivity of 100% for 3D-BSCTA and 98% for 3D-CTA was achieved by using combined images with multi-planar reconstruction (MPR) and maximum intensity projection (MIP). BSCTA detected and characterized the aneurysms as well as DSA, and BSCTA and DSA gave concordant results in detecting aneurysms. BSCTA is easily accessible, less time consuming, and most importantly, a non

  1. The Role of Bone Subtraction Computed Tomographic Angiography in Determining Intracranial Aneurysms in Non-Traumatic Subarachnoid Hemorrhage

    Science.gov (United States)

    Kayhan, Aysegul; Koc, Osman; Keskin, Suat; Keskin, Fatih

    2014-01-01

    Background: The presence of blood in the subarachnoid space is an acute pathology with a serious risk of death and complications. The most common etiology (approximately 80%) is intracranial aneurysm. Objectives: The aim of this study was to assess the role of bone subtracted computed tomographic angiography (BSCTA), a novel and noninvasive method for determining and characterizing intracranial aneurysms. Patients and Methods: Sixty consecutive patients with clinically suspected non-traumatic subarachnoid hemorrhage (SAH) were considered to enter the study. The subtraction quality was inadequate in ten patients; thus, they were excluded, leaving 50 patients (84.4%) in the study. Bone subtracted and non-subtracted 3D images were obtained from the BSCTA raw data sets. All images obtained by digital subtraction angiography (DSA), BSCTA, and computed tomographic angiography (CTA) were evaluated for the presence or absence of an aneurysm and the location, minimal sac diameter, and neck size ratio of the aneurysm. DSA was considered as the gold standard during the evaluation of the data. Results: Of the 50 patients who participated in this study, 11 had no aneurysms as determined by both CTA and DSA. Examination of the remaining 39 patients revealed the presence of 51 aneurysms. While 3D-CTA could not detect six aneurysms that were located in the base of the skull, 3D-BSCTA easily detected them. Moreover, five aneurysms were only partially detected by 3D-CTA. According to this data, the sensitivity of 3D-BSCTA and 3D-CTA was calculated as 98% and 86.3%, respectively; the specificity was calculated as 100% and 90.9%, respectively, per aneurysm; and the sensitivity of 100% for 3D-BSCTA and 98% for 3D-CTA was achieved by using combined images with multi-planar reconstruction (MPR) and maximum intensity projection (MIP). BSCTA detected and characterized the aneurysms as well as DSA, and BSCTA and DSA gave concordant results in detecting aneurysms. Conclusions: BSCTA is

  2. Acute non-traumatic marrow edema syndrome in the knee: MRI findings at presentation, correlation with spinal DEXA and outcome

    Energy Technology Data Exchange (ETDEWEB)

    Karantanas, Apostolos H. [Department of Radiology, University of Crete, Heraklion 711 10 Greece (Greece)], E-mail: apolsen@yahoo.com; Drakonaki, Elena [Department of Radiology, University of Crete, Heraklion 711 10 Greece (Greece); Karachalios, Theophilos [Department of Orthopaedic Surgery, University of Thessaly, Larissa 411 10 Greece (Greece); Korompilias, Anastasios V. [Department of Orthopaedic Surgery, University of Ioannina, Ioannina 451 10 (Greece); Malizos, Konstantinos [Department of Orthopaedic Surgery, University of Thessaly, Larissa 411 10 Greece (Greece)

    2008-07-15

    Purpose: The aim of the study was to present the MRI findings of non-traumatic edema-like lesions presented acutely in the adult knee and to correlate them with the 3-year outcome and the bone mineral density (BMD) in the spine. Materials and methods: Ninety-eight patients (40 men, 58 women, mean age 60.1 {+-} 11 years, age range 27-82 years), were followed up clinically as well as with MR imaging, when indicated, for at least 3 years. Patients were classified according to presentation in 3 groups (A: bone marrow edema (BME), B: BME and subchondral fracture, C: BME and articular collapse) and according to outcome in 2 groups (A: reversible BME, B: articular collapse). BMD measurements of the spine were carried out in males over 70 and females over 60 years old using DEXA. Results: The isolated BME pattern was observed in 64.3% (Group A), subchondral fractures without articular collapse in 11.2% (Group B) and articular collapse in 24.5% (Group C). Significant differences were found among the 3 groups at presentation, regarding the age, sex, BMD, affected area and duration of symptoms prior to imaging (p < 0.05). Localization of the lesions in the weight-bearing areas of the knee was shown in 100% of C, in 90.9% of B and in 50.8% of A. The duration of symptoms prior to imaging was longer in C (7.6 {+-} 2.8 m) than in A (2.5 {+-} 1.7 m) and B (4.0 {+-} 3.2 m) (p < 0.05). Group B progressed to articular collapse in 45.5%, the rest demonstrating a favourable outcome. Group C showed clinical improvement in 75% and persistent symptoms that required knee arthroplasty in 25% of cases. Articular collapse was the final outcome in 29.6% and transient BME in 70.4% of patients. These two groups showed significant differences regarding the age (p {approx} 0), sex (p = 0.002), low BMD (p = 0.004), affected area (p {approx} 0), presence of subchondral sparing (p {approx} 0), duration of symptoms prior to imaging (p {approx} 0), time from onset of symptoms to the final outcome (p

  3. Acute non-traumatic marrow edema syndrome in the knee: MRI findings at presentation, correlation with spinal DEXA and outcome

    International Nuclear Information System (INIS)

    Karantanas, Apostolos H.; Drakonaki, Elena; Karachalios, Theophilos; Korompilias, Anastasios V.; Malizos, Konstantinos

    2008-01-01

    Purpose: The aim of the study was to present the MRI findings of non-traumatic edema-like lesions presented acutely in the adult knee and to correlate them with the 3-year outcome and the bone mineral density (BMD) in the spine. Materials and methods: Ninety-eight patients (40 men, 58 women, mean age 60.1 ± 11 years, age range 27-82 years), were followed up clinically as well as with MR imaging, when indicated, for at least 3 years. Patients were classified according to presentation in 3 groups (A: bone marrow edema (BME), B: BME and subchondral fracture, C: BME and articular collapse) and according to outcome in 2 groups (A: reversible BME, B: articular collapse). BMD measurements of the spine were carried out in males over 70 and females over 60 years old using DEXA. Results: The isolated BME pattern was observed in 64.3% (Group A), subchondral fractures without articular collapse in 11.2% (Group B) and articular collapse in 24.5% (Group C). Significant differences were found among the 3 groups at presentation, regarding the age, sex, BMD, affected area and duration of symptoms prior to imaging (p < 0.05). Localization of the lesions in the weight-bearing areas of the knee was shown in 100% of C, in 90.9% of B and in 50.8% of A. The duration of symptoms prior to imaging was longer in C (7.6 ± 2.8 m) than in A (2.5 ± 1.7 m) and B (4.0 ± 3.2 m) (p < 0.05). Group B progressed to articular collapse in 45.5%, the rest demonstrating a favourable outcome. Group C showed clinical improvement in 75% and persistent symptoms that required knee arthroplasty in 25% of cases. Articular collapse was the final outcome in 29.6% and transient BME in 70.4% of patients. These two groups showed significant differences regarding the age (p ∼ 0), sex (p = 0.002), low BMD (p = 0.004), affected area (p ∼ 0), presence of subchondral sparing (p ∼ 0), duration of symptoms prior to imaging (p ∼ 0), time from onset of symptoms to the final outcome (p ∼ 0) and need for

  4. Patient-reported outcomes and adult patients' disease experience in the idiopathic inflammatory myopathies. report from the OMERACT 11 Myositis Special Interest Group.

    Science.gov (United States)

    Alexanderson, Helene; Del Grande, Maria; Bingham, Clifton O; Orbai, Ana-Maria; Sarver, Catherine; Clegg-Smith, Katherine; Lundberg, Ingrid E; Song, Yeong Wook; Christopher-Stine, Lisa

    2014-03-01

    The newly formed Outcome Measures in Rheumatology (OMERACT) Myositis Special Interest Group (SIG) was established to examine patient-reported outcome measures (PROM) in myositis. At OMERACT 11, a literature review of PROM used in the idiopathic inflammatory myopathies (IIM) and other neuromuscular conditions was presented. The group examined in more detail 2 PROM more extensively evaluated in patients with IIM, the Myositis Activities Profile, and the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire, through the OMERACT filter of truth, discrimination, and feasibility. Preliminary results from a qualitative study of patients with myositis regarding their symptoms were discussed that emphasized the range of symptoms experienced: pain, physical tightness/stiffness, fatigue, disease effect on emotional life and relationships, and treatment-related side effects. Following discussion of these results and following additional discussions since OMERACT 11, a research agenda was developed. The next step in evaluating PROM in IIM will require additional focus groups with a spectrum of patients with different myositis disease phenotypes and manifestations across a range of disease activity, and from multiple international settings. The group will initially focus on dermatomyositis and polymyositis in adults. Qualitative analysis will facilitate the identification of commonalities and divergent patient-relevant aspects of disease, insights that are critical given the heterogeneous manifestations of these diseases. Based on these qualitative studies, existing myositis PROM can be examined to more thoroughly assess content validity, and will be important to identify gaps in domain measurement that will be required to develop a preliminary core set of patient-relevant domains for IIM.

  5. Aneurysmal bone cyst and other nonneoplastic conditions

    International Nuclear Information System (INIS)

    Dahlin, D.C.; McLeod, R.A.

    1982-01-01

    Aneurysmal bone cyst is a benign proliferative tumefaction of bone. Histologic similarities indicate a kinship among classic aneurysmal bone cysts, essentially 'solid' proliferative lesions in bones; giant cell reparative granulomas of the jaws, at the base of the skull, and in the small bones of the hands and feet; skeletal lesions of hyperparathyroidism; and even pseudosarcomatous myositis ossificans, proliferative myositis, and proliferative fasciitis. (orig.)

  6. Parents' perception of self-advocacy of children with myositis: an anonymous online survey

    Directory of Open Access Journals (Sweden)

    Huber Adam M

    2011-06-01

    Full Text Available Abstract Background Children with complex medical issues experience barriers to the transition of care from pediatric to adult providers. We sought to identify these barriers by elucidating the experiences of patients with idiopathic inflammatory muscle disorders. Methods We collected anonymous survey data using an online website. Patients and their families were solicited from the US and Canada through established clinics for children with idiopathic inflammatory muscle diseases as well as with the aid of a nonprofit organization for the benefit of such individuals. The parents of 45 older children/young adults suffering from idiopathic inflammatory muscle diseases were surveyed. As a basis of comparison, we similarly collected data from the parents of 207 younger children with inflammatory muscle diseases. The survey assessed transition of care issues confronting families of children and young adults with chronic juvenile myositis. Results Regardless of age of the patient, respondents were unlikely to have a designated health care provider assigned to aid in transition of care and were unlikely to be aware of a posted policy concerning transition of care at their pediatrician's office. Additionally, regardless of age, patients and their families were unlikely to have a written plan for moving to adult care. Conclusions We identified deficiencies in the health care experiences of families as pertain to knowledge, self-advocacy, policy, and vocational readiness. Moreover, as children with complex medical issues grow up, parents attribute less self-advocacy to their children's level of independence.

  7. A further patient with parasitic myositis due to Haycocknema perplexum, a rare entity.

    Science.gov (United States)

    McKelvie, Penelope; Reardon, Katrina; Bond, Katherine; Spratt, David M; Gangell, Andrew; Zochling, Jane; Daffy, John

    2013-07-01

    A new genus of nematode, Haycocknema perplexum, causing polymyositis in humans, was first described in two Australian patients from Tasmania in 1998. Three patients with myositis due to the same nematode were reported from northern Queensland in 2008. We report the sixth case from Australia, a 50-year-old man, also from Tasmania. He had a 2-year history of progressive weakness, weight loss of 10 kg and dysphagia. Muscle biopsy was initially interpreted as polymyositis with eosinophils. Maximum creatine kinase (CK) level was 5700 U/L and full blood examination was normal. He deteriorated after several months of treatment with prednisolone and methotrexate and review of the muscle biopsy showed intramyofibre parasites of H. perplexum. After 3 months of treatment with albendazole therapy, he made a very good clinical recovery and his CK decreased to 470 U/L. This uniquely Australian parasite can mimic polymyositis and leads to significant irreversible morbidity (two of the previous patients still have weakness and elevated CK after years) and even mortality (one died), if diagnosed late or after corticosteroids. Diagnosis can only be made by histopathology of muscle biopsy. Copyright © 2012 Elsevier Ltd. All rights reserved.

  8. Miositis osificante de los aductores de la cadera Ossifying myositis of hip adductors

    Directory of Open Access Journals (Sweden)

    Feliberto Blanco Trujillo

    2011-08-01

    Full Text Available Se presenta el caso clínico de un paciente de 24 años de edad, asistido en la Escuela de Trabajadores Sociales de Santiago de Cuba por aumento de volumen en la cadera izquierda y limitación funcional total, no dolorosa, de esa parte del cuerpo. Se comprobó clínica y radiográficamente que se trataba de una miositis osificante de los aductores de la cadera, pero no pudo ser operado en esta provincia porque regresó a su país natal.The clinical case of a 24-year-old patient attended at Social Workers School in Santiago de Cuba due to increased left hip size and total functional limitation without pain of that body area was presented. It was clinically and radiographically documented that the diagnosis was ossifying myositis of hip adductors. However, surgery could not be done in that province because the patient travelled back to his country.

  9. Chaperone-mediated autophagy components are upregulated in sporadic inclusion-body myositis muscle fibres.

    Science.gov (United States)

    Cacciottolo, M; Nogalska, A; D'Agostino, C; Engel, W K; Askanas, V

    2013-12-01

    Sporadic inclusion-body myositis (s-IBM) is an age-associated degenerative muscle disease. Characteristic features are muscle-fibre vacuolization and intramuscle-fibre accumulations of multiprotein aggregates, which may result from the demonstrated impairments of the 26S proteasome and autophagy. Chaperone-mediated autophagy (CMA) is a selective form of lysosomal degradation targeting proteins carrying the KFERQ motif. Lysosome-associated membrane protein type 2A (LAMP2A) and the heat-shock cognate protein 70 (Hsc70) constitute specific CMA components. Neither CMA components nor CMA activity has been studied in normal or disease human muscle, to our knowledge. We studied CMA components by immunocytochemistry, immunoblots, real-time PCR and immunoprecipitation in: (a) 16 s-IBM, nine aged-matched normal and nine disease control muscle biopsies; and (b) cultured human muscle fibres (CHMFs) with experimentally inhibited activities of either the 26S proteasome or autophagy. Compared with age-matched controls, in s-IBM muscle, LAMP2A and Hsc70 were on a given transverse section accumulated as aggregates in approximately 5% of muscle fibres, where they (a) colocalized with each other and α-synuclein (α-syn), a CMA-targeted protein; and (b) were bound to each other and to α-syn by immunoprecipitation. By immunoblots, LAMP2A was increased sevenfold P pathogenic aspect in s-IBM. © 2013 British Neuropathological Society.

  10. A rapid and simple chemiluminescence method for screening levels of inosine and hypoxanthine in non-traumatic chest pain patients.

    Science.gov (United States)

    Farthing, Don E; Sica, Domenic; Hindle, Michael; Edinboro, Les; Xi, Lei; Gehr, Todd W B; Gehr, Lynne; Farthing, Christine A; Larus, Terri L; Fakhry, Itaf; Karnes, H Thomas

    2011-01-01

    A rapid and simple chemiluminescence method was developed for detection of inosine and hypoxanthine in human plasma. The method utilized a microplate luminometer with direct injectors to automatically dispense reagents during sample analysis. Enzymatic conversions of inosine to hypoxanthine, followed by hypoxanthine to xanthine to uric acid, generated superoxide anion radicals as a useful metabolic by-product. The free radicals react with Pholasin(®) , a sensitive photoprotein used for chemiluminescence detection, to produce measurable blue-green light. The use of Pholasin(®) and a chemiluminescence signal enhancer, Adjuvant-K™, eliminated the need for plasma clean-up steps prior to analysis. The method used 20 μL of heparinized plasma, with complete analysis of total hypoxanthine levels (inosine is metabolized to hypoxanthine using purine nucleoside phosphorylase) in approximately 3.7 min. The rapid chemiluminescence method demonstrated the capability of differentiating total hypoxanthine levels between healthy individuals, and patients presenting with non-traumatic chest pain and potential acute cardiac ischemia. The results support the potential use of chemiluminescence methodology as a diagnostic tool to rapidly screen for elevated levels of inosine and hypoxanthine in human plasma, potential biomarkers of acute cardiac ischemia. Copyright © 2009 John Wiley & Sons, Ltd.

  11. The characteristics of chronic pain after non-traumatic, non-compressive myelopathy: Focus on neuropathic pain.

    Science.gov (United States)

    Eom, Young In; Kim, Min; Joo, In Soo

    2017-05-01

    The aim of this study was to assess the characteristics of neuropathic pain after non-traumatic, non-compressive (NTNC) myelopathy and find potential predictors for neuropathic pain. We analyzed 54 patients with NTNC myelopathy. The Short Form McGill Pain Questionnaire (SF-MPQ) and the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) were used to assess pain. Health-related QOL was evaluated by the Short Form 36-item (SF-36) health survey. Out of 48 patients with pain, 16 (33.3%) patients experienced neuropathic pain. Mean age was significantly lower in patients with neuropathic pain than in patients with non-neuropathic pain (39.1 ± 12.5 vs. 49.8 ± 9.3, P = 0.002). There were no statistically significant differences in the other variables including sex, etiology of myelopathy, pain and QOL scores between the two groups. A binary logistic regression revealed that onset age under 40, and non-idiopathic etiology were independent predictors of the occurrence of neuropathic pain. Both SF-MPQ and LANSS scores were significantly correlated with SF-36 scores, adjusted by age, sex, presence of diabetes mellitus, and current EDSS scores (r = -0.624, P Neuropathic pain must be one of serious complications in patients with NTNC myelopathy and also affects their quality of life. Onset age and etiology of myelopathy are important factors in the development of neuropathic pain in NTNC myelopathy.

  12. OUTPATIENT PHYSICAL THERAPY EVALUATION AND TREATMENT OF A PATIENT DIAGNOSED WITH SPORADIC INCLUSION BODY MYOSITIS: A CASE STUDY

    Directory of Open Access Journals (Sweden)

    Tyler Harrigfeld

    2017-08-01

    Full Text Available Background: Sporadic inclusion body myositis is an autoimmune and degenerative disorder of skeletal muscle that affects people at random. It most commonly begins as progressive weakness and atrophy of lower extremity musculature, beginning with the proximal leg. These impairments in body structure adversely affect the performance of functional activities and mobility, resulting in a progressive decrease in independence and participation both at home and in the community. Physical therapy attempts to minimize these effects through educational and procedural interventions focused on treating impairments and limitations. The purpose of this case study was to provide a description of the physical therapy management of a patient diagnosed with sporadic inclusion body myositis. Case Summary: The patient was a 66-year-old male who was diagnosed with sporadic inclusion body myositis with a chief complaint of weakness and fall risk. He presented with generalized lower extremity weakness and atrophy of bilateral quadriceps, as well as impaired balance and increasing fatigue with activity. Therapeutic exercise, home exercise program, balance, gait, and stair training were delivered to address these impairments. Patient outcomes showed improvement in balance and safety with functional activities. Discussion: The patient was seen for seven visits that were 45 – 60 minutes in length, over a five-week period. The patient made subjective reports of improvement in functional activities and balance; however many objective outcome measures could not be reassessed. There is a need for further research on this population to determine the effectiveness and parameters of physical therapy interventions. Conclusion: Physical therapy may have helped improve balance as well as subjective reports from the patient of increased feeling of confidence while navigating stairs.

  13. Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain.

    Science.gov (United States)

    Nuño-Nuño, Laura; Joven, Beatriz Esther; Carreira, Patricia E; Maldonado-Romero, Valentina; Larena-Grijalba, Carmen; Cubas, Irene Llorente; Tomero, Eva Gloria; Barbadillo-Mateos, María Carmen; De la Peña Lefebvre, Paloma García; Ruiz-Gutiérrez, Lucía; López-Robledillo, Juan Carlos; Moruno-Cruz, Henry; Pérez, Ana; Cobo-Ibáñez, Tatiana; Almodóvar González, Raquel; Lojo, Leticia; García De Yébenes, María Jesús; López-Longo, Francisco Javier

    2017-11-01

    The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9.7 years. In the overall cohort, the 2-, 5-, and 10-year survival probabilities were 91.9, 86.7, and 77%, respectively. Main causes of death were infections and cancer (24% each). Multivariate model revealed that CAM (HR = 24.06), OM (HR = 12.00), DM (HR = 7.26), higher age at diagnosis (HR = 1.02), severe infections (HR = 3.66), interstitial lung disease (HR = 1.61), and baseline elevation of acute phase reactants (HR = 3.03) were associated with a worse prognosis, while edema of the hands (HR = 0.39), female gender (HR = 0.39), and longer disease duration (HR = 0.73) were associated with a better prognosis. The standardized mortality ratio was 1.56 (95% CI 1.28-1.87) compared to the Spanish general population. Our findings indicate that IIM has a high long-term mortality, with an excess of mortality compared to the Spanish population. A more aggressive therapy may be required in IIM patients presenting with poor predictive factors.

  14. Automated diagnosis of myositis from muscle ultrasound: Exploring the use of machine learning and deep learning methods.

    Science.gov (United States)

    Burlina, Philippe; Billings, Seth; Joshi, Neil; Albayda, Jemima

    2017-01-01

    To evaluate the use of ultrasound coupled with machine learning (ML) and deep learning (DL) techniques for automated or semi-automated classification of myositis. Eighty subjects comprised of 19 with inclusion body myositis (IBM), 14 with polymyositis (PM), 14 with dermatomyositis (DM), and 33 normal (N) subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally) were acquired. We considered three problems of classification including (A) normal vs. affected (DM, PM, IBM); (B) normal vs. IBM patients; and (C) IBM vs. other types of myositis (DM or PM). We studied the use of an automated DL method using deep convolutional neural networks (DL-DCNNs) for diagnostic classification and compared it with a semi-automated conventional ML method based on random forests (ML-RF) and "engineered" features. We used the known clinical diagnosis as the gold standard for evaluating performance of muscle classification. The performance of the DL-DCNN method resulted in accuracies ± standard deviation of 76.2% ± 3.1% for problem (A), 86.6% ± 2.4% for (B) and 74.8% ± 3.9% for (C), while the ML-RF method led to accuracies of 72.3% ± 3.3% for problem (A), 84.3% ± 2.3% for (B) and 68.9% ± 2.5% for (C). This study demonstrates the application of machine learning methods for automatically or semi-automatically classifying inflammatory muscle disease using muscle ultrasound. Compared to the conventional random forest machine learning method used here, which has the drawback of requiring manual delineation of muscle/fat boundaries, DCNN-based classification by and large improved the accuracies in all classification problems while providing a fully automated approach to classification.

  15. Automated diagnosis of myositis from muscle ultrasound: Exploring the use of machine learning and deep learning methods.

    Directory of Open Access Journals (Sweden)

    Philippe Burlina

    Full Text Available To evaluate the use of ultrasound coupled with machine learning (ML and deep learning (DL techniques for automated or semi-automated classification of myositis.Eighty subjects comprised of 19 with inclusion body myositis (IBM, 14 with polymyositis (PM, 14 with dermatomyositis (DM, and 33 normal (N subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally were acquired. We considered three problems of classification including (A normal vs. affected (DM, PM, IBM; (B normal vs. IBM patients; and (C IBM vs. other types of myositis (DM or PM. We studied the use of an automated DL method using deep convolutional neural networks (DL-DCNNs for diagnostic classification and compared it with a semi-automated conventional ML method based on random forests (ML-RF and "engineered" features. We used the known clinical diagnosis as the gold standard for evaluating performance of muscle classification.The performance of the DL-DCNN method resulted in accuracies ± standard deviation of 76.2% ± 3.1% for problem (A, 86.6% ± 2.4% for (B and 74.8% ± 3.9% for (C, while the ML-RF method led to accuracies of 72.3% ± 3.3% for problem (A, 84.3% ± 2.3% for (B and 68.9% ± 2.5% for (C.This study demonstrates the application of machine learning methods for automatically or semi-automatically classifying inflammatory muscle disease using muscle ultrasound. Compared to the conventional random forest machine learning method used here, which has the drawback of requiring manual delineation of muscle/fat boundaries, DCNN-based classification by and large improved the accuracies in all classification problems while providing a fully automated approach to classification.

  16. Injection of Botulinum Toxin a to Upper Esophageal Sphincter for Oropharyngeal Dysphagia in Two Patients with Inclusion Body Myositis

    Directory of Open Access Journals (Sweden)

    Louis WC Liu

    2004-01-01

    Full Text Available Inclusion body myositis (IBM is a progressive degenerative skeletal muscle disease leading to weakening and atrophy of both proximal and distal muscles. Dysphagia is reported in up to 86% of IBM patients. Surgical cricopharyngeal myotomy may be effective for cricopharyngeal dysphagia and there is one published report that botulinum toxin A, injected into the cricopharyngeus muscle using a hypopharyngoscope under general anesthesia, relieved IBM-associated dysphagia. This report presents the first documentation of botulinum toxin A injection into the upper esophageal sphincter using a flexible esophagogastroduodenoscope under conscious sedation, to reduce upper esophageal sphincter pressure and successfully alleviate oropharyngeal dysphagia in two IBM patients.

  17. Proposed criteria to differentiate heterogeneous eosinophilic gastrointestinal disorders of the esophagus, including eosinophilic esophageal myositis

    Science.gov (United States)

    Sato, Hiroki; Nakajima, Nao; Takahashi, Kazuya; Hasegawa, Go; Mizuno, Ken-ichi; Hashimoto, Satoru; Ikarashi, Satoshi; Hayashi, Kazunao; Honda, Yutaka; Yokoyama, Junji; Sato, Yuichi; Terai, Shuji

    2017-01-01

    AIM To define clinical criteria to differentiate eosinophilic gastrointestinal disorder (EoGD) in the esophagus. METHODS Our criteria were defined based on the analyses of the clinical presentation of eosinophilic esophagitis (EoE), subepithelial eosinophilic esophagitis (sEoE) and eosinophilic esophageal myositis (EoEM), identified by endoscopy, manometry and serum immunoglobulin E levels (s-IgE), in combination with histological and polymerase chain reaction analyses on esophageal tissue samples. RESULTS In five patients with EoE, endoscopy revealed longitudinal furrows and white plaques in all, and fixed rings in two. In one patient with sEoE and four with EoEM, endoscopy showed luminal compression only. Using manometry, failed peristalsis was observed in patients with EoE and sEoE with some variation, while EoEM was associated with hypercontractile or hypertensive peristalsis, with elevated s-IgE. Histology revealed the following eosinophils per high-power field values. EoE = 41.4 ± 7.9 in the epithelium and 2.3 ± 1.5 in the subepithelium; sEoE = 3 in the epithelium and 35 in the subepithelium (conventional biopsy); EoEM = none in the epithelium, 10.7 ± 11.7 in the subepithelium (conventional biopsy or endoscopic mucosal resection) and 46.8 ± 16.5 in the muscularis propria (peroral esophageal muscle biopsy). Presence of dilated epithelial intercellular space and downward papillae elongation were specific to EoE. Eotaxin-3, IL-5 and IL-13 were overexpressed in EoE. CONCLUSION Based on clinical and histological data, we identified criteria, which differentiated between EoE, sEoE and EoEM, and reflected a different pathogenesis between these esophageal EoGDs. PMID:28428721

  18. Clinical and laboratory description of a series of cases of acute viral myositis

    Directory of Open Access Journals (Sweden)

    Silvana Paula Cardin

    2015-10-01

    Full Text Available ABSTRACT OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK, lactate dehydrogenase (LDH, transaminases (AST and ALT, blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%, rhinorrhea (23%, and fever (63%, with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%, limited ambulation (57%, gait abnormality (40%, and muscle weakness in the lower limbs (71%, with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507 ± 9180 U/L, LDH (827 ± 598 U/L, and AST (199 ± 245 U/L, with a tendency to leukopenia (4590 ± 1420 leukocytes/mm3. The complete recovery of laboratory parameters was observed in 30 days (median, and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.

  19. Clinical and laboratory description of a series of cases of acute viral myositis.

    Science.gov (United States)

    Cardin, Silvana Paula; Martin, Joelma Gonçalves; Saad-Magalhães, Claudia

    2015-01-01

    Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK), lactate dehydrogenase (LDH), transaminases (AST and ALT), blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys) were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%), rhinorrhea (23%), and fever (63%), with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%), limited ambulation (57%), gait abnormality (40%), and muscle weakness in the lower limbs (71%), with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507±9180U/L), LDH (827±598U/L), and AST (199±245U/L), with a tendency to leukopenia (4590±1420) leukocytes/mm(3). The complete recovery of laboratory parameters was observed in 30 days (median), and laboratory and clinical recurrence was documented in one case after 10 months. Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  20. Nivolumab-related myasthenia gravis with myositis and myocarditis in Japan.

    Science.gov (United States)

    Suzuki, Shigeaki; Ishikawa, Nobuhisa; Konoeda, Fumie; Seki, Nobuhiko; Fukushima, Satoshi; Takahashi, Kikuko; Uhara, Hisashi; Hasegawa, Yoshikazu; Inomata, Shinichiro; Otani, Yasushi; Yokota, Kenji; Hirose, Takashi; Tanaka, Ryo; Suzuki, Norihiro; Matsui, Makoto

    2017-09-12

    To report the clinical features of myasthenia gravis (MG) induced by treatment with immune checkpoint inhibitors using 2-year safety databases based on postmarketing surveys in Japan. We studied 10,277 patients with cancer who had received monotherapy with either nivolumab or ipilimumab between September 2014 and August 2016. As the control group, 105 patients with idiopathic MG were used. There were 12 MG cases (0.12%) among 9,869 patients with cancer who had been treated with nivolumab, but none among 408 patients treated with ipilimumab. These 12 patients included 6 men and 6 women with a mean age of 73.5 ± 6.3 years. MG onset occurred in the early phase after nivolumab treatment and rapidly deteriorated. Nivolumab-related MG (nivoMG) included 4 patients with mild involvement and 8 patients with severe involvement. Bulbar symptoms and myasthenic crisis were observed more frequently in nivoMG than idiopathic MG. Ten patients were positive for anti-acetylcholine receptor antibodies. Serum creatine kinase levels were markedly elevated to an average level of 4,799 IU/L. Among the 12 patients with nivoMG, 4 had myositis and 3 had myocarditis, with 1 of these patients having both. Immunosuppressive therapy was effective. Postintervention status showed that pharmacologic remission or minimal manifestations were obtained in 4 patients; however, 2 patients died. Immune-related adverse events triggered by nivolumab impaired the patients' daily living activity. The prompt and correct recognition of MG following treatment with immune checkpoint inhibitors in patients with cancer is important. © 2017 American Academy of Neurology.

  1. Proposed criteria to differentiate heterogeneous eosinophilic gastrointestinal disorders of the esophagus, including eosinophilic esophageal myositis.

    Science.gov (United States)

    Sato, Hiroki; Nakajima, Nao; Takahashi, Kazuya; Hasegawa, Go; Mizuno, Ken-Ichi; Hashimoto, Satoru; Ikarashi, Satoshi; Hayashi, Kazunao; Honda, Yutaka; Yokoyama, Junji; Sato, Yuichi; Terai, Shuji

    2017-04-07

    To define clinical criteria to differentiate eosinophilic gastrointestinal disorder (EoGD) in the esophagus. Our criteria were defined based on the analyses of the clinical presentation of eosinophilic esophagitis (EoE), subepithelial eosinophilic esophagitis (sEoE) and eosinophilic esophageal myositis (EoEM), identified by endoscopy, manometry and serum immunoglobulin E levels (s-IgE), in combination with histological and polymerase chain reaction analyses on esophageal tissue samples. In five patients with EoE, endoscopy revealed longitudinal furrows and white plaques in all, and fixed rings in two. In one patient with sEoE and four with EoEM, endoscopy showed luminal compression only. Using manometry, failed peristalsis was observed in patients with EoE and sEoE with some variation, while EoEM was associated with hypercontractile or hypertensive peristalsis, with elevated s-IgE. Histology revealed the following eosinophils per high-power field values. EoE = 41.4 ± 7.9 in the epithelium and 2.3 ± 1.5 in the subepithelium; sEoE = 3 in the epithelium and 35 in the subepithelium (conventional biopsy); EoEM = none in the epithelium, 10.7 ± 11.7 in the subepithelium (conventional biopsy or endoscopic mucosal resection) and 46.8 ± 16.5 in the muscularis propria (peroral esophageal muscle biopsy). Presence of dilated epithelial intercellular space and downward papillae elongation were specific to EoE. Eotaxin-3, IL-5 and IL-13 were overexpressed in EoE. Based on clinical and histological data, we identified criteria, which differentiated between EoE, sEoE and EoEM, and reflected a different pathogenesis between these esophageal EoGDs.

  2. Mortality and Causes of Death in Patients with Sporadic Inclusion Body Myositis: Survey Study Based on the Clinical Experience of Specialists in Australia, Europe and the USA

    NARCIS (Netherlands)

    Price, Mark A.; Barghout, Victoria; Benveniste, Olivier; Christopher-Stine, Lisa; Corbett, Alastair; de Visser, Marianne; Hilton-Jones, David; Kissel, John T.; Lloyd, Thomas E.; Lundberg, Ingrid E.; Mastaglia, Francis; Mozaffar, Tahseen; Needham, Merrilee; Schmidt, Jens; Sivakumar, Kumaraswamy; DeMuro, Carla; Tseng, Brian S.

    2016-01-01

    There is a paucity of data on mortality and causes of death (CoDs) in patients with sporadic inclusion body myositis (sIBM), a rare, progressive, degenerative, inflammatory myopathy that typically affects those aged over 50 years. Based on patient records and expertise of clinical specialists, this

  3. Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study.

    Science.gov (United States)

    Picard, Cécile; Vincent, Thierry; Lega, Jean-Christophe; Hue, Sophie; Fortenfant, Françoise; Lakomy, Daniela; Humbel, René-Louis; Goetz, Joelle; Molinari, Nicolas; Bardin, Nathalie; Bertin, Daniel; Johanet, Catherine; Chretien, Pascale; Dubucquoi, Sylvain; Streichenberger, Nathalie; Desplat-Jégo, Sophie; Bossuyt, Xavier; Sibilia, Jean; Abreu, Isabelle; Chevailler, Alain; Fabien, Nicole

    2016-06-01

    Anti-signal recognition particle (SRP) antibodies are important serological markers for the diagnosis and the prognosis of idiopathic inflammatory myopathy (IIM), especially to distinguish immune-mediated necrotizing myopathy (IMNM). This study was set up to investigate the phenotype associated with anti-SRP antibodies and to evaluate the methods for detecting these antibodies. Clinical and biological data were retrospectively obtained from 60 adult patients with anti-SRP antibodies detected by a dot immunoassay from 12 centers. Thirty-six (60 %) out of these 60 patients suffered from an IIM, and among them, 21 patients were diagnosed as IMNM. Among patients with a definite IIM, proximal weakness and myalgia were prominent symptoms at the time of diagnosis. Only few patients displayed severe extra-muscular symptoms such as cardiac involvement or severe myositis. Mean creatine kinase levels were high for all patients except for two of them. When testing by indirect immunofluorescence (IIF) on HEp2 cells, the fraction of patients displaying the typical anti-SRP fine speckled staining of the cytoplasm was higher in patients with IIM (30/36) (83 %) than in patients with non-IIM (3/24) (12.5 %) (p myositis, whereas only 6 (22 %) out of 27 patients with a positive immunodot but a negative cytoplasmic pattern suffered from a myositis (p myositis.

  4. Effects of blood-flow-restricted resistance training on muscle function in a 74-year-old male with sporadic inclusion body myositis

    DEFF Research Database (Denmark)

    Jørgensen, Anders Nørkær; Aagaard, P; Nielsen, J L

    2016-01-01

    Sporadic inclusion body myositis (sIBM) is a systemic disease that is characterized by substantial skeletal muscle weakness and muscle inflammation, leading to impaired physical function. The objective was to investigate the effect of low-load resistance exercise with concurrent partial blood flow...

  5. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes.

    Science.gov (United States)

    Miller, F W; Chen, W; O'Hanlon, T P; Cooper, R G; Vencovsky, J; Rider, L G; Danko, K; Wedderburn, L R; Lundberg, I E; Pachman, L M; Reed, A M; Ytterberg, S R; Padyukov, L; Selva-O'Callaghan, A; Radstake, T R; Isenberg, D A; Chinoy, H; Ollier, W E R; Scheet, P; Peng, B; Lee, A; Byun, J; Lamb, J A; Gregersen, P K; Amos, C I

    2015-10-01

    Autoimmune muscle diseases (myositis) comprise a group of complex phenotypes influenced by genetic and environmental factors. To identify genetic risk factors in patients of European ancestry, we conducted a genome-wide association study (GWAS) of the major myositis phenotypes in a total of 1710 cases, which included 705 adult dermatomyositis, 473 juvenile dermatomyositis, 532 polymyositis and 202 adult dermatomyositis, juvenile dermatomyositis or polymyositis patients with anti-histidyl-tRNA synthetase (anti-Jo-1) autoantibodies, and compared them with 4724 controls. Single-nucleotide polymorphisms showing strong associations (Pmyositis phenotypes together, as well as for the four clinical and autoantibody phenotypes studied separately. Imputation and regression analyses found that alleles comprising the human leukocyte antigen (HLA) 8.1 ancestral haplotype (AH8.1) defined essentially all the genetic risk in the phenotypes studied. Although the HLA DRB1*03:01 allele showed slightly stronger associations with adult and juvenile dermatomyositis, and HLA B*08:01 with polymyositis and anti-Jo-1 autoantibody-positive myositis, multiple alleles of AH8.1 were required for the full risk effects. Our findings establish that alleles of the AH8.1 comprise the primary genetic risk factors associated with the major myositis phenotypes in geographically diverse Caucasian populations.

  6. The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome.

    Science.gov (United States)

    Lega, Jean-Christophe; Fabien, Nicole; Reynaud, Quitterie; Durieu, Isabelle; Durupt, Stéphane; Dutertre, Marine; Cordier, Jean-François; Cottin, Vincent

    2014-09-01

    To describe the clinical spectrum associated with aminoacyl-transfer RNA synthetase (ARS) autoantibodies in patients with idiopathic inflammatory myositis defined according to Peter and Bohan's criteria. Cohort studies were selected from MEDLINE and Embase up to August 2013. Two investigators independently extracted data on study design, patient characteristics, and clinical features (interstitial lung disease [ILD], fever, mechanic's hands [MH], Raynaud's phenomenon [RPh], arthralgia, sclerodactyly, cancer and dermatomyositis-specific rash) according to the presence of myositis-specific (anti-aminoacyl-transfer RNA synthetase [ARS], anti-signal recognition particle [anti-SRP] and anti-Mi2) and myositis-associated (anti-PM/Scl, anti-U1-RNP and anti-Ku) autoantibodies. 27 studies (3487 patients) were included in the meta-analysis. Arthralgia (75%, CI 67-81) and ILD (69%, CI 63-74) were the most prevalent clinical signs associated with anti-ARS autoantibodies. Anti-Mi2 and anti-SRP autoantibodies were associated with few extramuscular signs. ARS autoantibodies were identified in 13% of patients with cancer-associated myositis (5-25). Patients with non-anti-Jo1 ARS had greater odds of presenting fever (RR 0.63, CI 0.52-0.90) and ILD (RR 0.87, CI 0.81-0.93) compared to those with anti-Jo1 autoantibodies. The frequencies of myositis (RR 1.60, CI 1.38-1.85), arthralgia (RR 1.52, CI 1.32-1.76) and MH (RR 1.47, CI 1.11-1.94) were almost 50% higher in patients with anti-Jo1 compared to non-anti-Jo1 ARS autoantibodies. Patients with anti-PM/Scl differed from those with anti-ARS autoantibodies by a greater prevalence of RPh (RR 0.70, CI 0.53-0.94) and sclerodactyly (RR 0.47, CI 0.25-0.89). ILD was less frequent in patients with anti-U1-RNP autoantibodies (RR 3.35, CI 1.07-10.43). No difference was observed between anti-ARS and myositis-associated autoantibodies for other outcomes. The presence of anti-ARS autoantibodies delimits a heterogeneous subset of patients with a high

  7. TLR4-HMGB1 signaling pathway affects the inflammatory reaction of autoimmune myositis by regulating MHC-I.

    Science.gov (United States)

    Wan, Zemin; Zhang, Xiujuan; Peng, Anping; He, Min; Lei, Zhenhua; Wang, Yunxiu

    2016-12-01

    To analyze the effects of TLR4 on the expression of the HMGB1, MHC-I and downstream cytokines IL-6 and TNF-α, and to investigate the biological role of the TLR4-HMGB1 signaling pathway in the development of the autoimmune myositis. We built mice models with experimental autoimmune myositis (EAM) and used the inverted screen experiment to measure their muscle endurance; we also examined inflammatory infiltration of muscle tissues after HE staining; and we assessed the expression of MHC-I using immunohistochemistry. In addition, peripheral blood mononuclear cells (PBMC) were extracted and flow cytometry was utilized to detect the effect of IFN-γ on the expression of MHC-I. Furthermore, PBMCs were treated with IFN-γ, anti-TLR4, anti-HMGB1 and anti-MHC-I. Real-time PCR and western blotting were employed to examine the expressions of TLR4, HMGB1 and MHC-I in different groups. The ELISA method was also utilized to detect the expression of the downstream cytokines TNF-α and IL-6. The expressions of TLR4, HMGB1 and MHC-I in muscle tissues from mice with EAM were significantly higher than those in the control group (all Pmyositis inflammation by regulating the expression of MHC-I and other pro-inflammatory cytokines. Copyright © 2016 Elsevier B.V. All rights reserved.

  8. Is intra-articular pathology associated with MCL edema on MR imaging of the non-traumatic knee?

    International Nuclear Information System (INIS)

    Blankenbaker, Donna G.; De Smet, Arthur A.; Fine, Jason P.

    2005-01-01

    Edema surrounding the medial collateral ligament (MCL) is seen on MR imaging in patients with MCL injuries and in patients with radiographic osteoarthritis in the non-traumatic knee. Because we noted MCL edema in patients without prior trauma or osteoarthritis, we studied the association between intra-articular pathology and MCL edema in patients without knee trauma. We evaluated the MR examinations of 247 consecutive patients (121 male, 126 female with a mean age of 44 years) without recent trauma for the presence of edema surrounding the MCL, meniscal and ACL tears, medial meniscal extrusion, medial compartment chondromalacia, and osteoarthritis. The percentages of patients illustrating MCL edema with and without each type of pathology were compared using Fisher's exact test to determine if there was a statistically significant association. We found MCL edema in 60% of 247 patients. MCL edema was present in 67% of patients with medial meniscal tears, 35% with lateral meniscal tears, 100% with meniscal extrusion of 3 mm or more, 78% with femoral chondromalacia, 82% with tibial chondromalacia, and 50% with osteoarthritis. The percentage of patients with edema increased with the severity of the chondromalacia. These associations were all statistically significant (p <0.02). The mean age of those with MCL edema was 49.7 years compared with 34.9 years without MCL edema (p <0.001). Patient gender and ACL tear did not correlate with MCL edema. Nine (4%) of the 247 patients had MCL edema without intra-articular pathology. None of these 9 patients had MCL tenderness or joint laxity on physical examination. We confirmed that MCL edema is associated with osteoarthritis, but is also associated with meniscal tears, meniscal extrusion, and chondromalacia. In addition, MCL edema can be seen in patients without intra-articular pathology, recent trauma or MCL abnormality on physical examination. (orig.)

  9. Non-traumatic incidental findings in patients undergoing whole-body computed tomography at initial emergency admission.

    Science.gov (United States)

    Kroczek, Eduard K; Wieners, Gero; Steffen, Ingo; Lindner, Tobias; Streitparth, Florian; Hamm, Bernd; Maurer, Martin H

    2017-10-01

    To evaluate the number, localisation and importance of non-traumatic incidental findings (IFs) in patients with suspected or obvious multiple trauma undergoing whole-body CT (WBCT) in a level-1 trauma centre. Between January 2009 and December 2013, a total of 2440 patients with trauma undergoing WBCT at admission to a level-1 trauma centre of a university hospital were retrospectively analysed, through imaging IFs unrelated to trauma with the radiological reports. All IFs were grouped into four categories according to their clinical relevance. Category 1: urgent treatment or further clarification needed; category 2: further examination and follow-up within 3-6 months required; category 3: findings with no immediate consequences for the treatment of the patient but of potential relevance in the future; category 4: harmless findings. Altogether, 5440 IFs in 2440 patients (1735 male, 705 female; mean age 45.1 years) were documented. In 204 patients (8.4%) urgent category 1 findings were reported, 766 patients (31.4%) had category 2 findings, 1236 patients (50.7%) had category 3 findings and 1173 patients (48.1%) had category 4 findings. Most IFs were detected in the abdomen/pelvis (42.5%). 602 (24.7%) of the patients had no IFs. WBCT scans of unrelated trauma patients demonstrate a high rate of IF. A substantial percentage (8.4%) of patients had urgent category 1IFs and a high percentage (31.4%) had category 2 IFs requiring a follow-up. This high number of patients with polytrauma undergoing WBCT, having IFs of high relevance, poses a major challenge for the level-1 trauma centre in the acute and postacute management of these patients. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  10. Is intra-articular pathology associated with MCL edema on MR imaging of the non-traumatic knee?

    Energy Technology Data Exchange (ETDEWEB)

    Blankenbaker, Donna G.; De Smet, Arthur A. [University of Wisconsin Medical School, Division of Musculoskeletal Imaging, Department of Radiology, Madison (United States); Fine, Jason P. [University of Wisconsin, Department of Statistics, Madison (United States); University of Wisconsin, Department of Biostatistics and Informatics, Madison (United States)

    2005-08-01

    Edema surrounding the medial collateral ligament (MCL) is seen on MR imaging in patients with MCL injuries and in patients with radiographic osteoarthritis in the non-traumatic knee. Because we noted MCL edema in patients without prior trauma or osteoarthritis, we studied the association between intra-articular pathology and MCL edema in patients without knee trauma. We evaluated the MR examinations of 247 consecutive patients (121 male, 126 female with a mean age of 44 years) without recent trauma for the presence of edema surrounding the MCL, meniscal and ACL tears, medial meniscal extrusion, medial compartment chondromalacia, and osteoarthritis. The percentages of patients illustrating MCL edema with and without each type of pathology were compared using Fisher's exact test to determine if there was a statistically significant association. We found MCL edema in 60% of 247 patients. MCL edema was present in 67% of patients with medial meniscal tears, 35% with lateral meniscal tears, 100% with meniscal extrusion of 3 mm or more, 78% with femoral chondromalacia, 82% with tibial chondromalacia, and 50% with osteoarthritis. The percentage of patients with edema increased with the severity of the chondromalacia. These associations were all statistically significant (p <0.02). The mean age of those with MCL edema was 49.7 years compared with 34.9 years without MCL edema (p <0.001). Patient gender and ACL tear did not correlate with MCL edema. Nine (4%) of the 247 patients had MCL edema without intra-articular pathology. None of these 9 patients had MCL tenderness or joint laxity on physical examination. We confirmed that MCL edema is associated with osteoarthritis, but is also associated with meniscal tears, meniscal extrusion, and chondromalacia. In addition, MCL edema can be seen in patients without intra-articular pathology, recent trauma or MCL abnormality on physical examination. (orig.)

  11. Management of non-traumatic chest pain by the French Emergency Medical System: Insights from the DOLORES registry.

    Science.gov (United States)

    Manzo-Silberman, Stéphane; Assez, Nathalie; Vivien, Benoît; Tazarourte, Karim; Mokni, Tarak; Bounes, Vincent; Greffet, Agnès; Bataille, Vincent; Mulak, Geneviève; Goldstein, Patrick; Ducassé, Jean Louis; Spaulding, Christian; Charpentier, Sandrine

    2015-03-01

    The early recognition of acute coronary syndromes is a priority in health care systems, to reduce revascularization delays. In France, patients are encouraged to call emergency numbers (15, 112), which are routed to a Medical Dispatch Centre where physicians conduct an interview and decide on the appropriate response. However, the effectiveness of this system has not yet been assessed. To describe and analyse the response of emergency physicians receiving calls for chest pain in the French Emergency Medical System. From 16 November to 13 December 2009, calls to the Medical Dispatch Centre for non-traumatic chest pain were included prospectively in a multicentre observational study. Clinical characteristics and triage decisions were collected. A total of 1647 patients were included in the study. An interview was conducted with the patient in only 30.5% of cases, and with relatives, bystanders or physicians in the other cases. A Mobile Intensive Care Unit was dispatched to 854 patients (51.9%) presenting with typical angina chest pains and a high risk of cardiovascular disease. Paramedics were sent to 516 patients (31.3%) and a general practitioner was sent to 169 patients (10.3%). Patients were given medical advice only by telephone in 108 cases (6.6%). Emergency physicians in the Medical Dispatch Centre sent an effecter to the majority of patients who called the Emergency Medical System for chest pain. The response level was based on the characteristics of the chest pain and the patient's risk profile. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  12. Nontraumatic femoral head necrosis. Classification of bone scintigraphic findings and diagnostic value of SPECT following planar imaging

    Energy Technology Data Exchange (ETDEWEB)

    Minoshima, Satoshi; Uchida, Yoshitaka; Anzai, Yoshimi; Uno, Kimiichi; Arimizu, Noboru [Chiba Univ. (Japan). School of Medicine

    1994-09-01

    This study was conducted to determine bone scintigraphic findings in nontraumatic femoral head avascular necrosis and diagnostic value of SPECT imaging following a conventional planar imaging. Forty-three femoral heads in twenty-six cases with idiopathic femoral head necrosis (n=2), systemic lupus erythematosus (n=22), aplastic anemia (n=1), and renal transplantation (n=1) were studied. The diagnosis for femoral head necrosis was based on magnetic resonance imaging as well as other diagnostic studies in all cases. Scintigraphic findings of planar and SPECT images were classified into six categories: normal (N); cold or decrease (C); partial increase with cold or decrease (PH+C); ring-like increase with a cold center (RH+C); partial increase (PH); diffuse and/or irregular increase (DH). Avascular necrosis was confirmed in twenty-four femoral heads, in which planar and SPECT images showed scintigraphic findings of N (n=3, 2), C (n=1, 3), PH+C (n=2, 8), RH+C (n=2, 3), PH (n=9, 2), and DH (n=7, 6), respectively. Femoral heads without avascular necrosis demonstrated planar and SPECT findings of N (n=16, 12), C (n=0, 6), and DH (n=3, 1), respectively. When considering C, PH+C, and RH+C as diagnostic findings for avascular necrosis, sensitivities of planar and SPECT images were 21% and 58%, and specificities were 100% and 68%, respectively. In nineteen femoral heads with normal planar findings (N), SPECT correctly identified avascular necrosis in two femoral heads and misidentified six normal femoral heads as avascular necrosis. In nineteen femoral heads with nondiagnostic abnormalities (PH, DH), SPECT correctly identified avascular necrosis in seven femoral heads and showed no false positive. Diagnostic planar findings in five femoral heads were concordant with SPECT diagnosis. These results indicate that SPECT imaging is most valuable when planar images show nondiagnostic abnormalities based on the proposed classification of scintigraphic findings. (author).

  13. Nontraumatic femoral head necrosis. Classification of bone scintigraphic findings and diagnostic value of SPECT following planar imaging

    International Nuclear Information System (INIS)

    Minoshima, Satoshi; Uchida, Yoshitaka; Anzai, Yoshimi; Uno, Kimiichi; Arimizu, Noboru

    1994-01-01

    This study was conducted to determine bone scintigraphic findings in nontraumatic femoral head avascular necrosis and diagnostic value of SPECT imaging following a conventional planar imaging. Forty-three femoral heads in twenty-six cases with idiopathic femoral head necrosis (n=2), systemic lupus erythematosus (n=22), aplastic anemia (n=1), and renal transplantation (n=1) were studied. The diagnosis for femoral head necrosis was based on magnetic resonance imaging as well as other diagnostic studies in all cases. Scintigraphic findings of planar and SPECT images were classified into six categories: normal (N); cold or decrease (C); partial increase with cold or decrease (PH+C); ring-like increase with a cold center (RH+C); partial increase (PH); diffuse and/or irregular increase (DH). Avascular necrosis was confirmed in twenty-four femoral heads, in which planar and SPECT images showed scintigraphic findings of N (n=3, 2), C (n=1, 3), PH+C (n=2, 8), RH+C (n=2, 3), PH (n=9, 2), and DH (n=7, 6), respectively. Femoral heads without avascular necrosis demonstrated planar and SPECT findings of N (n=16, 12), C (n=0, 6), and DH (n=3, 1), respectively. When considering C, PH+C, and RH+C as diagnostic findings for avascular necrosis, sensitivities of planar and SPECT images were 21% and 58%, and specificities were 100% and 68%, respectively. In nineteen femoral heads with normal planar findings (N), SPECT correctly identified avascular necrosis in two femoral heads and misidentified six normal femoral heads as avascular necrosis. In nineteen femoral heads with nondiagnostic abnormalities (PH, DH), SPECT correctly identified avascular necrosis in seven femoral heads and showed no false positive. Diagnostic planar findings in five femoral heads were concordant with SPECT diagnosis. These results indicate that SPECT imaging is most valuable when planar images show nondiagnostic abnormalities based on the proposed classification of scintigraphic findings. (author)

  14. Injury and subsequent regeneration of muscles for activation of local innate immunity to facilitate the development and relapse of autoimmune myositis in C57BL/6 mice.

    Science.gov (United States)

    Kimura, Naoki; Hirata, Shinya; Miyasaka, Nobuyuki; Kawahata, Kimito; Kohsaka, Hitoshi

    2015-04-01

    To determine whether injury and regeneration of the skeletal muscles induce an inflammatory milieu that facilitates the development and relapse of autoimmune myositis. The quadriceps of C57BL/6 mice were injured with bupivacaine hydrochloride (BPVC) and evaluated histologically. Macrophages and regenerating myofibers in the treated muscles and differentiating C2C12 myotubes were examined for cytokine expression. Mice were immunized with C protein fragments at the base of the tail and in the right hind footpads (day 0) to evoke systemic anti-C protein immunity and to induce local myositis in the right hind limbs. The contralateral quadriceps muscles were injured with BPVC or phosphate buffered saline (PBS) on day 7 or after spontaneous regression of myositis (day 42). The quadriceps muscle in nonimmunized mice was injured with BPVC on day 7. The muscles were examined histologically 14 days after treatment. The BPVC-injured muscles had macrophage infiltration most abundantly at 3 days after the injection, with emergence of regenerating fibers from day 5. The macrophages expressed inflammatory cytokines, including tumor necrosis factor α, interleukin-1β, and CCL2. Regenerating myofibers and C2C12 myotubes also expressed the cytokines. The BPVC-injected muscles from nonimmunized mice had regenerating myofibers with resolved cell infiltration 14 days after treatment. In mice preimmunized with C protein fragments, the muscles injected with BPVC on day 7 as well as on day 42, but not those injected with PBS, had myositis accompanied by CD8+ T cell infiltration. Injury and regeneration could set up an inflammatory milieu in the muscles and facilitate the development and relapse of autoimmune myositis. Copyright © 2015 by the American College of Rheumatology.

  15. Daily Supplementation of D-ribose Shows No Therapeutic Benefits in the MHC-I Transgenic Mouse Model of Inflammatory Myositis

    Science.gov (United States)

    Coley, William; Rayavarapu, Sree; van der Meulen, Jack H.; Duba, Ayyappa S.; Nagaraju, Kanneboyina

    2013-01-01

    Background Current treatments for idiopathic inflammatory myopathies (collectively called myositis) focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite successful immunosuppression. We have previously shown that decreased expression of a purine nucleotide cycle enzyme, adenosine monophosphate deaminase (AMPD1), leads to muscle weakness in a mouse model of myositis and may provide a mechanistic basis for muscle weakness. One of the downstream metabolites of this pathway, D-ribose, has been reported to alleviate symptoms of myalgia in patients with a congenital loss of AMPD1. Therefore, we hypothesized that supplementing exogenous D-ribose would improve muscle function in the mouse model of myositis. We treated normal and myositis mice with daily doses of D-ribose (4 mg/kg) over a 6-week time period and assessed its effects using a battery of behavioral, functional, histological and molecular measures. Results Treatment with D-ribose was found to have no statistically significant effects on body weight, grip strength, open field behavioral activity, maximal and specific forces of EDL, soleus muscles, or histological features. Histological and gene expression analysis indicated that muscle tissues remained inflamed despite treatment. Gene expression analysis also suggested that low levels of the ribokinase enzyme in the skeletal muscle might prevent skeletal muscle tissue from effectively utilizing D-ribose. Conclusions Treatment with daily oral doses of D-ribose showed no significant effect on either disease progression or muscle function in the mouse model of myositis. PMID:23785461

  16. Daily supplementation of D-ribose shows no therapeutic benefits in the MHC-I transgenic mouse model of inflammatory myositis.

    Directory of Open Access Journals (Sweden)

    William Coley

    Full Text Available BACKGROUND: Current treatments for idiopathic inflammatory myopathies (collectively called myositis focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite successful immunosuppression. We have previously shown that decreased expression of a purine nucleotide cycle enzyme, adenosine monophosphate deaminase (AMPD1, leads to muscle weakness in a mouse model of myositis and may provide a mechanistic basis for muscle weakness. One of the downstream metabolites of this pathway, D-ribose, has been reported to alleviate symptoms of myalgia in patients with a congenital loss of AMPD1. Therefore, we hypothesized that supplementing exogenous D-ribose would improve muscle function in the mouse model of myositis. We treated normal and myositis mice with daily doses of D-ribose (4 mg/kg over a 6-week time period and assessed its effects using a battery of behavioral, functional, histological and molecular measures. RESULTS: Treatment with D-ribose was found to have no statistically significant effects on body weight, grip strength, open field behavioral activity, maximal and specific forces of EDL, soleus muscles, or histological features. Histological and gene expression analysis indicated that muscle tissues remained inflamed despite treatment. Gene expression analysis also suggested that low levels of the ribokinase enzyme in the skeletal muscle might prevent skeletal muscle tissue from effectively utilizing D-ribose. CONCLUSIONS: Treatment with daily oral doses of D-ribose showed no significant effect on either disease progression or muscle function in the mouse model of myositis.

  17. The emergency department as a 'last resort': why parents seek care for their child's nontraumatic dental problems in the emergency room.

    Science.gov (United States)

    Mostajer Haqiqi, Azadeh; Bedos, Christophe; Macdonald, Mary Ellen

    2016-10-01

    Over the last two decades, there has been an increasing trend in the number of families using emergency departments (EDs) for treating their children's nontraumatic dental problems. We do not know why families use the ED in this way; to date, little research has addressed parents' decisions. The purpose of this study was to explore the reasons that lead parents to select the ED over a dental clinic for their child's nontraumatic dental problem. Using a qualitative descriptive design, we conducted semi-structured interviews with parents of children under age 10 who sought care for nontraumatic dental problems in an ED of a pediatric hospital. The interviews were audio-recorded, transcribed, and coded for thematic analysis using Grembowski's dental care process model as a sensitizing construct. Fifteen parents were recruited (ten mothers and five fathers). Three salient themes were identified: (i) parental beliefs and socioeconomic challenges which contributed to their care seeking, (ii) barriers parents faced in finding oral healthcare options for their children in their communities (e.g., poor access to care and poor quality of care), and (iii) parent's high satisfaction with the care provided through the ED. The ED was families' last resort; parents took their child to the ED because of the lack of other options in their communities rather than a belief that the ED was the best choice for dental care. The current pattern of ED use resulted in stress for these parents and repercussions for the children (e.g., pain, longer waiting, and increased complications); further, it has been shown in the literature to be an economic strain on the health system. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. A Systematic Review of Non-Traumatic Spinal Cord Injuries in Sub-Saharan Africa and a Proposed Diagnostic Algorithm for Resource-Limited Settings

    Directory of Open Access Journals (Sweden)

    Abdu Kisekka Musubire

    2017-12-01

    Full Text Available BackgroundNon-traumatic myelopathy is common in Africa and there are geographic differences in etiology. Clinical management is challenging due to the broad differential diagnosis and the lack of diagnostics. The objective of this systematic review is to determine the most common etiologies of non-traumatic myelopathy in sub-Saharan Africa to inform a regionally appropriate diagnostic algorithm.MethodsWe conducted a systemic review searching Medline and Embase databases using the following search terms: “Non traumatic spinal cord injury” or “myelopathy” with limitations to epidemiology or etiologies and Sub-Saharan Africa. We described the frequencies of the different etiologies and proposed a diagnostic algorithm based on the most common diagnoses.ResultsWe identified 19 studies all performed at tertiary institutions; 15 were retrospective and 13 were published in the era of the HIV epidemic. Compressive bone lesions accounted for more than 48% of the cases; a majority were Pott’s disease and metastatic disease. No diagnosis was identified in up to 30% of cases in most studies; in particular, definitive diagnoses of non-compressive lesions were rare and a majority were clinical diagnoses of transverse myelitis and HIV myelopathy. Age and HIV were major determinants of etiology.ConclusionCompressive myelopathies represent a majority of non-traumatic myelopathies in sub-Saharan Africa, and most were due to Pott’s disease. Non-compressive myelopathies have not been well defined and need further research in Africa. We recommend a standardized approach to management of non-traumatic myelopathy focused on identifying treatable conditions with tests widely available in low-resource settings.

  19. Evaluation of reduced-dose CT for acute non-traumatic abdominal pain: evaluation of diagnostic accuracy in comparison to standard-dose CT.

    Science.gov (United States)

    Othman, Ahmed E; Bongers, Malte Niklas; Zinsser, Dominik; Schabel, Christoph; Wichmann, Julian L; Arshid, Rami; Notohamiprodjo, Mike; Nikolaou, Konstantin; Bamberg, Fabian

    2018-01-01

    Background Patients with acute non-traumatic abdominal pain often undergo abdominal computed tomography (CT). However, abdominal CT is associated with high radiation exposure. Purpose To evaluate diagnostic performance of a reduced-dose 100 kVp CT protocol with advanced modeled iterative reconstruction as compared to a linearly blended 120 kVp protocol for assessment of acute, non-traumatic abdominal pain. Material and Methods Two radiologists assessed 100 kVp and linearly blended 120 kVp series of 112 consecutive patients with acute non-traumatic pain (onset diagnostic confidence. Both 100 kVp and linearly blended 120 kVp series were quantitatively evaluated regarding radiation dose and image noise. Comparative statistics and diagnostic accuracy was calculated using receiver operating curve (ROC) statistics, with final clinical diagnosis/clinical follow-up as reference standard. Results Image quality was high for both series without detectable significant differences ( P = 0.157). Image noise and artifacts were rated low for both series but significantly higher for 100 kVp ( P ≤ 0.021). Diagnostic accuracy was high for both series (120 kVp: area under the curve [AUC] = 0.950, sensitivity = 0.958, specificity = 0.941; 100 kVp: AUC ≥ 0.910, sensitivity ≥ 0.937, specificity = 0.882; P ≥ 0.516) with almost perfect inter-rater agreement (Kappa = 0.939). Diagnostic confidence was high for both dose levels without significant differences (100 kVp 5, range 4-5; 120 kVp 5, range 3-5; P = 0.134). The 100 kVp series yielded 26.1% lower radiation dose compared with the 120 kVp series (5.72 ± 2.23 mSv versus 7.75 ± 3.02 mSv, P diagnostic accuracy for the assessment of acute non-traumatic abdominal pain.

  20. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

    NARCIS (Netherlands)

    Rider, Lisa G.; Aggarwal, Rohit; Pistorio, Angela; Bayat, Nastaran; Erman, Brian; Feldman, Brian M.; Huber, Adam M.; Cimaz, Rolando; Cuttica, Rubén J.; de Oliveira, Sheila Knupp; Lindsley, Carol B.; Pilkington, Clarissa A.; Punaro, Marilynn; Ravelli, Angelo; Reed, Ann M.; Rouster-Stevens, Kelly; van Royen-Kerkhof, Annet; Dressler, Frank; Magalhaes, Claudia Saad; Constantin, Tamás; Davidson, Joyce E.; Magnusson, Bo; Russo, Ricardo; Villa, Luca; Rinaldi, Mariangela; Rockette, Howard; Lachenbruch, Peter A.; Miller, Frederick W.; Vencovsky, Jiri; Ruperto, Nicolino; Hansen, Paul; Apaz, Maria; Bowyer, Suzanne; Curran, Megan; Davidson, Joyce; Griffin, Thomas; Huber, Adam H.; Jones, Olcay; Kim, Susan; Lang, Bianca; Lindsley, Carol; Lovell, Daniel; Saad Magalhaes, Claudia; Pachman, Lauren M.; Pilkington, Clarissa; Ponyi, Andrea; Quartier, Pierre; Ramanan, Athimalaipet V.; Reed, Ann; Rennebohm, Robert

    2017-01-01

    Objective. To develop response criteria for juvenile dermatomyositis (DM). Methods. We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials

  1. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis : An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

    NARCIS (Netherlands)

    Rider, Lisa G.; Aggarwal, Rohit; Pistorio, Angela; Bayat, Nastaran; Erman, Brian; Feldman, Brian M.; Huber, Adam M.; Cimaz, Rolando; Cuttica, Rubén J.; De Oliveira, Sheila Knupp; Lindsley, Carol B.; Pilkington, Clarissa A.; Punaro, Marilynn; Ravelli, Angelo; Reed, Ann M.; Rouster-Stevens, Kelly; van Royen-Kerkhof, Annet; Dressler, Frank; Magalhaes, Claudia Saad; Constantin, Tamás; Davidson, Joyce E.; Magnusson, Bo; Russo, Ricardo; Villa, Luca; Rinaldi, Mariangela; Rockette, Howard; Lachenbruch, Peter A.; Miller, Frederick W.; Vencovsky, Jiri; Ruperto, Nicolino; Rider, Lisa G.; Ruperto, Nicolino; Miller, Frederick W.; Aggarwal, Rohit; Erman, Brian; Bayat, Nastaran; Pistorio, Angela; Huber, Adam M.; Feldman, Brian M.; Hansen, Paul; Rockette, Howard; Lachenbruch, Peter A.; Ruperto, Nicolino; Rider, Lisa G.; Apaz, Maria T; Bowyer, Suzanne; Cimaz, Rolando; Constantin, Tamás; Curran, Megan; Davidson, Joyce E.; Feldman, Brian M.; Griffin, Thomas; Huber, Adam H.; Jones, Olcay; Kim, Susan; Lang, Bianca; Lindsley, Carol; Lovell, Daniel J.; Saad Magalhaes, Claudia; Pachman, Lauren M.; Pilkington, Clarissa; Ponyi, Andrea; Punaro, Marilynn; Quartier, Pierre; Ramanan, Athimalaipet V; Ravelli, Angelo; Reed, Ann M.; Rennebohm, Robert; Sherry, David D.; Silva, Clovis A.; Stringer, Elizabeth; van Royen-Kerkhof, Annet; Wallace, Carol; Miller, Frederick W.; Oddis, Chester V.; Reed, Ann M.; Rider, Lisa G.; Ruperto, Nicolino; Apaz, Maria T; Avcin, Tadej; Becker, Mara; Beresford, Michael W.; Cimaz, Rolando; Constantin, Tamás; Curran, Megan; Cuttica, Ruben; Davidson, Joyce E.; Dressler, Frank; Dvergsten, Jeffrey; Feitosa de Oliveira, Sheila Knupp; Feldman, Brian M.; Leme Ferriani, Virginia Paes; Flato, Berit; Gerloni, Valeria; Griffin, Thomas; Henrickson, Michael; Hinze, Claas; Hoeltzel, Mark; Huber, Adam M.; Ibarra, Maria; Ilowite, Norman T; Imundo, Lisa; Jones, Olcay; Kim, Susan; Kingsbury, Daniel; Lang, Bianca; Lindsley, Carol; Lovell, Daniel J.; Martini, Alberto; Saad Magalhaes, Claudia; Magnusson, Bo; Maguiness, Sheilagh; Maillard, Susan; Mathiesen, Pernille; McCann, Liza J.; Nielsen, Susan; Pachman, Lauren M.; Passo, Murray; Pilkington, Clarissa; Punaro, Marilynn; Quartier, Pierre; Rabinovich, Egla; Ramanan, Athimalaipet V; Ravelli, Angelo; Reed, Ann M.; Rennebohm, Robert; Rider, Lisa G.; Rivas-Chacon, Rafael; Byun Robinson, Angela; Rouster-Stevens, Kelly; Russo, Ricardo; Rutkowska-Sak, Lidia; Sallum, Adriana; Sanner, Helga; Schmeling, Heinrike; Selcen, Duygu; Shaham, Bracha; Sherry, David D.; Silva, Clovis A.; Spencer, Charles H.; Sundel, Robert; Tardieu, Marc; Thatayatikom, Akaluck; van der Net, Janjaap; van Royen-Kerkhof, Annet; Wahezi, Dawn; Wallace, Carol; Zulian, Francesco; analysis, Conjoint; Cimaz, Rolando; Constantin, Tamás; Cuttica, Ruben; Davidson, Joyce E.; Dressler, Frank; Knupp Feitosa de Oliveira, Sheila; Feldman, Brian M.; Griffin, Thomas; Henrickson, Michael; Huber, Adam M.; Imundo, Lisa; Lang, Bianca; Lindsley, Carol; Saad Magalhaes, Claudia; Magnusson, Bo; Maillard, Susan; Pachman, Lauren M.; Passo, Murray; Pilkington, Clarissa; Punaro, Marilynn; Ravelli, Angelo; Reed, Ann M.; Rider, Lisa G.; Rouster-Stevens, Kelly; Russo, Ricardo; Shaham, Bracha; Sundel, Robert; van der Net, Janjaap; van Royen-Kerkhof, Annet; Cimaz, Rolando; Cuttica, Rubén J.; Knupp Feitosa de Oliveira, Sheila; Feldman, Brian M.; Huber, Adam M.; Lindsley, Carol B.; Pilkington, Clarissa; Punaro, Marilynn; Ravelli, Angelo; Reed, Ann M.; Rouster-Stevens, Kelly; van Royen-Kerkhof, Annet; Amato, Anthony A; Chinoy, Hector; Cooper, Robert G.; Dastmalchi, Maryam; de Visser, Marianne; Fiorentino, David; Isenberg, David; Katz, James; Mammen, Andrew; Oddis, Chester V.; Ytterberg, Steven R.

    2017-01-01

    Objective: To develop response criteria for juvenile dermatomyositis (DM). Methods: We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials

  2. The role of tumor necrosis factor-α-related apoptosis-inducing ligand (TRAIL) in mediating autophagy in myositis skeletal muscle: A potential non-immune mechanism of muscle damage

    Science.gov (United States)

    Alger, Heather M.; Raben, Nina; Pistilli, Emidio; Francia, Dwight; Rawat, Rashmi; Getnet, Derese; Ghimbovschi, Svetlana; Chen, Yi-Wen; Lundberg, Ingrid E.; Nagaraju, Kanneboyina

    2011-01-01

    Objective Multinucleated cells are relatively resistant to classical apoptosis, and the factors initiating cell-death and damage in myositis are not well defined. We hypothesized that non-immune autophagic cell death may play a role in muscle fiber damage. Recent literature indicates that tumor necrosis factor-alpha-related apoptosis inducing ligand (TRAIL) may induce both NFκB (nuclear factor kappa-light chain enhancer of activated B cells) activation and autophagic cell death in other systems. Here, we have investigated its role in cell death and pathogenesis in vitro and in vivo using myositis (human and mouse) muscle tissues. Methods Gene expression profiling indicated that expression of TRAIL and several autophagy markers was specifically upregulated in myositis muscle tissue; these results were confirmed by immunohistochemistry and immunoblotting. We also analyzed TRAIL-induced cell death (apoptosis and autophagy) and NFκB activation in vitro in cultured cells. Results TRAIL was expressed predominantly in muscle fibers of myositis, but not in biopsies from normal or other dystrophic-diseased muscle. Autophagy markers were upregulated in human and mouse models of myositis. TRAIL expression was restricted to regenerating/atrophic areas of muscle fascicles, blood vessels, and infiltrating lymphocytes. TRAIL induced NFκB activation and IκB degradation in cultured cells that are resistant to TRAIL-induced apoptosis but undergo autophagic cell death. Conclusion Our data demonstrate that TRAIL is expressed in myositis muscle and may mediate both activation of NFκB and autophagic cell death in myositis. Thus, this non-immune pathway may be an attractive target for therapeutic intervention in myositis. PMID:21769834

  3. Sleep disordered breathing in a cohort of patients with sporadic inclusion body myositis.

    Science.gov (United States)

    Della Marca, Giacomo; Sancricca, Cristina; Losurdo, Anna; Di Blasi, Chiara; De Fino, Chiara; Morosetti, Roberta; Broccolini, Aldobrando; Testani, Elisa; Scarano, Emanuele; Servidei, Serenella; Mirabella, Massimiliano

    2013-08-01

    The aims of the study were: (1) to evaluate subjective sleep quality and daytime sleepiness in patients affected by sporadic inclusion-body myositis (IBM); (2) to define the sleep and sleep-related respiratory pattern in IBM patients. Thirteen consecutive adult patients affected by definite IBM were enrolled, six women and seven men, mean age 66.2 ± 11.1 years (range: 50-80). Diagnosis was based on clinical and muscle biopsy studies. All patients underwent subjective sleep evaluation (Pittsburgh Sleep Quality Index, PSQI and Epworth Sleepiness Scale, ESS), oro-pharingo-esophageal scintigraphy, pulmonary function tests, psychometric measures, anatomic evaluation of upper airways, and laboratory-based polysomnography. Findings in IBM patients were compared to those obtained from a control group of 25 healthy subjects (13 men and 12 women, mean age 61.9 ± 8.6 years). Disease duration was >10 years in all. Mean IBM severity score was 28.8 ± 5.4 (range 18-36). Dysphagia was present in 10 patients. Nine patients had PSQI scores ≥ 5; patients had higher mean PSQI score (IBM: 7.2 ± 4.7, CONTROLS: 2.76 ± 1.45, p=0.005); one patient (and no controls) had EES>9. Polysomnography showed that IBM patients, compared to controls, had lower sleep efficiency (IBM: 78.8 ± 12.0%, 94.0 ± 4.5%, p<0.001), more awakenings (IBM: 11.9 ± 11.0, CONTROLS: 5.2 ± 7.5, p=0.009) and increased nocturnal time awake (IBM: 121.2 ± 82.0 min., 46.12 ± 28.8 min., p=0.001). Seven Patients (and no controls) had polysomnographic findings consistent with sleep disordered breathing (SDB). Data suggest that sleep disruption, and in particular SDB, might be highly prevalent in IBM. Data indicate that IBM patients have poor sleep and high prevalence of SDB. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  4. Interferon-regulated chemokine score associated with improvement in disease activity in refractory myositis patients treated with rituximab.

    Science.gov (United States)

    López De Padilla, Consuelo M; Crowson, Cynthia S; Hein, Molly S; Strausbauch, Michael A; Aggarwal, Rohit; Levesque, Marc C; Ascherman, Dana P; Oddis, Chester V; Reed, Ann M

    2015-01-01

    The purpose of this study was to investigate whether serum interferon (IFN)-regulated chemokine and distinct cytokine response profiles are associated with clinical improvement in patients with refractory inflammatory myopathy treated with rituximab. In a randomised, placebo-phase trial Rituximab in Myositis Trial (RIM), 200 refractory adult and paediatric myositis subjects received rituximab. Following rituximab, clinical response and disease activity were assessed. Serum samples and clinical data were collected at baseline and several time-points after rituximab treatment. Multiplexed sandwich immunoassays quantified serum levels of IFN-regulated chemokines and other pro-inflammatory cytokines. Composite IFN-regulated chemokine and Th1, Th2, Th17 and regulatory cytokine scores were computed. Baseline IFN-regulated chemokine, Th1, Th2, Th17 and regulatory cytokine scores correlated with baseline physician global VAS, whereas the baseline Th1, Th2 and Th17 cytokine scores correlated with baseline muscle VAS. We also found baseline IFN-regulated chemokine scores correlated with specific non-muscular targets such as baseline cutaneous (r=0.29; p=0.002) and pulmonary (r=0.18; p=0.02) VAS scores. Among all cytokine/chemokines examined, the baseline score of IFN-regulated chemokines demonstrated the best correlation with changes in muscle VAS at 8 (r=-0.19; p=0.01) and 16 weeks (r=-0.17; p=0.03) following rituximab and physician global VAS at 16 weeks (r=-0.16; p=0.04). In vitro experiments showed increased levels of IL-8 (p=0.04), MCP-1 (p=0.04), IL-6 (p=0.03), IL-1β (p=0.04), IL-13 (p=0.04), IL-10 (p=0.02), IL-2 (p=0.04) and IFN-γ (p=0.02) in supernatants of TLR-3 stimulated PBMCs from non-responder compared to patients responders to rituximab. IFN-regulated chemokines before treatment is associated with improvement in disease activity measures in refractory myositis patients treated with rituximab.

  5. Diagnostic accuracy of low-dose CT compared with abdominal radiography in non-traumatic acute abdominal pain: prospective study and systematic review

    Energy Technology Data Exchange (ETDEWEB)

    Alshamari, Muhammed; Geijer, Haakan [Oerebro University, Department of Radiology, Faculty of Medicine and Health, Oerebro (Sweden); Norrman, Eva [Oerebro University, Department of Medical Physics, Faculty of Medicine and Health, Oerebro (Sweden); Geijer, Mats [Lund University and Skaane University Hospital, Department of Medical Imaging and Physiology, Lund (Sweden); Jansson, Kjell [Oerebro University, Department of Surgery, Faculty of Medicine and Health, Oerebro (Sweden)

    2016-06-15

    Abdominal radiography is frequently used in acute abdominal non-traumatic pain despite the availability of more advanced diagnostic modalities. This study evaluates the diagnostic accuracy of low-dose CT compared with abdominal radiography, at similar radiation dose levels. Fifty-eight patients were imaged with both methods and were reviewed independently by three radiologists. The reference standard was obtained from the diagnosis in medical records. Sensitivity and specificity were calculated. A systematic review was performed after a literature search, finding a total of six relevant studies including the present. Overall sensitivity with 95 % CI for CT was 75 % (66-83 %) and 46 % (37-56 %) for radiography. Specificity was 87 % (77-94 %) for both methods. In the systematic review the overall sensitivity for CT varied between 75 and 96 % with specificity from 83 to 95 % while the overall sensitivity for abdominal radiography varied between 30 and 77 % with specificity 75 to 88 %. Based on the current study and available evidence, low-dose CT has higher diagnostic accuracy than abdominal radiography and it should, where logistically possible, replace abdominal radiography in the workup of adult patients with acute non-traumatic abdominal pain. (orig.)

  6. Active extravasation of contrast within the hemorrhage (spot sign: a multidetector computed tomography finding that predicts growth and a worse prognosis in non-traumatic intracerebral hemorrhage

    Directory of Open Access Journals (Sweden)

    Marcos Rosa Junior

    2013-10-01

    Full Text Available Intracerebral hemorrhage (ICH causes high rates of disability and neurological sequelae Objective To evaluate spot signs as predictors of expansion and worse prognosis in non-traumatic ICH in a Brazilian cohort. Method We used multidetector computed tomography angiography to study 65 consecutive patients (40 men, 61.5%, with ages varying from 33 to 89 years (median age 55 years. Clinical and imaging findings were correlated with the findings based on the initial imaging. Results Of the individuals who presented a spot sign, 73.7% died (in-hospital mortality, whereas in the absence of a spot sign the mortality rate was 43.0%. Although expansion of ICH was detected in 75% of the patients with a spot sign, expansion was observed in only 9.0% of the patients who did not present a spot sign. Conclusions The spot sign strongly predicted expansion in non-traumatic ICH and an increased risk of in-hospital mortality.

  7. Using external lumbar CSF drainage to treat communicating external hydrocephalus in adult patients after acute traumatic or non-traumatic brain injury.

    Science.gov (United States)

    Manet, Romain; Payen, Jean-François; Guerin, Romain; Martinez, Orianne; Hautefeuille, Serge; Francony, Gilles; Gergelé, Laurent

    2017-10-01

    Despite various treatments to control intracranial pressure (ICP) after brain injury, patients may present a late onset of high ICP or a poor response to medications. External lumbar drainage (ELD) can be considered a therapeutic option if high ICP is due to communicating external hydrocephalus. We aimed at describing the efficacy and safety of ELD used in a cohort of traumatic or non-traumatic brain-injured patients. In this multicentre retrospective analysis, patients had a delayed onset of high ICP after the initial injury and/or a poor response to ICP treatments. ELD was considered in the presence of radiological signs of communicating external hydrocephalus. Changes in ICP values and side effects following the ELD procedure were reported. Thirty-three patients with a median age of 51 years (25-75th percentile: 34-61 years) were admitted after traumatic (n = 22) or non-traumatic (n = 11) brain injuries. Their initial Glasgow Coma Scale score was 8 (4-11). Eight patients underwent external ventricular drainage prior to ELD. Median time to ELD insertion was 5 days (4-8) after brain insult. In all patients, ELD was dramatically effective in lowering ICP: 25 mmHg (20-31) before versus 7 mmHg (3-10) after (p hydrocephalus has been made.

  8. Diagnostic accuracy of low-dose CT compared with abdominal radiography in non-traumatic acute abdominal pain: prospective study and systematic review

    International Nuclear Information System (INIS)

    Alshamari, Muhammed; Geijer, Haakan; Norrman, Eva; Geijer, Mats; Jansson, Kjell

    2016-01-01

    Abdominal radiography is frequently used in acute abdominal non-traumatic pain despite the availability of more advanced diagnostic modalities. This study evaluates the diagnostic accuracy of low-dose CT compared with abdominal radiography, at similar radiation dose levels. Fifty-eight patients were imaged with both methods and were reviewed independently by three radiologists. The reference standard was obtained from the diagnosis in medical records. Sensitivity and specificity were calculated. A systematic review was performed after a literature search, finding a total of six relevant studies including the present. Overall sensitivity with 95 % CI for CT was 75 % (66-83 %) and 46 % (37-56 %) for radiography. Specificity was 87 % (77-94 %) for both methods. In the systematic review the overall sensitivity for CT varied between 75 and 96 % with specificity from 83 to 95 % while the overall sensitivity for abdominal radiography varied between 30 and 77 % with specificity 75 to 88 %. Based on the current study and available evidence, low-dose CT has higher diagnostic accuracy than abdominal radiography and it should, where logistically possible, replace abdominal radiography in the workup of adult patients with acute non-traumatic abdominal pain. (orig.)

  9. Nontraumatic Exertional Rhabdomyolysis Leading to Acute Kidney Injury in a Sickle Trait Positive Individual on Renal Biopsy

    Directory of Open Access Journals (Sweden)

    Kalyana C. Janga

    2018-01-01

    Full Text Available A 26-year-old African American male with a history of congenital cerebral palsy, sickle cell trait, and intellectual disability presented with abdominal pain that started four hours prior to the hospital visit. The patient denied fever, chills, diarrhea, or any localized trauma. The patient was at a party at his community center last evening and danced for 2 hours, physically exerting himself more than usual. Labs revealed blood urea nitrogen (BUN level of 41 mg/dL and creatinine (Cr of 2.8 mg/dL which later increased to 4.2 mg/dL while still in the emergency room. Urinalysis revealed hematuria with RBC > 50 on high power field. Imaging of the abdomen revealed no acute findings for abdominal pain. With fractional excretion of sodium (FeNa > 3%, findings suggested nonoliguric acute tubular necrosis. Over the next couple of days, symptoms of dyspepsia resolved; however, BUN/Cr continued to rise to a maximum of 122/14 mg/dL. With these findings, along with stable electrolytes, urine output matching the intake, and prior use of proton pump inhibitors, medical decision was altered for the possibility of acute interstitial nephritis. Steroids were subsequently started and biopsy was taken. Biopsy revealed heavy deposits of myoglobin. Creatinine phosphokinase (CPK levels drawn ten days later after the admission were found to be elevated at 334 U/dl, presuming the levels would have been much higher during admission. This favored a diagnosis of acute kidney injury (AKI secondary to exertional rhabdomyolysis. We here describe a case of nontraumatic exertional rhabdomyolysis in a sickle cell trait (SCT individual that was missed due to findings of microscopic hematuria masking underlying myoglobinuria and fractional excretion of sodium > 3%. As opposed to other causes of ATN, rhabdomyolysis often causes FeNa < 1%. The elevated fractional excretion of sodium in this patient was possibly due to the underlying inability of SCT positive individuals

  10. Shoulder pain in primary care: diagnostic accuracy of clinical examination tests for non-traumatic acromioclavicular joint pain

    Science.gov (United States)

    2013-01-01

    .41) and four positive clinical features resulted in 95% specificity (95% CI 0.90, 0.98) and a LR+ of 4.98 (95% CI 1.69, 13.84). Conclusions In this cohort of primary care patients with predominantly subacute or chronic ACJ pain of non-traumatic onset, traditional ACJ tests were of limited diagnostic value. Combinations of other history and physical examination findings were able to more accurately identify injection-confirmed ACJ pain in this cohort. PMID:23634871

  11. Expression of BAFF receptors in muscle tissue of myositis patients with anti-Jo-1 or anti-Ro52/anti-Ro60 autoantibodies.

    Science.gov (United States)

    Kryštůfková, Olga; Barbasso Helmers, Sevim; Venalis, Paulius; Malmström, Vivianne; Lindroos, Eva; Vencovský, Jiří; Lundberg, Ingrid E

    2014-10-10

    Anti-Jo-1 and anti-Ro52 autoantibodies are common in patients with myositis, but the mechanisms behind their production are not known. Survival of autoantibody-producing cells is dependent on B-cell-activating factor of the tumour necrosis factor family (BAFF). BAFF levels are elevated in serum of anti-Jo-1-positive myositis patients and are influenced by type-I interferon (IFN). IFN-producing cells and BAFF mRNA expression are present in myositis muscle. We investigated expression of the receptors for BAFF in muscle tissue in relation to anti-Jo-1 and anti-Ro52/anti-Ro60 autoantibodies and type-I IFN markers. Muscle biopsies from 23 patients with myositis selected based on autoantibody profile and 7 healthy controls were investigated for expression of BAFF receptor (BAFF-R), B-cell maturation antigen (BCMA) and transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI). Nineteen samples were assessed for plasma (CD138) and B-cell (CD19) markers. The numbers of positive cells per area were compared with the expression of plasmacytoid dendritic cell (pDC) marker blood dendritic cell antigen-2 (BDCA-2) and IFNα/β-inducible myxovirus resistance-1 protein (MX-1). BAFF-R, BCMA and TACI were expressed in five, seven and seven patients, respectively, and more frequently in anti-Jo-1-positive and/or anti-Ro52/anti-Ro60-positive patients compared to controls and to patients without these autoantibodies (P = BAFF-R: 0.007, BCMA: 0.03 and TACI: 0.07). A local association of receptors with B and plasma cells was confirmed by confocal microscopy. The numbers of CD138-positive and BCMA-positive cells were correlated (r = 0.79; P = 0.001). Expression of BDCA-2 correlated with numbers of CD138-positive cells and marginally with BCMA-positive cells (r = 0.54 and 0.42, respectively; P = 0.04 and 0.06, respectively). There was a borderline correlation between the numbers of positively stained TACI cells and MX-1 areas (r = 0.38, P = 0.08). The expression

  12. Radiation recall dermatitis, panniculitis, and myositis following cyclophosphamide therapy: histopathologic findings of a patient affected by multiple myeloma.

    Science.gov (United States)

    Borroni, Giovanni; Vassallo, Camilla; Brazzelli, Valeria; Martinoli, Sara; Ardigò, Marco; Alessandrino, Paolo Emilio; Borroni, Riccardo Giovanni; Franchini, Pietro

    2004-06-01

    Radiation recall dermatitis is one of the skin sequelae that may affect oncology patients. It occurs in a previously irradiated field, when subsequent chemotherapy is given. The eruption may be elicited by chemotherapy, even several months after radiotherapy. Its mechanism is poorly understood, and the histopathologic findings have received, to date, only sketchy descriptions. A 55-year-old male affected by multiple myeloma received radiation therapy both on his left coxofemoral area, and lumbar region (D11-L1). After cyclophosphamide administration, he developed 2 well defined square-shaped, infiltrated erythematoviolaceous plaques in the prior irradiated fields. Histopathologic findings revealed a diffusely fibrosclerosing process, involving deep dermis, hypodermis, as well as the underlying muscle, while sparing the epidermis and superficial-mid dermis. Histopathology was indistinguishable from deep radio-dermatitis, panniculitis, and myositis. This is the first case providing clear evidence of the causative role of cyclophosphamide in inducing a cutaneous and subcutaneous radiation recall reaction.

  13. Case report: A rare case of focal myositis presenting as Sartorius muscle contracture: A case report and review of literature.

    Science.gov (United States)

    Wang, Jin; Jiao, Juyang; Zhao, Guanglei; Shi, Jingsheng; Xia, Jun

    2018-05-01

    Focal myositis (FM) is a very rare myopathy of unknown etiology characterized by focal enlargement within one single skeletal muscle. In particular, it occurs only involving the Sartorius muscle has never been reported. A 25-year-old man was admitted to the hospital with progressive restricted left hip joint extension, left thigh discomfort and gait disturbance for 6 years. Combining clinical manifestations with results of radiological and pathological examinations, it was consistent with the diagnosis of FM INTERVENTIONS:: The patient received a surgery under general anesthesia to release the contracted Sartorius tendon. The range of motion of the patient's left hip and ipsilateral knee has significantly improved as well as the discomfort of his left thigh relieved obviously after the surgery. This case report is the first to report FM presenting as sartorius muscle contracture and the surgery is an alternative therapy for these patients.

  14. A Study on Clinical Activity of Myositis by the Use of Radioisotope Bone Scan in the Patients with Dermatomyositis - polymyositis

    International Nuclear Information System (INIS)

    Choi, Sung Jae; Koh, Chang Soon

    1982-01-01

    To evaluate the diagnostic usefulness of radioisotope bone scan and clinical activity of myositis, bone scan using 99m Tc-Methylene diphosphonate was serially done before and after treatment with prednisolone in 10 patients with well- documented dermatomyositis-polymyositis. The observed results were as follows. 1. In all 10 patients before treatment with prednisolone, the bone scans showed evidence of increased muscle uptake. Muscle uptake was markedly increased in 4 patients, moderately increased in 3 patients and minimally increased in 3 patients. 2. The site of increased muscle uptake was consistent with the site of clinically involved muscle which was weak and tender. 3. The degree of muscle uptake correlated with the severity of the muscle weakness and tenderness at the scan was done. In all 10 patients treated with high dose prednisolone, muscle uptake was decreased following therapy. Above results suggest the radioisotope bone scanning may be useful in the diagnosis and treatment of patient with dermatomyositis-polymyositis.

  15. Myositis, Ganglioneuritis, and Myocarditis with Distinct Perifascicular Muscle Atrophy in a 2-Year-Old Male Boxer

    Directory of Open Access Journals (Sweden)

    Paul M. Rossman

    2018-02-01

    Full Text Available A 2-year-old male, intact Boxer was referred for chronic diarrhea, hyporexia, labored breathing, weakness and elevated creatine kinase, and alanine aminotransferase activities. Initial examination and diagnostics revealed a peripheral nervous system neurolocalization, atrial premature complexes, and generalized megaesophagus. Progressive worsening of the dog’s condition was noted after 36 h; the dog developed aspiration pneumonia, was febrile and oxygen dependent. The owners elected humane euthanasia. Immediately postmortem biopsies of the left cranial tibial and triceps muscles and the left peroneal nerve were obtained. Postmortem histology revealed concurrent myositis, myocarditis, endocarditis, and ganglioneuritis. Mixed mononuclear cell infiltrations and a distinct perifascicular pattern of muscle fiber atrophy was present in both muscles. This is a novel case of diffuse inflammatory myopathy with a distinct perifascicular pattern of atrophy in addition to endocarditis, myocarditis, and epicarditis.

  16. Role of Innate Immunity in a Model of Histidyl-tRNA Synthetase (Jo-1)-mediated Myositis

    Science.gov (United States)

    Soejima, Makoto; Kang, Eun Ha; Gu, Xinyan; Katsumata, Yasuhiro; Clemens, Paula R.; Ascherman, Dana P.

    2010-01-01

    Objectives Previous work in humans and in animal models supports a key role for histidyl-tRNA synthetase (HRS=Jo-1) in the pathogenesis of idiopathic inflammatory myopathy. While most investigations have focused on the ability of HRS to trigger adaptive immune responses, in vitro studies clearly indicate that HRS possesses intrinsic chemokine-like properties capable of activating the innate immune system. The purpose of this study was therefore to examine the ability of HRS to direct innate immune responses in a murine model of myositis. Methods Following intramuscular immunization with soluble HRS in the absence of exogenous adjuvant, selected strains of mice were evaluated at different time points for histopathologic evidence of myositis. ELISA-based assessment of autoantibody formation and CFSE proliferation studies provided complementary measures of B and T cell responses triggered by HRS immunization. Results Compared to appropriate control proteins, a murine HRS fusion protein induced robust, statistically significant muscle inflammation in multiple congenic strains of C57BL/6 and NOD mice. Time course experiments revealed that this inflammatory response occurred as early as 7 days post immunization and persisted for up to 7 weeks. Parallel immunization strategies in DO11.10/Rag2−/− and C3H/HeJ (TLR4−/−) mice indicated that the ability of murine HRS to drive muscle inflammation was not dependent on B cell receptor or T cell receptor recognition and did not require TLR4 signaling. Conclusion Collectively, these experiments support a model in which HRS can trigger both innate and adaptive immune responses which culminate in severe muscle inflammation that is the hallmark of idiopathic inflammatory myopathy. PMID:21280002

  17. Longitudinal course of disease in a large cohort of myositis patients with autoantibodies recognizing the signal recognition particle

    Science.gov (United States)

    Werner, Jessie L.; Albayda, Jemyma; Paik, Julie; Danoff, Sonye K.; Casciola-Rosen, Livia; Christopher-Stine, Lisa; Mammen, Andrew L.

    2016-01-01

    Objective Patients with immune-mediated necrotizing myopathy (IMNM) often have autoantibodies recognizing the signal recognition particle (SRP) or HMG-CoA reductase (HMGCR). Here, we studied a cohort of anti-SRP patients to identify factors associated with disease severity and clinical improvement; we also compared the severity of weakness in those with anti-SRP versus anti-HMGCR autoantibodies. Methods All anti-SRP patients in the Johns Hopkins Myositis Cohort from 2002 to 2015 were included. Longitudinal information regarding proximal muscle strength, creatine kinase (CK) levels, and immunosuppressive therapy were recorded at each visit. Univariate and multivariate multilevel regression models were used to assess prognostic factors influencing recovery. Strength in the anti-SRP patients was compared to strength in 49 previously described anti-HMGCR subjects. Results Data from 37 anti-SRP patients and 380 total clinic visits was analyzed. Younger age at onset was associated with more severe weakness at the first visit (p=0.02) and all subsequent visits (p=0.002). Only 50% of patients reached near-full or full strength after 4 years of treatment and most of these continued to have elevated CK levels. Rituximab appeared to be effective in 13 of 17 anti-SRP patients. Anti-SRP patients were significantly weaker than those with anti-HMGCR autoantibodies (−1.3 strength points, p=0.001). Conclusions Younger age at onset is associated with more severe weakness in anti-SRP myositis. Furthermore, even among anti-SRP patients whose strength improved with immunosuppression, most had ongoing disease activity as demonstrated by elevated CK levels. Finally, anti-SRP patients were significantly weaker than anti-HMGCR patients, providing evidence that these autoantibodies are associated with distinct forms of IMNM. PMID:27111848

  18. Diagnosis and treatment of Sarcocystis neurona-induced myositis in a free-ranging California sea lion.

    Science.gov (United States)

    Carlson-Bremer, Daphne P; Gulland, Frances M D; Johnson, Christine K; Colegrove, Kathleen M; Van Bonn, William G

    2012-02-01

    An underweight, lethargic adult female California sea lion (Zalophus californianus) became stranded along the California shore and was captured and transported to a rehabilitation hospital for assessment and care. Initial physical assessment revealed the sea lion was lethargic and in poor body condition. Active myositis was diagnosed on the basis of concurrent elevations in activities of alanine aminotransferase and creatine kinase detected during serum biochemical analysis. Infection with Sarcocystis neurona was diagnosed after serologic titers increased 4-fold over a 3-week period. Diagnosis was confirmed on the basis of histopathologic findings, positive results on immunohistochemical staining, and results of quantitative PCR assay on biopsy specimens obtained from the diaphragm and muscles of the dorsal cervical region. Anticoccidial treatment was instituted with ponazuril (10 mg/kg [4.5 mg/lb], PO, q 24 h) and continued for 28 days. Prednisone (0.2 mg/kg [0.09 mg/lb], PO, q 12 h) was administered for 2 days and then every 24 hours for 5 days to treat associated inflammation. At the end of treatment, the sea lion was clinically normal, alanine aminotransferase and creatine kinase values were within reference limits, and antibody titers against S neurona had decreased 6-fold. The sea lion was released approximately 3 months after becoming stranded. S neurona-induced myositis was diagnosed in a free-ranging California sea lion. On the basis of the successful treatment and release of this sea lion, anticoccidial treatment should be considered for marine mammals in which protozoal disease is diagnosed.

  19. Nontraumatic temporal subcortical hemorrhage

    International Nuclear Information System (INIS)

    Weisberg, L.A.; Stazio, A.; Shamsnia, M.; Elliott, D.; Charity Hospital, New Orleans, LA

    1990-01-01

    Thirty patients with temporal hematomas were analyzed. Four with frontal extension survived. Of 6 with ganglionic extension, three had residual deficit. Of 8 with parietal extension, 4 had delayed deterioration and died, two patients recovered, and two with peritumoral hemorrhage due to glioblastoma multiforme died. Five patients with posterior temporal hematomas recovered. In 7 patients with basal-inferior temporal hematomas, angiography showed aneurysms in 3 cases, angiomas in 2 cases and no vascular lesion in 2 cases. Of 23 cases with negative angiography and no systemic cause for temporal hematoma, 12 patients were hypertensive and 11 were normotensive. Ten hypertensive patients without evidence of chronic vascular disease had the largest hematomas, extending into the parietal or ganglionic regions. Seven of these patients died; 3 had residual deficit. Eleven normotensive and two hypertensive patients with evidence of chronic vascular change had smaller hematomas. They survived with good functional recovery. (orig.)

  20. Novel assessment tools to evaluate clinical and laboratory responses in a subset of patients enrolled in the Rituximab in Myositis trial.

    Science.gov (United States)

    Rider, Lisa G; Yip, Adrienne L; Horkayne-Szakaly, Iren; Volochayev, Rita; Shrader, Joseph A; Turner, Maria L; Kong, Heidi H; Jain, Minal S; Jansen, Anna V; Oddis, Chester V; Fleisher, Thomas A; Miller, Frederick W

    2014-01-01

    We aimed to assess changes in myositis core set measures and ancillary clinical and laboratory data from the National Institutes of Health's subset of patients enrolled in the Rituximab in Myositis trial. Eighteen patients (5 dermatomyositis, 8 polymyositis, 5 juvenile dermatomyositis) completed more in-depth testing of muscle strength and cutaneous assessments, patient-reported outcomes, and laboratory tests before and after administration of rituximab. Percentage change in individual measures and in the definitions of improvement (DOIs) and standardized response means were examined over 44 weeks. Core set activity measures improved by 18-70% from weeks 0-44 and were sensitive to change. Fifteen patients met the DOI at week 44, 9 patients met a DOI 50% response, and 4 met a DOI 70% response. Muscle strength and function measures were more sensitive to change than cutaneous assessments. Constitutional, gastrointestinal, and pulmonary systems improved 44-70%. Patient-reported outcomes improved up to 28%. CD20+ B cells were depleted in the periphery, but B cell depletion was not associated with clinical improvement at week 16. This subset of patients had high rates of clinical response to rituximab, similar to patients in the overall trial. Most measures were responsive, and muscle strength had a greater degree of change than cutaneous assessments. Several novel assessment tools, including measures of strength and function, extra-muscular organ activity, fatigue, and health-related quality of life, are promising for use in future myositis trials. Further study of B cell-depleting therapies in myositis, particularly in treatment-naïve patients, is warranted.

  1. Histogram analysis derived from apparent diffusion coefficient (ADC) is more sensitive to reflect serological parameters in myositis than conventional ADC analysis.

    Science.gov (United States)

    Meyer, Hans Jonas; Emmer, Alexander; Kornhuber, Malte; Surov, Alexey

    2018-05-01

    Diffusion-weighted imaging (DWI) has the potential of being able to reflect histopathology architecture. A novel imaging approach, namely histogram analysis, is used to further characterize tissues on MRI. The aim of this study was to correlate histogram parameters derived from apparent diffusion coefficient (ADC) maps with serological parameters in myositis. 16 patients with autoimmune myositis were included in this retrospective study. DWI was obtained on a 1.5 T scanner by using the b-values of 0 and 1000 s mm - 2 . Histogram analysis was performed as a whole muscle measurement by using a custom-made Matlab-based application. The following ADC histogram parameters were estimated: ADCmean, ADCmax, ADCmin, ADCmedian, ADCmode, and the following percentiles ADCp10, ADCp25, ADCp75, ADCp90, as well histogram parameters kurtosis, skewness, and entropy. In all patients, the blood sample was acquired within 3 days to the MRI. The following serological parameters were estimated: alanine aminotransferase, aspartate aminotransferase, creatine kinase, lactate dehydrogenase, C-reactive protein (CRP) and myoglobin. All patients were screened for Jo1-autobodies. Kurtosis correlated inversely with CRP (p = -0.55 and 0.03). Furthermore, ADCp10 and ADCp90 values tended to correlate with creatine kinase (p = -0.43, 0.11, and p = -0.42, = 0.12 respectively). In addition, ADCmean, p10, p25, median, mode, and entropy were different between Jo1-positive and Jo1-negative patients. ADC histogram parameters are sensitive for detection of muscle alterations in myositis patients. Advances in knowledge: This study identified that kurtosis derived from ADC maps is associated with CRP in myositis patients. Furthermore, several ADC histogram parameters are statistically different between Jo1-positive and Jo1-negative patients.

  2. Heterotopic ossification revisited.

    Science.gov (United States)

    Mavrogenis, Andreas F; Soucacos, Panayotis N; Papagelopoulos, Panayiotis J

    2011-03-11

    Heterotopic ossification is the abnormal formation of mature lamellar bone within extraskeletal soft tissues where bone does not exist. Heterotopic ossification has been classified into posttraumatic, nontraumatic or neurogenic, and myositis ossificans progressiva or fibrodysplasia ossificans progressive. The pathophysiology is unknown. Anatomically, heterotopic ossification occurs outside the joint capsule without disrupting it. The new bone can be contiguous with the skeleton but generally does not involve the periosteum. Three-phase technetium-99m (99mTc) methylene diphosphonate bone scan is the most sensitive imaging modality for early detection and assessing the maturity of heterotopic ossification. Nonsurgical treatment with indomethacin and radiation therapy is appropriate for prophylaxis or early treatment of heterotopic ossification. Although bisphosphonates are effective prophylaxis if initiated shortly after the trauma, mineralization of the bone matrix resumes after drug discontinuation. During the acute inflammatory stage, the patient should rest the involved joint in a functional position; once acute inflammatory signs subside, passive range of motion exercises and continued mobilization are indicated. Surgical indications for excision of heterotopic ossification include improvement of function, standing posture, sitting or ambulation, independent dressing, feeding and hygiene, and repeated pressure sores from underlying bone mass. The optimal timing of surgery has been suggested to be a delay of 12 to 18 months until radiographic evidence of heterotopic ossification maturation and maximal recovery after neurological injury. The ideal candidate for surgical treatment before 18 months should have no joint pain or swelling, a normal alkaline phosphatase level, and 3-phase bone scan indicating mature heterotopic ossification. Copyright 2011, SLACK Incorporated.

  3. Number of test trials needed for performance stability and interrater reliability of the one leg stand test in patients with a major non-traumatic lower limb amputation

    DEFF Research Database (Denmark)

    Kristensen, Morten Tange; Nielsen, Anni Østergaard; Madsen Topp, Ulla

    2014-01-01

    Balance is beneficial for daily functioning of patients with a lower limb amputation and sometimes assessed by the one-leg stand test (OLST). The aims of the study were to examine (1) the number of trials needed to achieve performance stability, (2) the interrater reliability of the OLST in patie......Balance is beneficial for daily functioning of patients with a lower limb amputation and sometimes assessed by the one-leg stand test (OLST). The aims of the study were to examine (1) the number of trials needed to achieve performance stability, (2) the interrater reliability of the OLST...... in patients with a major non-traumatic lower limb amputation, and (3) to provide a test procedure....

  4. Nontraumatic fracture risk with diabetes mellitus and impaired fasting glucose in older white and black adults: the health, aging, and body composition study.

    Science.gov (United States)

    Strotmeyer, Elsa S; Cauley, Jane A; Schwartz, Ann V; Nevitt, Michael C; Resnick, Helaine E; Bauer, Douglas C; Tylavsky, Frances A; de Rekeneire, Nathalie; Harris, Tamara B; Newman, Anne B

    2005-07-25

    Diabetes mellitus (DM) and related complications may increase clinical fracture risk in older adults. Our objectives were to determine if type 2 diabetes mellitus or impaired fasting glucose was associated with higher fracture rates in older adults and to evaluate how diabetic individuals with fractures differed from those without fractures. The Health, Aging, and Body Composition Study participants were well-functioning, community-dwelling men and women aged 70 to 79 years (N = 2979; 42% black), of whom 19% had DM and 6% had impaired fasting glucose at baseline. Incident nontraumatic clinical fractures were verified by radiology reports for a mean +/- SD of 4.5 +/- 1.1 years. Cox proportional hazards regression models determined how DM and impaired fasting glucose affected subsequent risk of fracture. Diabetes mellitus was associated with elevated fracture risk (relative risk, 1.64; 95% confidence interval, 1.07-2.51) after adjustment for a hip bone mineral density (BMD) and fracture risk factors. Impaired fasting glucose was not significantly associated with fractures (relative risk, 1.34; 95% confidence interval, 0.67-2.67). Diabetic participants with fractures had lower hip BMD (0.818 g/cm(2) vs 0.967 g/cm(2); Pbattery score (5.0 vs 7.0), and falls (37% vs 21%) compared with diabetic participants without fractures (P<.05). These results indicate that older white and black adults with DM are at higher fracture risk compared with nondiabetic adults with a similar BMD since a higher risk of nontraumatic fractures was found after adjustment for hip BMD. Fracture prevention needs to target specific risk factors found in older adults with DM.

  5. Emergency department CT screening of patients with nontraumatic neurological symptoms referred to the posterior fossa: comparison of thin versus thick slice images.

    Science.gov (United States)

    Kamalian, Shervin; Atkinson, Wendy L; Florin, Lauren A; Pomerantz, Stuart R; Lev, Michael H; Romero, Javier M

    2014-06-01

    Evaluation of the posterior fossa (PF) on 5-mm-thick helical CT images (current default) has improved diagnostic accuracy compared to 5-mm sequential CT images; however, 5-mm-thick images may not be ideal for PF pathology due to volume averaging of rapid changes in anatomy in the Z-direction. Therefore, we sought to determine if routine review of 1.25-mm-thin helical CT images has superior accuracy in screening for nontraumatic PF pathology. MRI proof of diagnosis was obtained within 6 h of helical CT acquisition for 90 consecutive ED patients with, and 88 without, posterior fossa lesions. Helical CT images were post-processed at 1.25 and 5-mm-axial slice thickness. Two neuroradiologists blinded to the clinical/MRI findings reviewed both image sets. Interobserver agreement and accuracy were rated using Kappa statistics and ROC analysis, respectively. Of the 90/178 (51 %) who were MR positive, 60/90 (66 %) had stroke and 30/90 (33 %) had other etiologies. There was excellent interobserver agreement (κ > 0.97) for both thick and thin slice assessments. The accuracy, sensitivity, and specificity for 1.25-mm images were 65, 44, and 84 %, respectively, and for 5-mm images were 67, 45, and 85 %, respectively. The diagnostic accuracy was not significantly different (p > 0.5). In this cohort of patients with nontraumatic neurological symptoms referred to the posterior fossa, 1.25-mm-thin slice CT reformatted images do not have superior accuracy compared to 5-mm-thick images. This information has implications on optimizing resource utilizations and efficiency in a busy emergency room. Review of 1.25-mm-thin images may help diagnostic accuracy only when review of 5-mm-thick images as current default is inconclusive.

  6. Eosinophilic esophageal myositis diagnosed by endoscopic ultrasound-guided fine-needle aspiration biopsy: a case report.

    Science.gov (United States)

    Igarashi, Ryo; Irisawa, Atsushi; Shibukawa, Goro; Yamabe, Akane; Fujisawa, Mariko; Sato, Ai; Maki, Takumi; Arakawa, Noriyuki; Yoshida, Yoshitsugu; Yamamoto, Shogo; Ikeda, Tsunehiko

    2016-10-01

    Eosinophilic esophagitis (EoE) is diagnosed by microscopic findings of eosinophilic infiltration into the squamous epithelium. In contrast, another disease concept termed "eosinophilic esophageal myositis (EoEM)" has been proposed, whereby there is eosinophilic infiltration into the muscularis propria instead. A 60-year-old man was referred to our hospital for chest pain, dysphagia, and several episodes of esophageal food impaction. Although EoE was suspected based on clinical features, biopsy specimens showed no mucosal eosinophilic infiltration. Endoscopic ultrasound (EUS) showed thickening of the muscularis propria layer and subsequent EUS-guided fine-needle aspiration biopsy (EUS-FNA) revealed eosinophilic infiltration into the muscularis propria. Although the patient's symptoms gradually improved after steroid administration, complete remission was not achieved after 1 year of treatment. This case may reflect a disorder distinct from typical EoE based on eosinophilic infiltration of the muscularis propria but not the squamous epithelium, and we, therefore, diagnosed it as EoEM using the EUS-FNA findings as reference.

  7. Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause.

    Science.gov (United States)

    Dardis, Christopher; Antezana, Ariel; Tanji, Kurenai; Maccabee, Paul J

    2017-06-23

    BACKGROUND Sporadic inclusion body myositis (IBM) is the most common acquired myopathy seen in adults aged over 50 years, with a prevalence estimated at between 1 and 70 per million. Weakness of the diaphragm with loss of vital capacity is almost universal in IBM. This is almost always asymptomatic. When respiratory complications occur, they are most often due to aspiration. Respiratory failure due to diaphragmatic weakness is exceptionally rare, particularly as the presenting symptom of the disease. It is not currently considered to be a paraneoplastic syndrome. CASE REPORT Our patient presented with hypercarbic respiratory failure. This is the first such reported case without signs of weakness elsewhere of which we are aware. We suspected IBM based on her history of progressive weakness and findings on electromyography. There was a delay of 5 years in obtaining biopsy for confirmation, during which she presented with recurrent episodes of respiratory failure despite using non-invasive ventilation. An autopsy revealed the presence of papillary thyroid carcinoma with spread to local lymph nodes. On the basis that these co-morbidities are unlikely to have occurred by chance (we estimate 1×10-17), we hypothesize that IBM may be a paraneoplastic condition. We acknowledge that proof would require demonstrating a pathogenic antibody. CONCLUSIONS IBM should be considered in older patients (age >45) presenting with otherwise unexplained respiratory failure. A workup for possible malignancy in this setting appears reasonable.

  8. Upregulation of immunoproteasome subunits in myositis indicates active inflammation with involvement of antigen presenting cells, CD8 T-cells and IFNΓ.

    Directory of Open Access Journals (Sweden)

    Khetam Ghannam

    Full Text Available OBJECTIVE: In idiopathic inflammatory myopathies (IIM infiltration of immune cells into muscle and upregulation of MHC-I expression implies increased antigen presentation and involvement of the proteasome system. To decipher the role of immunoproteasomes in myositis, we investigated individual cell types and muscle tissues and focused on possible immune triggers. METHODS: Expression of constitutive (PSMB5, -6, -7 and corresponding immunoproteasomal subunits (PSMB8, -9, -10 was analyzed by real-time RT-PCR in muscle biopsies and sorted peripheral blood cells of patients with IIM, non-inflammatory myopathies (NIM and healthy donors (HD. Protein analysis in muscle biopsies was performed by western blot. Affymetrix HG-U133 platform derived transcriptome data from biopsies of different muscle diseases and from immune cell types as well as monocyte stimulation experiments were used for validation, coregulation and coexpression analyses. RESULTS: Real-time RT-PCR revealed significantly increased expression of immunoproteasomal subunits (PSMB8/-9/-10 in DC, monocytes and CD8+ T-cells in IIM. In muscle biopsies, the immunosubunits were elevated in IIM compared to NIM and exceeded levels of matched blood samples. Proteins of PSMB8 and -9 were found only in IIM but not NIM muscle biopsies. Reanalysis of 78 myositis and 20 healthy muscle transcriptomes confirmed these results and revealed involvement of the antigen processing and presentation pathway. Comparison with reference profiles of sorted immune cells and healthy muscle confirmed upregulation of PSMB8 and -9 in myositis biopsies beyond infiltration related changes. This upregulation correlated highest with STAT1, IRF1 and IFNγ expression. Elevation of T-cell specific transcripts in active IIM muscles was accompanied by increased expression of DC and monocyte marker genes and thus reflects the cell type specific involvement observed in peripheral blood. CONCLUSIONS: Immunoproteasomes seem to indicate

  9. Are repeated single-limb heel raises and manual muscle testing associated with peak plantar-flexor force in people with inclusion body myositis?

    Science.gov (United States)

    Harris-Love, Michael O; Shrader, Joseph A; Davenport, Todd E; Joe, Galen; Rakocevic, Goran; McElroy, Beverly; Dalakas, Marinos

    2014-04-01

    Repeated heel raises have been proposed as a method of ankle plantar-flexor strength testing that circumvents the limitations of manual muscle testing (MMT). The study objective was to examine the relationships among ankle plantar-flexion isometric maximum voluntary contraction (MVC), repeated single-limb heel raises (SLHRs), and MMT in people with myositis. This was a cross-sectional study with a between-group design. The ability to complete 1 SLHR determined group assignment (SLHR group, n=24; no-SLHR group, n=19). Forty-three participants with myositis (13 women; median age=64.9 years) participated. Outcome measures included MVC, predicted MVC, Kendall MMT, and Daniels-Worthingham MMT. The Kendall MMT was unable to detect significant ankle plantar-flexor weakness established by quantitative methods and was unable to discriminate between participants who could and those who could not perform the SLHR task. Ankle plantar-flexion MVC was not associated with the number of heel-raise repetitions in the SLHR group (pseudo R(2)=.13). No significant relationship was observed between MVC values and MMT grades in the SLHR and no-SLHR groups. However, a moderate relationship between MVC values and MMT grades was evident in a combined-group analysis (ρ=.50-.67). The lower half of both MMT grading scales was not represented in the study despite the profound weakness of the participants. Both Kendall MMT and Daniels-Worthingham MMT had limited utility in the assessment of ankle plantar-flexor strength. Repeated SLHRs should not be used as a proxy measure of ankle plantar-flexion MVC in people with myositis.

  10. Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase Myositis-related interstitial lung disease and antisynthetase syndrome

    Directory of Open Access Journals (Sweden)

    Joshua Solomon

    2011-02-01

    Full Text Available Em pacientes com miosite, é comum o comprometimento pulmonar, e a presença de anticorpos anti-aminoacil-RNAt sintetase (anti-ARS é preditora da presença ou do desenvolvimento de doença pulmonar intersticial (DPI. Uma entidade clínica distinta - a síndrome antissintetase - é caracterizada pela presença de anticorpos anti-ARS, miosite, DPI, artrite, fenômeno de Raynaud e "mãos de mecânico". O mais comum anticorpo anti-ARS é o anti-Jo-1. Anticorpos anti-ARS mais recentemente descritos podem conferir um fenótipo que é distinto daquele de pacientes com positividade para anti-Jo-1, sendo caracterizado por uma menor incidência de miosite e uma maior incidência de DPI. Nos pacientes com DPI relacionada à síndrome antissintetase, a resposta a medicações imunossupressoras é em geral favorável.In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS antibodies marks the presence or predicts the development of interstitial lung disease (ILD. A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

  11. Contribution of dot-blot assay to the diagnosis and management of myositis: a three-year practice at a university hospital centre.

    Science.gov (United States)

    Martel, Clothilde; Vignaud, Guillaume; Liozon, Eric; Magy, Laurent; Gallouedec, Gael; Ly, Kim; Bezanahary, Holly; Cypierre, Anne; Lapébie, François-Xavier; Palat, Sylvain; Gondran, Guillaume; Jauberteau, Marie-Odile; Fauchais, Anne-Laure

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases with wide clinical spectrum that may lead to delayed diagnosis. The aim of this study was to examine the impact of IIM-specific dot-blot assay on diagnostic process of patients presenting with muscular or systemic symptoms evocating of IIM. We collected all the prescriptions of an IIM specific dot-blot assay (8 autoantigens including Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, PM/Scl and Scl-70) over a 38-month period. 316 myositis dot-blot assays (MSD) were performed in 274 patients (156 women, mean age 53±10.6 years) referring for muscular and/or systemic symptoms suggesting IIM. The timing of dot prescription through the diagnostic process was highly variable: without (35%), concomitantly (16%) or after electromyographic studies (35%). Fifty-nine patients (22%) had IIM according to Bohan and Peter's criteria. Among them, 29 (49%) had positive dot (8 Jo-1, 6 PM-Scl, 5 PL-12, 5 SRP, 2 Mi-2, 2 PL-7 and 1 Ku). Various other diagnoses were performed including 35 autoimmune disease or granulomatosis (12%), 19 inflammatory rheumatic disease (7%), 16 non inflammatory muscular disorders (6%), 10 drug-induced myalgia (4%), 11 infectious myositis (4%). Except 11 borderline SRP results and one transient PM-Scl, MSD was positive only in one case of IIM. Dot allowed clinicians to correct diagnosis in 4 cases and improved the diagnosis of IIM subtypes in 4 cases. This study reflects the interest of myositis dot in the rapid diagnosis process of patients with non-specific muscular symptoms leading to various diagnoses including IIM.

  12. Upregulation of Immunoproteasome Subunits in Myositis Indicates Active Inflammation with Involvement of Antigen Presenting Cells, CD8 T-Cells and IFNγ

    Science.gov (United States)

    Ghannam, Khetam; Martinez-Gamboa, Lorena; Spengler, Lydia; Krause, Sabine; Smiljanovic, Biljana; Bonin, Marc; Bhattarai, Salyan; Grützkau, Andreas; Burmester, Gerd-R.

    2014-01-01

    Objective In idiopathic inflammatory myopathies (IIM) infiltration of immune cells into muscle and upregulation of MHC-I expression implies increased antigen presentation and involvement of the proteasome system. To decipher the role of immunoproteasomes in myositis, we investigated individual cell types and muscle tissues and focused on possible immune triggers. Methods Expression of constitutive (PSMB5, -6, -7) and corresponding immunoproteasomal subunits (PSMB8, -9, -10) was analyzed by real-time RT-PCR in muscle biopsies and sorted peripheral blood cells of patients with IIM, non-inflammatory myopathies (NIM) and healthy donors (HD). Protein analysis in muscle biopsies was performed by western blot. Affymetrix HG-U133 platform derived transcriptome data from biopsies of different muscle diseases and from immune cell types as well as monocyte stimulation experiments were used for validation, coregulation and coexpression analyses. Results Real-time RT-PCR revealed significantly increased expression of immunoproteasomal subunits (PSMB8/-9/-10) in DC, monocytes and CD8+ T-cells in IIM. In muscle biopsies, the immunosubunits were elevated in IIM compared to NIM and exceeded levels of matched blood samples. Proteins of PSMB8 and -9 were found only in IIM but not NIM muscle biopsies. Reanalysis of 78 myositis and 20 healthy muscle transcriptomes confirmed these results and revealed involvement of the antigen processing and presentation pathway. Comparison with reference profiles of sorted immune cells and healthy muscle confirmed upregulation of PSMB8 and -9 in myositis biopsies beyond infiltration related changes. This upregulation correlated highest with STAT1, IRF1 and IFNγ expression. Elevation of T-cell specific transcripts in active IIM muscles was accompanied by increased expression of DC and monocyte marker genes and thus reflects the cell type specific involvement observed in peripheral blood. Conclusions Immunoproteasomes seem to indicate IIM activity and

  13. MANAGEMENT OF ACUTE RENAL FAILURE WITH DELAYED HYPERCALCEMIA SECONDARY TO SARCOCYSTIS NEURONA-INDUCED MYOSITIS AND RHABDOMYOLYSIS IN A CALIFORNIA SEA LION (ZALOPHUS CALIFORNIANUS).

    Science.gov (United States)

    Alexander, Amy B; Hanley, Christopher S; Duncan, Mary C; Ulmer, Kyle; Padilla, Luis R

    2015-09-01

    A 3-yr-old captive-born California sea lion (Zalophus californianus) developed Sarcocystis neurona-induced myositis and rhabdomyolysis that led to acute renal failure. The sea lion was successfully managed with fluid therapy, antiprotozoals, antibiotics, anti-inflammatories, antiemetics, gastroprotectants, and diuretics, but developed severe delayed hypercalcemia, a syndrome identified in humans after traumatic or exertion-induced rhabdomyolysis. Treatment with calcitonin was added to the management, and the individual recovered fully. The case emphasizes that animals with rhabdomyolysis-induced renal failure risk developing delayed hypercalcemia, which may be life threatening, and calcium levels should be closely monitored past the resolution of renal failure.

  14. A case of anti-Jo1 myositis with pleural effusions and pericardial tamponade developing after exposure to a fermented Kombucha beverage.

    Science.gov (United States)

    Derk, Chris T; Sandorfi, Nora; Curtis, Mark T

    2004-08-01

    The pathogenesis of the idiopathic inflammatory myopathies has been postulated to be an environmental trigger causing the expression of the disease in a genetically predisposed patient. We report a case of anti-Jo1 antibody-positive myositis which was associated with pleural effusions, pericardial effusion with tamponade, and 'mechanic's hands', probably related to the consumption of a fermented Kombucha beverage. Kombucha 'mushroom', a symbiosis of yeast and bacteria, is postulated to be the trigger for our patient's disease owing to the proximity of his symptoms to the consumption of the Kombucha beverage.

  15. Extraarticular bony ankylosis in a child with supracondylar fracture of humerus

    Directory of Open Access Journals (Sweden)

    Naranje Sameer

    2012-11-01

    Full Text Available 【Abstract】 Myositis ossificans is defined as forma-tion of bone at the site of injured muscle. It is one of the rare complications of supracondylar fracture of humerus in children. Myositis mass usually develops on the anterior aspect in the brachialis muscle and produces restriction of range of motion, but complete ankylosis is rare. To the best of our knowledge, this is the first case to be reported in the literature as a consequence of myositis ossificans traumatica. In this case, a six-year-old child presented to the casualty department with pain in the right elbow after a fall on out-stretched hand during play. After surgical excision through the anterior approach, the child had no symptoms referable to the elbow and a residual flexion deformity of 15 degrees with further painless flexion up to 100 degrees at last follow-up of one year after surgery. Key words: Humerus; Fractures, Bone; Myositis; Ankylosis

  16. The validity of the Brain Injury Cognitive Screen (BICS) as a neuropsychological screening assessment for traumatic and non-traumatic brain injury.

    Science.gov (United States)

    Vaughan, Frances L; Neal, Jo Anne; Mulla, Farzana Nizam; Edwards, Barbara; Coetzer, Rudi

    2017-04-01

    The Brain Injury Cognitive Screen (BICS) was developed as an in-service cognitive assessment battery for acquired brain injury patients entering community rehabilitation. The BICS focuses on domains that are particularly compromised following TBI, and provides a broader and more detailed assessment of executive function, attention and information processing than comparable screening assessments. The BICS also includes brief assessments of perception, naming, and construction, which were predicted to be more sensitive to impairments following non-traumatic brain injury. The studies reported here examine preliminary evidence for its validity in post-acute rehabilitation. In Study 1, TBI patients completed the BICS and were compared with matched controls. Patients with focal lesions and matched controls were compared in Study 2. Study 3 examined demographic effects in a sample of normative data. TBI and focal lesion patients obtained significantly lower composite memory, executive function and attention and information processing BICS scores than healthy controls. Injury severity effects were also obtained. Logistic regression analyses indicated that each group of BICS memory, executive function and attention measures reliably differentiated TBI and focal lesion participants from controls. Design Recall, Prospective Memory, Verbal Fluency, and Visual Search test scores showed significant independent regression effects. Other subtest measures showed evidence of sensitivity to brain injury. The study provides preliminary evidence of the BICS' sensitivity to cognitive impairment caused by acquired brain injury, and its potential clinical utility as a cognitive screen. Further validation based on a revised version of the BICS and more normative data are required.

  17. Women with a history of childhood maltreatment exhibit more activation in association areas following non-traumatic olfactory stimuli: a fMRI study.

    Directory of Open Access Journals (Sweden)

    Ilona Croy

    2010-02-01

    Full Text Available The aim of this study was investigating how women with a history of childhood maltreatment (CM process non-threatening and non-trauma related olfactory stimuli. The focus on olfactory perception is based on the overlap of brain areas often proposed to be affected in CM patients and the projection areas of the olfactory system, including the amygdala, orbitofrontal cortex, insula and hippocampus.Twelve women with CM and 10 controls participated in the study. All participants were, or have been, patients in a psychosomatic clinic. Participants underwent a fMRI investigation during olfactory stimulation with a neutral (coffee and a pleasant (peach odor. Furthermore, odor threshold and odor identification (Sniffin' Sticks were tested.Both groups showed normal activation in the olfactory projection areas. However, in the CM-group we found additionally enhanced activation in multiple, mainly neocortical, areas that are part of those involved in associative networks. These include the precentral frontal lobe, inferior and middle frontal structures, posterior parietal lobe, occipital lobe, and the posterior cingulate cortex.The results indicate that in this group of patients, CM was associated with an altered processing of olfactory stimuli, but not development of a functional olfactory deficit. This complements other studies on CM insofar as we found the observed pattern of enhanced activation in associative and emotional regions even following non-traumatic olfactory cues.

  18. Expert consensus on facilitators and barriers to return-to-work following surgery for non-traumatic upper extremity conditions: a Delphi study.

    Science.gov (United States)

    Peters, S E; Johnston, V; Ross, M; Coppieters, M W

    2017-02-01

    This Delphi study aimed to reach consensus on important facilitators and barriers for return-to-work following surgery for non-traumatic upper extremity conditions. In Round 1, experts ( n = 42) listed 134 factors, which were appraised in Rounds 2 and 3. Consensus (⩾85% agreement) was achieved for 13 facilitators (high motivation to return-to-work; high self-efficacy for return-to-work and recovery; availability of modified/alternative duties; flexible return-to-work arrangements; positive coping skills; limited heavy work exertion; supportive return-to-work policies; supportive supervisor/management; no catastrophic thinking; no fear avoidance to return-to-work; no fear avoidance to pain/activity; return to meaningful work duties; high job satisfaction) and six barriers (mood disorder diagnosis; pain/symptoms at more than one musculoskeletal site; heavy upper extremity exertions at work; lack of flexible return-to-work arrangements; lack of support from supervisor/management; high level of pain catastrophizing). Future prognostic studies are required to validate these biopsychosocial factors to further improve return-to-work outcomes. V.

  19. Non-traumatic acute epidural spinal hematomas diagnosed by magnetic resonance; Hematomas espinales epidurales agudos no traumaticos: diagnostico por resonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Rovira, A.; Grive, E.; Pedraza, S.; Capellades, J.; Nos, C.; Alarcon, M.; Rovira, A. [Hospital Universitari Vall d' Hebron. Barcelona (Spain)

    2000-07-01

    The non-traumatic spinal epidural hematoma (NTSEH) is a rare entity that can be the cause of an acute spinal compression syndrome. the objective of this review is to identify the characteristics by MRI and NTSEH and to analyze the factors that influence in its prognosis. In the years 1994 and 1999, 12 patients with NTSEH have been diagnosed in our hospital, and a MRI was performed during the acute phase. the characteristics of the lesions have been analyzed by MRI, with special emphasis on the topographic data and resonance signal and the factors that can influence in the clinical prognosis of the patients. Initially, all of the patients presented pain in the cervical dorsal or interscapular site, followed by a sensitive-motor deficit picture. The MRI showed a lesion of expansive character and posterior epidural location in every case that would produce varying degrees of compression on the spinal cord. The NTSEH should be considered as one of the causes of acute spinal cord compression. The clinical association of intense cervical, dorsal or interscapular pain followed by a sensomotor deficit picture should lead to the suspicion of this entity, that would require an immediate examination with MRI to verify its diagnosis. Both the clinical manifestations as well as the characteristics observed by MRI of the NTSEH have a prognostic value and determine the therapeutic decision. (Author) 34 refs.

  20. Sodium phenylbutyrate reverses lysosomal dysfunction and decreases amyloid-β42 in an in vitro-model of inclusion-body myositis.

    Science.gov (United States)

    Nogalska, Anna; D'Agostino, Carla; Engel, W King; Askanas, Valerie

    2014-05-01

    Sporadic inclusion-body myositis (s-IBM) is a severe, progressive muscle disease for which there is no enduring treatment. Pathologically characteristic are vacuolated muscle fibers having: accumulations of multi-protein aggregates, including amyloid-β(Aβ) 42 and its toxic oligomers; increased γ-secretase activity; and impaired autophagy. Cultured human muscle fibers with experimentally-impaired autophagy recapitulate some of the s-IBM muscle abnormalities, including vacuolization and decreased activity of lysosomal enzymes, accompanied by increased Aβ42, Aβ42 oligomers, and increased γ-secretase activity. Sodium phenylbutyrate (NaPB) is an orally bioavailable small molecule approved by the FDA for treatment of urea-cycle disorders. Here we describe that NaPB treatment reverses lysosomal dysfunction in an in vitro model of inclusion-body myositis, involving cultured human muscle fibers. NaPB treatment improved lysosomal activity, decreased Aβ42 and its oligomers, decreased γ-secretase activity, and virtually prevented muscle-fiber vacuolization. Accordingly, NaPB might be considered a potential treatment of s-IBM patients. Copyright © 2014 Elsevier Inc. All rights reserved.