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Sample records for syringomyelia

  1. Syringomyelia

    Science.gov (United States)

    ... of syringomyelia may include: Stiffness in your back, shoulders, arms and legs Pain in your neck, arms and back Bowel and bladder function problems Muscle weakness and spasms in your legs Spinal curvature ( ...

  2. Syringomyelia

    Science.gov (United States)

    ... 800-533-3231 theacpa.org American Syringomyelia & Chiari Alliance Project P.O. Box 1586 Longview, TX 75606- ... Legislative Updates Impact NINDS Contributions to Approved Therapies Strategic Plans & Evaluations Strategic Plans Program Evaluations Workforce Diversity ...

  3. Syringomyelia

    DEFF Research Database (Denmark)

    Blegvad, C; Grotenhuis, J A; Juhler, M

    2014-01-01

    .0) and hydromyelia (Q06.4) existing as independent ICD-10 entities, we have shown that the use of classifying terminology for fluid-filled cavities in the spinal cord is indiscriminate and inconsistent. Even though a general agreement on the believed pathogenetic mechanism exists, and the general treatment methods...... are used in accordance with this mechanism, the terminology fails to function as a simple and universal link between theory and treatment. CONCLUSIONS: We propose a new causal concept for an ICD classification with syringomyelia (G95.0) as the only describing terminology, thus abandoning the use...

  4. Fetal syringomyelia.

    Science.gov (United States)

    Guo, Anne; Chitayat, David; Blaser, Susan; Keating, Sarah; Shannon, Patrick

    2014-08-06

    We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.

  5. MRI diagnosis of syringomyelia

    International Nuclear Information System (INIS)

    Takahashi, Mutsumasa; Sakamoto, Yuji; Kojima, Ryutaro; Matsuno, Yasuji

    1989-01-01

    Eighteen cases of syringomyelia were classified into communicating type (8 cases), traumatic type (5 cases) and tumor type (5 cases). Although there was similarity in the appearance of MRI regardless of the causes, there were some characteristics which were found more prevalently in one type of syringomyelia than the other. Traumatic syringomyelia involved longer segments, frequently entire length of the spinal cord. Chiari I malformation was more often associated with the communicating type, while the cystic appearance of syringomyelia was more frequently associated with the tumor type. Enlargement of the spinal cord was often seen with the tumor type, followed by traumatic type. T2-weighted images showed increased intensity in the area of the spinal cord trauma in all trauma cases, while tumor types also showed increased signal intensity within the tumor on T2-weighted images. Flow void sign of the syrinx was observed frequently in the communicating type. Although there was overlapping of the findings among syringomyelias of different causes, it was often possible to make differentiation among 3 types. When clinical and MR findings were considered together, MRI was one of the most important radiologic examinations in the evaluation of syringomyelia. (author)

  6. Scoliosis Associated With Syringomyelia

    Directory of Open Access Journals (Sweden)

    Gh Fathi

    2007-01-01

    Full Text Available Introduction: The differential diagnosis of idiopathic and syringomyelia associated scoliosis is important because corrective surgery for scoliosis associated with syringomyelia prior to management of syringomyelia can be dangerous. There are important imaging indicators for diagnosis of syringomyelia associated with scoliosis. A few of these indicators have been assessed in our study. Methods: A retrospective descriptive study including 38 patients with both scoliosis and syringomyelia was performed at the Shafa Yahyaeian center. Standard scoliosis series radiographs and MRI of all patients were studied. The type of scoliosis, location and magnitude of deformity, kyphosis or lordosis in the sagittal plane, location and size of syrinx were assessed. Results: Thoracic kyphosis was present in 94.7% of patients. 37% of patients had scoliosis with convexity to left. Arnold –chiari malformation was present in 36% and cord tethering in 21% of patients. The locations of syrinx were as follows: 47.4%cervical, 44.6 thoracic, 2.7% lumbar and 5.3% were holocord. Conclusion: Kyphosis, abscence of lordosis in sagittal plane, progressive scoliosis and scoliosis with convexity to left are atypical findings and could be indicators of the presence of syringomyelia. If these indicators are present, a diagnosis of idiopathic scoliosis should be made with caution.

  7. Acute syringomyelia: case report.

    Science.gov (United States)

    Milhorat, Thomas H; Bolognese, Paolo A; Black, Karen S; Woldenberg, Rona F

    2003-11-01

    Syringomyelia is generally regarded as a chronic, slowly progressive disorder. We describe a case of acute dilation of the central canal of the spinal cord that presented with rapidly progressive segmental signs. A 29-year-old female patient who had previously undergone surgical treatment for a Chiari I malformation, syringomyelia, and hydrocephalus presented with an 8-day history of headaches, progressive paraparesis, and urinary retention. Magnetic resonance imaging scans demonstrated panventricular hydrocephalus in association with a holocord syrinx that extended to the obex. Magnetic resonance imaging scans that had been coincidentally obtained just 3 days before the onset of symptoms had revealed no evidence of hydrocephalus or syringomyelia. The patient underwent emergency revision of a failed ventriculoperitoneal shunt. Postoperatively, there was prompt resolution of the syringomyelia, hydrocephalus, and associated neurological deficits. Among patients with communicating syringomyelia, the central canal of the spinal cord participates as a "fifth ventricle" and can undergo rapid dilation in association with acute hydrocephalus. Appropriate treatment in such cases involves placement of a ventriculoperitoneal shunt.

  8. Pan, Syrinx and syringomyelia.

    Science.gov (United States)

    Palacios-Sánchez, Leonardo; Botero-Meneses, Juan Sebastián; Vélez-Flórez, María Camila

    2017-12-01

    Many myths and legends have had a deep influence on modern language, and on modern medical vernacular. The terms "syrinx" and "panic"are two of the most characteristic examples and their use in neurology and other specialties is well known. This article reviews the history of these words in Greek mythology and their use in modern medicine. It is known by very few that clinical symptoms or conditions, such as syringomyelia and panic attacks, have a mythological origin in their definition and naming.

  9. Post-traumatic syringomyelia

    International Nuclear Information System (INIS)

    Freund, M.; Sartor, K.; Aschoff, A.; Spahn, B.

    1999-01-01

    The improvement of preclinical emergency medicine, better surgical and conservative therapies, and the development of intensive care units and specialized centers have improved the survival rate for patients with serious spinal cord injuries. Therefore, more sequelae of chronic spinal cord injuries such as post-traumatic spinal cord cavitations also occur. The first such case was described by Bastian in 1867. Generally, these cavitations were diagnosed from 2 months up to 32 years after the trauma. The overall prevalence of post-traumatic syringomyelia (PTS) is not known; however, with the increasing use of magnetic resonance imaging (MRI), its diagnosis has increased, ranging from 2.3% of paraplegic and tetraplegic patients in 1976 and 3.2% in 1985, to nearly 50% in a selected group of patients in 1991 and 1993. In 1995, a 4.45% incidence was reported. In our clinic we are currently treating 440 cases of syringomyelia, 140 of which are PTS. Several observations suggest more than one potential mechanism for the evolution of a post-traumatic cyst or PTS. Various factors, such as hemorrhage or, in particular, ischemia within the spinal cord, blockage of the cerebrosinal fluid (CSF) pathways around the cord or localized meningeal fibrosis either alone or in combination with other factors, may be involved. Clinically, sensory disturbances, loss of motor function, pain, and modification of the deep tendon reflexes are observed in most patients. On MRI, PTS is seen as a longitudinal, cystic cavity within the spinal cord, giving a hypointense signal on T 1 -weighted images and a hyperintense signal on T 2 -weighted images. For treatment planning it is mandatory to identify the lower and upper end of the PTS on the MRI. (orig.) [de

  10. Exercise Concepts for Individuals with Syringomyelia

    Science.gov (United States)

    ... Center Research Great Expectations Post navigation ← Previous Next → Exercise Concepts for Individuals with Syringomyelia Posted on November ... related duties? 3. Do you have questions about exercise? Do you exercise regularly? Are you involved in ...

  11. Syringomyelia coexisting with guillain-barre syndrome.

    Science.gov (United States)

    Kim, Hee-Sang; Yun, Dong Hwan; Chon, Jinmann; Lee, Jong Eon; Park, Min Ho; Han, Yoo Jin

    2013-10-01

    Guillain-Barre syndrome (GBS) and syringomyelia are diseases of different entities. GBS is an acute post-infectious autoimmune disease which is mediated by autoantibodies against the myelin of peripheral nerves. Syringomyelia is a chronic disease characterized by a cavity extending longitudinally inside the spinal cord. A 67-year-old man is being hospitalized due to severe numbness and ascending weakness in all limbs. On neurological examination, the motor power of all limbs are decreased and show absence of deep tendon reflexes (DTRs). The patient is being diagnosed with GBS on the basis of the acute clinical course, nerve conduction studies of segmental demyelinating polyneuropathy, and a finding of albuminocytologic dissociation in the cerebrospinal fluid. The patient is presented with a new set of symptoms thereafter, which composes of sensory changes in the upper extremities, the urinary dysfunction including frequency and residual urine, spastic bilateral lower extremities, and increased reflexes of the knee and the biceps at follow-up examinations. The spinal magnetic resonance imaging in the sagittal section revealed a syrinx cavity between the fifth cervical and the first thoracic vertebral segment in the cord. The somatosensory evoked potential show sensory pathway defects between both the brachial plexus and the brain stem. Thus, this patient is being diagnosed with both GBS and syringomyelia. We report a case of symptomatic syringomyelia coexisting with GBS. Since the GBS is presented with a progressive muscle weakness and reduced DTRs, the muscle weakness and stiffness in the extremities suggests a concurrent syringomyelia might be easily overlooked.

  12. Cervical spinal canal narrowing in idiopathic syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Struck, Aaron F. [Massachusetts General Hospital, Department of Neurology, Boston, MA (United States); Carr, Carrie M. [Mayo Clinic, Department of Radiology, Rochester, MN (United States); Shah, Vinil [University of California San Francisco, Department of Radiology, San Francisco, CA (United States); Hesselink, John R. [University of California San Diego, Department of Radiology, San Diego, CA (United States); Haughton, Victor M. [University of Wisconsin, Department of Radiology, Madison, WI (United States)

    2016-08-15

    The cervical spine in Chiari I patient with syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without syringomyelia. We tested the hypothesis that patients with idiopathic syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of syringomyelia. Local institutional review boards approved this retrospective study. Patients with IS were compared to age-matched controls with normal sagittal spine MR. All subjects had T1-weighted spin-echo (500/20) and T2-weighted fast spin-echo (2000/90) sagittal cervical spine images at 1.5 T. Readers blinded to demographic data and study hypothesis measured anteroposterior diameters at each cervical level. The spinal canal diameters were compared with a Mann-Whitney U test. The overall difference was assessed with a Friedman test. Seventeen subjects were read by two reviewers to assess inter-rater reliability. Fifty IS patients with 50 age-matched controls were studied. IS subjects had one or more syrinxes varying from 1 to 19 spinal segments. Spinal canal diameters narrowed from C1 to C3 and then enlarged from C5 to C7 in both groups. Diameters from C2 to C4 were narrower in the IS group (p < 0.005) than in controls. The ratio of the C3 to the C7 diameters was also smaller (p = 0.004) in IS than controls. Collectively, the spinal canal diameters in the IS were significantly different from controls (Friedman test p < 0.0001). Patients with IS have abnormally narrow upper and mid cervical spinal canal diameters and greater positive tapering between C3 and C7. (orig.)

  13. Idiopathic Syringomyelia in a Military Helicopter Pilot.

    Science.gov (United States)

    Schiemer, Anthony

    2017-10-01

    A syrinx is a fluid-filled cavity within the spinal cord. They can lead to a variety of symptoms, including limb weakness and back pain. Incidental finding of syringomyelia provides a challenge for clinicians due to the wide variety of possible symptoms. In military aviation, neurological findings in pilots can result in extensive investigation that can lead to potentially invasive management. Conversely, the potential for chronic progression of a spinal syrinx and subsequent neurological deterioration makes early identification critical. Ultimately, the discovery of a lesion may have implications for flying status and operational capability. A 25-yr-old man working as a navy Seahawk helicopter pilot presented with episodes of right arm paraesthesia and pain between the scapulae. On at least one occasion, these symptoms woke him at night. Upon magnetic resonance imaging, dilatation of the central canal in a syrinx-like pattern in the lower cervical region was noted. Neurology review suggested the finding was persistent and unlikely to be responsible for his symptoms. No surgical input was recommended. His symptoms were attributed to mild cervical spondylosis, which resolved with ongoing physiotherapy, and he was returned to flying status. This case highlights several issues involved with the incidental finding of a syringomyelia. Surgical intervention has been known to worsen symptoms. Conversely, studies have identified minimal radiological progression in cases of idiopathic syringomyelia, with fewer individuals displaying neurological deterioration. For aircrew, potentially unnecessary neurosurgical intervention poses risks to a flying career and overall operational capability.Schiemer A. Idiopathic syringomyelia in a military helicopter pilot. Aerosp Med Hum Perform. 2017; 88(10):962-965.

  14. Acute Idiopathic Syringomyelia: A Case Report

    Directory of Open Access Journals (Sweden)

    June-Kai Chen

    2004-08-01

    Full Text Available There have been isolated reports in the literature of the natural history and less than complete knowledge of the epidemiology and pathophysiology of syringomyelia. This article describes a clinically acute-onset abnormal patient with localized dilatation of the central canal of the spinal cord who had no evidence of present or prior Chiari's malformation, trauma, infection, tumor, or other predisposing pathologic condition. It is suggested that this distinct condition be labeled “acute idiopathic syringomyelia”.

  15. CT in three cases of syringomyelia

    International Nuclear Information System (INIS)

    Ugawa, Yoshikazu; Sakuta, Manabu; Yagishita, Akira; Inoue, Kiyoharu.

    1983-01-01

    We presented the results of our experience with metrizamide computed tomographic myelography (MCTM) and myelography in three cases of syringomyelia. In case 1, MCTM revealed Chiari malformation, even though this malformation was not clear in the myelography. In case 3, Chiari malformation was apparent in MCTM and in the myelography. In case 1, CT of the spinal cord without enhancement (plain CT) showed a syrinx in the cervical spinal cord, and MCTM made the syrinx clearer with enhancement. MCTM also demonstrated a cavity at the levels of Th 6 and L 1 of the vertebra. In case 2, a syrinx was disclosed in MCTM, in spite of normal myelography. In case 3, myelography demonstrated a spinal cord swelling. MCTM made a syrinx less clear than plain CT. Not only MCTM but also plain CT is an excellent method for the recognition of syringomyelia. The non-invasive plain CT is the first choice of the examinations in cases with syrinogomyelia, because MCTM may make a syrinx obscure. CT and MCTM have to be performed at many levels of the spinal cord in case with syringomyelia. CT or MCTM may demonstrate a syrinx or a cavity at the levels of the spinal cord, where no syrinx is suspected to exist by clinical features, as well as at the levels appearing normal in the myelography. (author)

  16. Syringomyelia and cervical spondylosis: A clinicoradiological investigation

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Y.L.; Moseley, I.F.

    1987-03-01

    Eight eight patients with proven syringomyelia have been studied retrospectively to explore the relationship between syringomyelia and spondylotic degeneration of the cervical spine. When compared with age and sex matched control subjects, they showed no significant increase in incidence or severity of spondylosis or of vertebral subluxation. Duration of symptoms, degree of disablity, and the pattern of analgesia or lower motor neurone lesions in the arms did not appear to be related to the development of spondylosis. The onset of moderate spondylosis, was however, earlier than in the controls, and individual cases with a remarkably early onset or unusual site and/or severity of spondylosis and subluxation were encountered, particularly following laminectomy. It was also noted that in a small number of patients the concomitant development of spondylosis contributed to clinical deterioration. It is concluded that while syringomyelia does not have a consistent, major role in the development of cervical spondylosis or vertebral luxation, it may contribute to progression of spinal degenerative disease in some patients.

  17. Syringomyelia and cervical spondylosis: A clinicoradiological investigation

    International Nuclear Information System (INIS)

    Yu, Y.L.; Moseley, I.F.

    1987-01-01

    Eight eight patients with proven syringomyelia have been studied retrospectively to explore the relationship between syringomyelia and spondylotic degeneration of the cervical spine. When compared with age and sex matched control subjects, they showed no significant increase in incidence or severity of spondylosis or of vertebral subluxation. Duration of symptoms, degree of disablity, and the pattern of analgesia or lower motor neurone lesions in the arms did not appear to be related to the development of spondylosis. The onset of moderate spondylosis, was however, earlier than in the controls, and individual cases with a remarkably early onset or unusual site and/or severity of spondylosis and subluxation were encountered, particularly following laminectomy. It was also noted that in a small number of patients the concomitant development of spondylosis contributed to clinical deterioration. It is concluded that while syringomyelia does not have a consistent, major role in the development of cervical spondylosis or vertebral luxation, it may contribute to progression of spinal degenerative disease in some patients. (orig.)

  18. Foraminal syringomyelia: suggestion for a grading system.

    Science.gov (United States)

    Versari, P P; D'Aliberti, G; Talamonti, G; Collice, M

    1993-01-01

    The standard treatment of foraminal syringomyelia includes foramen magnum decompression and duraplasty. Improvement or stabilization of the disease are achieved in most of cases. However, at least one third of patients are reported to receive little or no benefit. In this paper we retrospectively reviewed a series of 40 consecutive foramen magnum decompressions in order to identify the possible pre-operative outcome predictors. Based on clinical evolution, neurological impairment and radiological features, a scale of severity was fixed and retrospectively tested. A pre-operative score was obtained for each patient and was correlated with the surgical results. Then a four level grading system was derived. All grade I and grade II patients achieved good results (improvement or stabilization), whereas grade III patients showed intermediate behaviour and grade IV invariably worsened. On this basis, surgical results of foramen magnum decompression might be further improved provided that a careful pre-operative selection is made.

  19. Radiographic studies of the ventricles in syringomyelia

    International Nuclear Information System (INIS)

    West, R.J.; Williams, B.

    1980-01-01

    Radiographic investigations of 171 patients with communicating syringomyelia have been reviewed. Hydrocephalus was found in one third of the cases and has occasionally progressed after operation on the posterior fossa, sometimes with accompanying clinical deterioration. The outlets of the fourth ventricle were usually abnormal; tonsillar descent, arachnoiditis and both together were seen. Arachnoiditis correlated strongly with a history of difficult birth. The foramen of Magendie was sometimes patent and sometimes blocked. There was no consistent level of occulusion corresponding to a persistent roof of the fourth ventricle. The cisterna magna was usually small or obliterated but some examples of large cisterns or subarachnoid pouches were found. Radiological demonstration of a communication from the fourth ventricle to the syrinx occurred in only seven patients by positive contrast material and not by air. It is suggested that a sizable communication is rare at the time when patients seek treatment. (orig.)

  20. MR imaging of syringomyelia secondary to arachnoid adhesions

    International Nuclear Information System (INIS)

    Mark, A.S.; Andrews, B.; Sanches, J.; Manelfe, C.; Norman, D.; Newton, T.H.

    1987-01-01

    Arachnoid adhesions have been recognized as a cause of syringomyelia. Ten patients with surgically proven syringomyelia secondary to arachnoid adhesions (seven postraumatic, two postinfectious, one post-Pantopaque) were examined by MR imaging using sagittal and axial T1-weighted and sagittal T2-weighted sequences. A syrinx with loss of the sharp edge between the cord and the subarachnoid space as compared with the sharp interface seen in syringomyelia associated with Chiari I, was demonstrated on T1-weighted sequences in all patients. Intradural arachnoid cysts were found in nine patients. After surgical fenestration of the cyst, four patients improved and five remained unchanged. In one patient the syrinx decreased after fenestration of the cyst. Early detection and fesestration of these cavities may improve the patients' outcome

  1. Spontaneous drainage in syringomyelia: magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Bogdanov, E.I.; Mendelevich, E.G.; Ibatullin, M.M.

    2000-01-01

    We present five cases of syringomyelia associated with Chiari I or other causes of partial obstructions at the cervicomedullary junction, with spontaneous disruption of the wall of a cervical syrinx and formation of a communication between the cavity and the subarachnoid space, shown on axial MRI. MRI can be used to investigate the hydrodynamics, showing the liquid inside the disrupted syrinx wall and the pathway of drainage. The finding of spontaneous drainage may be important for understanding the pathogenesis of syringomyelia and may be helpful for choosing a surgical approach. (orig.)

  2. Syringomyelia presenting with unilateral optic neuropathy: a case report

    Directory of Open Access Journals (Sweden)

    Ngoo QZ

    2017-03-01

    Full Text Available Qi Zhe Ngoo, Evelyn Li Min Tai, Wan Hazabbah Wan Hitam Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia Purpose: In this case report, we present two cases of syringomyelia with optic neuropathy.Findings: In Case 1, a 36-year-old Malay lady presented to our clinic with acute onset of blurring of vision in her left eye that she experienced since past 1 month. She was diagnosed with syringomyelia 12 years ago and was on conservative management. Her visual acuity was 6/6 in the right eye and counting fingers at 1 m in the left. There was a positive relative afferent pupillary defect in her left eye. Optic nerve functions of her left eye were reduced. Visual field showed a left inferior field defect. Her extraocular muscle movements were full. Magnetic resonance imaging of the brain and spine showed syringomyelia at the level of C2–C6 and T2–T9. Both of her optic nerves were normal. Her condition improved with intravenous and oral corticosteroids. In Case 2, a 44-year-old Malay lady presented to our clinic with a progressive central scotoma in her right eye that she experienced since past 1 month. She had previous history of recurrent episodes of weakness in both of her lower limbs from past 8 months. Visual acuity in her right and left eye was 6/9 and 6/6, respectively. The relative afferent pupillary defect in her right eye was positive. Optic nerve functions of her right eye were affected. Visual field showed a central scotoma in her right eye. Her extraocular muscle movements were full. Fundoscopy of her right eye showed a pale optic disc. Her left eye fundus was normal. Magnetic resonance imaging of the brain and spine showed syringomyelia at T3–T6. Both of her optic nerves were normal. A diagnosis of syringomyelia with right optic atrophy was performed. Her condition improved with intravenous and oral corticosteroids.Conclusion: Optic neuropathy is a rare neuro

  3. Post-traumatic syringomyelia; Die posttraumatische Syringomyelie

    Energy Technology Data Exchange (ETDEWEB)

    Freund, M.; Sartor, K. [Universitaetsklinikum Heidelberg (Germany). Abt. Neuroradiologie; Aschoff, A. [Universitaetsklinikum Heidelberg (Germany). Neurochirurgische Klinik; Spahn, B. [Universitaetsklinikum Heidelberg (Germany). Abt. Orthopaedie 2

    1999-12-01

    The improvement of preclinical emergency medicine, better surgical and conservative therapies, and the development of intensive care units and specialized centers have improved the survival rate for patients with serious spinal cord injuries. Therefore, more sequelae of chronic spinal cord injuries such as post-traumatic spinal cord cavitations also occur. The first such case was described by Bastian in 1867. Generally, these cavitations were diagnosed from 2 months up to 32 years after the trauma. The overall prevalence of post-traumatic syringomyelia (PTS) is not known; however, with the increasing use of magnetic resonance imaging (MRI), its diagnosis has increased, ranging from 2.3% of paraplegic and tetraplegic patients in 1976 and 3.2% in 1985, to nearly 50% in a selected group of patients in 1991 and 1993. In 1995, a 4.45% incidence was reported. In our clinic we are currently treating 440 cases of syringomyelia, 140 of which are PTS. Several observations suggest more than one potential mechanism for the evolution of a post-traumatic cyst or PTS. Various factors, such as hemorrhage or, in particular, ischemia within the spinal cord, blockage of the cerebrosinal fluid (CSF) pathways around the cord or localized meningeal fibrosis either alone or in combination with other factors, may be involved. Clinically, sensory disturbances, loss of motor function, pain, and modification of the deep tendon reflexes are observed in most patients. On MRI, PTS is seen as a longitudinal, cystic cavity within the spinal cord, giving a hypointense signal on T{sub 1}-weighted images and a hyperintense signal on T{sub 2}-weighted images. For treatment planning it is mandatory to identify the lower and upper end of the PTS on the MRI. (orig.) [German] Aufgrund der verbesserten medizinischen Versorgung schwer Rueckenmarkverletzter hat die Zahl der (Langzeit-) Ueberlebenden erheblich zugenommen. Allerdings koennen Spaetkomplikationen auftreten, die zu einer deutlichen Zunahme der

  4. MRI of secondary cervical syringomyelia in four cats

    International Nuclear Information System (INIS)

    Okada, Midori; Itou, Takuya; Sakai, Takeo; Kitagawa, Masato; Ito, Daisuke; Kanayama, Kiichi

    2009-01-01

    This report describes the use of magnetic resonance imaging (MRI) to diagnose cervical syringomyelia in 4 cats. MRI revealed enlargement of the lateral ventricle in all the cats. Of the 4 cases, MRI revealed herniation of the cerebellum in 3 cats, an isolated fourth ventricle in 1 cat, severe hydrocephalus in 2 cats and brain masses in 1 cat. In this report, the cervical syringomyelia in these cats may have been due to formation of a secondary syrinx (enlargement of the central canal) as a result of blockage of flow in the outlet of the fourth ventricle caused by feline infectious peritonitis (FIP) encephalomyelitis or secondary cerebellar tonsillar herniation caused by increased intracranial pressure due to intracranial masses or may have been due to caudal compression of the cerebellum caused by increased intracranial pressure due to hydrocephalus. (author)

  5. ANAESTHETIC MANAGEMENT IN A PATIENT WITH ARNOLD-CHI ARI MALFORMATION TYPE I AND SYRINGOMYELIA

    OpenAIRE

    Kartika; Pratap; Vijayalaxmi; Kalyan Chakravarthy; Nagaraju

    2013-01-01

    ABSTRACT: Syringomyelia is an unusual neurological condition characterised by the the presence of cystic cavity in the spinal cord resultin g in neurological manifestations. Here, we report a safe anesthetic management of patient with Arnold-Ch iari malformation type I and syringomyelia posted for foramen magnum decompression . INTRODUCTION: Arnold-Chiari malformation (ACM) is a developmental malformation characterised by downward displacement of cere...

  6. Volume change theory for syringomyelia: A new perspective.

    Science.gov (United States)

    Rai, Survendra Kumar Rajdeo; Rai, Pooja Survendra Kumar

    2015-01-01

    The etiopathogenesis of syringomyelia is still an enigma. The authors present a novel theory based on fluid dynamics at the craniovertebral (CV) junction to explain the genesis of syringomyelia (SM). The changes in volume of spinal canal, spinal cord, central canal and spinal subarachnoid space (SSS) in relation to the posterior fossa have been analysed, specifically during postural movements of flexion and extension. The effect of fluctuations in volume of spinal canal and its contents associated with cerebrospinal fluid (CSF) flow dynamics at the CV junction have been postulated to cause the origin and propagation of the syringomyelia. The relevant literature on the subject has been reviewed and the author's theory has been discussed. Volume of spinal canal in flexion is always greater than that in extension. Flexion of spine causes narrowing of the ventral subarachnoid space (SAS) and widening of dorsal SAS while extension causes reverse changes leading to fluid movement in dorsal spinal SAS in flexion and ventral spinal SAS in extension. Cervical and lumbar spinal region with maximum bulk hence maximum area and volume undergo maximum deformation during postural changes. SSS CSF is the difference between the volume of spinal canal and spinal cord, varies in flexion and extension which is compensated by changes in posterior fossa (CSF) volume in normal circumstances. Blocked SAS at foramen magnum donot permit spinal SAS CSF exchange which during postural changes is compensated by cavitatory/cystic (syrinx) change at locations in cervical and lumbar spine with propensity for maximum deformation. Augmentation of posterior fossa volume by decompression helps by normalization of this CSF exchange dynamics but immobilizing the spinal movement theoretically will cease any dynamic volume changes thereby minimizing the destructive influence of the fluid exchange on the cord. Thus, this theory strengthens the rational of treating patients by either methodology.

  7. [Oropharyngeal dysphagia associated with Chiari I malformation and syringomyelia J].

    Science.gov (United States)

    Cortés-Medina, Julio César; Cárdenas-Lara, Armando; Guerrero-Rascón, Carlos Alberto; Rodríguez-Bautista, Heber

    2014-01-01

    Dysphagia associated with neurological disease is an important clinical manifestation in the diagnosis of injury that justifies the compression of the brainstem and lower cranial nerves. To emphasize the study of dysphagia in a patient with Chiari I malformation associated with syringomyelia in the absence of primary gastroenterological symptoms. We describe the case of a 62 year-old woman with oropharyngeal dysphagia of six years of evolution, cervicobrachialgia, ptosis and facial diplexia. Magnetic resonance imaging is an essential element for establishing the etiologic diagnosis of neurogenic dysphagia.

  8. [Hemihypertrophy and scoliosis revealing a Chiari 1 malformation with syringomyelia].

    Science.gov (United States)

    Benjamin, M-D; Santiago, J; Hebert, J-C; Thirion, S; Ranaivojaona, S; Alvarez, C; Atallah, A; Sibille, G; Bataille, H; Porlys, M; Ebrad, P

    2011-11-01

    We report the case of a 9-year-old boy with progressive thoracic scoliosis and crossed hemihypertrophy who was discovered with a Chiari 1 malformation and syringomyelia. These disorders are connected by complex physiopathological mechanisms; their association deserves attention. This observation reviews the importance of the clinical examination, particularly the neurological exam, in childhood scoliosis. The features suggesting a neurogenic background of spine deformation should be sought. Scoliosis with hemihypertrophy can be the sign of an underlying neurological abnormality. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  9. Giant posterior fossa arachnoid cyst causing tonsillar herniation and cervical syringomyelia

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    Vijay P Joshi

    2013-01-01

    Full Text Available Acquired cerebellar tonsillar herniation and syringomyelia associated with posterior fossa mass lesions is an exception rather than the rule. In the present article, we describe the neuroimaging findings in a case of 28-year-old female patient presented with a history of paraesthesia involving right upper limb of 8-month duration. Magnetic resonance imaging showed a giant retrocerebellar arachnoid causing tonsillar herniation with cervical syringomyelia. The findings in the present case supports that the one of the primary mechanism for the development of syringomyelia may be the obstruction to the flow of cerebrospinal fluid causing alterations in the passage of extracellular fluid in the spinal cord and leading to syringomyelia.

  10. Difficult intubation in a parturient with syringomyelia and Arnold–Chiari malformation: Use of Airtraq™ laryngoscope

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    Mustapha, Bensghir; Chkoura, K.; Elhassani, M.; Ahtil, R.; Azendour, H.; Kamili, N. Drissi

    2011-01-01

    Anesthetic technique in parturient with syringomyelia and Arnold–Chiari malformation is variable depending on the teams. Difficult intubation is one of the risks when general anesthesia is opted. Different devices have been used to manage the difficult intubation in pregnant women. We report the use of Airtraq™ laryngoscope after failed standard laryngoscopy in a parturient with syringomyelia and Arnold–Chiari type I malformation. PMID:22144932

  11. Difficult intubation in a parturient with syringomyelia and Arnold-Chiari malformation: Use of Airtraq™ laryngoscope

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    Bensghir Mustapha

    2011-01-01

    Full Text Available Anesthetic technique in parturient with syringomyelia and Arnold-Chiari malformation is variable depending on the teams. Difficult intubation is one of the risks when general anesthesia is opted. Different devices have been used to manage the difficult intubation in pregnant women. We report the use of Airtraq™ laryngoscope after failed standard laryngoscopy in a parturient with syringomyelia and Arnold-Chiari type I malformation.

  12. Acquired Chiari Malformation and Syringomyelia Secondary to Space-Occupying Lesions: A Systematic Review.

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    Wang, Justin; Alotaibi, Naif M; Samuel, Nardin; Ibrahim, George M; Fallah, Aria; Cusimano, Michael D

    2017-02-01

    Acquired Chiari malformations (ACM) and associated syringomyelia secondary to space-occupying lesions can cause neurologic deficits independent of or in combination with the offending mass. Although type I Chiari malformations are traditionally treated with posterior fossa decompression, optimal surgical management of ACM and associated syringomyelia remains unclear. The purpose of this study is to review the current literature surrounding the management of ACM. A systematic review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Using the relevant keywords, articles were identified through multiple databases from inception to April 2016. Our primary outcome was postoperative resolution of tonsillar herniation, syringomyelia, and clinical symptoms and signs. Thirty studies (27 case reports and 3 case series), encompassing 44 patients, were included in the review. Meningiomas (36%) and arachnoid cysts (32%) were the lesions most commonly associated with ACM. Offending lesions were usually large (85%) and almost all were found in the posterior fossa (89%). Syringomyelia was present in 82% of cases. Overall, all but 1 patient had improvement or resolution of their syringomyelia after surgery and none required a syrinx shunt. Rates of tonsillar ascent, syrinx resolution, and neurologic recovery were similar in patients who underwent lesion removal alone versus those who underwent posterior fossa decompression and lesion removal. Space-occupying lesions in the posterior fossa are a rare cause of ACM and syringomyelia. Surgical management of the underlying lesion improves ACM and associated syringomyelia without the need for syrinx shunting. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Shunting of recurrent post-traumatic syringomyelia into the fourth ventricle: a case report

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    Lin Chih-Lung

    2010-07-01

    Full Text Available Abstract Introduction Post-traumatic syringomyelia is a progressive degenerative disorder that is a well-recognized sequela of spinal cord injury. There is currently no optimal intervention capable of producing satisfactory long-term clinical results. Case presentation In this report, we present a 55-year-old Asian man with recurrent syringomyelia after shunt treatment. The syrinx extended from the thoracic cord into the medulla. We used a silicone tube to create a channel connecting the syrinx cavity directly to the fourth ventricle. The patient made a good recovery and follow-up magnetic resonance imaging revealed a considerable diminution in the size of the syrinx. Conclusions We present a new approach that has the potential to improve the outcome of patients with recurrent post-traumatic syringomyelia, who cannot be treated by conventional methods.

  14. Choroid Plexus in the Central Canal of the Spinal Cord Causing Recurrent Syringomyelia.

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    Shtaya, Anan; Sadek, Ahmed-Ramadan; Nicoll, James A R; Nader-Sepahi, Ali

    2018-03-01

    Syringomyelia is a fluid-filled cavitation within the substance of the spinal cord. This condition usually follows a primary pathology that disrupts the normal cerebrospinal fluid circulation or disturbs the microcirculation and cytoarchitecture of the spinal cord parenchyma. However, an etiology of recurrent syringomyelia resulting from an ectopic choroid plexus (CP) has not been discussed. Ectopic CP rests may be found within the central nervous system. Although there has been a single report, describing ectopic intramedullary spinal cord CP, to our knowledge, extra-cranial nonmalignant CP in the central canal of the spinal cord has not been reported. We report CP in the central canal of the spinal cord in a 23-year-old male patient who had developmental delay and diabetes mellitus type I who presented with dissociated sensory changes and muscle wastage predominantly on the right upper and lower limbs. Magnetic resonance imaging demonstrated a multiloculated spinal cord syringomyelia stretching from cervical (C3) to the conus medullaris causing recurrent neurologic deficits. A biopsy of the central canal spinal cord lesion revealed CP. Decompression and syringosubarachnoid shunt insertion stabilized the patient's neurology. Our illustrative case reveals the presence of CP in the central canal of the spinal cord that may suggest a role in the etiology of recurrent syringomyelia. Although management poses a challenge to neurosurgeons, prompt decompression and shunting of the syringomyelia remains a favorable approach with acceptable outcomes. Further investigation into the pathophysiology of central canal CP ectopic causing recurrent syringomyelia and its correlation with spinal cord development may help future treatments. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Anesthetic and Obstetric Management of Syringomyelia During Labor and Delivery: A Case Series and Systematic Review.

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    Garvey, Gráinne Patricia; Wasade, Vibhangini S; Murphy, Kellie E; Balki, Mrinalini

    2017-09-01

    Syringomyelia is a rare, slowly progressive neurological condition characterized by the presence of a syrinx within the spinal cord. Consensus regarding the safest mode of delivery and anesthetic management in patients with syringomyelia remains controversial and presents management dilemmas. This study reviews the cases of syringomyelia at our institution and provides a systematic review of the literature to guide decisions regarding labor and delivery management. A retrospective review of cases at our hospital from 2002 to 2014 and a systematic review of the literature from 1946 to 2014 were undertaken. Hospital records and electronic databases were interrogated using International Classification of Diseases, 10th Revision codes and the keywords "syringomyelia," "syringobulbia," and "pregnancy." Data regarding demographics, diagnosis, radiology reports, neurological symptoms, mode of delivery, anesthetic management, and maternal-fetal outcomes were collected. We collected and analyzed data on a total of 43 pregnancies in 39 patients. The most common location for syrinx was in the cervicothoracic region (41.9%). The large majority of patients (n = 34; 87%) demonstrated signs and symptoms associated with syringomyelia before delivery. Syringomyelia associated with Arnold Chiari malformation was documented in 49% (n = 21) cases. General anesthesia was the most commonly used (n = 21/30, 70%) anesthetic technique for cesarean delivery. The majority (n = 9/13, 69%) of patients had an epidural sited for labor analgesia. There were no maternal or neonatal complications associated with neuraxial anesthesia; however, 3 cases (14%) raised concerns regarding general anesthesia including difficult intubation, transient worsening of neurological symptoms postpartum, and prolonged muscle paralysis after atracurium. Despite concerns regarding aggravation of the syrinx with vaginal delivery, this mode of delivery has never caused any documented long-term worsening of neurological

  16. Magnetic resonance imaging of post-traumatic syringomyelia and its surgical treatment

    International Nuclear Information System (INIS)

    Isu, Toyohiko; Iwasaki, Yoshinobu; Nunomura, Mitsuru; Akino, Minoru; Koyanagi, Izumi; Abe, Hiroshi; Saito, Hisatoshi.

    1991-01-01

    The purpose of this study was to review magnetic resonance imaging (MRI) scans of post-traumatic syringomyelia and to assess the outcome of surgical treatment. The subjects were 16 patients (13 men and 3 women) whose ages ranged from 22 to 69 years, with a mean of 42 years. Nine patients had delayed neurologic symptoms 2 years and 2 months through 32 years after spinal injuries. The site of initial spinal cord injury was the lower cervical region in 4 patients, the thoracic region in 8, and the upper lumbar region in 4. In all patients, post-traumatic syringomyelia was easy to diagnose on MRI. MRI showed the syrinx extending superiorly and/or inferiorly from the area of old trauma, sometimes extending to the medulla oblongata. In the cervical cord and the upper thoracic cord, the syrinx was unilaterally or bilaterally situated in the postero-lateral portion. Below the middle thoracic cord, the syrinx was centrally located. Surgery was performed in 6 patients. At an average follow up of 2 years and 9 months, both pain and numbness were relieved in all patients (100%) and neurologic symptoms improved in 5 patients (83%). Post-traumatic syringomyelia should be considered in all patients having delayed onset or aggravation of neurologic symptoms after spinal injury. MRI appears promising for the early diagnosis of post-traumatic syringomyelia that can be treated favorably by surgical procedures. (N.K.)

  17. Syringomyelia following tuberculous meningitis. Report of three cases diagnosed by MR imaging

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    Tsuchiya, Kazuhiro; Takeshita, Koji; Makita, Kozo; Furui, Shigeru; Takenaka, Eiichi

    1988-11-01

    We present three cases with syringomyelia after tuberculous meningitis. The MR findings suggested the syrinx was formed by blockage of the CSF flow at the outlets of the fourth ventricle. We consider this complication is not a rare condition following tuberculous meningitis.

  18. A rat model of chronic syringomyelia induced by epidural compression of the lumbar spinal cord.

    Science.gov (United States)

    Lee, Ji Yeoun; Kim, Shin Won; Kim, Saet Pyoul; Kim, Hyeonjin; Cheon, Jung-Eun; Kim, Seung-Ki; Paek, Sun Ha; Pang, Dachling; Wang, Kyu-Chang

    2017-10-01

    OBJECTIVE There has been no established animal model of syringomyelia associated with lumbosacral spinal lipoma. The research on the pathophysiology of syringomyelia has been focused on Chiari malformation, trauma, and inflammation. To understand the pathophysiology of syringomyelia associated with occult spinal dysraphism, a novel animal model of syringomyelia induced by chronic mechanical compression of the lumbar spinal cord was created. METHODS The model was made by epidural injection of highly concentrated paste-like kaolin solution through windows created by partial laminectomy of L-1 and L-5 vertebrae. Behavioral outcome in terms of motor (Basso-Beattie-Bresnahan score) and urinary function was assessed serially for 12 weeks. Magnetic resonance images were obtained in some animals to confirm the formation of a syrinx and to monitor changes in its size. Immunohistochemical studies, including analysis for glial fibrillary acidic protein, NeuN, CC1, ED-1, and caspase-3, were done. RESULTS By 12 weeks after the epidural compression procedure, syringomyelia formation was confirmed in 85% of the rats (34 of 40) on histology and/or MRI. The syrinx cavities were found rostral to the epidural compression. Motor deficit of varying degrees was seen immediately after the procedure in 28% of the rats (11 of 40). In 13 rats (33%), lower urinary tract dysfunction was seen. Motor deficit improved by 5 weeks after the procedure, whereas urinary dysfunction mostly improved by 2 weeks. Five rats (13%, 5 of 40) died 1 month postoperatively or later, and 3 of the 5 had developed urinary tract infection. At 12 weeks after the operation, IHC showed no inflammatory process, demyelination, or accelerated apoptosis in the spinal cords surrounding the syrinx cavities, similar to sham-operated animals. CONCLUSIONS A novel experimental model for syringomyelia by epidural compression of the lumbar spinal cord has been created. The authors hope that it will serve as an important research

  19. Surgical Management of Syringomyelia Associated with Spinal Adhesive Arachnoiditis, a Late Complication of Tuberculous Meningitis: A Case Report.

    Science.gov (United States)

    Lee, Jun Seok; Song, Geun Sung; Son, Dong Wuk

    2017-04-01

    Syringomyelia associated with tuberculous meningitis is an extremely rare condition. Only a few studies have reported clinical experience with syringomyelia as a late complication of tuberculous meningitis. Twenty-six years after a tuberculous meningitis episode, a 44-year-old man presented with progressively worsening spastic paresis of the lower limbs and impaired urinary function for 2 years. Radiological examination revealed syringomyelia extending from the level of C2 to T9 and arachnoiditis with atrophy of the spinal cord between C2 and T3. We performed laminectomy from C7 to T1, dissected the arachnoid adhesion and placed a syringo-pleural shunt via keyhole myelotomy. One year after the operation, his neurological condition improved. The postoperative control magnetic resonance imaging revealed the correctly located shunt and significantly diminished syringomyelia cavities. We aim to discuss the mechanism of syrinx formation following tuberculous meningitis and to share our surgical therapeutic experience with this rare disease entity.

  20. Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformation.

    Science.gov (United States)

    Nacir, Baris; Arslan Cebeci, S; Cetinkaya, E; Karagoz, A; Erdem, H R

    2010-05-01

    Neuropathic arthropathy (NA), known as Charcot neuroarthropathy, is a chronic, degenerative arthropathy and is associated with decreased sensory innervation. Numerous causes of this arthropathy have been described. Neuropathic joint, although first described by Charcot in tabes dorsalis in 1868, has subsequently been observed in a variety of conditions including syringomyelia, diabetes mellitus and peripheral nerve disorders. Syringomyelia is characterized by slow progression. The shoulders and elbows are the most frequently involved joints in syringomyelia. Involvement of the hand is a quite rarely seen in the cases of NA caused by syringomyelia. In this article, we reported a case of NA secondary to syringomyelia. The characteristics of this presented case is the presence of Arnold-Chiari malformation accompanying with syringomyelia and involvement of the shoulder, elbow and hand (multiple joint involvement).

  1. Clinical findings in syringomyelia in contrast to the lesions seen in NMR imaging

    International Nuclear Information System (INIS)

    Besser, R.

    1987-01-01

    Syringomyelia is a congenital disease of the spinal cord that can progress throughout the patient's lifetime. The primary symptoms of this disorder are described. The authors discuss how the diagnosis of syringomyelia is difficult because the illness progresses very slowly and can remain stable for decades. They describe their utilization of a superior method for the diagnosis of syringomyelic cavities in the spinal cord: NMR-imaging. It is not invasive and the entire brain stem and cervical cord can be shown on one picture. The caudal spinal cord can also be examines. Other diseases, such as, meningioma and glioma, can be excluded and one can assess the possibility whether a Arnold-Chiari Malformation may be present

  2. Syringomyelia secondary to cervical spondylosis: Case report and review of literature

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    Savitr Sastri Bhagavathula Venkata

    2014-01-01

    Full Text Available Syringomyelia secondary to cervical spondylosis is a rare entity to encounter in clinical practice. We discuss the case of a 53-year-old lady who presented with a syringomyelic syndrome and was found to have cervical spondylosis on imaging. Cine-MRI revealed an obstruction of cerebrospinal fluid (CSF flow in the cervical spinal subarachnoid space. Decompression of the same led to clinical and radiological improvement. There is a potential causal association between cervical spondylosis and syringomyelia. MRI CSF flow studies may help in deciding the course of treatment in such cases. A subset of patients with cervical spondylosis and concurrent spinal cord signal intensity changes may show reversal of the same following intervention.

  3. Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia.

    Science.gov (United States)

    Haldar, Rudrashish; Gyanesh, Prakhar; Samanta, Sukhen

    2013-10-01

    Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM), also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.

  4. Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia

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    Rudrashish Haldar

    2013-01-01

    Full Text Available Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM, also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.

  5. Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation

    International Nuclear Information System (INIS)

    Hite, S.H.; Krivit, W.; Haines, S.J.; Whitley, C.B.

    1997-01-01

    We present the imaging findings in a patient with mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) who developed holocord syringomyelia. This represents the only reported case of syrinx formation in a child with MPS VI. Clinical, neurologic and spinal magnetic resonance imaging findings are presented. The patient has maintained a stable clinical and neurologic course over the period following allogeneic bone marrow transplant. (orig.). With 3 figs

  6. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

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    Jing-cheng XIE

    2016-04-01

    Full Text Available Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS.  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 years old. The course of disease ranged from 3 months to 20 years (average 42.17 months. Among those patients, one patient presented hydromyelia, 8 patients suffered from meningeal cyst within the sacral canal, and one patient were concurrent with sacral dermal sinus. The weakness of lower extremities, especially distal limbs, was the main clinical manifestation. Five patients were accompanied with bowel and bladder dysfunction and 5 patients with sensory disturbance below the level of syringomyelia, especially hypesthesia. Preoperative MRI showed conus medullaris disappeared at the end of spinal cord, and there was fluid signal in the lower spinal cord with hypo-intensity signal in T1WI and hyper-intensity signal in T2WI without enhancement. All patients underwent surgical procedures. Under microscope, filum terminale was cut off, drainage was performed, meningeal cyst within the sacral canal was removed, and tethered cord was released. The success rate of operations was 100%. The duration of surgery ranged from 1.52 to 3.07 h (average 2.15 h, with average intraoperative blood loss 220 ml (ranged from 100 to 410 ml. The tethering filum had been totally resected and histological examination showed typical filum tissue in all cases. No operative complication was found. Visual Analogue Scale (VAS score was decreased, and the lower limbs weakness as well as bowel and bladder dysfunction was gradually relieved after operation. The period of follow-up was ranged from 6

  7. Clinical and radiological outcome of craniocervical osteo-dural decompression for Chiari I-associated syringomyelia.

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    Spena, Giannantonio; Bernucci, Claudio; Garbossa, Diego; Valfrè, Walter; Versari, Pietro

    2010-07-01

    The aim of this study was to analyze the long-term clinical and radiological outcomes of craniocervical decompression for patients affected by Chiari I-related syringomyelia. We performed a retrospective analysis of a group of patients affected by Chiari I-associated syringomyelia treated by craniocervical decompression (CCD). Surgical and technical aspects and preoperative factors predicting outcome were discussed. A total of 36 patients were reviewed. There were 17 men and 19 women (female/male ratio 1.11), and the mean age was 40.4 (range 18-68). The most important preoperative symptoms were related to myelopathy (pain, weakness, atrophy, spasticity, sensory loss, and dysesthesias). Most syrinxes were in the cervico-thoracic region (61.1%), and the majority of patients had tonsillar descent between the foramen magnum and C1. All patients underwent a craniectomy less than 3 cm in diameter followed by a duroplasty with dura substitute. No arachnoid manipulation was necessary. Three patients (8.1%) experienced cerebrospinal fluid leaks that resolved without complications. At a mean follow up of 40 months (range 16-72) 80.5% of patients exhibited improvement over their preoperative neurological examination while 11.1% stabilized. The syrinx shrank in 80.5% of patients. Chi-square test showed that preoperative syrinx extension and degree of tonsillar descent did not correlate with clinical and neuroradiological postoperative evolution. Treating syringomyelia associated in Chiari I malformation with CCD leads to a large percentage of patients with satisfying results and no irreversible complications.

  8. The nicotinic acid balance in patients with syringomyelia in the course of deep X-ray therapy

    International Nuclear Information System (INIS)

    Oganesyan, A.A.

    1975-01-01

    Patients with syringomyelia showed a lowered nicotinic acid content, which deficit increased with deep x-ray therapy. Differences in the total dose of penetrating radiation did not seem to affect the providing of nicotinic acid to the patients' organism. (V.A.P.)

  9. Sphenoid Sinus Carcinoma with Intramedullary Spinal Cord Metastasis and Syringomyelia - Report of A Case -

    International Nuclear Information System (INIS)

    Oh, Yoon Kyeong; Kim, Young Sook

    1996-01-01

    Purpose : Primary sphenoid carcinoma is rare. It accounts for 0.3% of all primary paranasal sinus malignancies. Because of the rarity of sphenoid carcinoma, large series of patients with outcome and survival statistics are currently unavailable. So we followed up the 1 case of sphenoid sinus carcinoma treated in our hospital and reported the course of the disease. In a review of case reports and small series of patients, 2-year survival was 7%. Our case is alive at 29 months after diagnosis of sphenoid sinus carcinoma. Intramedullary spinal cord metastasis (ISCM) is an unusual complication of cancer. In our case rapidly progressive paraparesis and urinary retention developed at 25 months after diagnosis of sphenoid sinus carcinoma. MRI of the thoracic spines showed the intramedullary spinal cord tumor mass at T3 and T4 level with accompanying syringomyelia. Here we report a case of ISCM associated with syringomyelia which has developed after primary sphenoid sinus carcinoma with a review of literature about the clinical behavior and treatment of this lesion

  10. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia.

    Science.gov (United States)

    Dlouhy, Brian J; Dawson, Jeffrey D; Menezes, Arnold H

    2017-12-01

    OBJECTIVE The pathophysiology underlying tonsillar herniation and CSF obstruction in Chiari malformation Type I (CM-I) is unclear, and the cause of CM-I-associated syringomyelia is not well understood. A better understanding of this pathophysiology is important for an improved treatment strategy. Therefore, the authors sought to identify, characterize, and examine the intradural pathology and CSF flow pathophysiology in the posterior fossa and at the level of the foramen magnum that occurs in the setting of CM-I. They determined the incidence of these intradural findings and assessed differences across age, with the degree of tonsillar herniation, and in the presence and absence of syringomyelia. METHODS A prospective database initiated in March 2003 recorded all intraoperative findings during surgical treatment of children and adults with CM-I with or without syringomyelia. A total of 389 surgeries for CM-I were performed in 379 patients between March 2003 and June 2016. A total of 109 surgeries were performed in 109 patients with CM-I (without osseoligamentous abnormalities) in whom both a posterior fossa extradural and intradural decompression with duraplasty was performed (first-time intradural procedures). Using a surgical microscope, intradural pathology and obstruction of CSF channels were identified and assessed. Student t-tests and Fisher's exact tests compared groups in a series of univariate analyses, followed by multivariate logistic regression. RESULTS The following intradural pathological entities were observed (prevalence noted in parentheses). These include those that did not obstruct CSF flow channels: opacified arachnoid (33.0%), thickened arachnoid (3.7%), ischemic and gliotic tonsils (40.4%), tonsillar cysts (0.9%), and inferior descent of the fourth ventricle and cervicomedullary junction (CMJ) (78.0%). The following intradural pathological entities were observed to obstruct CSF flow channels: medialized tonsils (100%), tonsil overlying and

  11. Foramen magnum decompression versus terminal ventriculostomy for the treatment of syringomyelia.

    Science.gov (United States)

    Filizzolo, F; Versari, P; D'Aliberti, G; Arena, O; Scotti, G; Mariani, C

    1988-01-01

    The A.A review 30 consecutive cases of syringomyelia operated on during the last seven years. Six terminal ventriculostomies (TV) and twenty-seven procedures for foramen magnum decompression (FMD) were performed. All patients of TV group had CT-myelography (CTM) and/or NMR controls at different times after surgery. Clinical results are as follows: 1) of the 6 patients who had TV, only one showed an improvement while five continued to deteriorate and three of them needed a FMD, one a cysto-peritoneal shunt and the last one died from lung cancer. 2) of the 27 patients who had FMD, twenty improved, four were unchanged and three worsened. 3) no surgical deaths occurred in this series. Postoperative NMR monitoring represents an effective non-invasive neuroradiological procedure that allows follow-up of syrinx evolution over the years.

  12. Association of HTLV-I with Arnold Chiari syndrome and syringomyelia

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    Graça Maria de Castro Viana

    Full Text Available HTLV-I is associated with a broad spectrum of manifestations, including tropical spastic paraparesis and adult T-cell leukemia/lymphoma. Arnold Chiari syndrome is a condition characterized by herniation of the cerebellar tonsils through the foramen magnum. This condition should be suspected in all patients with headache and impaired motor coordination. Syringomyelia is a developmental anomaly that leads to the formation of an intramedullary cavity. Its clinical presentation is classically characterized by syringomyelic dissociation of sensation, with suspended distribution in the proximal portion of the trunk and upper limbs and preservation in other regions. We report here a case of association of the three diseases, which is rare in clinical practice, illustrating the difficulty in the diagnosis and therapeutic management of these conditions.

  13. Syringomyelia and Arnold-Chiari malformation associated with neck pain and left arm radiculopathy treated with spinal manipulation.

    Science.gov (United States)

    Tieppo Francio, Vinicius

    2014-11-09

    An 18-year-old female patient presented with left dominant neck pain after a motor vehicle collision. Her cervical spine MRI revealed syringomyelia with associated Type I Arnold-Chiari malformation. Some researchers have reported that these might be considered contraindications to spinal manipulation. Nevertheless, her benign and functional clinical examination suggested otherwise and she underwent four manipulative treatments in 2 weeks. By the end of the treatment plan and after 1-month follow-up, she was asymptomatic, no adverse effects were noted and her outcome assessment score decreased from 56% to 0%. This case illustrates that spinal manipulation may be a useful adjunctive treatment procedure for spinal pain, even in the presence of syringomyelia and Chiari malformation, which may not necessarily be a contraindication to spinal manipulation, when performed by a skilled and well-trained physician. 2014 BMJ Publishing Group Ltd.

  14. Anesthetic management during Cesarean section in a woman with residual Arnold–Chiari malformation Type I, cervical kyphosis, and syringomyelia

    Science.gov (United States)

    Ghaly, Ramsis F.; Candido, Kenneth D.; Sauer, Ruben; Knezevic, Nebojsa Nick

    2012-01-01

    Background: Type I Arnold–Chiari malformation (ACM) has an adult onset and consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum. There is paucity of literature on the anesthetic management during pregnancy of residual ACM Type I, with cervical xyphosis and persistent syringomyelia. Case Description: A 34-year-old woman with surgically corrected ACM Type I presented for Cesarean delivery. A recent MRI demonstrated worsening of cervical xyphosis after several laminectomies and residual syringomyelia besides syringopleural shunt. Awake fiberoptic intubation was performed under generous topical anesthesia to minimize head and neck movement during endotracheal intubation. We used a multimodal general anesthesia without neuromuscular blockade. The neck was maintained in a neutral position. Following delivery, the patient completely recovered in post-anesthesia care unit (PACU), with no headache and no exacerbation or worsening of neurological function. Conclusions: The present case demonstrates that patients with partially corrected ACM, syringomyelia, cervical kyphosis, and difficult intubation undergoing Cesarean delivery require an interdisciplinary team approach, diligent preparation, and skilled physicians. PMID:22439117

  15. Anesthetic management during Cesarean section in a woman with residual Arnold-Chiari malformation Type I, cervical kyphosis, and syringomyelia.

    Science.gov (United States)

    Ghaly, Ramsis F; Candido, Kenneth D; Sauer, Ruben; Knezevic, Nebojsa Nick

    2012-01-01

    Type I Arnold-Chiari malformation (ACM) has an adult onset and consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum. There is paucity of literature on the anesthetic management during pregnancy of residual ACM Type I, with cervical xyphosis and persistent syringomyelia. A 34-year-old woman with surgically corrected ACM Type I presented for Cesarean delivery. A recent MRI demonstrated worsening of cervical xyphosis after several laminectomies and residual syringomyelia besides syringopleural shunt. Awake fiberoptic intubation was performed under generous topical anesthesia to minimize head and neck movement during endotracheal intubation. We used a multimodal general anesthesia without neuromuscular blockade. The neck was maintained in a neutral position. Following delivery, the patient completely recovered in post-anesthesia care unit (PACU), with no headache and no exacerbation or worsening of neurological function. The present case demonstrates that patients with partially corrected ACM, syringomyelia, cervical kyphosis, and difficult intubation undergoing Cesarean delivery require an interdisciplinary team approach, diligent preparation, and skilled physicians.

  16. Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression.

    Science.gov (United States)

    Gil, Z; Rao, S; Constantini, S

    2000-09-01

    Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI.

  17. Comparison of gabapentin versus topiramate on clinically affected dogs with Chiari-like malformation and syringomyelia.

    Science.gov (United States)

    Plessas, I N; Volk, H A; Rusbridge, C; Vanhaesebrouck, A E; Jeffery, N D

    2015-09-19

    To date there is no evidence-based data for efficacious treatment of neuropathic pain in dogs with Chiari-like malformation (CM) and syringomyelia (SM). The objective of this prospective cross-over study was to compare the effect of gabapentin versus topiramate, as an add-on treatment to carprofen, on quality of life (QoL) of dogs experiencing signs of neuropathic pain due to CM/SM. A visual analogue scale (VAS) was used to assess the QoL: (1) on day 0; (2) after 1 week of carprofen only; (3) after 2 weeks on carprofen and gabapentin; and (4) after 2 weeks on carprofen and topiramate. No significant difference was observed between VAS after gabapentin or topiramate (P=0.91). However, an improvement in QoL was observed when gabapentin was compared with baseline (P=0.009), but not for topiramate. In conclusion, the addition of gabapentin was more effective in improving QoL than carprofen alone, but the study failed to identify that gabapentin was more efficacious than topiramate. Perhaps the more favourable side effect profile of the former makes it more suitable for the treatment of neuropathic pain associated with CM/SM but further placebo-controlled trials are required to assess the efficacy of these drugs. British Veterinary Association.

  18. Delamination technique together with longitudinal incisions for treatment of Chiari I/syringomyelia complex: a prospective clinical study

    Directory of Open Access Journals (Sweden)

    Savas Yildiray

    2009-06-01

    Full Text Available Abstract Background Treatment modalities in Chiari malformation type 1(CMI accompanied by syringomyelia have not yet been standardized. Pathologies such as a small posterior fossa and thickened dura mater have been discussed previously. Various techniques have been explored to enlarge the foramen magnum and to expand the dura. The aim of this clinical study was to explore a new technique of excision of the external dura accompanied by widening the cisterna magna and making longitudinal incisions in the internal dura, without disturbing the arachnoid. Methods Ten patients with CMI and syringomyelia, operated between 2004 and 2006, formed this prospective series. All cases underwent foramen magnum decompression of 3 × 3 cm area with C1–C2 (partial laminectomy, resection of foramen magnum fibrous band, excision of external dura, delamination and widening of internal dura with longitudinal incisions. Results Patients were aged between 25 and 58 years and occipital headache was the most common complaint. The mean duration of preoperative symptoms was 4 years and the follow-up time was 25 months. Clinical progression was halted for all patients; eight patients completely recovered and two reported no change. In one patient, there was a transient cerebrospinal fluid (CSF fistula that was treated with tissue adhesive. While syringomyelia persisted radiologically with radiological stability in five patients; for three patients the syringomyelic cavity decreased in size, and for the remaining two it regressed completely. Conclusion Removal of the fibrous band and the outer dural layer, at level of foramen magnum, together with the incision of inner dural layer appears to be good technique in adult CMI patients. The advantages are short operation time, no need for duraplasty, sufficient posterior fossa decompression, absence of CSF fistulas as a result of extra arachnoidal surgery, and short duration of hospitalization. Hence this surgical technique has

  19. The efficacy of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation typeⅠwith syringomyelia

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    LI Peng-chao

    2012-08-01

    Full Text Available Objective To report the method and effect of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation type Ⅰ with syringomyelia. Methods Ninty-three patients suffered from Chiari malformation typeⅠwith syringomyelia were treated by microinvasive cisterna magna reconstruction. The skin incision was 1.50-3.00 cm. The bone removal of foramen magnum was 1.50 cm × 2.00 cm with C1 reserved. Dura and arachnoid were incised and sutured linearly. All of the patients underwent cerebellar tonsillar resection and exploration of median aperture of fourth ventricle. Intraoperative ultrasonography was performed both before and after cerebellar tonsillar resection to judge the effect of cisterna magna reconstruction. According to Tator method, the curative effect was divided into 3 groups, improved, stable and worsen. MRI were reviewed at the same time, and the result was divided into syrinx disappeared, reduced, no change and expanded. Results The operation was successful in all patients. Postoperative complications included cerebellum hemorrhage (n = 1, cerebral infarction (n = 1, hydrocephalus (n = 1, subcutaneous dropsy (n = 2 and were recovered after specific treatment. All patients were followed up for 6 months to 12 months after operation. Thirty-six cases were improved, 55 cases were stable, and 2 cases got worse. The MRI showed that the syringomyelia shrinked or disappeared in 90 cases, no change in 3 cases and no expansion. Eighty cases were followed up for 30 months to 36 months after operation, 12 stable cases improved, 1 stable case got worse, while the others remained unchanged. The MRI showed no change was compared with previous follow-up imaging. Conclusion Microinvasive cisterna magna reconstruction is a surgical procedure with mininal injury, quick recovery, stable effect, fewer complications, and high security. Intraoperative ultrasonography provides reliable data and is easy to perform.

  20. Histologic findings and comparison with sputum smears in pulmonary aspiration of vegetable particles in syringomyelia: a case report.

    Science.gov (United States)

    Martínez-Girón, Rafael; Ribas-Barceló, Andrés; Astudillo-González, Aurora; Calvo-Temprano, David

    2007-01-01

    Pulmonary aspiration of vegetable matter may be a serious cause of lung damage, requiring, almost always, the help of a histopathologic examination. We report the case of a 55-year-old woman with a granulomatous pneumonitis due to repeated pulmonary aspiration of vegetable particles and affected by a syringomyelia. Histologic findings from a lobectomy section are shown and compared with those observed in different cytologic sputum samples. The recognition of uncommon morphologic structures, and their comparison with others already known, both in histologic sections and in cellular smears, may be useful when it comes to establishing a sure diagnosis.

  1. Volume reduction of the jugular foramina in Cavalier King Charles Spaniels with syringomyelia

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    Schmidt Martin

    2012-09-01

    Full Text Available Abstract Background Understanding the pathogenesis of the chiari-like malformation in the Cavalier King Charles Spaniel (CKCS is incomplete, and current hypotheses do not fully explain the development of syringomyelia (SM in the spinal cords of affected dogs. This study investigates an unconventional pathogenetic theory for the development of cerebrospinal fluid (CSF pressure waves in the subarachnoid space in CKCS with SM, by analogy with human diseases. In children with achondroplasia the shortening of the skull base can lead to a narrowing of the jugular foramina (JF between the cranial base synchondroses. This in turn has been reported to cause a congestion of the major venous outflow tracts of the skull and consequently to an increase in the intracranial pressure (ICP. Amongst brachycephalic dog breeds the CKCS has been identified as having an extremely short and wide braincase. A stenosis of the JF and a consequential vascular compromise in this opening could contribute to venous hypertension, raising ICP and causing CSF jets in the spinal subarachnoid space of the CKCS. In this study, JF volumes in CKCSs with and without SM were compared to assess a possible role of this pathologic mechanism in the development of SM in this breed. Results Computed tomography (CT scans of 40 CKCSs > 4 years of age were used to create three-dimensional (3D models of the skull and the JF. Weight matched groups (7–10 kg of 20 CKCSs with SM and 20 CKCSs without SM were compared. CKCSs without SM presented significantly larger JF -volumes (median left JF: 0.0633 cm3; median right JF: 0.0703 cm3; p 3; median right JF: 0.0434 cm3; p Conclusion A stenosis of the JF and consecutive venous congestion may explain the aetiology of CSF pressure waves in the subarachnoid space, independent of cerebellar herniation, as an additional pathogenetic factor for the development of SM in this breed.

  2. A historical review of syringomyelia in the cranio-cervical region and new aspects for operative treatment possible after NMR examination

    International Nuclear Information System (INIS)

    Freckmann, N.; Herrmann, H.D.

    1987-01-01

    The authors discuss how radiotherapy of syringomyelia is obsolete and the necessary operational choice should be made according to the etiopathogenesis. This is essentially determined by the exact diagnosis bases on NMR imaging which, according to this review, is superior to any other neuroradiological procedures

  3. Twelve years of chiari-like malformation and syringomyelia scanning in Cavalier King Charles Spaniels in the Netherlands : Towards a more precise phenotype

    NARCIS (Netherlands)

    Wijnrocx, Katrien; Van Bruggen, Leonie W L; Eggelmeijer, Wieteke; Noorman, Erik; Jacques, Arnold; Buys, Nadine; Janssens, Steven; Mandigers, Paul J J

    2017-01-01

    Chiari-like malformation (CM), syringomyelia (SM) and middle ear effusion (also called PSOM) are three conditions that frequently occur in Cavalier King Charles Spaniels (CKCS). Both CM and SM are currently screened in the Netherlands prior to breeding and are graded according to the British

  4. Magnetic resonance imaging-based measures of atlas position: Relationship to canine atlantooccipital overlapping, syringomyelia and clinical signs.

    Science.gov (United States)

    Cerda-Gonzalez, S; Bibi, K F; Gifford, A T; Mudrak, E L; Scrivani, P V

    2016-03-01

    Canine atlantooccipital overlapping (AOO) is characterized by a decreased distance between the dorsal arch of the atlas and the supraoccipital bone. Current diagnostic criteria for this condition are subjective and clinician-dependent; objective criteria are needed to allow a reliable and reproducible diagnosis in clinical and research settings and assess clinical significance. We propose four standardized MRI-based measurements to objectively assess the proximity of the atlas to the foramen magnum. Inclusion criteria for dogs in this study were bodyweight 5 months, and availability of a complete MRI study performed with the craniocervical junction in extension. Exclusion criteria were space-occupying lesions and poor image quality (i.e. unclear bony margins). Measurements also included blinded determinations of skull type, presence of craniocervical junction anomalies and presence and severity of syringomyelia. Clinical status at the time of imaging was noted. Measurements were obtained in 271 dogs; these were reproducible and reliable. Findings varied by skull type: dolichocephalic dogs had smaller foramen magnum, whereas brachycephalic dogs had more cranially and dorsally positioned atlas bodies in comparison to the other skull types. Measurements also increased with increases in bodyweight. This study demonstrated a close association between AOO, syringomyelia and clinical signs. Toy and small breed dogs (including Cavalier King Charles spaniels) showed higher than previously reported prevalence of AOO; its occurrence was also associated with lower bodyweights within the study population of <15 kg toy/small breed dogs. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida.

    Science.gov (United States)

    Williams, Helen

    2008-04-11

    This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described.Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF) volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage.The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of posterior fossa hypoplasia, where

  6. A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida

    Directory of Open Access Journals (Sweden)

    Williams Helen

    2008-04-01

    Full Text Available Abstract This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of

  7. Comparison of the scoliosis curve patterns and MRI syrinx cord characteristics of idiopathic syringomyelia versus Chiari I malformation.

    Science.gov (United States)

    Zhu, Zezhang; Sha, Shifu; Chu, Winnie C C; Yan, Huang; Xie, Dingding; Liu, Zhen; Sun, Xu; Zhu, Weiguo; Cheng, Jack C Y; Qiu, Yong

    2016-02-01

    Although the more readily available MR imaging has brought about more incidental findings of idiopathic syringomyelia (IS), no published study has specifically addressed the clinical and imaging features of IS-associated scoliosis. Since IS and Chiari I malformation (CMI)-type syringomyelia are hypothesized to share a common underlying developmental pathomechanism, this study aimed to investigate the scoliosis curve patterns and MRI syrinx cord characteristics of patients with IS comparing with those seen in CMI. Sixty-one patients with scoliosis secondary to IS were identified and reviewed retrospectively. The curve pattern and specific curve features were recorded and compared with historic CMI controls. Location, size, and morphological appearance of the syrinx were systematically assessed on MR images. The maximal syrinx/cord ratio and rostrocaudal length of the syrinx in IS averaged 0.43 ± 0.16 (range 0.17-0.78) and 4.6 ± 2.5 (range 2-15) vertebral levels, respectively, both of which were smaller than those reported in CMI-type syringomyelia. Regarding the characteristics of IS-related scoliosis, sagittal profiles as well as the frequency of curve patterns and atypical features were all found to resemble those in patients with CMI (P > .05). Among the 47 individuals with a single thoracic curve, Fisher exact test revealed a significant correlation between curve convexity and the dominant side of deviated syrinx (83.3 % concordance rate, P = .021). In addition, apex of the thoracic curve trended toward being significantly correlated with the level of maximum expansion of the syrinx (P = .066). Radiological characteristics of scoliosis were found to be similar between idiopathic and CMI-type syrinx in both the coronal and sagittal planes, adding further evidence to the concept that these entities may be part of a spectrum of disease sharing a common pathophysiological mechanism. The thoracic spine in IS patients tended to be convex to the deviated side of

  8. Anesthetic Management in Arnold Chiari Malformation with Syringomyelia: Case Report [Siringomiyelinli Arnold Chiari Malformasyonda Anestezik Yaklaşım: Olgu Sunumu

    OpenAIRE

    Şanli, Mukadder; Durak, Mehmet Akif; Şanli, Bayram; Gülhaş, Nurçin; Gülerce, Hacer; Ersoy, Mehmet Özcan

    2014-01-01

    AbstractArnold Chiari is a congenitally developed malformation. Syringomyelia is accompanied in some of the cases. Four types of malformation have been identified and type I is frequent in adults. Cerebellar tonsils are displaced downward from the foramen magnum in this type. In order to prevent tonsillar herniation, anesthesist should avoid central blocks and increasing intracranial pressure in the management of anesthesia. We aimed to rewiew the management of anesthesia in a patient with de...

  9. Dystonia of the legs induced by walking or passive movement of the big toe in a patient with cerebellar ectopia and syringomyelia.

    Science.gov (United States)

    Berardelli, A; Thompson, P D; Day, B L; Rothwell, J C; O'Brien, M D; Marsden, C D

    1986-01-01

    A 45-year-old man with dystonia of the legs was found to have cerebellar ectopia and syringomyelia. Both walking and passive movements of the big toes evoked dystonic postures of the legs. Electrophysiologic studies suggested that the dystonic movements were reflex in origin. We propose that the cervical spinal cord lesion, involving propriospinal pathways, resulted in an abnormal response of spinal interneurons to peripheral stimuli.

  10. Dogs attending primary-care practice in England with clinical signs suggestive of Chiari-like malformation/syringomyelia.

    Science.gov (United States)

    Sanchis-Mora, S; Pelligand, L; Thomas, C L; Volk, H A; Abeyesinghe, S M; Brodbelt, D C; Church, D B; Thomson, P C; McGreevy, P D; O'Neill, D G

    2016-10-29

    Chiari-like malformation/syringomyelia (CM/SM) in dogs describes a developmental disorder that can cause pain and reduced quality of life. This retrospective study aimed to report the period prevalence, clinical signs and risk factors for diagnosis of symptomatic CM/SM in the veterinary primary-care setting using a cross-sectional design. The study population included all dogs within the VetCompass Programme (September 1, 2009-June 13, 2014). Overall, the period prevalence of symptomatic CM/SM was 0.05 per cent (95 per cent CI 0.04 per cent to 0.06 per cent) for all breeds. The period prevalence in the Cavalier King Charles Spaniel (CKCS) was 1.6 per cent (95 per cent CI 1.2 to 2.06). Other breeds at increased odds included the King Charles Spaniel (KCS), Affenpinscher, Chihuahua and Pomeranian. Insured dogs had 4.6 times the odds (95 per cent CI 2.95 to 7.17) of having a diagnosis of CM/SM compared with uninsured dogs. Pain was the most commonly associated clinical sign (67 dogs, 72 per cent). Analgesics were prescribed to 72 (77.4 per cent) of the symptomatic dogs. Despite its low overall period prevalence, the high proportion of affected dogs identified with chronic pain suggests a significant welfare issue. Financial implications could impede the diagnostic process and lead to underestimation of the true prevalence. This study may help to inform clinicians about the clinical relevance and the need for improved awareness of clinical signs, particularly in high-risk breeds, to optimise the management of CM/SM in primary-care practice. British Veterinary Association.

  11. One-Stage Correction Surgery of Scoliosis Associated With Syringomyelia: Is it Safe to Leave Untreated a Syrinx Without Neurological Symptom?

    Science.gov (United States)

    Wang, Guodong; Sun, Jianmin; Jiang, Zhensong; Cui, Xingang; Cui, Jiangchao

    2015-06-01

    Retrospective study. To investigate the safety to leave a syrinx untreated in 1-stage correction surgery of scoliosis associated with syringomyelia without progressive neurological symptom. The present protocol for patients with scoliosis secondary to syringomyelia advocated to treat the syrinx first because of the increased risk in correction surgery. However, in daily life, these patients could still do lateral bending, in which spinal cord distracted albeit without any neurological symptom occurred. Twenty-one consecutive patients with scoliosis associated with syringomyelia with or without Chiari malformation underwent surgery in our department from 2003 to 2010 were included in this study. Patients with progressive neural deficits were excluded. Every patient received detailed neurological and radiologic examination before the surgery, including whole spine films, lateral-bending and fulcrum-bending films, 3-dimensional computed tomography scan, and magnetic resonance imaging. All the patients underwent 1-stage correction surgery without treatment of syrinx. During the surgery, Spinal Cord Monitor (SCM) and wake-up test were used to prevent serious neurological complications. At follow-up, patients received neurological examination and whole spine x-ray films. There were 13 male and 8 female patients. Before the surgery, 3 patients complained wasting of the intrinsic muscles of hand, 1 complained numbness of left upper extremity, and 4 complained back pain. Negative abdomen reflex occurred on 12 of 21 patients. All the patients were single major curve, including 14 thoracic curves and 7 thoracolumbar curves. The mean preoperative Cobb angle of scoliosis was 68.05±20.1 degrees, on bending films was 39.48±21.56 degrees, postoperative was 23.19±14.14 degrees, at final follow-up was 25.76±14.46 degrees. The mean flexibility was 0.452±0.158, correction ratio was 0.685±0.140. During the operation, SCM showed motor evoked potential (MEP) loss transiently in 2

  12. Posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression for Chiari malformation type Ⅰ with basilar invagination, atlantoaxial subluxation, and syringomyelia

    Directory of Open Access Journals (Sweden)

    HU Peng

    2012-08-01

    Full Text Available Background Chiari malformation type Ⅰ(CM-Ⅰ is one of the soft tissue anomalies in craniovertebral junction (CVJ. This kind of soft tissue anomaly usually develops with bone anomaly, such as atlantoaxial subluxation, basilar invagination, platybasia, C1 assimilation, etc. For these complex combined anomalies, the treatment remains unaddressed. This study was performed to evaluate the effect of posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression for Chiari malformation type Ⅰ with basilar invagination, atlantoaxial subluxation, and syringomyelia. Methods Patients with basilar invagination and atlantoaxial subluxation treated from July 2004 to September 2011 were reviewed. Including criterions were made to screen matching patients. Including patients were retrospectively analyzed on both clinical outcomes and radiographical results. Japanese Orthopaedic Association (JOA score was used to evaluate the clinical outcomes, while the syrinx maximum size was measured on transverse view of MRI T2 image. The results were analyzed by SPSS 17.0 using t -text. Significant difference was considered when P ≤ 0.05. Results Fourteen patients met the including criterions, including 4 male patients and 10 female patients, with a mean age of 31.86 ± 11.36 (standard deviation, range: 17-51 years. Mean JOA score preoperatively of 14 patients was 13.07 ± 1.59 (standard deviation, while that was 15.57 ± 1.02 (standard deviation postoperatively (t = 9.946, P = 0.000. The mean syrinx size was (7.05 ± 1.98 mm (standard deviation, while that was (2.21 ± 1.91 mm (standard deviation postoperatively (t = 7.271, P = 0.000. There were no procedure-related morbidity or mortality happened. Conclusion Direct posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression can obviously improve the clinical outcomes and shrink syrinx for patients suffered from Chiari malformation typeⅠ with

  13. Hemiplegia cruzada associada a impressão basilar, malformação de Arnold-Chiari e siringomielia: relato de caso Cruciate hemiplegia associated with basilar impression, Arnold-Chiari malformation and syringomyelia: case report

    Directory of Open Access Journals (Sweden)

    José Alberto Gonçalves da Silva

    1996-12-01

    Full Text Available Os autores apresentam um caso de hemiplegia cruzada associada a impressão basilar, malformação de Arnold-Chiari e siringomielia. Discutem as propostas anatômicas e a fiopatogenia desta síndrome de ocorrência bastante rara.The authors report a case of cruciate hemiplegia associated with basilar impression, Chiari malformation and syringomyelia. The neuroanatomical controversy, the surgical treatment and the good outcome of the patient are discussed.

  14. A Poroelastic Fluid/Structure-Interaction Model of Cerebrospinal Fluid Dynamics in the Cord With Syringomyelia and Adjacent Subarachnoid-Space Stenosis.

    Science.gov (United States)

    Bertram, C D; Heil, M

    2017-01-01

    An existing axisymmetric fluid/structure-interaction (FSI) model of the spinal cord, pia mater, subarachnoid space, and dura mater in the presence of syringomyelia and subarachnoid-space stenosis was modified to include porous solids. This allowed investigation of a hypothesis for syrinx fluid ingress from cerebrospinal fluid (CSF). Gross model deformation was unchanged by the addition of porosity, but pressure oscillated more in the syrinx and the subarachnoid space below the stenosis. The poroelastic model still exhibited elevated mean pressure in the subarachnoid space below the stenosis and in the syrinx. With realistic cord permeability, there was slight oscillatory shunt flow bypassing the stenosis via the porous tissue over the syrinx. Weak steady streaming flow occurred in a circuit involving craniocaudal flow through the stenosis and back via the syrinx. Mean syrinx volume was scarcely altered when the adjacent stenosis bisected the syrinx, but increased slightly when the syrinx was predominantly located caudal to the stenosis. The fluid content of the tissues over the syrinx oscillated, absorbing most of the radial flow seeping from the subarachnoid space so that it did not reach the syrinx. To a lesser extent, this cyclic swelling in a boundary layer of cord tissue just below the pia occurred all along the cord, representing a mechanism for exchange of interstitial fluid (ISF) and cerebrospinal fluid which could explain recent tracer findings without invoking perivascular conduits. The model demonstrates that syrinx volume increase is possible when there is subarachnoid-space stenosis and the cord and pia are permeable.

  15. Assessment of cerebellar pulsation in dogs with and without Chiari-like malformation and syringomyelia using cardiac-gated cine magnetic resonance imaging.

    Science.gov (United States)

    Driver, C J; Watts, V; Bunck, A C; Van Ham, L M; Volk, H A

    2013-10-01

    Canine Chiari-like malformation (CM) is characterised by herniation of part of the cerebellum through the foramen magnum. In humans with Chiari type I malformation (CM-I), abnormal pulsation of the cerebellum during the cardiac cycle has been documented and is pivotal to theories for the pathogenesis of syringomyelia (SM). In this retrospective study, cardiac-gated cine balanced fast field echo (bFEE) magnetic resonance imaging (MRI) was used to assess pulsation of the brain in dogs and to objectively measure the degree of cerebellar pulsation with the neck in a flexed position. Overall, 17 Cavalier King Charles Spaniels (CKCS) with CM, including eight with SM and nine without SM, were compared with six small breed control dogs. Linear regions of interest were generated for the length of cerebellar herniation from each phase of the cardiac cycle and the degree of cerebellar pulsation was subsequently calculated. Age, bodyweight and angle of neck flexion were also compared. CKCS with CM and SM had significantly greater pulsation of the cerebellum than control dogs (P=0.003) and CKCS with CM only (P=0.031). There was no significant difference in age, bodyweight and angle of neck flexion between the three groups. Cardiac-gated cine bFEE MRI permitted the dynamic visualisation of cerebellar pulsation in dogs. These findings support the current theories regarding the pathogenesis of SM secondary to CM and further highlight the similarities between canine CM and human CM-I. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Questionnaire-based Analysis of Owner-reported Scratching and Pain Signs in Cavalier King Charles Spaniels Screened for Chiari-like Malformation and Syringomyelia.

    Science.gov (United States)

    Sparks, C R; Cerda-Gonzalez, S; Griffith, E H; Lascelles, B D X; Olby, N J

    2018-01-01

    Chiari-like malformation (CM) and syringomyelia (SM) cause a pain syndrome in Cavalier King Charles spaniels (CKCS). Clinical signs are not consistently apparent on neurologic examination, and owner reporting of signs provides vital clinical history. However, owner questionnaires for this disease are not well developed. To develop a tool to capture owner-reported clinical signs for use in clinical trials and to compare owner-reported signs with the presence of pain on neurologic examination and SM on magnetic resonance imaging (MRI). Fifty client-owned CKCS. Owners completed a questionnaire and pain/scratch map. Each dog underwent a neurologic examination and craniocervical magnetic resonance imaging (MRI). Questionnaire responses were developed into scores, area of shading for pain/scratch maps was measured, and consistency of responses between these tools was assessed. Owner-reported findings were compared with neurologic examination findings and presence and severity of SM on MRI. Thirty-three dogs were symptomatic and 17 asymptomatic; 30 had SM. The most common sign of pain was crying out when lifted (n = 11). Extent of shaded areas on maps positively correlated with questionnaire scores for pain (r 2  = 0.213, P = 0.006) and scratch (r 2  = 0.104, P = 0.089). Owner-reported findings were not significantly associated with presence or severity of SM or neurologic examination findings. Owner-reported lateralization of signs was significantly associated with SM lateralization (P owner-reported signs with SM highlights our lack of understanding of the pathophysiology of pain in this disease. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc., on behalf of the American College of Veterinary Internal Medicine.

  17. Craniometric Analysis of the Hindbrain and Craniocervical Junction of Chihuahua, Affenpinscher and Cavalier King Charles Spaniel Dogs With and Without Syringomyelia Secondary to Chiari-Like Malformation.

    Science.gov (United States)

    Knowler, Susan P; Kiviranta, Anna-Mariam; McFadyen, Angus K; Jokinen, Tarja S; La Ragione, Roberto M; Rusbridge, Clare

    2017-01-01

    To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS). Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios) of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB) using Discriminant Function Analysis and ANOVA with post-hoc corrections. The analysis identified 14/22 significant traits for SM in the three dog breeds, five of which were identical to those reported for the GB and suggest inclusion of a common aetiology. One ratio, caudal fossa height to the length of the skull base extended to an imaginary point of alignment between the atlas and supraoccipital bones, was common to all three breeds (p values 0.029 to Chihuahua had a smaller angle between the dens, atlas and basioccipital bone (p value < 0.001); Affenpinschers had a smaller distance from atlas to dens (p value 0.009); CKCS had a shorter distance between the spheno-occipital synchondrosis and atlas (p value 0.007). The selected morphometries successfully characterised conformational changes in the brain and craniocervical junction that might form the basis of a diagnostic tool for all breeds. The severity of SM involved a spectrum of abnormalities, incurred by changes in both angulation and size that could alter neural parenchyma compliance and/or impede cerebrospinal fluid channels.

  18. Use of Morphometric Mapping to Characterise Symptomatic Chiari-Like Malformation, Secondary Syringomyelia and Associated Brachycephaly in the Cavalier King Charles Spaniel.

    Directory of Open Access Journals (Sweden)

    Susan P Knowler

    Full Text Available To characterise the symptomatic phenotype of Chiari-like malformation (CM, secondary syringomyelia (SM and brachycephaly in the Cavalier King Charles Spaniel using morphometric measurements on mid-sagittal Magnetic Resonance images (MRI of the brain and craniocervical junction.This retrospective study, based on a previous quantitative analysis in the Griffon Bruxellois (GB, used 24 measurements taken on 130 T1-weighted MRI of hindbrain and cervical region. Associated brachycephaly was estimated using 26 measurements, including rostral forebrain flattening and olfactory lobe rotation, on 72 T2-weighted MRI of the whole brain. Both study cohorts were divided into three groups; Control, CM pain and SM and their morphometries compared with each other.Fourteen significant traits were identified in the hindbrain study and nine traits in the whole brain study, six of which were similar to the GB and suggest a common aetiology. The Control cohort had the most elliptical brain (p = 0.010, least olfactory bulb rotation (p = 0.003 and a protective angle (p = 0.004 compared to the other groups. The CM pain cohort had the greatest rostral forebrain flattening (p = 0.007, shortest basioccipital (p = 0.019, but a greater distance between the atlas and basioccipital (p = 0.002 which was protective for SM. The SM cohort had two conformation anomalies depending on the severity of craniocervical junction incongruities; i the proximity of the dens (p <0.001 ii increased airorhynchy with a smaller, more ventrally rotated olfactory bulb (p <0.001. Both generated 'concertina' flexures of the brain and craniocervical junction.Morphometric mapping provides a diagnostic tool for quantifying symptomatic CM, secondary SM and their relationship with brachycephaly. It is hypothesized that CM pain is associated with increased brachycephaly and SM can result from different combinations of abnormalities of the forebrain, caudal fossa and craniocervical junction which compromise

  19. Long-term outcome of Cavalier King Charles spaniel dogs with clinical signs associated with Chiari-like malformation and syringomyelia.

    Science.gov (United States)

    Plessas, I N; Rusbridge, C; Driver, C J; Chandler, K E; Craig, A; McGonnell, I M; Brodbelt, D C; Volk, H A

    2012-11-17

    The disease complex Chiari-like malformation (CM) and syringomyelia (SM) has been associated with the development of neuropathic pain (NeP), and commonly affects Cavalier King Charles spaniels (CKCS). This prospective cohort study followed 48 CKCSs with CM and/or SM and clinical signs suggestive of NeP for a period of 39 (±14.3) months from diagnosis. At the end of the study, 36 dogs were still alive; five dogs died of an unrelated or unknown cause, and seven were euthanased due to severe clinical signs suggestive of NeP. During the follow-up period, the clinical signs of scratching, facial rubbing behaviour, vocalisation and exercise ability were evaluated. Nine out of 48 dogs stopped scratching (Pdogs exhibiting exercise intolerance, vocalisation or facial rubbing behaviour. The overall severity of clinical signs based on a visual analogue scale (VAS) (0 mm: no clinical signs 100 mm: severe clinical signs) increased (from median 75 mm (interquartile ranges (IQR) 68-84) to 84 mm (IQR 71.5-91), Pdogs were static or improved. In general, the majority of the owners felt that the quality of life of their dogs was acceptable. Medical treatments received were gabapentin or pregabalin and/or intermittently, carprofen. The owner's perception of their animal's progress, and progress based on VAS, had strong positive correlation (Spearman's rank correlation (s(r)) 0.74, P<0.001). Overall, this study suggests that clinical signs suggestive of NeP progress in three-quarters of CKCSs with CM and/or SM.

  20. Craniometric Analysis of the Hindbrain and Craniocervical Junction of Chihuahua, Affenpinscher and Cavalier King Charles Spaniel Dogs With and Without Syringomyelia Secondary to Chiari-Like Malformation.

    Directory of Open Access Journals (Sweden)

    Susan P Knowler

    Full Text Available To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS.Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB using Discriminant Function Analysis and ANOVA with post-hoc corrections.The analysis identified 14/22 significant traits for SM in the three dog breeds, five of which were identical to those reported for the GB and suggest inclusion of a common aetiology. One ratio, caudal fossa height to the length of the skull base extended to an imaginary point of alignment between the atlas and supraoccipital bones, was common to all three breeds (p values 0.029 to <0.001. Associated with SM were a reduced occipital crest and two acute changes in angulation i 'sphenoid flexure' at the spheno-occipital synchondrosis ii 'cervical flexure' at the foramen magnum allied with medulla oblongata elevation. Comparing dogs with and without SM, each breed had a unique trait: Chihuahua had a smaller angle between the dens, atlas and basioccipital bone (p value < 0.001; Affenpinschers had a smaller distance from atlas to dens (p value 0.009; CKCS had a shorter distance between the spheno-occipital synchondrosis and atlas (p value 0.007.The selected morphometries successfully characterised conformational changes in the brain and craniocervical junction that might form the basis of a diagnostic tool for all breeds. The severity of SM involved a spectrum of abnormalities, incurred by changes in both angulation and size that could alter neural parenchyma compliance and/or impede cerebrospinal fluid channels.

  1. A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients.

    Science.gov (United States)

    Thakar, Sumit; Sivaraju, Laxminadh; Jacob, Kuruthukulangara S; Arun, Aditya Atal; Aryan, Saritha; Mohan, Dilip; Sai Kiran, Narayanam Anantha; Hegde, Alangar S

    2018-01-01

    OBJECTIVE Although various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome. METHODS Data from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed. Various preoperative clinical and radiological variables in the 57 patients who formed the study cohort were assessed in a bivariate analysis to determine their ability to predict clinical outcome (as measured on the Chicago Chiari Outcome Scale [CCOS]) and the resolution of syrinx at the last follow-up. The variables that were significant in the bivariate analysis were further analyzed in a multiple linear regression analysis. Different regression models were tested, and the model with the best prediction of CCOS was identified and internally validated in a subcohort of 25 patients. RESULTS There was no correlation between CCOS score and syrinx resolution (p = 0.24) at a mean ± SD follow-up of 40.29 ± 10.36 months. Multiple linear regression analysis revealed that the presence of gait instability, obex position, and the M-line-fourth ventricle vertex (FVV) distance correlated with CCOS score, while the presence of motor deficits was associated with poor syrinx resolution (p ≤ 0.05). The algorithm generated from the regression model demonstrated good diagnostic accuracy (area under curve 0.81), with a score of more than 128 points demonstrating 100% specificity for clinical improvement (CCOS score of 11 or greater). The model had excellent reliability (κ = 0.85) and was validated with

  2. Twelve years of chiari-like malformation and syringomyelia scanning in Cavalier King Charles Spaniels in the Netherlands: Towards a more precise phenotype.

    Directory of Open Access Journals (Sweden)

    Katrien Wijnrocx

    Full Text Available Chiari-like malformation (CM, syringomyelia (SM and middle ear effusion (also called PSOM are three conditions that frequently occur in Cavalier King Charles Spaniels (CKCS. Both CM and SM are currently screened in the Netherlands prior to breeding and are graded according to the British Veterinary Association's Kennel Club (BVA/KC scheme. This study evaluated the prevalence and estimated genetic parameter of CM, SM and middle ear effusion from 12 years of screening results. For SM, the classical method using the BVA/KC scheme, was compared with exact measuring of the central canal dilation. For CM, the BVA/KC scheme was compared with a more detailed scheme. Next to this the presence of microchip artifacts was assessed. 1249 screening of 1020 dogs were re-evaluated. Results indicated the presence of CM in all dogs, suggesting it has become a breed-specific characteristic. And although different grades of CM were observed, the condition did not deteriorate over time. SM was present in 39% of the dogs and a clear age effect was demonstrated, with SM increasing with age. This emphasizes the importance of screening at appropriate age, since SM can worsen with increasing age. One alternative is to promote repeated measures. The presence of middle ear effusion in this study was 19%-21% for dogs younger than 3 years, and 32%-38% for dogs older than 3 years. In as much as 60%, microchip artifacts were noticed, leading to the recommendation to place microchips in another location in breeds that are susceptible to developing SM. Finally, this study estimated the heritability of CM in this population, due to the lack of phenotypic variance, to be very low at 0.02-0.03. The heritability for SM central canal dilatation to be 0.30, compared to 0.13 for the classical BVA/KC method, using a model including the age effect and the combined effect of veterinary clinic and year of the evaluation. Genetic correlations were rather small, ranging from 0.16-0.33. As a

  3. Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients Impressão basilar, malformação de Chiari e siringomielia: estudo retrospecivo de 53 casos operados

    Directory of Open Access Journals (Sweden)

    José Alberto Gonçalves da Silva

    2003-06-01

    Full Text Available The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI, Chiari malformation (CM, and syringomyelia (SM. The patients were divided into two groups. Group I (24 patients underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior- caudal direction, avoiding, in this way, its adherence to the cerebellum.São analisados os resultados obtidos com o tratamento cirúrgico de 53 casos de impressão basilar (IB, malformação de Chiari (MC e siringomielia (SM. Os pacientes foram divididos em dois grupos: no grupo I (24 casos foi realizada a descompressão osteodural, caracterizada por craniectomia ampla occipital inferior, laminectomia variável de C 1 a C 3, abertura da dura-máter em forma de Y, dissecção das aderências aracnóideas das tonsilas cerebelares com o bulbo e medula cervical, abertura ampla do quarto ventrículo e enxerto dural; no grupo II (29 casos foi utilizada a descompressão osteodural-neural, caracterizada pelos mesmos detalhes técnicos empregados no grupo I, acrescidos da

  4. Management of parturients in active labor with Arnold Chiari malformation, tonsillar herniation, and syringomyelia

    Science.gov (United States)

    Ghaly, Ramsis F.; Tverdohleb, Tatiana; Candido, Kenneth D.; Knezevic, Nebojsa Nick

    2017-01-01

    Background: Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? Case Description: A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery. A diagnosis of ACM-1 was made 12 years ago when a brain magnetic resonance imaging (MRI) was performed for right-sided numbness following a rear-end motor vehicle collision. The patient had been asymptomatic since then and had been seen by an outside neurologist frequently for the past 10 years. During the anesthesia evaluation, it was noted that she had an exaggerated patellar reflex, and a questionable left-sided Babinski; subsequently, an MRI study was requested. Review of a brain MRI demonstrated an advanced form of ACM with a 1.7 cm transtonsillar herniation and a large syrinx extending from C1 down to C5. Following a discussion with the patient, family, and primary OB team, a plan for elective cesarean section was made per neurosurgical recommendations. This was conducted uneventfully under general anesthesia. The patient had no complaints in the post-anesthesia care unit. Conclusion: Unfamiliarity of health care providers with regards to ACM-1 parturients can be countered by increasing awareness of this condition throughout medical specialties involved in their care. The Ghaly Obstetric Guide to Arnold-Chiari malformation Type 1, along with proper training of anesthesia care providers regarding the specificities of ACM-1 parturients aids in better management and understanding of this complex condition. PMID:28217389

  5. [Arnold-Chiari malformation and syringomyelia in primary care. A case report].

    Science.gov (United States)

    Martínez-Sabater, A; Sancho-Cantus, D

    2012-01-01

    Rare diseases, due to their epidemiological characteristics, and sometimes to the non-specific symptoms, are difficult to diagnose routinely at Primary Care Level. A case is presented of Arnold Chiari malformation in a young male patient with early insidious presentation (neck pain and sub-occipital headaches) consulting due to the emergence of new symptoms (paresthesias, muscular weakness, cervicobrachial and radiating lumbar pain, and headaches after mild exertion). Copyright © 2011 Elsevier España, S.L. y SEMERGEN. All rights reserved.

  6. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Roya Memarpour

    2015-01-01

    Full Text Available Neuroarthropathy (neuropathic osteoarthropathy, also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis. We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

  7. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

    Science.gov (United States)

    Memarpour, Roya; Gonzalez-Ibarra, Fernando

    2015-01-01

    Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine. PMID:25692057

  8. Endoscopic Management of Arnold-Chiari Malformation Type I with or without Syringomyelia.

    Science.gov (United States)

    Ratre, Shailendra; Yadav, Nishtha; Yadav, Yad Ram; Parihar, Vijay Singh; Bajaj, Jitin; Kher, Yatin

    2018-01-01

     Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. Endoscope-assisted decompression of the posterior fossa has been found to be safe and effective. We report our initial experience of endoscopic management of ACM-I.  This was a prospective study of 15 symptomatic patients. Pre- and postoperative clinical status and computed tomography and magnetic resonance imaging findings were recorded. Suboccipital bone of ∼ 3 cm distance from the foramen of magnum and posterior arch of atlas was removed. Partial splitting of the dura mater with preservation of the inner portion and the arachnoid membrane was performed. Any change in axial and sagittal length of the syrinx, tonsillar ascension, shape of the tonsil tip, appearance of cerebrospinal fluid posterior to the tonsil, and formation of the cisterna magna were recorded. Patients with atlantoaxial instability, tethered cord, associated myelomeningocele, hydrocephalus, or elevated intracranial pressure were excluded.  Age of patients ranged from 26 to 48 years. There were nine female patients. There were six patients with ACM-I without and nine with ACM-I with syrinx. Average pre- and postoperative Karnofsky performance score was 78 and 93, respectively. Average operative time was 130 minutes (110-190 minutes), and blood loss was 30 mL (20-180 mL). Follow-up ranged from 9 to 21 months.  Although the study is limited by the small number of patients with a short follow-up, endoscopic decompression in selected patients of ACM-I with or without syrinx with dural splitting was a safe and effective alternative to microsurgical treatment. Georg Thieme Verlag KG Stuttgart · New York.

  9. Management of parturients in active labor with Arnold Chiari malformation, tonsillar herniation, and syringomyelia.

    Science.gov (United States)

    Ghaly, Ramsis F; Tverdohleb, Tatiana; Candido, Kenneth D; Knezevic, Nebojsa Nick

    2017-01-01

    Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery. A diagnosis of ACM-1 was made 12 years ago when a brain magnetic resonance imaging (MRI) was performed for right-sided numbness following a rear-end motor vehicle collision. The patient had been asymptomatic since then and had been seen by an outside neurologist frequently for the past 10 years. During the anesthesia evaluation, it was noted that she had an exaggerated patellar reflex, and a questionable left-sided Babinski; subsequently, an MRI study was requested. Review of a brain MRI demonstrated an advanced form of ACM with a 1.7 cm transtonsillar herniation and a large syrinx extending from C1 down to C5. Following a discussion with the patient, family, and primary OB team, a plan for elective cesarean section was made per neurosurgical recommendations. This was conducted uneventfully under general anesthesia. The patient had no complaints in the post-anesthesia care unit. Unfamiliarity of health care providers with regards to ACM-1 parturients can be countered by increasing awareness of this condition throughout medical specialties involved in their care. The Ghaly Obstetric Guide to Arnold-Chiari malformation Type 1, along with proper training of anesthesia care providers regarding the specificities of ACM-1 parturients aids in better management and understanding of this complex condition.

  10. Massive reduction of tumour load and normalisation of hyperprolactinaemia after high dose cabergoline in metastasised prolactinoma causing thoracic syringomyelia.

    NARCIS (Netherlands)

    Uum, S.H.M. van; Alfen, N. van; Wesseling, P.; Lindert, E.J. van; Pieters, G.F.F.M.; Nooijen, P.T.G.A.; Hermus, A.R.M.M.

    2004-01-01

    In 1970 a 20 year old woman presented with a pituitary chromophobe adenoma for which she underwent transfrontal pituitary surgery. In 1978 she had to be reoperated on because of local tumour recurrence, resulting in hypopituitarism. Bromocriptine (5 mg/day) was given for 15 years, but the plasma

  11. Bilateral vocal cord palsy causing stridor as the only symptom of syringomyelia and Chiari I malformation, a case report

    Directory of Open Access Journals (Sweden)

    Saif Yousif

    2016-01-01

    Conclusion: Although uncommon, formation of a syrinx should be considered for patients who present with stridor and reiterates the importance of MRI as an important investigative tool of bilateral vocal cord palsy.

  12. Prevalence and heritability of symptomatic syringomyelia in Cavalier King Charles Spaniels and long-term outcome in symptomatic and asymptomatic littermates

    DEFF Research Database (Denmark)

    Thøfner, Maria Søndergaard; Stougaard, C. L.; Westrup, Ulrik

    2015-01-01

    (P euthanasia in 20%. Dogs with syringes, which expressed no clinical signs at the age of 6, remained asymptomatic in 14/15 cases (93%). CONCLUSIONS AND CLINICAL IMPORTANCE: The prevalence of symptomatic SM is high...

  13. Chiari Malformation

    Science.gov (United States)

    ... available from the following organizations: American Syringomyelia & Chiari Alliance Project P.O. Box 1586 Longview, TX 75606- ... Legislative Updates Impact NINDS Contributions to Approved Therapies Strategic Plans & Evaluations Strategic Plans Program Evaluations Workforce Diversity ...

  14. Symptomatic Chiari malformation in infancy and adolescence

    International Nuclear Information System (INIS)

    Fukushima, Takeo; Matsuda, Toshihiro; Tsugu, Hitoshi; Sakamoto, Seisaburou; Tomonaga, Masamichi; Asakawa, Koji; Mitsudome, Akihisa; Hashimoto, Takahisa.

    1994-01-01

    Of the 7 children and adolescents with Chiari malformation reported here 5 had Chiari type 1 and 2 had Chiari type 2 disease. All 5 Chiari type 1 patients had syringomyelia and scoliosis, but the Chiari type 2 patients did not. All the patients except one with localized syringomyelia (case 3) underwent posterior decompression with suboccipital craniectomy, upper cervical laminectomy and duraplasty, and their clinical signs and symptoms improved. Case 3 received a syringo-subarachnoid shunt and improved clinically. Motor function was restored better than sensory function. Cranial nerve signs were cleared up in the Chiari type 2 patients. In the Chiari type 1 patients postoperative neurological improvement correlated well with the collapse of syringomyelia. Cine MR imaging was useful in the evaluation of CSF dynamics at the cranio-vertebral junction and in the syringomyelic cavity before and after surgery. (author)

  15. Does Gd-DTPA help in separating tumor from syrinx or cyst in the spinal cord

    International Nuclear Information System (INIS)

    Slasky, B.S.; Niendorf, H.P.; Steiner, R.E.; Bydder, G.M.; Young, I.R.

    1987-01-01

    There is some difficulty in differentiating among syringomyelia, syringomyelia in association with tumor, and cystic tumor in the spinal cord with MR imaging. The role of the paramagnetic contrast agent Gd-DTPA in evaluating this problem was studied in 16 patients. Contrast enhancement was observed in all 12 patients with histologically proved tumors. Enhancement was marked in eight cases, moderate in two cases, and mild in two cases. Compared to the unenhanced images, the images obtained after Gd-DTPA administration delineated more clearly and accurately the exact location and extent of tumor in cord cavitation. This information was extremely helpful in biopsy, definitive surgery, and radiation therapy

  16. CRANIOVERTEBRAL JUNCTION ANOMALIES SEEN AT ...

    African Journals Online (AJOL)

    hi-tech

    2000-03-03

    Mar 3, 2000 ... anomalies that give rise to symptoms in this area are basilar impression, occipitalisation of the atlas, odontoid process abnormalities and atlanto-axial dislocation. Neuromeningeal anomalies in this region include Arnold-. Chiari malformation, syringomyelia and basal arachnoiditis. The clinical presentation ...

  17. Cutis Marmorata Telangiectatica and Chiari Type I

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-12-01

    Full Text Available A 10-year-old girl born with telangiectasias of the lip, trunk, extremities, palms, and soles was found to have an asymptomatic Chiari I malformation without syringomyelia on MRI, and is reported from Children’s Hospital, Alabama.

  18. Posttraumatische syringomyelie bij 2 patiënten met een thoracale dwarslaesie

    NARCIS (Netherlands)

    Bollen, A E; Hoving, E W; Kuks, J B

    2000-01-01

    Two patients, men aged 42 and 40 years, developed new neurological symptoms 3 months and 22 years, respectively, after a traumatic high thoracic spinal cord injury. The MRI scan showed a cavity in the central part of the spinal cord, on which the diagnosis of 'posttraumatic syringomyelia' could be

  19. Co-existence of Chiari malformation type I and Epstein-Barr virus meningoencephalitis in a 3-year-old child: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Solomou, E.K.; Kotsarini, C.; Badra, F.A. [University Hospital of Patras, Department of Radiology, Rio (Greece); Krepis, A.; Papanastasiou, D. [University Hospital of Patras, Pediatric Clinic, Patras (Greece); Patriarcheas, G. [Diagnostic Centre B Diagnosis, Patras (Greece)

    2005-01-01

    In the present report we describe an unusual case of a 3-year-old girl who was admitted to our hospital with Epstein-Barr virus meningoencephalitis. Brain magnetic resonance imaging revealed diffuse abnormalities in white matter and Chiari I malformation with cervical and thoracic hydro-syringomyelia. (orig.)

  20. Syringomyelie en een chiari-malformatie bij een kind met scoliose

    NARCIS (Netherlands)

    Weijenberg, A; Brouwer, O F; Hoving, E W

    2008-01-01

    A 9-year-old boy who since 3 years had been treated with a brace for presumed idiopathic thoracic scoliosis was referred because of severe syringomyelia on MRI of the spinal cord. Neuroimaging of the spinal cord had been performed because of back pain, although the patient had no neurological signs

  1. The quality of life and psychiatric morbidity in patients operated for Arnold-Chiari malformation type I.

    Science.gov (United States)

    Bakim, Bahadir; Goksan Yavuz, Burcu; Yilmaz, Adem; Karamustafalioglu, Oguz; Akbiyik, Meral; Yayla, Sinan; Yuce, Ismail; Alpak, Gokay; Tankaya, Onur

    2013-10-01

    There are some case reports that highlight the association of Arnold-Chiari malformation (ACM) with psychiatric symptoms. We assessed the association between ACM and psychiatric symptoms and risk factors in terms of psychiatric morbidity and evaluated the quality of life after surgery. This study consisted of sixteen patients who underwent decompression operation at the Department of Neurosurgery of Sisli Etfal Hospital. The MINI plus, Short-Form McGill Pain Questionnaire and WHOQOL-BREF-TR were administered to patients. About 43.8% of the patients had a psychiatric disorder. About 50% of the patients had co-existing syringomyelia of which 50% with syringomyelia had a psychiatric disorder. Patients with syringomyelia without any psychiatric disorder had significantly lower scores on physical domain of WHOQOL-BREF-TR (p = 0.02) than the patients without syringomyelia and psychiatric disorder. Subjects with a psychiatric disorder had lower scores on four domains of WHOQOL-BREF-TR. The patients with psychiatric diagnoses had significantly higher scores on affective pain index (p = 0.021) and total pain index (p = 0.037) than the patients without any psychiatric disorder. The presence of a psychiatric condition influences not only the physical aspect but also deteriorates the psychological and social relations and environmental aspect. Moreover the presence of a psychiatric disorder increases the perception of pain and causes more discomfort.

  2. Magnetic resonance imaging of spinal dysraphism

    Energy Technology Data Exchange (ETDEWEB)

    Akino, Minoru; Isu, Toyohiko; Iwasaki, Yoshinobu; Abe, Hiroshi; Abe, Satoru; Miyasaka, Kazuo; Nomura, Mikio; Saito, Hisatoshi.

    1988-04-01

    Nineteen patients with lumbosacral spina bifida were studied by magnetic resonance imaging (MRI) and were divided into two groups: those with lumbosacral lipoma and those with meningomyelocele. All of the patients with meningomyelocele underwent surgery soon after birth for closure of the skin defect. Whenever possible, examination was not confined to the lumbosacral area but also included the brain and other portions of the spinal cord. Certain similarities and differences in pathology were ascertained in the two groups. The tethered cords were the same in both groups. However, Chiari malformations were observed only in patients with meningomyelocele, and hydrocephalus occurred only in patients with Chiari malformations. Syringomyelia and scoliosis were detected in both groups, but scoliosis was more prevalent in the meningomyelocele group. There appeared to be a correlation between scoliosis and syringomyelia; in five of the seven cases of syringomyelia, the locations of the scoliosis and syringomyelia were the same. With MRI, these complex pathologies, including tethered cord, syringomyelia, scoliosis, Chiari malformations, and hydrocephalus, were easily visualized. The superiority of MRI over conventional X-ray technology has been well established. First, a direct image of the spinal cord is obtained. Second, there is no necessity for injection of contrast material into the intrathecal space. Third, any scanning field is possible. There are also some disadvantages with MRI. First, the spatial resolution is inferior to that of high-resolution computed tomography. Second, MRI cannot provide information concerning bone cortex. Therefore, bone involvement cannot be accurately diagnosed. However, in the assessment of spinal dysraphism, MRI is an excellent diagnostic tool and should be the preferred method of diagnosing spinal dysraphism.

  3. Guillain-Barre syndrome: A possibility in a spinal cord injured patient

    Directory of Open Access Journals (Sweden)

    Jagatsinh Yogendrasinh

    2007-01-01

    Full Text Available A 28-year-old male had paraplegia as a result of fracture dislocation of T12/L1 six years ago. He was functioning independently until four weeks ago, when he started complaining of trunkal paraesthesia which later progressed to include the upper extremities. The initial diagnosis was that of posttraumatic syringomyelia (PTS. While awaiting the MRI scan he developed weakness of upper limbs. The weakness restricted his self-care activities including transfers. The MRI did not show any evidence of syringomyelia. Neurological consultation and assessment yielded provisional diagnosis of Guillain-Barre syndrome (GBS. The patient was treated with immunoglobulins and regained 90% of his previous neurological status. This case is reported to raise awareness among clinicians to include the possibility of the GBS in the differential diagnosis of progressive neurological loss on top of existing neurological deficiency in spinal cord injured patients.

  4. Syringo-hydromyelic syndrome in six calves

    International Nuclear Information System (INIS)

    Testoni, S.; Sconza, S.; Giuliani, A.; Rossi, M.; Gentile, A.

    2006-01-01

    The clinical, diagnostic and anatomo-pathologic aspects of syringo-hydromyelic syndrome are described in six calves. The animals were submitted because of inability to rise since birth. At physical examination, calves appeared bright, alert, and in good condition; they were recumbent and, despite repeated attempts to stand up, were unable to. When forced to move, they showed a characteristic posterior bunny-hopping. Radiographically, one calf presented emivertebrae. The myelography was performed. In five calves a swelling was observed in lumbar spinal cord. At postmortem examination the main lesions were confined to the lumbar spinal cord with cystic cavities. The histological diagnosis were: syringo-hydromyelia in four calves; syringomyelia in one calf; syringomyelia and hydromyelia, simultaneously, in one calf [it

  5. Nuclear magnetic resonance imaging of the spine

    International Nuclear Information System (INIS)

    Modic, M.T.; Weinstein, M.A.; Pavlicek, W.; Starnes, D.L.; Duchesneau, P.M.; Boumphrey, F.; Hardy, R.J. Jr.

    1984-01-01

    Forty subjects were examined to determine the accuracy and clinical usefulness of nuclear magnetic resonance (NMR) examination of the spine. The NMR images were compared with plain radiographs, high-resolution computed tomograms, and myelograms. The study included 15 patients with normal spinal cord anatomy and 25 patients whose pathological conditions included canal stenosis, herniated discs, metastatic tumors, primary cord tumor, trauma, Chiari malformations, syringomyelia, and developmental disorders. Saturation recovery images were best in differentiating between soft tissue and cerebrospinal fluid. NMR was excellent for the evaluation of the foramen magnum region and is presently the modality of choice for the diagnosis of syringomyelia and Chiari malformation. NMR was accurate in diagnosing spinal cord trauma and spinal canal block

  6. MR imaging of the spine in children with spina bifida

    International Nuclear Information System (INIS)

    Williamson, M.R.; Glasier, C.M.; Angtuaco, E.J.C.; McConnell, J.R.; Chadduck, W.M.; Binet, E.F.

    1986-01-01

    Twenty infants and children with spina bifida were examined as outpatients with spinal MR imaging. Studies were performed using a 0.6-T imager and a surface coil. These examinations were performed in patients previously operated on who had progressive neurologic symptoms and in newly diagnosed patients to exclude lipomyeloschisis, low-lying cord, diastematomyelia, or syringomyelia. Predominantly T1-weighted sequences were obtained in at least two orthogonal planes with a 5-mm section thickness. Eighteen patients had a low-lying spinal cord. Six patients had syringomyelia, usually lumbar. Five patients had intraspinal lipomas, and three had diastematomyelia. MR imaging was diagnostic in 17 instances. Patient motion or severe kyphoscoliosis comprised three examinations. MR imaging is the screening examination of choice in children with spina bifida

  7. Clinical and radiological findings in arnold chiari malformation

    International Nuclear Information System (INIS)

    Khan, A.A.; Bhatti, S.N.; Ahmed, E.; Aurangzeb, A.; Ali, A.; Khan, A.; Afzal, S.; Khan, G.

    2010-01-01

    Background: The Chiari Malformation I (CMI) is a disorder of uncertain origin that has been traditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum. The anomaly is a leading cause of syringomyelia and occurs in association with osseous abnormalities at the cranio vertebral junction. In contrast to other Chiari malformations, CMI tends to present in the second or third decade of life and is sometimes referred to as the 'adult-type' Chiari malformation. The objective was to document clinical and radiological findings in Arnold Chiari Malformation-I. Method: This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad at Neurosurgery Department during July 2008 - July 2010. We examined a prospective cohort of 60 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine. Results: There were 40 female and 20 male patients. The age of onset was 24.9 +- 15.8 years. Common associated radiological problems included syringomyelia (60%), scoliosis (25%), and basilar invagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%). The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), and varying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-like episodes, a Meniere's disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances in the absence of syringomyelia. Conclusion: These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible

  8. Assessment of rat spinal cord injury models

    OpenAIRE

    Xu, Ning

    2015-01-01

    Traumatic spinal cord injury (SCI) is a complicated and devastating condition, causing different extents of motor, sensory and autonomic dysfunctions. In addition, there is a risk for secondary complications after SCI including posttraumatic syringomyelia (PTS) that can cause further functional loss. Since there is no available effective treatment, tremendous efforts have been made to develop new therapeutic strategies to promote functional recovery after SCI. In experimental r...

  9. Siringomielia idiopática a propósito de un caso

    Directory of Open Access Journals (Sweden)

    Marlon Martínez

    2013-10-01

    Full Text Available Title: Idiopathic syringomyelia: a case report.ResumenLa siringomielia es un trastorno que consiste en la formación de uno o varios quistes en la médula espinal, que generalmente inician en la región cervical y pueden extenderse hasta la región torácica o hacia el tronco encefálico. Su etiología es multifactorial, pudiendo ser secundaria a malformación de Arnold-Chiari, causas idiopáticas, y traumatismos. Las manifestaciones clínicas son variadas presentando un curso crónico-progresivo. Se presenta el caso de una mujer de 47 años de edad con diagnóstico de siringomielia cervicotorácica, cuya manifestación inicial fue inusual, aguda y progresiva, ameritando procedimiento quirúrgico con buenos resultados. Se discute sobre la presentación clínica atípica y las medidas terapéuticas quirúrgicas empleadas en éste caso. (DUAZARY 2012 No. 1, 60 - 64AbstractSyringomyelia is a disorder that involves the formation of one o more cysts in the spinal cord, usually begin in the cervical region and extend to the thoracic region or to the brain stem. The etiology is multifactorial and usually is secondarily to Arnold-Chiari malformation, idiopathic causes and trauma. These clinical manifestation are various with a progressive chronic course. The following case report is about a woman of 47 years old, whit diagnosis of cervico-thoracic syringomyelia, whose initial manifestation was unusual, acute and progressive; the patient requires Surgical Procedures with good results. In this case discussed about the clinical atypical presentation and the therapeutic surgical measures used in this case.Keywords: Syringomyelia; cysts; spinal cord; brain stem; Arnold-Chiari malformation; surgical procedures. (MeSH.

  10. MR imaging of the spine in children

    International Nuclear Information System (INIS)

    Duthoy, M.J.; Lund, G.

    1988-01-01

    MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor, primary spinal cord tumor, metastatic disease, neuroectodermal disorder, congenital malformation, trauma, and demyelinating, neurodegenerative, or metabolic disorders. MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR. (orig./MG)

  11. MR imaging of the spine in children

    Energy Technology Data Exchange (ETDEWEB)

    Duthoy, M.J.; Lund, G.

    1988-08-01

    MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor, primary spinal cord tumor, metastatic disease, neuroectodermal disorder, congenital malformation, trauma, and demyelinating, neurodegenerative, or metabolic disorders. MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR.

  12. Clinical and radiological findings in Arnold Chiari malformation.

    Science.gov (United States)

    Khan, Abdul Aziz; Bhatti, Sajid Nazir; Khan, Ghayyur; Ahmed, Ehtisham; Aurangzeb, Ahsan; Ali, Asghar; Khan, Amjad; Afzal, Saleem

    2010-01-01

    The Chiari Malformation I (CMI) is a disorder of uncertain origin that has been traditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum. The anomaly is a leading cause of syringomyelia and occurs inassociation with osseus abnormalities at the craniovertebral junction. In contrast to other Chiari malformations, CMI tends to present in the second or third decade of life and is sometimes referred to as the 'adult-type' Chiari malformation. The objective was to document clinical and radiological findings in Arnold Chiari Malformation-I. This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad at Neurosurgery Department during July 2008-July 2010. We examined a prospective cohort of 60 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine. There were 40 female and 20 male patients. The age of onset was 24.9 +/- 15.8 years. Common associated radiological problems included syringomyelia (60%), scoliosis (25%), and basilarinvagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%). The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), and varying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-like episodes, a Meniere's disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances in the absence of syringomyelia. These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes

  13. Magnetic resonance imaging of spinal cord injury in chronic stage

    International Nuclear Information System (INIS)

    Tobimatsu, Haruki; Nihei, Ryuichi; Kimura, Tetsuhiko; Yano, Hideo; Touyama, Tetsuo; Tobimatsu, Yoshiko; Suyama, Naoto; Yoshino, Yasumasa

    1991-01-01

    Magnetic resonance (MR) images of a total of 195 patients with cervical (125) or thoracic (70) spinal cord injury were reviewed. The imaging studies of the spinal cord lesions were correlated with clinical manifestations. Sequential MR imaging revealed hypointensity on T1-weighted images (T1WI) and hyperintensity on T2-weighted images (T2WI) in all patients, except for five patients showing no signal changes and two showing isointensity, suggesting gliosis, myelomalacia, and syringomyelia. Spinal cord lesions were classified into four types: small lesions, large lesions, complete transverse, and longitudinal rupture. These lesions were well correlated with the severity of injury and paralysis. Complete paralysis was frequently associated with enlarged, complete transverse for cervical spinal cord injury, and longitudinal ruptured or thinned complete transverse for thoracic spinal cord injury. The height of paralysis was well in agreement with that of lesions. For incomplete paralysis, localized lesions were seen within the spinal cord, coinciding with the paralysis or severity. Traumatic syringomyelia was seen in 17 patients (8.7%)-- for the cervical site (10 patients, 8%) and the thoracic site (7 patients, 10%). When homogeneous and marginally clear hypointensity is shown on T1-weighted images and vacuolated hyperintensity is shown on T2-weighted images, in addition to lesions spreading two or more cords or 1.5 or more cords above the nervous root level of paralysis, traumatic syringomyelia is strongly suspected, requiring the follow up observation. (N.K.)

  14. Imaging of the late sequelae of spinal cord injuries

    International Nuclear Information System (INIS)

    Bodley, R.

    1994-01-01

    With an increasing ability to diagnose and treat the neurological complications, surveillance of the state of the spinal cord has now assumed great importance. Magnetic resonance imaging (MRI), or computerized tomography (CT) with myelography if MRI is contra-indicated is the method of choice and can demonstrate the pathology with great clarity. In most patients, midline sagittal T1W images are sufficient for screening purposes and for monitoring the success of treatment. Operative, imaging and postmortem studies have shown that the two main changes that occur are: (a) atrophic and (b) cystic - the microcystic and myxoid gel changes of myelomalacia, focal cysts and the larger, more expansive, syringomyelia. As yet, there is no standardization of terminology to describe the various pathological and radiological states. This is critical as only one condition, syringomyelia, is currently amenable to definitive surgery and without conformity, comparisons of incidence in different populations and assessment of the results of surgery are impossible. The published small studies of predominantly symptomatic patients at varying stages of chronicity give differing incidences of changes. Preliminary results of a surveillance MRI study of the spinal cord changes in 153 patients who had had a spinal cord injury over 20 years previously are presented. Altrophy was present in 62%, myelomalacia in 54%, syringomyelia in 22%, focal cysts in 9% and disruption in 7%. (orig./VHE) [de

  15. Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome.

    Science.gov (United States)

    Segal, Devorah; Heary, Robert F; Sabharwal, Sanjeev; Barry, Maureen T; Ming, Xue

    2016-07-01

    The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1-3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement. Surgery was complicated by bleeding from intracranial vascular malformations. This report describes a very rapidly developing, massive holocord syringomyelia related to CSF obstruction due to an unusual congenital brain malformation and associated vascular overgrowth at the site. Serial, premorbid MRI studies demonstrated the very rapid progression from no Chiari malformation, to progressively greater cerebellar tonsillar herniation, to holocord syrinx. This complication has never been reported in MCAP syndrome and should be considered in any affected MCAP patient with a progressive neurological decline, even if previous spine imaging findings were normal. Surgical complications due to hemorrhage also need to be considered in this vascular brain malformation.

  16. Focal adhesive arachnoiditis of the spinal cord: Imaging diagnosis and surgical resolution

    Directory of Open Access Journals (Sweden)

    Hiroki Morisako

    2010-01-01

    Full Text Available Background: Although adhesive arachnoiditis of the spinal cord can cause progressive symptoms associated with syringomyelia or myelomalacia, its surgical resolution based on the imaging diagnosis is not well characterized. This study aims to describe the use of imaging for the diagnosis of focal adhesive arachnoiditis of the spinal cord and its surgical resolution using microsurgical arachnoidolysis. Materials and Methods: Four consecutive patients with symptomatic syringomyelia or myelomalacia caused by focal adhesive arachnoiditis underwent microsurgical arachnoidolysis. Comprehensive imaging evaluation using constructive interference in steady-state (CISS magnetic resonance imaging (MRI or myelographic MR imaging using true fast imaging with steady-state precession (TrueFISP sequences was included before surgery to determine the surgical indication. Results: In all four patients a focal adhesion was identified at the cervical or thoracic level of the spinal cord, a consequence of infection or trauma. Three patients showed modest or minor improvement in neurological function, and one patient was unchanged after surgery. The syringomyelia or myelomalacia resolved after surgery and no recurrence was noted within the follow-up period, which ranged from 5 months to 30 months. Conclusions: MRI diagnosis of focal adhesive arachnoiditis is critical to determine the surgical indication. Microsurgical arachnoidolysis appears to be a straightforward method for stabilizing the progressive symptoms, though the procedure is technically demanding.

  17. Posterior fossa decompression combined with resection of the cerebellomedullary fissure membrane and expansile duraplasty: a radical and rational surgical treatment for Arnold-Chiari type I malformation.

    Science.gov (United States)

    Liang, Chen Jian; Dong, Qiu Jian; Xing, You Heng; Shan, Ma; Wen, Lian Xiao; Qiang, Zhong Yuan; Ping, Zhang Qing; Tao, Peng Zhi; Ping, Huang Xiao

    2014-12-01

    This study aims to introduce a new surgical procedure for the treatment of Arnold-Chiari type I malformation (ACM-1) and to compare its effectiveness with the techniques described in previous studies. We performed the following procedures: foramen magnum decompression combined with the removal of both the atlanto-occipital membrane, extended resection of the cerebellomedullary fissure arachnoid membrane, and artificial duraplasty to enlarge the membranic posterior fossa without resecting the cerebellar tonsils and syringosubarachnoid shunting. There were 21 ACM-1 patients: 12 cases had osteo-compression on the cerebellar hemisphere, 18 cases had thickened adhered fabric ring that stretched from arachnoid membrane to cerebellar hemisphere, and 15 cases with syringomyelia. The patients were followed up for 6 months to 3 years after the surgery. All patients showed a remarkable recovery of syringomyelia. There were no morbidity or death related to the surgery. Most of ACM-1 patients, the osteo- and membrane compression on cerebellar hemisphere and tonsil were observed during the operation. Therefore, decompression of foramen magnum and posterior craniocervical combined with the removal of cerebellomedullary fissure arachnoid membrane and placement of an artificial dural graft should be considered as a comprehensive option of minimally invasive surgery and rational and radical treatment of ACM-1. Our experience showed that, by using our procedure, shunting becomes no longer necessary in the treatment of ACM-1-associated syringomyelia.

  18. Does the mesodermal derangement in Chiari Type I malformation extend to the cervical spine? Evidence from an analytical morphometric study on cervical paraspinal muscles.

    Science.gov (United States)

    Thakar, Sumit; Kurudi Siddappa, Avinash; Aryan, Saritha; Mohan, Dilip; Sai Kiran, Narayanam Anantha; Hegde, Alangar S

    2017-10-01

    OBJECTIVE The mesodermal derangement in Chiari Type I malformation (CMI) has been postulated to encompass the cervical spine. The objectives of this study were to assess the cross-sectional areas (CSAs) of cervical paraspinal muscles (PSMs) in patients with CMI without syringomyelia, compare them with those in non-CMI subjects, and evaluate their correlations with various factors. METHODS In this retrospective study, the CSAs of cervical PSMs in 25 patients were calculated on T2-weighted axial MR images and computed as ratios with respect to the corresponding vertebral body areas. These values and the cervical taper ratios were then compared with those of age- and sex-matched non-CMI subjects and analyzed with respect to demographic data and clinicoradiological factors. RESULTS Compared with the non-CMI group, the mean CSA values for the rectus capitis minor and all of the subaxial PSMs were lower in the study group, and those of the deep extensors were significantly lower (p = 0.004). The cervical taper ratio was found to be significantly higher in the study cohort (p = 0.0003). A longer duration of symptoms and a steeper cervical taper ratio were independently associated with lower CSA values for the deep extensors (p = 0.04 and p = 0.03, respectively). The presence of neck pain was associated with a lower CSA value for the deep flexors (p = 0.03). CONCLUSIONS Patients with CMI demonstrate alterations in their cervical paraspinal musculature even in the absence of coexistent syringomyelia. Their deep extensor muscles undergo significant atrophic changes that worsen with the duration of their symptoms. This could be related to a significantly steeper cervical taper ratio that their cervical cords are exposed to. Neck pain in these patients is related to atrophy of their deep flexor muscles. A steeper cervical taper ratio and alterations in the PSMs could be additional indicators for surgery in patients with CMI without syringomyelia.

  19. Posterior Fossa Decompression with Duraplasty in Chiari-1 Malformations

    International Nuclear Information System (INIS)

    Rehman, L.; Akbar, H.; Bokhari, I.; Babar, A. K.; Hahim, A. S. M.; Arain, S. H.

    2015-01-01

    Objective: To evaluate the symptomatic outcome after PFD (Posterior Fossa Decompression) with duraplasty in Chiari-1 malformations. Study Design: Case series. Place and Duration of Study: Department of Neurosurgery, JPMC, Karachi, from July 2008 to September 2012. Methodology: This included 21 patients of Chiari 1 malformations admitted in department through OPD with clinical features of headache, neck pain, numbness, neurological deficit, and syringomyelia. Diagnosis was confirmed by MRI. PFD followed by C1 laminectomy with duraplasty was done in all cases and symptomatic outcome was assessed in follow-up clinic. Results: Among 21 patients, 13 were females and 8 were males. Age ranged from 18 to 40 years. All the patients had neck pain and numbness in hands. Only 3 patients had weakness of all four limbs and 12 with weakness of hands. Symptoms evolved over a mean of 12 months. Syringomyelia was present in all cases. All patients underwent posterior fossa decompression with duraplasty with an additional C1 laminectomy and in 2 cases C2 laminectomy was done. Syringo-subarachnoid shunt was placed in one patient and ventriculo-peritoneal shunt was placed in 2 patients. Pain was relieved in all cases. Weakness was improved in all cases and numbness was improved in 19 cases. Syringomyelia was improved in all cases. Postoperative complications included CSF leak in 2 patients and wound infection in one patient. However, there was no mortality. Conclusion: Posterior fossa decompression with duraplasty is the best treatment option for Chiari-1 malformations because of symptomatic improvement and less chances of complications. (author)

  20. Richard Bright and his neurological studies.

    Science.gov (United States)

    Pearce, J M S

    2009-01-01

    Richard Bright was one of the famous triumvirate of Guy's Hospital physicians in the Victorian era. Remembered for his account of glomerulonephritis (Bright's disease) he also made many important and original contributions to medicine and neurology. These included his work on cortical epileptogenesis, descriptions of simple partial (Jacksonian) seizures, infantile convulsions, and a variety of nervous diseases. Most notable were his reports of neurological studies including papers on traumatic tetanus, syringomyelia, arteries of the brain, contractures of spinal origin, tumours of the base of the brain, and narcolepsy. His career and these contributions are outlined. Copyright 2009 S. Karger AG, Basel.

  1. Imaging in scoliosis from the orthopaedic surgeon's point of view

    Energy Technology Data Exchange (ETDEWEB)

    Thomsen, Marc [Stiftung Orthopaedische Universitaetsklinik Heidelberg, Schlierbacher Landstr. 200a, 69118 Heidelberg (Germany); Abel, Rainer [Stiftung Orthopaedische Universitaetsklinik Heidelberg, Schlierbacher Landstr. 200a, 69118 Heidelberg (Germany)]. E-mail: rainer.abel@ok.uni-heidelberg.de

    2006-04-15

    For treating patients with scoliosis orthopaedic surgeons need diagnostic imaging procedures in order to provide answers about a possible underlying disease, choice of treatment, and prognosis. Once treatment is instituted, imaging is also critical for monitoring changes of the deformity so as to optimize therapy. The combined effort of orthopaedic surgeons and radiologists helps detect treatable causes of scoliosis at an early stage, define the need and timing for surgery, and ensure that every precaution is taken to minimize the risks of surgery. Neurosurgical causes, with particular reference to spinal cord tumours and syringomyelia, need to be addressed before scoliosis surgery can be contemplated.

  2. Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.

    Science.gov (United States)

    Aryan, Saritha; Ghosal, Nandita; Aziz, Zarina A; Hegde, A S; Dadlani, Ravi

    2014-01-01

    We present a case of infantile holocord ependymoma in a 4-month-old boy who presented with infection of ventriculoperitoneal shunt done elsewhere for a communicating hydrocephalus. On magnetic resonance imaging, a diffuse holocord T2-hyperintense, T1-hypointense intramedullary bulky lesion with syringomyelia in the cervical level was seen. To the best of our knowledge, this is the first case of infantile holocord ependymoma. As the extent of morbidity associated with a spinal cord tumor is high, an increased level of suspicion and the need for a complete spinal cord screening in a case of infantile hydrocephalus without obvious clinical and radiological evidence of intracranial abnormality is emphasized.

  3. Fixed cord

    International Nuclear Information System (INIS)

    Levy, L.M.; DiChiro, G.; DeSouza, B.; McCullough, D.C.; McVeigh, E.; Hefffez, D.

    1989-01-01

    Pulsatile longitudinal motion of the spinal cord was examined with MR phase imaging in healthy subjects and in cases involving cord tethering and compression. Asymptomatic patients with a low conus medullaris demonstrated normal cord motion. Clinical improvement was associated with improved cord motion after surgical untethering, provided permanent neurologic damage had not occurred. Decreased and unchanged cord motion was associated with unchanged neurologic deficits. In cases of normal cord motion and possible retethering versus syringomyelia, clinical improvement occurred after shunting only. MR imaging of pulsatile cord motion can be clinically useful in the evaluation of diseases restricting motion of the neuraxis

  4. A Retrospective 2D Morphometric Analysis of Adult Female Chiari Type I Patients with Commonly Reported and Related Conditions

    Directory of Open Access Journals (Sweden)

    Maggie S. Eppelheimer

    2018-01-01

    Full Text Available Purpose: Researchers have sought to better understand Chiari type I malformation (CMI through morphometric measurements beyond tonsillar position (TP. Soft tissue and bone structures within the brain and craniocervical junction have been shown to be different for CMI patients compared to healthy controls. Yet, several morphological characteristics have not been consistently associated with CMI. CMI is also associated with different prevalent conditions (PCs such as syringomyelia, pseudotumor, Ehlers-Danlos syndrome (EDS, scoliosis, and craniocervical instability. The goal of this study was two-fold: (1 to identify unique morphological characteristics of PCs, and (2 to better explain inconsistent results from case-control comparisons of CMI.Methods: Image, demographic, and PC information was obtained through the Chiari1000, a self-report web-accessed database. Twenty-eight morphometric measurements (MMs were performed on the cranial MR images of 236 pre-surgery adult female CMI participants and 140 female healthy control participants. Custom software was used to measure 28 structures within the posterior cranial fossa (PCF compartment, craniocervical junction, oral cavity, and intracranial area on midsagittal MR images for each participant.Results: Morphometric analysis of adult females indicated a smaller McRae line length in CMI participants with syringomyelia compared to those without syringomyelia. TP was reduced in CMI participants with EDS than those without EDS. Basion to posterior axial line was significantly longer in CMI participants with scoliosis compared to those without scoliosis. No additional MMs were found to differ between CMI participants with and without a specific PC. Four morphometric differences were found to be consistently different between CMI participants and healthy controls regardless of PC: larger TP and a smaller clivus length, fastigium, and corpus callosum height in CMI participants.Conclusion: Syringomyelia, EDS

  5. Magnetic resonance imaging of the central nervous system. Comparison with X-ray CT

    Energy Technology Data Exchange (ETDEWEB)

    Kajima, Toshio; Kagawa, Yoshihiro; Katsuta, Shizutomo.

    1987-06-01

    Magnetic resonance imaging (MRI) and X-ray computed tomography (X-ray CT) have been performed in 169 consecutive patients with central nervous system diseases. The findings from the two methods were compared for the capacity to defect lesions. Magnetic resonance imaging was more sensitive than or equivalent to X-ray CT in detecting lesions - especially detecting. Arnold-Chiari malformation, syringomyelia, spinal cord injury, and pituitary adenoma - in 158 patients (94 %). In six patients (10 %), lesion detection was possible only by MRI. Magnetic resonance imaging was inferior to X-ray CT in 11 patients (7 %) in detecting calcified lesions, meningioma, and cavernous hemangioma. (Namekawa, K.).

  6. Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

    Energy Technology Data Exchange (ETDEWEB)

    Yoshino, Kimihiro; Suga, Masakazu; Takemoto, Motohisa

    1987-06-01

    We evaluated the usefulness of magnetic resonance imaging (MRI) in the diagnosis of the Arnold-Chiari malformation. The patient, a 52-year-old man, complained of dizziness on walking. He initially refused to undergone myelography, but 4 months later, underwent MRI test, which lead to the diagnosis of Arnold-Chiari malformation (I type). Not all patients with symptoms of Arnold-Chiari malformation, syringomyelia, syringobulbia and cervical spinal tumor, undergone myelography, which is an invasive technique, therefore MRI should be the first examination for the patients with disorders involving the craniocervical junction.

  7. Atlantooccipital Overlap and Other Craniocervical Junction Abnormalities in Dogs.

    Science.gov (United States)

    Loughin, Catherine A; Marino, Dominic J

    2016-03-01

    The term craniocervical junction abnormality (CJA) is an umbrella term for a variety of malformations that occur in the craniocervical region. These abnormalities include Chiari-like malformation, atlantooccipital overlapping, atlantoaxial instability, occipitoatlantoaxial malformations, atlantoaxial dural bands, and dens abnormalities. Syringomyelia can occur secondary to any of these malformations or a combination. Clinical signs in dogs with a CJA typically include neck pain and varying signs associated with a cervical myelopathy. MRI of CJAs with computed tomography imaging is necessary for a full evaluation. Some dogs with CJAs respond to medical management, but for most surgery is the treatment of choice. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Think of the Conus Medullaris at the Time of Diagnosis of Fetal Sacral Agenesis.

    Science.gov (United States)

    Mottet, Nicolas; Martinovic, Jelena; Baeza, Claire; Guimiot, Fabien; Bault, Jean-Philippe; Aubry, Marie Cécile; Riethmuller, Didier; Zerah, Michel; Cretolle, Celia; Benachi, Alexandra

    2017-01-01

    There is no precise prenatal indicator to refine an accurate prognosis in case of sacral agenesis and to define the diagnostic approach and outcome criteria in case of fetal sacral agenesis using 3 characteristics of the conus medullaris (CM): its position, its appearance, and associated spinal abnormalities. Ten cases of prenatally diagnosed sacral agenesis were included between 1995 and 2014 after collating ultrasound findings and prenatal computed tomography data. Two cases of total sacral agenesis and 8 of partial agenesis were included. There were 1 or more spinal abnormalities in 8/10 cases: 6 lipomas, 4 low-lying tethered cords, 2 diastematomyelias, and 1 syringomyelia. Three situations were distinguished: sacral agenesis with low-lying tethered cord, sacral agenesis with a truncated CM, and sacral agenesis with CM in place. If the sacral agenesis is isolated, a lipoma should be sought. Lipomas of the filum have a good prognosis, whereas lipomas of the CM cause neurological deficits in 1/3 of cases. When there is a low-lying tethered cord, a diastematomyelia or a syringomyelia may be associated. In truncated CM, there may be a severe form suggestive of caudal regression syndrome. Serious ultrasound signs are immobility of the lower limbs, talipes equinovarus, impaired bladder emptying, and dilatation of the upper urinary tract. A precise description of the morphology of the CM, its position, and associated spinal malformations are important in defining the neurological, urinary, gastrointestinal, and motor functions prognosis in cases of fetal sacral agenesis. © 2016 S. Karger AG, Basel.

  9. MR imaging of spinal trauma

    International Nuclear Information System (INIS)

    Buchberger, W.; Springer, P.; Birbamer, G.; Judmaier, W.; Kathrein, A.; Daniaux, H.

    1995-01-01

    To assess the value of MR imaging in the acute and chronic stages of spinal trauma. 126 MR examinations of 120 patients were evaluated retrospectively. In 15 cases of acute spinal cord injury, correlation of MR findings with the degree of neurological deficit and eventual recovery was undertaken. Cord anomalies in the acute stage were seen in 16 patients. Intramedullary haemorrhage (n=6) and cord transection (n=2) were associated with complete injuries and poor prognosis, whereas patients with cord oedema (n=7) had incomplete injuries and recovered significant neurological function. In the chronic stage, MR findings included persistent cord compression in 8 patients, syringomyelia or post-traumatic cyst in 12, myelomalacia in 6, cord atrophy in 9, and cord transection in 7 patients. In acute spinal trauma, MR proved useful in assessing spinal cord compression and instability. In addition, direct visualisation and characterisation of posttraumatic changes within the spinal cord may offer new possibilities in establishing the prognosis for neurological recovery. In the later stages, potentially remediable causes of persistent or progressive symptoms, such as chronic spinal cord compression or syringomyelia can be distinguished from other sequelae of spinal trauma, such as myelomalacia, cord transection or atrophy. (orig.) [de

  10. MR imaging of intramedullary tumors of the spinal cord: comparison with surgical findings

    International Nuclear Information System (INIS)

    Choe, Du Whan; Hwang, Hee Young; Lee, Hyeon Kyeong; Han, Moon Hee; Kim, In One; Kim, Hyen Jip; Chang, Kee Hyung

    1991-01-01

    To evaluate the capability of MR imaging to localize intramedullary tumors of the spinal cord and to distinguish solid from cystic components. MR images of 12 patients with surgically-proven intramedullary spinal cord tumor were retrospectively reviewed and correlated with surgical findings. Histologic diagnosis consisted of 3 astrocytomas, 4 ependymomas, 2 gangliogliomas, and one case of lipoma, lymphoma, and glioblastoma multiform each. MR scans were obtained on a 2.0T or a 0.5T with T1-and T2-weighted spin-echo pulse sequences in sagittal and axial planes. Contrast enhancement was performed with Gd-DTPA in 9 patients. All tumors expanded the spinal cord and showed an extent of involvement ranging from 2 to 5 vertebral heights, except for the gangliogliomas which involved the spinal cord more extensively. Contrast enhancement was seen in all 9 patients. Intratumoral cavities were observed in 1 out of 3 astrocytomas and 2 gangliogliomas. Peritumoral syringomyelia was seen in 2 out of 4 ependymonas and 2 gangliogliomas. In most cases, the MR findings correlated well with the surgical findings with respect to the extent, distinctness of the tumor margin, intratumoral cavity, and associated syringomyelia. It is concluded that MR imaging is a very useful diagnostic tool in the evaluation of intramedullary spinal cord tumors

  11. Surgical treatment of Arnold-Chiari malformation type I in an adult patient

    Directory of Open Access Journals (Sweden)

    Marković Marko

    2008-01-01

    Full Text Available Background. Herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal with obliteration of the cerebellomedullary cistern is the primary feature of Arnold-Chiari type I malformation (ACM I. It is considered to be congenital malformation, although there have been reported cases of an acquired form. Case report. We presented a female patient, 45-year old, with ACM I without syringomyelia as a rare and unusual clinical image, as well as the effect of decompressive surgery in the treatment of this malformation. The patient was admitted to the Department of Neurosurgery with clinical signs of truncal ataxia worsening during the last six years. Moderate quadriparesis with predominant lower extremity involvement and the signs of the cranial nerves damages occured during the last seven months before admission, with progressive clinical course up to the date of admission. Neurosurgical treatment that included suboccipital medial craniectomy with resection of posterior arch C1 vertebrae and C2 laminectomy resulted in a significant clinical improvement and a much better quality of life. Conclusion. Posterior craniovertebral decompression with microsurgical reduction of the cerebellar tonsils and placement of an artificial dural graft is a treatment of choice in severe forms of ACM I without syringomyelia. .

  12. Usefulness of MRI in the diagnosis of atlanto-axial dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Ko; Fukui, Keiji; Sadamoto, Kazuhiko; Miki, Hitoshi; Niida, Akira; Sakaki, Saburo.

    1988-08-01

    We studied 14 cases of atlanto-axial dislocation (AAD) by means of a 0.5 Tesla superconductive magnetic resonance imaging (MRI) system using saturation recovery sequences (Tr: 500 msec, Te: 20 - 30 msec) with a 5 - 10 mm slice thickness, and directly evaluated the degree of the spinal-cord compression in flexion, neutral, and extension of the head on the midsagittal images. The patients ranged from 11 to 76 years of age, and 8 of the 14 were male. In all cases we could demonstrate the cord compression in various degrees by either the odontoid process or the posterior arch of the atlas. The spinal cords were compressed from ventral in flexion in 6 cases, from dorsal in flexion in 2 cases, from dorsal in extension in 2 cases, and from ventral in both flexion and extension in 2 cases each. In addition, syringomyelia could be revealed by MRI in combination with AAD in 2 cases. MRI is useful in revealing the direction and degree of spinal-cord compression in patients with AAD, and in evaluating combined diseases such as syringomyelia and Arnold-Chiari malformations.

  13. [Acute paraplegia and intramedullary cavitation in a patient with pulmonary tuberculosis].

    Science.gov (United States)

    Schapira, M; Presas, J L; Speiser, E; Klimovsky, S; Barro, A; Nogués, M

    1992-01-01

    This 42-year-old male patient voluntarily discontinued treatment for lung TBC and twenty days later developed acute paraplegia. Magnetic resonance imaging (MRI) demonstrated a large intramedullary cavity extending from T2 to the conus medullaris. Having resumed anti-TBC treatment, the patient progressed favourably, despite any change in cavity size. Tuberculous meningitis may be complicated by the appearance of intramedullary cavities by two distinct mechanisms: 1) adhesive arachnoiditis at the skull base with obstruction of Luschka and Magendie foramina, followed by hydrocephalus and hydromyelia; and 2) spinal cord arachnoiditis with the development of arachnoidal and intramedullary cysts. In either case, symptoms are of late presentation. To the best of our knowledge, this is the first report in the literature of lung tuberculosis associated with syringomyelia but without basal arachnoiditis. Acute clinical presentation with paraplegia is exceptional.

  14. Diseases of the spine, spinal cord, and extremities

    International Nuclear Information System (INIS)

    Tanaka, H.

    1983-01-01

    This paper covers the following topics: acupuncture needle in the spinal canal of the neck; thoracic tuberculous spondylitis: cold abscess; comminuted fracture of the fifth cervical vertebra; lumbar spondylolysis; lumbar spondylosis; ossification of the posterior longitudinal ligament; disk herniation of the cervical spine: narrowing of the root tunnel; ankylosing spondylitis: narrowing of the root tunnel; ossification of the ligamentum flavum; narrow spinal canal syndrome; syringomyelia; epidural neurinoma of the cervical spine; neurofibromatosis; spinal arteriovenous malformation; atrophy of the thoracic spinal cord; osteomyelitis tuberculosa of the rib; osteomyelitis tuberculosa of the left femur; chronic osteomyelitis of the left humerus; malignant giant cell tumor of the left femur; rheumatoid arthritis of the left hip joint; arthrosis deformans of the left hip joint; and enchondroma of the left index finger: Proximal Phalanx

  15. Magnetic resonance imaging of traumatic cervical injury

    International Nuclear Information System (INIS)

    Juhng, S. K.; Lee, K. S.; Sohn, K. J.; Choi, S. S.; Won, J. J.

    1994-01-01

    To evaluate magnetic resonance imaging (MRI) findings of cevical injuries. MRI studies of 34 patients with cervical spinal injuries were analyzed retrospectively. All MRI scans were obtained with an 1.0T superconductive MRI scanner (Siemens Magnetom 42SPE) and their findings were analyzed regarding the spinal cord, bony spine, ligaments, and intervertebral disks. A variety of abnormal findings were detected: 25 cord abnormalities including cord compression (15 cases), cord edema (4 cases), syringomyelia (4 cases), myelomalacia (1 case), and hemorrhagic contusion (1 case), 18 ligamentous injuries, 22 disk herniations (9 post-traumatic, 13 chronic degenerative), 11 spine fractures, and 4 subluxations. MRI is useful in evaluating the spinal cord itself, in depicting ligamentous injuries, in establishing the presence of disc herniation, and in assessing the alignment of cervical spine

  16. Fast STIR (Short TI Inversion Recovery) imaging of the spine. The assessment of the role for the depiction of intramedullary spinal cord lesions and the usefulness of the additional transverse images

    International Nuclear Information System (INIS)

    Okumura, Ryosuke; Kan, Tomoko; Hirose, Tomohiro; Hara, Tadashi; Shibata, Toyomichi; Ueno, Makoto; Takagi, Takehisa; Kohno, Shigene

    2002-01-01

    It is known that the fast STIR images of the spine achieve more excellent lesion contrast than the usual fast spin echo (FSE) T2-weighted images, because the elongation of T1 and T2 relaxation time of the lesion additively contribute to the contrast. The fast STIR images showed advantages in the depiction of acute and chronic lesion of multiple sclerosis and tolerable disadvantages in the depiction of other lesions, such as spondylotic myelopathy or syringomyelia. Transverse images showed less usefulness than sagittal images except for their superior gray-to-white matter contrast. Fast STIR imaging can replace FSE T2-weighted imaging in the study of restricted patients' population. (author)

  17. Tectal glioma presenting with clinical triad of obesity, amenorrhea and central cord syndrome with radiological pentad of hydrocephalus, empty sella, suprapineal diverticula, Chiari and syrinx.

    Science.gov (United States)

    Dhandapani, Sivashanmugam; Karthigeyan, Madhivanan; Sahoo, Sushanta K; Gupta, Vivek

    2017-02-01

    Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma. She was subjected to endoscopic third ventriculostomy (ETV). All of her symptoms improved after ETV and the tumor is being followed up. The possible pathogenesis of such a highly atypical clinico-radiological presentation is described. This unusual syndromic presentation of tectal glioma without clinical features of increased intracranial pressure is probably due to increased compliance of third ventricular walls, and is relieved with ETV.

  18. Comparison of CT and MR in 400 patients with suspected disease of the brain and cervical spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Bradley, W.G. Jr.; Waluch, V.; Yadley, R.A.; Wycoff, R.R.

    1984-09-01

    Magnetic resonance imaging (MR) (0.35T) and computed tomography (CT) were compared in 400 consecutive patients with suspected disease of the brain and cervical spinal cord. Of 325 positive diagnoses, MR detected abnormality while CT was normal in 93; MR was more specific in 68; MR and CT gave equivalent information in 129; CT was more specific in 32; and CT was positive while MR was normal in 3. MR was superior to CT in detection of multiple sclerosis, subcortical arteriosclerotic encephalopathy, posterior fossa infarcts and tumors, small extra-axial fluid collections, and cervical syringomyelia. CT was preferable in evaluation of meningiomas and separation of tumor from edema. CT takes less time and may be preferable in patients with acute trauma as well as very young or elderly individuals. Thus the two studies should be considered complementary.

  19. Comparison of CT and MR in 400 patients with suspected disease of the brain and cervical spinal cord

    International Nuclear Information System (INIS)

    Bradley, W.G. Jr.; Waluch, V.; Yadley, R.A.; Wycoff, R.R.

    1984-01-01

    Magnetic resonance imaging (MR) (0.35T) and computed tomography (CT) were compared in 400 consecutive patients with suspected disease of the brain and cervical spinal cord. Of 325 positive diagnoses, MR detected abnormality while CT was normal in 93; MR was more specific in 68; MR and CT gave equivalent information in 129; CT was more specific in 32; and CT was positive while MR was normal in 3. MR was superior to CT in detection of multiple sclerosis, subcortical arteriosclerotic encephalopathy, posterior fossa infarcts and tumors, small extra-axial fluid collections, and cervical syringomyelia. CT was preferable in evaluation of meningiomas and separation of tumor from edema. CT takes less time and may be preferable in patients with acute trauma as well as very young or elderly individuals. Thus the two studies should be considered complementary

  20. Magnetic resonance tomography for trauma of the cervical spine

    International Nuclear Information System (INIS)

    Meydam, K.; Sehlen, S.; Schlenkhoff, D.; Kiricuta, J.C.; Beyer, H.K.

    1986-01-01

    Twenty patients who had suffered spinal trauma were examined by magnetic resonance tomography. Fifteen patients with first degree trauma in Erdmann's classification showed no abnormality. Magnetic resonance tomography of the cervical spine appears to be a suitable method for investigating patients with whiplash injuries. It is indicated following severe flexion injuries with subluxations and neurological symptoms, since it is the only method that can demonstrate the spinal cord directly and completely and show the extent of cord compression. For patients with thoracic trauma and rapidly developing neurological symptoms, magnetic resonance tomography is ideal for showing post-traumatic syringomyelia. Magnetic resonance tomography following whiplash injuries is recommended if plain films of the cervical spine show any abnormalities, as well as for the investigation of acute or sub-acute neurological abnormalities. The various findings are discussed. (orig.) [de

  1. Posterior fossa decompression with duraplasty in Chiari surgery: A technical note

    Directory of Open Access Journals (Sweden)

    Marcelo Ferreira Sabba

    Full Text Available Summary Chiari malformation (CM is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI. Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.

  2. Nuclear magnetic resonance (NMR) imaging of Arnold-Chiari type I malformation with hydromyelia

    Energy Technology Data Exchange (ETDEWEB)

    DeLaPaz, R.L.; Brady, T.J.; Buonanno, F.S.; New, P.F.; Kistler, J.P.; McGinnis, B.D.; Pykett, I.L.; Taveras, J.M.

    1983-02-01

    Saturation recovery nuclear magnetic resonance (NMR) images and metrizamide computed tomography (CT) scans were obtained in an adult patient with a clinical history suggestive of syringomyelia. Both NMR and CT studies showed low lying cerebellar tonsils. The CT study demonstrated central cavitation of the spinal cord from the midthoracic to midcervical levels but could not exclude an intramedullary soft tissue mass at the cervico-medullary junction. The NMR images in transverse, coronal, and sagittal planes demonstrated extension of an enlarged central spinal cord cerebrospinal fluid space to the cervico-medullary junction. This was felt to be strong evidence for exclusion of an intramedullary soft tissue mass and in favor of a diagnosis of Arnold-Chiari Type I malformation with hydromyelia. The noninvasive nature of spinal cord and cervico-medullary junction evaluation with NMR is emphasized.

  3. Nuclear magnetic resonance tomography of the cervical canal

    Energy Technology Data Exchange (ETDEWEB)

    Terwey, B.; Koschorek, F.; Jensen, H.P.

    1985-12-01

    170 patients with suspected lesions of the cervical part of the medulla were examined using nuclear magnetic resonance (NMR) tomography. 27 cases revealed no pathological changes in the regions of the cervical medulla, the cervical canal and of the cervical spine. 143 cases produced pathological findings whose diagnoses determined therapeutical approach. Verified pathological changes comprised anomalies of the cranio-cervical junction like basilar impression and Arnold-Chiari malformation, various types of cavity formation in the cervical medulla (syringomyelia, hydromyelia), demyelinization processes, intramedullary and extramedullary tumours, intervertebral disk degeneration processes, dislocation of intervertebral disks and spondylophytes with spinal stenoses. Sagittal sections in different functional positions allowed to demonstrate the biomechanical effects of extramedullary masses on the cervical medulla. However, proven tumours could not be differentiated successfully using histological methods. Nevertheless, NMR tomography will replace invasive methods like conventional cervical myelography and CT myelography in diagnostic clarification of diseases of the cervical medulla.

  4. Diagnostic value and clinical problems of MR imaging in congenital anomalies of the central nervous system, 2. Spinal dysraphisms

    Energy Technology Data Exchange (ETDEWEB)

    Oi, Shizuo; Urui, Seishiro; Asano, Noboru; Masumura, Michio; Shose, Yoshiteru; Matsumoto, Satoshi

    1987-06-01

    Spina bifida and associated congenital anomalies in the central nervous system were evaluated by means of MRI, and the results compared with those obtained by conventional diagnostic procedures. Using the two-dimensional Fourier transform technique, a three-radiofrequency-pulse sequence (inversion recovery: IR 2100/500; spin-echo: SE 2100/40 or 2100/80) was routinely applied. Compared with X-ray CT, MR proved to be more accurate in the detection of the pathoanatomical relation between the lesion and the spinal cord, or that between the spinal dysraphic state and associated intracranial anomalies. MRI was also superior in the anatomical diagnosis of a spinal lipoma, a tethered cord, syringobulbia, syringomyelia, the Chiari anomaly, and so forth. The most considerable disadvantage of MRI in the diagnosis of the spina bifida is the poor information it provides about the bifid spine itself, but this information may be obtained by the use of conventional diagnostic procedures. Also, a regular-conducting MRI system is still insufficient to demonstrate the precise location of the canda equina nerve roots, especially in relation to a lipoma, although the spin-echo MR myelographic technique was helpful in demarcating the major structures, such as the lipoma and the cord. In syringomyelia and syringobulbia, further invasive study in analyzing the fluid dynamics is needed to determine the proper operative procedure. It was emphasized in this study that MRI is an extremely valuable diagnostic tool also in the diagnosis of spinal dysraphism, especially in the detection of a pathoanatomical structure, but can also be expected to be improved so as to make possible finer anatomical analysis and provide a higher quality of information on the fluid dynamics, at least so as to indicate operative procedures without any invasive methods. (J.P.N.).

  5. Gd-DTPA MR imaging enhancement of spinal cord tumors

    International Nuclear Information System (INIS)

    Dillon, W.P.; Bolla, K.; Mark, A.S.; Tsudura, J.S.; Norman, D.; Newton, T.H.

    1987-01-01

    Nineteen patients with suspected spinal cord tumors were imaged with T1- and T2-weighted sequences before and after the administration of Gd-DTPA (0.1 mmol/kg). Eleven of the 19 patients had spinal cord tumors (three unproven). Eight of 11 patients had intramedullary tumors (four astrocytomas, two ependymomas) and two had extra-medullary tumors (one meningioma, one metastatic melanoma). Other lesions studied include idiopathic syringomyelia (two), spinal arteriovenous malformation (AVM) (one), posttraumatic arachnoiditis (one), and cord infarct (one). All of the tumors enhanced after the administration of Gd-DTPA. Spinal cord enhancement was also noted in association with a spinal cord AVM, a suspected cord infarct, and in the patient with severe arachnoiditis. No enhancement was present in patients with idiopathic or posttraumatic syringomyelia or in the three normal patients. In six of the patients, enhancement was critical in confirming disease that was questionable on the precontrast MR images. Gadolinium enhancement allowed differentiation of tumor from postoperative changes in two patients with spinal cord tumors. Enhanced images localized the lesion more accurately than precontrast MR images in eight patients. In four patients a lesion could only be detected after the administration of contrast. The postcontrast images better defined the margin of tumor from surrounding edema, operative scarring, and cord cavitation. The AVM case had enhancement of slowly flowing veins with Gd-DTPA posterior to an ischemic cord segment. Gd-DTPA enhancement is extremely useful in the detection and therapeutic assessment of spinal cord tumors; however, enhancement is not specific for tumors and should be interpreted in light of the clinical setting

  6. Quality of life in individuals affected by Arnold Chiari malformation: comparison and validation of a measurement instrument.

    Science.gov (United States)

    Martinez-Sabater, Antonio; Ballestar-Tarin, Maria Luisa; Vazquez-Seoane, Miriam; Mari-Avargues, Lucia; Saus-Ortega, Carlos; Casal-Angulo, Maria Del Carmen

    2017-11-23

    Introduction. Arnold Chiari Malformation (ACM) type I is a pathology whose symptomatology has repercussions for the quality of life of those affected by it. Quality-of-life measurement instruments can allow the severity of the impact of Chiari type I malformation on patients' lives to be monitored. The Chiari Symptom Profile (CSP) is a valid and reliable instrument designed for this purpose. The aim of the study was to adapt the CSP to Spanish and to explore the reliability and validity of this construct in the context of Spanish-speaking patients with ACM. The English CSP instrument has a good internal validity and consistency. We used a standardized procedure for the linguistic validation of the translated scale. For the psychometric validation, we recruited 215 individuals with ACM and calculated the Cronbach's alpha for the sample. The construct was validated by analyzing the age, sex, and presence of syringomyelia, as well as by correlating the results with the sickness impact profile 30 (SIP-30) questionnaire, which can also evaluate quality of life in this type of patient. The Spanish version of the CSP has good internal consistency and validity (Cronbach's alpha of 0.90); age, sex, and the presence of syringomyelia do not significantly affect the quality of life of patients with ACM. There was a direct and significant correlation between the Spanish CSP and the validated SIP-30 questionnaire results (p < 0.05). Further analysis showed a positive correlation for the physical and psychological scopes of the CSP and SIP-30 questionnaires, but not for their functional and social scopes. This version of the CSP is a valid and reliable instrument for measuring quality of life in patients with ACM in the Spanish context. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  7. Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.

    Science.gov (United States)

    Langridge, Benjamin; Phillips, Edward; Choi, David

    2017-08-01

    Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical decompression, but little is known about the natural history of patients who do not undergo surgical management. This information is critical to determine how these patients should be managed. We conducted a systematic literature review to determine the natural history of CM-1, particularly in patients who did not undergo surgery and in asymptomatic individuals, to help patients and physicians determine when surgery is likely to be beneficial. The literature search was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the electronic databases PubMed, Scopus, Cochrane Library, and Web of Science. Inclusion and exclusion criteria were predefined. In symptomatic patients who did not undergo surgery, headaches and nausea often improved, whereas ataxia and sensory disturbance tended not to improve spontaneously. Of patients, 27%-47% had an improvement in symptoms after 15 months, and 37%-40% with cough headache and 89% with nausea who were managed nonoperatively improved at follow-up. Most asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the presence of syringomyelia. The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient's symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Early onset scoliosis with intraspinal anomalies: management with growing rod.

    Science.gov (United States)

    Jayaswal, Arvind; Kandwal, Pankaj; Goswami, Ankur; Vijayaraghavan, G; Jariyal, Ashok; Upendra, B N; Gupta, Ankit

    2016-10-01

    To evaluate clinical and radiological outcomes of growing rod (GR) in the management of Early Onset Scoliosis (EOS) with intraspinal anomalies. The effect of repeated distractions following GR, in the presence of intraspinal anomalies has not been studied. During 2007-2012, 46 patients underwent fusionless surgery. Out of these 46 patients, 13 patients had one or more intraspinal anomalies. 11 patients had undergone prior neurosurgical procedure while 2 (filum terminale lipoma and syringomyelia) did not. A total of 88 procedures were conducted during the treatment period; 13 index surgeries, 74 distractions of GR and 1 unplanned surgery. The age at surgery was 6.8 ± 2.5 years (3.5-12 years). 11 patients had congenital scoliosis and 2 had idiopathic scoliosis. A total of 19 (41.30 %) intraspinal anomalies [Tethered Cord Syndrome (TCS) 08, Split Cord Malformation (SCM) 08, Syringomyelia 01, Meningomyelocele 01, Filum terminale Lipoma 01] were seen. The average lengthening procedures per patient were 5.7 (4-9) with distraction interval of 6.7 (6-7.25) months. Pre-operative Cobb angle was 78.50 ± 18.1 (54-114°) and improved to 53.10 ± 16.70 (36-84°) at final follow-up. A total of 15 complications related to implant (9), wound (2), anesthesia (2) and neurological (2) occurred in 7 patients. Among the two neurological complications, one patient sustained fall in the post-op period and reported to the emergency department with paraplegia and broken proximal screw. While other patient experienced MEP changes during index procedure. None of the patients had any neurological complications during repeated lengthening procedures. The most common cord anomalies associated with EOS in our study are TCS and SCM. Although presence of previous intraspinal anomaly does not seem to increase the incidence of neurological deficit, use of neuromonitoring is advisable for all index procedure and selected distractions. Level 4 (case series).

  9. [Papillary oedema revealing Arnold Chiari malformation type 1: about a case].

    Science.gov (United States)

    Imane, Mouhoub; Asmae, Maadane; Toufik, Ramdani; Rachid, Sekhsoukh

    2016-01-01

    Arnold Chiari malformation type 1 is defined as a herniation of the cerebellar tonsils into the foramen magnum of more than 5 mm. Symptoms are most commonly dominated by occipital headache, torticollis and sometimes swallowing disorders. Ophthalmologically abnormal convergences, oculomotor palsy and diplopia are the main clinical signs. We report the case of a 9 year old child, who presented with visual loss evolving since 6 months. Ophthalmologic examination showed visual acuity of 4/10 in both eyes, retained ocular motility and rotational nystagmus. The examination of the anterior segment of the eye showed megalocornea with no evidence of goniodysgenesis, iridodonesis associated with atrophy of the dilator muscle and microcoria with lazy photomotor reflex. Normal intraocular pressure was 14 mmHg. Ocular fundus examination, despite difficulties in performing it, objectified bilateral papilledema (stage II). General physical examination showed torticollis, scoliosis and a tetra-pyramidal syndrome. MRI showed Chiari malformation type I associated with hydrocephalus and syringomyelia. Neurosurgical intervention based on internal CSF drainage with occipitocervical osteo-dural decompression was proposed. The evolution was favorable with regression of clinical signs. Ophthalmologically, there was a regression of papilledema but visual acuity remained stationary. The occurrence of papilledema associated with Chiari malformation type 1 is rare, it has been only reported in 2% of symptomatic patients. Its pathophysiology is still poorly understood. The originality of our study consists in the association of cerebellar malformations with ocular malformations including megalocornea and microcoria which make ophthalmologic examination more difficult to perform.

  10. Unusual Ventilatory Response to Exercise in Patient with Arnold-Chiari Type 1 Malformation after Posterior Fossa Decompression

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    Keely Smith

    2016-01-01

    Full Text Available We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2 resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship. No new pathology of the brainstem was discovered by MRI or neurological evaluation to explain this phenomenon. The patient was placed on continuous noninvasive open ventilation (NIOV during the day and CPAP at night for a period of 6 months. His pCO2 level decreased to normal limits and his symptoms improved; specifically, he experienced less headaches and fatigue during exercise. In this report, we describe the abnormal response to exercise that patients with AC-Type 1 could potentially experience, even after decompression, characterized by the impairment of ventilator response to hypercapnia during exertion, reflecting a complete loss of chemical influence on breathing with no evidence of abnormality in the corticospinal pathway.

  11. George Marinesco in the Constellation of Modern Neuroscience

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    Ioan Opris

    2017-12-01

    Full Text Available George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was “La Cellule Nerveuse” and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time.

  12. George Marinesco in the Constellation of Modern Neuroscience.

    Science.gov (United States)

    Opris, Ioan; Nestianu, Valeriu S; Nestianu, Adrian; Bilteanu, Liviu; Ciurea, Jean

    2017-01-01

    George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was "La Cellule Nerveuse" and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time.

  13. Spinal cord involvement in tuberculous meningitis.

    Science.gov (United States)

    Garg, R K; Malhotra, H S; Gupta, R

    2015-09-01

    To summarize the incidence and spectrum of spinal cord-related complications in patients of tuberculous meningitis. Reports from multiple countries were included. An extensive review of the literature, published in English, was carried out using Scopus, PubMed and Google Scholar databases. Tuberculous meningitis frequently affects the spinal cord and nerve roots. Initial evidence of spinal cord involvement came from post-mortem examination. Subsequent advancement in neuroimaging like conventional lumbar myelography, computed tomographic myelography and gadolinium-enhanced magnetic resonance-myelography have contributed immensely. Spinal involvement manifests in several forms, like tuberculous radiculomyelitis, spinal tuberculoma, myelitis, syringomyelia, vertebral tuberculosis and very rarely spinal tuberculous abscess. Frequently, tuberculous spinal arachnoiditis develops paradoxically. Infrequently, spinal cord involvement may even be asymptomatic. Spinal cord and spinal nerve involvement is demonstrated by diffuse enhancement of cord parenchyma, nerve roots and meninges on contrast-enhanced magnetic resonance imaging. High cerebrospinal fluid protein content is often a risk factor for arachnoiditis. The most important differential diagnosis of tuberculous arachnoiditis is meningeal carcinomatosis. Anti-tuberculosis therapy is the main stay of treatment for tuberculous meningitis. Higher doses of corticosteroids have been found effective. Surgery should be considered only when pathological confirmation is needed or there is significant spinal cord compression. The outcome in these patients has been unpredictable. Some reports observed excellent recovery and some reported unfavorable outcomes after surgical decompression and debridement. Tuberculous meningitis is frequently associated with disabling spinal cord and radicular complications. Available treatment options are far from satisfactory.

  14. Traumatic spinal cord injury caused by suspected hyperflexion of the atlantoaxial joint in a 10-year-old cat

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    Annette Wessmann

    2015-06-01

    Full Text Available Case summary A 10-year-old cat presented 5 days after a traumatic event with acute recumbency followed by some clinical improvement. The neuroanatomical localisation was the C1–C5 spinal cord segments. Initial survey radiographs, including lateral flexed views, showed no convincing abnormalities. Magnetic resonance imaging (MRI revealed a marked focal intramedullary lesion at the level of the dens and suspected oedema extending over C2–C3 vertebrae, suggesting early syrinx formation. The cat made an initial excellent recovery on restricted exercise without medical treatment. The MRI changes largely resolved on follow-up MRI 4 weeks later yet recurred following a relapse 4 months later. At this stage, a post-traumatic syrinx had developed. Moreover, the suspected atlantoaxial instability was finally diagnosed on radiography with fully flexed lateral views. A hyperflexion injury causing tearing of the atlantoaxial ligaments was considered most likely given the lack of malformations or fractures. The cat made a full recovery on conservative management. Relevance and novel information This is the first report of sequential MRI findings in a cat with atlantoaxial instability. Moreover, post-traumatic syringomyelia formation following atlantoaxial injury has not been reported. Sequential MRI aids in the diagnosis of hyperflexion injury if survey radiographs fail to identify atlantoaxial instability.

  15. Sonography for detection of spinal dermal sinus tracts.

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    Lin, Kuang-Lin; Wang, Huei-Shyong; Chou, Ming-Liang; Lui, Ta-Ngar

    2002-08-01

    It is well known that spinal dysraphism may be complicated by meningitis as a result of infection traveling from the skin along a patent dermal sinus tract. The only clue is the lower back cutaneous lesions. Our aim was to investigate the correlation between cutaneous lesions, patent dermal sinus tracts, and spinal dysraphism and their complications. Five patients (3 female and 2 male) with spinal patent dermal sinus tracts were studied. We used a 7-MHz linear transducer with a two-dimensional real-time sonographic system to insonate and obtain transverse and longitudinal views of the spinal cord and subcutaneous area, extending from the cervical cord to the sacral areas. Subsequently, we performed spinal magnetic resonance imaging in every patient to confirm the diagnosis and to evaluate the intraspinal conditions. The associated central nervous system anomalies and complications were tethered cords (n = 5), dermoid cysts (n = 3), lipoma (n = 2), central nervous system infections (n = 2), and syringomyelia (n = 1). The outcomes were better in those who received surgical intervention before they were infected. Early detection of spinal patent dermal sinus tracts and related anomalies was accomplished with spinal sonography and allowed for prophylactic treatment (e.g., early surgical intervention) before the onset of neurologic deficits.

  16. Automated single-trial assessment of laser-evoked potentials as an objective functional diagnostic tool for the nociceptive system.

    Science.gov (United States)

    Hatem, S M; Hu, L; Ragé, M; Gierasimowicz, A; Plaghki, L; Bouhassira, D; Attal, N; Iannetti, G D; Mouraux, A

    2012-12-01

    To assess the clinical usefulness of an automated analysis of event-related potentials (ERPs). Nociceptive laser-evoked potentials (LEPs) and non-nociceptive somatosensory electrically-evoked potentials (SEPs) were recorded in 37 patients with syringomyelia and 21 controls. LEP and SEP peak amplitudes and latencies were estimated using a single-trial automated approach based on time-frequency wavelet filtering and multiple linear regression, as well as a conventional approach based on visual inspection. The amplitudes and latencies of normal and abnormal LEP and SEP peaks were identified reliably using both approaches, with similar sensitivity and specificity. Because the automated approach provided an unbiased solution to account for average waveforms where no ERP could be identified visually, it revealed significant differences between patients and controls that were not revealed using the visual approach. The automated analysis of ERPs characterized reliably and objectively LEP and SEP waveforms in patients. The automated single-trial analysis can be used to characterize normal and abnormal ERPs with a similar sensitivity and specificity as visual inspection. While this does not justify its use in a routine clinical setting, the technique could be useful to avoid observer-dependent biases in clinical research. Copyright © 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  17. Recapping hemilaminoplasty for spinal surgical disorders using ultrasonic bone curette.

    Science.gov (United States)

    Matsuoka, Hidenori; Itoh, Yasunobu; Numazawa, Shinichi; Tomii, Masato; Watanabe, Kazuo; Hirano, Yoshitaka; Nakagawa, Hiroshi

    2012-01-01

    The authors present a novel method of the recapping hemilaminoplasty in a retrospective study of patients with spinal surgical disorders. This report describes the surgical technique and the results of hemilaminoplasty using an ultrasonic bone curette. The aim of this study was to examine the safety and effectiveness of the hemilaminoplasty technique with ultrasonic bone curette. Between April 2003 and July 2011, 33 patients with various spinal diseases (17 spinal tumors, 5 dural arteriovenous fistulas, 3 syringomyelia, 2 sacral perineural cysts, and 2 arachnoid cysts) were treated microsurgically by using an ultrasonic bone curette with scalpel blade and lightweight handpiece. The ultrasonic bone curette was used for division of lamina. After resection of the lesion, the excised lamina was replaced exactly in situ to its original anatomic position with a titanium plate and screw. Additional fusion technique was not required and the device was easy to handle. All patients were observed both neurologically and radiologically by dynamic plain radiographs and computed tomography (CT) scan. The operation was performed successfully and there were no instrument-related complications such as dural laceration, nerve root injury, and vessels injury. The mean number of resected and restored lamina was 1.7. CT confirmed primary bone fusion in all patients by 12 months after surgery. The ultrasonic bone curette is a useful instrument for recapping hemilaminoplasty in various spinal surgeries. This method allows anatomical reconstruction of the excised bone to preserve the posterior surrounding tissues.

  18. [Contributions of professor Xue Chong-cheng in acupuncture and neuropsychiatry].

    Science.gov (United States)

    Yang, Qiu-li; Liu, Wan-ting; Xu, Rui

    2008-07-01

    Professor Xue Chong-cheng devotes himself in clinical and research of integrative works of TCM, acupuncture, neuropsychiatry, neurosurgery and medicopsychology for more than 70 years. He firstly confirmed acupoints are identical with nerve and motor points, found propagating meridian sensation on phantom limbs of acquired and congenital amputates. The sensation can pass over fresh incision wound and it exits and disappears with the cortical sensation. He proposed a central theory that a meridians model is present in the brain. He suggested examination of general afferent system, cortical sensation and deep pain of syringomyelia with acupuncture as they are absent in routine methods but still present during needling. He firstly reported meridian type of sensory epilepsy and it was recognized by the Epilepsy Center of the USA. He treated psychosis with electroacupuncture convulsive therapy, the dosage of current used was less than 4% of the conventional method. The Journal of Psychiatry of USA recognized it is a dramatic progress for more than 40 years. He compiled first integrative medical questionnaire for assessment of pain. According to TCM theories and modern method of standardization he established inventories and national norms of personality and constitution for the corresponding examinations. They fulfill the gap of China. He firstly proposed the model of TCM is temporo-spatio-socio-psycho-biological. Now he is nearly ninety years old but is still struggling on the first line.

  19. Craniocervical junction abnormalities with atlantoaxial subluxation caused by ventral subluxation of C2 in a dog

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    Harumichi Itoh

    2017-03-01

    Full Text Available Craniocervical junction abnormalities with atlantoaxial subluxation caused by ventral subluxation of C2 were diagnosed in a 6-month-old female Pomeranian with tetraplegia as a clinical sign. Lateral survey radiography of the neck with flexion revealed atlantoaxial subluxation with ventral subluxation of C2. Computed tomography revealed absence of dens and atlanto-occipital overlapping. Magnetic resonance imaging showed compression of the spinal cord and indentation of caudal cerebellum. The diagnosis was Chiari-like malformation, atlantoaxial subluxation with ventral displacement of C2, atlanto-occipital overlapping, and syringomyelia. The dog underwent foramen magnum decompression, dorsal laminectomy of C1, and ventral fixation of the atlantoaxial joint. Soon after the operation, voluntary movements of the legs were recovered. Finally, the dog could stand and walk without assistance. The dog had complicated malformations at the craniocervical junction but foramen magnum decompression and dorsal laminectomy for Chiari-like malformation, and ventral fixation for atlantoaxial subluxation resulted in an excellent clinical outcome.

  20. Measurement of normal cervical spinal cord in metrizamide CT myelography

    International Nuclear Information System (INIS)

    Suzuki, Fumio; Koyama, Tsunemaro; Aii, Heihachirou

    1985-01-01

    The shape of the spinal cord is the most important factor in diagnosis of spinal disorders by metrizamide CT myelography (met. CT). Even in cases where the spinal cord looks normal in shape its size might be abnormal, for example in cases with spinal cord atrophy, syringomyelia, intramedullary tumor and several other conditions. In detecting the slightest abnormality in such cases, it is absolutely necessary to have in hand the knowledge of the nomal size of the spinal cord at each level. We measured, therefore, the sagittal and transverse diameters of the cervical spinal cord in 55 patients with no known lesions on met. CT (Fig. 1). Comparing our results with those by others, we found some differences as to the size of the spinal cord. We assume that these differences are due to the differences in resolution of the CT scanners used. The size of the spinal cord tends to measure larger with a CT scanner with high resolution than with others. Previous authors reported that the size of the spinal cord would vary by window center settings. Our experimental results indicate, however, that window center settings do not significantly affect the measurements. It is concluded that the normal values of the spinal cord dimensions at each level somewhat differ by CT equipments used. One should have normal values with one's own equipment in hand in order to take full advantage of this sophisticated diagnostic technique. (author)

  1. Various MRI findings of spinal ependymoma

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    Shin, Yoon Joo; Lee, Eugene; Lee, Joon Woo; Kang, Yu Suhn; Hyun, Seung Jae; Kim, Ki Jeong; Jahng, Tae Ahn; Kim, Hyun Jib; Kang, Heung Sik [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of)

    2017-06-15

    To present the typical and atypical magnetic resonance image (MRI) findings of intramedullary spinal ependymomas, and compare these findings with pathological subtypes. Between January 2003 to November 2014, 47 patients who had spinal ependymoma with pathologic confirmation, were retrospectively reviewed with all electronic medical records and MR images. MR imaging was done in all cases, and the images of spinal ependymomas and associated enhancement patterns were correlated with pathologic findings. The enhancement patterns were categorized into four categories: homogeneous, heterogeneous, rim-enhancement, and non-enhancement. Heterogeneous enhancement was observed in 50% of the cases. Among the 47 cases, 35 cases were well-marginated, with 21 being cervically located. All lesions were centrally located in the axial axis. Most of the cases showed T1-iso signal intensity (81%) and T2-high signal intensity (72%). The hemosiderin cap sign, syringomyelia, tumoral and non-tumoral cysts were well demonstrated. The most common pathologic type was cellular ependymoma. However, due to the small sample size, we believe it inappropriate to statistically discuss the MRI findings according to the pathologic subtype. Most spinal ependymoma showed T1-iso signal intensity and T2-high signal intensity, with enhancement patterns other than homogeneous enhancement.

  2. Iopamidol vs Metrizamide: a double blind study for cervical myelography

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    Drayer, P.; Warner, M.A.; Allen, S.; Bates, M.; Sudilovsky, A.; Luther, J.; Wilkins, R.

    1982-11-01

    A double-blind study was performed on 20 patients comparing the safety and efficacy of Iopamidol and Metrizamide in cervical myelography. The radiographic qualities of the Iopamidol and Metrizamide examinations were equivalent when using the same volume (12 to 13 ml), concentration (200 mg I/ml) and a C1-2 route of administration. The performance of a CT scan on selected patients in specified areas of interest provided additional diagnostic information in some patients (e.g. syringomyelia, degenerative spondylosis). The adverse reactions were mild in the Iopamidol group with 4 of the 10 patients experiencing no adverse reactions. The more severe reactions, including disorientation, agitation, dysarthria, asterixis, hyperreflexia and EEG abnormalities were limited to the Metrizamide group with one or more occurring in 2 of the 10 patients studies. Only one Metrizamide patient experienced no adverse reactions. In this study containing a limited number of patients, Iopamidol was shown to be a diagnostically effective and safer contrast medium for performing cervical myelography.

  3. Hemifacial spasm in a patient with neurofibromatosis and Arnold-Chiari malformation: a unique case association Espasmo hemifacial em paciente com neurofibromatose e malformação de Arnold-Chiari: uma associação rara

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    Andre Carvalho Felício

    2007-09-01

    Full Text Available BACKGROUND: The association of hemifacial spasm (HFS, Chiari type I malformation (CIM and neurofibromatosis type 1 (NF1 has not been described yet. CASE REPORT: We report the case of a 31-year-old woman with NF1 who developed a right-sided HFS. On magnetic resonance imaging (MRI a CIM was seen without syringomyelia. The patient has been successfully treated with botulinum toxin type A injections for 5 years without major side effects. CONCLUSION:Clinical features of HFS, CMI and NF1 are highlighted together with their possible relationship. Also, therapeutic strategies are also discussed.INTRODUÇÃO: A associação entre espasmo hemifacial (EHF, malformação de Chiari tipo I (MCI e neurofibromatose tipo I (NFI ainda não foi descrita. RELATO DO CASO: Relatamos o caso de mulher com 31 anos com NFI que desenvolveu EHF à direita. Na ressonância magnética (RM foi observada MCI sem seringomielia associada. A paciente foi tratada com sucesso com toxina botulínica tipo A por 5 anos sem efeitos colaterais. CONCLUSÃO: Ressaltamos as características clínicas do EHF, MCI e NFI assim como uma possível relação entre elas. Além disto, discutimos também estratégias terapêuticas.

  4. Subdural Fluid Collection and Hydrocephalus After Foramen Magnum Decompression for Chiari Malformation Type I: Management Algorithm of a Rare Complication.

    Science.gov (United States)

    Rossini, Zefferino; Milani, Davide; Costa, Francesco; Castellani, Carlotta; Lasio, Giovanni; Fornari, Maurizio

    2017-10-01

    Chiari malformation type I is a hindbrain abnormality characterized by descent of the cerebellar tonsils beneath the foramen magnum, frequently associated with symptoms or brainstem compression, impaired cerebrospinal fluid circulation, and syringomyelia. Foramen magnum decompression represents the most common way of treatment. Rarely, subdural fluid collection and hydrocephalus represent postoperative adverse events. The treatment of this complication is still debated, and physicians are sometimes uncertain when to perform diversion surgery and when to perform more conservative management. We report an unusual occurrence of subdural fluid collection and hydrocephalus that developed in a 23-year-old patient after foramen magnum decompression for Chiari malformation type I. Following a management protocol, based on a step-by-step approach, from conservative therapy to diversion surgery, the patient was managed with urgent external ventricular drainage, and then with conservative management and wound revision. Because of the rarity of this adverse event, previous case reports differ about the form of treatment. In future cases, finding clinical and radiologic features to identify risk factors that are useful in predicting if the patient will benefit from conservative management or will need to undergo diversion surgery is only possible if a uniform form of treatment is used. Therefore, we believe that a management algorithm based on a step-by-step approach will reduce the use of invasive therapies and help to create a standard of care. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

    Science.gov (United States)

    Sgulò, Francesco Giovanni; Spennato, Pietro; Aliberti, Ferdinando; Di Martino, Giuliana; Cascone, Daniele; Cinalli, Giuseppe

    2017-01-01

    Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

  6. Magnetic resonance imaging of cervical cord injury and its correlation with the patient's outcome

    International Nuclear Information System (INIS)

    Panggabean, F.; Nakamura, Tsutomu

    1991-01-01

    Thirty four patients with cervical cord injuries were studied by magnetic resonance imaging (MRI) with reference to neurological deficits. Studies in the acute or subacute stage were made in 25 patients, of whom 17 patients were studied consecutively up to the chronic stage. Chronic studies were made in 26 patients. In acute or subacute MRI studies 12 patients had an intramedullary high signal intensity (HSI) in the traumatized area on T2-weighted image (T2-WI). In consecutive studies HSI appeared persistently up to the chronic stage in four patients, and a low signal intensity (LSI) appeared a few months after the injury at T1-WI, to this point disclosing the area of iso-signal intensity. The HSI in the remaining eight patients disappeared at least in three months. The former MRI findings might suggest the development of myelomalacia from acute hemorrhagic necrosis while the latter might be edema of the traumatized spinal cord. Nine patients of chronic MRI studies showed myelomalacia in six, syringomyelia in two, and transection of the spinal cord in one patient respectively. Significant correlations between MRI findings and neurological deficits in acute and chronic stage were present. It was concluded that those who had no intramedullary abnormality in MRI showed less severe neurological deficits and better outcomes, while those who had intramedullary abnormality in MRI showed severe neurological deficits and poor outcomes. (author)

  7. Visibility of the central canal on MRI

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    Petit-Lacour, M.C.; Lasjaunias, P.; Iffenecker, C.; Benoudiba, F.; Hadj Rabia, M.; Doyon, D. [Service de Neuroradiologie, Faculte de Paris Sud (France); Hurth, M. [Department of Neurosurgery, Faculte Paris Sud, Kremlin-Bicetre (France)

    2000-10-01

    The central canal of the spinal cord is present at birth and becomes progressively obliterated. Cadaver studies have shown that it may persiste partially or completely. To our knowledge, this entity has not been described on MRI. We reviewed 794 MRI studies of the spinal cord, and found 12 patients (aged 14 to 65 years) who had an intramedullary cavity. The cavity was at the junction of the ventral {sup 1}/{sub 3} and dorsal {sup 2}/{sub 3} of the spinal cord, except at the level of the lumbar enlargement, where it was central. It was filiform in most cases, although sometimes fusiform (3 to 4 mm in diameter), and had regular contours. The cavity were thoracic in 69 % of cases. The clinical features were totally unrelated to the image, and there were no anatomical factors (Chiari malformation, dysraphism) predisposing to syringomyelia. The images were perfectly compatible with a persistent central canal, which we interpret as a variant of normal anatomy. Therefore it is important to regard these findings as normal, to avoid unnecessary treatment and follow-up. (orig.)

  8. Visibility of the central canal on MRI

    International Nuclear Information System (INIS)

    Petit-Lacour, M.C.; Lasjaunias, P.; Iffenecker, C.; Benoudiba, F.; Hadj Rabia, M.; Doyon, D.; Hurth, M.

    2000-01-01

    The central canal of the spinal cord is present at birth and becomes progressively obliterated. Cadaver studies have shown that it may persiste partially or completely. To our knowledge, this entity has not been described on MRI. We reviewed 794 MRI studies of the spinal cord, and found 12 patients (aged 14 to 65 years) who had an intramedullary cavity. The cavity was at the junction of the ventral 1 / 3 and dorsal 2 / 3 of the spinal cord, except at the level of the lumbar enlargement, where it was central. It was filiform in most cases, although sometimes fusiform (3 to 4 mm in diameter), and had regular contours. The cavity were thoracic in 69 % of cases. The clinical features were totally unrelated to the image, and there were no anatomical factors (Chiari malformation, dysraphism) predisposing to syringomyelia. The images were perfectly compatible with a persistent central canal, which we interpret as a variant of normal anatomy. Therefore it is important to regard these findings as normal, to avoid unnecessary treatment and follow-up. (orig.)

  9. Habitual biting of a finger in a child

    Directory of Open Access Journals (Sweden)

    K N Sarveswari

    2017-01-01

    Full Text Available A 3-year-old male child was brought by his parents with a nonhealing ulcer on the right middle finger having no significant history except for an injury sustained to the right elbow in December 2013. On further probing, the mother revealed that the child used to indulge in habitual biting of his right middle finger while watching TV. Initially he was investigated extensively by a vascular surgeon and no abnormality was detected. He was later referred to the dermatology department and on examination, the patient was attentive with normal behaviour. The right upper limb was slightly larger than left. There was no deformity of the right elbow. The right third fingertip was enlarged and mutilated. There was no nerve thickening or hypopigmented patch. There was loss of sensation on the right hand and arm. Differential diagnosis of Lesch–Nyhan syndrome and congenital sensory neuropathy were considered. The patient was referred to a neurologist who investigated further with magnetic resonance imaging (MRI, and the final diagnosis of syringomyelia was made based on MRI findings.

  10. CT diagnosis in the evaluation of vertebral trauma

    International Nuclear Information System (INIS)

    Emori, Takumi; Kadoya, Satoru; Nakamura, Tsutomu; Ito, Shotaro; Kwak, Ryungchan

    1984-01-01

    The diagnostic capability of the CT scan of the vertebral trauma and a comparison with the results of a routine roentgenogram and tomogram were studied in 11 patients. In total, there were 15 fractured vertebrae: 3 in the upper cervical, 3 in the lower cervical, and 9 in the thoracic and thoraco-lumbar vertebrae. In the detailed evaluation of the vertebral fractures, CT provided more information than plain films in all 15 fractured vertebrae, with a better visualization of the spinal bony details, particularly at the upper cervical, thoracic, and thoraco-lumbar levels, where the interpretation of the spinal abnormalities is usually difficult because of adjacent structures such as the skull and thorax. Only CT was able to demonstrate impingements on the vertebral canal by bony fragments. Post-traumatic syringomyelia was incidentally demonstrated in one patient on a plain CT. In 6 patients, conventional tomography was done, but no additional information with regard to spinal instability and spinal-cord compression was obtained. The usage of sagittal tomography was also limited, because it required a change in the patient's position, which might worsen the neurological deficits. On the other hand, a plain roentgenogram and conventional tomography were superior in the evaluation of spinal malalignment and fractures running horizontally. In summary, both plain roentgenograms and CT images provided detailed information about vertebral injury, whereas conventional tomography is judged to be inferior and not always necessary. Based on these results, our new diagnostic and therapeutic approaches using CT for the vertebral injuries were presented. (author)

  11. The advantages of computed tomography over conventional x-ray examination in the treatment of cervical spinal diseases

    International Nuclear Information System (INIS)

    Shin, Hideo; Yamaura, Akira; Makino, Hiroyasu

    1982-01-01

    Computed tomography (CT) of the cervical spinal column was carried out in 42 patients using a General Electric CT/T of a Toshiba TCT60 Type A scanner. There were 22 cervical disk lesions, 4 spinal neoplasms, 6 narrow spinal canals with or without ossification of posterior longitudinal ligament, 2 syringomyelias, 6 traumas and 2 Arnold-Chiari malformations. In all patients, CT-examination followed conventional spinal X-ray studies. Correlation between the CT and conventional X-ray findings revealed the better diagnostic capability of the CT. For example, the measured midline sagittal diameter of the spinal canal in a patient with the narrowest canal in this series was 7.4 mm on the CT and 9.6 mm on the conventioned plain film at the C 5 level. To know the precise sagittal diameter of the cord itself, CT myelography (CTM) is indispensable. CTM is useful in determining the nature of the disease, the risk and approach of surgery, and for evaluation after the surgical procedure. Although the range of motion of cervical joints and intervertebral foramen are visible with conventional films, the size and extension of a tumor, the degree of bony errosion and the spinal subarachnoid space can be precisely identified only by CT. CT study of the spine and spinal cord is a simple procedure and less likely to produce complication, even with CTM, although there are certain limitations in the examination which are also presented. (author)

  12. Ogilvie's syndrome following posterior spinal arthrodesis for scoliosis.

    Science.gov (United States)

    Tsirikos, Athanasios I; Sud, Alok

    2013-07-01

    We report Ogilvie's syndrome following posterior spinal arthrodesis on a patient with thoracic and lumbar scoliosis associated with intraspinal anomalies. Postoperative paralytic ileus can commonly complicate scoliosis surgery. Ogilvie's syndrome as a cause of abdominal distension and pain has not been reported following spinal deformity correction and can mimic post-surgical ileus. 12 year old female patient with double thoracic and lumbar scoliosis associated with Arnold-Chiari 1 malformation and syringomyelia. The patient underwent posterior spinal fusion from T4 to L3 with segmental pedicle screw instrumentation and autogenous iliac crest grafting. She developed abdominal distension and pain postoperatively and this deteriorated despite conservative management. Repeat ultrasounds and abdominal computer tomography scans ruled out mechanical obstruction. The clinical presentation and blood parameters excluded toxic megacolon and cecal volvulus. As the symptoms persisted, a laparotomy was performed on postoperative day 16, which demonstrated ragged tears of the colon and cecum. A right hemi-colectomy followed by ileocecal anastomosis was required. The pathological examination of surgical specimens excluded inflammatory bowel disease and vascular abnormalities. The patient made a good recovery following bowel surgery and at latest followup 3.2 years later she had no abdominal complaints and an excellent scoliosis correction. Ogilvie's syndrome should be included in the differential diagnosis of postoperative ileus in patients developing prolonged unexplained abdominal distension and pain after scoliosis correction. Early diagnosis and instigation of conservative management can prevent major morbidity and mortality due to bowel ischemia and perforation.

  13. Percutaneous minimally invasive fetoscopic surgery for spina bifida aperta. Part III: neurosurgical intervention in the first postnatal year.

    Science.gov (United States)

    Graf, K; Kohl, T; Neubauer, B A; Dey, F; Faas, D; Wanis, F A; Reinges, M H T; Uhl, E; Kolodziej, M A

    2016-02-01

    To evaluate the need for postnatal neurosurgical intervention after fetoscopic patch coverage of spina bifida aperta (SBA). This was a retrospective analysis of a cohort of 71 fetuses which underwent minimally invasive fetoscopic patch coverage of SBA between 21 + 0 and 29 + 1 weeks of gestation. Postnatal neurosurgical procedures were classified into two types: re-coverage of the SBA within the first 3 months following birth, and shunt placement as treatment of associated hydrocephalus within the first year. Location of the SBA was lumbosacral in 59 cases, lumbar in seven, thoracic in three and sacral in two. In total, 20/71 (28%) patients underwent early postnatal neurosurgical intervention by means of re-coverage of the SBA. This was performed because of cerebrospinal fluid leakage in seven (35%), adhesions with functional deterioration in three (15%), incomplete coverage in five (25%) and skin defect in five (25%) cases. Ventriculoperitoneal shunt placement within 1 year was required in 32 (45%) cases and was preceded by ventriculostomy in two. Three (4%) infants needed Chiari decompression surgery in the first 12 months following birth, because of syringomyelia or gait disturbance. Fetoscopic patch coverage of SBA may require postnatal re-coverage in some cases. In most cases, conservative wound treatment shows good results, without requiring neurosurgical intervention. The low 1-year-shunt rate is comparable to data of the Management of Myelomeningocele Study and lower compared with published data of patients with postnatal only coverage of SBA. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

  14. September 2012 critical care case of the month

    Directory of Open Access Journals (Sweden)

    Raschke RA

    2012-09-01

    Full Text Available No abstract available. Article truncated at 150 words. History of Present IllnessA 45 year old man was transferred from another medical center. He was found unresponsive, with muscle spasticity. After arrival at the outside medical center his vital signs were temperature 106.4 degrees F, heart rate 160 beats/min, respiratory rate 44 breaths per minute, and BP of 70/45 mm Hg. He was orally intubated for respiratory distress with induced by vecuronium. His white blood cell count was 21,000 cells/μL. Chest x-ray showed bilateral consolidations and he was given fluids and gatifloxacin. His blood pressure improved to 130/94 and he was transferred. PMH, SH, FHHe has a past medical history of quadriplegia at the C6 level with a history of severe back pain because syringomyelia. He has a history of autonomic dysreflexia. Despite his disability he is quite functional working as a personal injury lawyer. He had been managed with a variety of medications including benzodiazepams, narcotics …

  15. Ogilvie′s syndrome following posterior spinal arthrodesis for scoliosis

    Directory of Open Access Journals (Sweden)

    Athanasios I Tsirikos

    2013-01-01

    Full Text Available We report Ogilvie′s syndrome following posterior spinal arthrodesis on a patient with thoracic and lumbar scoliosis associated with intraspinal anomalies. Postoperative paralytic ileus can commonly complicate scoliosis surgery. Ogilvie′s syndrome as a cause of abdominal distension and pain has not been reported following spinal deformity correction and can mimic post-surgical ileus. 12 year old female patient with double thoracic and lumbar scoliosis associated with Arnold-Chiari 1 malformation and syringomyelia. The patient underwent posterior spinal fusion from T 4 to L 3 with segmental pedicle screw instrumentation and autogenous iliac crest grafting. She developed abdominal distension and pain postoperatively and this deteriorated despite conservative management. Repeat ultrasounds and abdominal computer tomography scans ruled out mechanical obstruction. The clinical presentation and blood parameters excluded toxic megacolon and cecal volvulus. As the symptoms persisted, a laparotomy was performed on postoperative day 16, which demonstrated ragged tears of the colon and cecum. A right hemi-colectomy followed by ileocecal anastomosis was required. The pathological examination of surgical specimens excluded inflammatory bowel disease and vascular abnormalities. The patient made a good recovery following bowel surgery and at latest followup 3.2 years later she had no abdominal complaints and an excellent scoliosis correction. Ogilvie′s syndrome should be included in the differential diagnosis of postoperative ileus in patients developing prolonged unexplained abdominal distension and pain after scoliosis correction. Early diagnosis and instigation of conservative management can prevent major morbidity and mortality due to bowel ischemia and perforation.

  16. Unusual Ventilatory Response to Exercise in Patient with Arnold-Chiari Type 1 Malformation after Posterior Fossa Decompression.

    Science.gov (United States)

    Smith, Keely; Gomez-Rubio, Ana M; Harris, Tomika S; Brooks, Lauren E; Mosquera, Ricardo A

    2016-01-01

    We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship. No new pathology of the brainstem was discovered by MRI or neurological evaluation to explain this phenomenon. The patient was placed on continuous noninvasive open ventilation (NIOV) during the day and CPAP at night for a period of 6 months. His pCO2 level decreased to normal limits and his symptoms improved; specifically, he experienced less headaches and fatigue during exercise. In this report, we describe the abnormal response to exercise that patients with AC-Type 1 could potentially experience, even after decompression, characterized by the impairment of ventilator response to hypercapnia during exertion, reflecting a complete loss of chemical influence on breathing with no evidence of abnormality in the corticospinal pathway.

  17. Acromesomelic dysplasia (Marotaeux type associated with craniovertebral junction anomaly: A report of a rare case and review of literature

    Directory of Open Access Journals (Sweden)

    Jayesh C Sardhara

    2014-01-01

    Full Text Available Acromesomelic dysplasia Maroteaux type (AMDM is a rare autosomal recessive osteochondrodysplasia. The responsible gene, AMDM gene, in human beings, has been mapped on 9p13-q12 chromosome by homozygous mapping and pathogenic mutation was later identified in natriuretic receptor B (NPR-B which has been implicated in the regulation of skeletal growth. Till now, around 40 to 50 cases of AMDM have been described in the world literature. Association of the congenital craniovertebral (CV junction anomaly has not been reported. Here we are presenting a case of AMDM, with CV junction anomaly. A 10-year boy presented with short stature (122 cm with short distal limbs, symptomatic for thoracic kyphoscoliosis with back pain. On examination there were no neurological deficits. On radiological investigation, he was found to have short and broad phalanges and toes, thoracic kyphoscoliosis, abnormal pelvic ring, mild ventriculomegaly, cervical syringomyelia and tonsillar descent below foramen magnum, hydrocephalus, os odontoideum with Klippel-Feil anomaly. This was diagnosed as AMDM with congenital os odontoideum, Klippel-Feil anomaly with Arnold-Chiari malformation (ACM type-1. The patient underwent posterior fossa decompression by removal of foramen magnum ring along with C1 arch for ACM type-1. Kyphosis was left for conservative treatment till further observation and required orthopedic correction in his further age. To the best of our knowledge this is a very rare entity of AMDM with congenital CV junction anomaly.

  18. Arnold-Chiari 1 malformation type 1 with syringohydromyelia presenting as acute tetraparesis: a case report.

    Science.gov (United States)

    Schneider, Byron; Birthi, Pravardhan; Salles, Sara

    2013-03-01

    A 19-year-old woman who presented to a community hospital after awakening with tetraparesis, generalized paresthesia, and severe neck pain, and was transferred to an acute care hospital. Magnetic resonance imaging of the head and spine was performed and revealed a cystic lesion extending from the C1 level to the C6 level as well as an Arnold-Chiari type 1 malformation. Emergent surgical posterior fossa decompression with duraplasty and C1 laminectomy was undertaken. Most symptoms improved immediately postoperatively. On post-operative day 15, the patient was transferred to our acute rehabilitation hospital for an additional 16 days. With continued aggressive therapy, she demonstrated complete resolution of tetraparesis as well as significant improvement in muscle strength and function in addition to resolution of paresthesia and neck pain. Functional independence measure scores were 69/126 on admission to 110/126 on discharge from the rehabilitation hospital. Her tetraparesis eventually resolved; manual muscle testing scores on follow-up 2 months later were 5/5 in all four extremities. This is the first reported case of Chiari I malformation with syringohydromyelia presenting as acute tetraparesis, generalized paresthesia, and neck pain. Surgical decompression leading to resolution of symptoms made other etiologies extremely unlikely and there was no history of trauma. The different theories on the pathogenesis of syringomyelia are discussed.

  19. Advantages of computed tomography over conventional x-ray examination in the treatment of cervical spinal diseases

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hideo; Yamaura, Akira; Makino, Hiroyasu (Chiba Univ. (Japan). School of Medicine)

    1982-07-01

    Computed tomography (CT) of the cervical spinal column was carried out in 42 patients using a General Electric CT/T of a Toshiba TCT60 Type A scanner. There were 22 cervical disk lesions, 4 spinal neoplasms, 6 narrow spinal canals with or without ossification of posterior longitudinal ligament, 2 syringomyelias, 6 traumas and 2 Arnold-Chiari malformations. In all patients, CT-examination followed conventional spinal X-ray studies. Correlation between the CT and conventional X-ray findings revealed the better diagnostic capability of the CT. For example, the measured midline sagittal diameter of the spinal canal in a patient with the narrowest canal in this series was 7.4 mm on the CT and 9.6 mm on the conventioned plain film at the C/sub 5/ level. To know the precise sagittal diameter of the cord itself, CT myelography (CTM) is indispensable. CTM is useful in determining the nature of the disease, the risk and approach of surgery, and for evaluation after the surgical procedure. Although the range of motion of cervical joints and intervertebral foramen are visible with conventional films, the size and extension of a tumor, the degree of bony errosion and the spinal subarachnoid space can be precisely identified only by CT. CT study of the spine and spinal cord is a simple procedure and less likely to produce complication, even with CTM, although there are certain limitations in the examination which are also presented.

  20. Arnold–Chiari malformation type 1 complicated by sudden onset anterior spinal artery thrombosis, tetraparesis and respiratory arrest

    Science.gov (United States)

    Wani, Abdul Majid; Zayyani, Najah R; Al Miamini, Wail; Khoujah, Amer M; Alharbi, Zeyad; Diari, Mohd S

    2011-01-01

    Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari’s types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold–Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold–Chiari malformation without any of the above predisposing factors. PMID:22701030

  1. Arnold-Chiari malformation type 1 complicated by sudden onset anterior spinal artery thrombosis, tetraparesis and respiratory arrest.

    Science.gov (United States)

    Wani, Abdul Majid; Zayyani, Najah R; Al Miamini, Wail; Khoujah, Amer M; Alharbi, Zeyad; Diari, Mohd S

    2011-04-15

    Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari's types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold-Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold-Chiari malformation without any of the above predisposing factors.

  2. Clinical and MR findings of tethered cord syndrome

    International Nuclear Information System (INIS)

    Chung, Ho In; Kim, Hyae Young; Chung, Eun Chul; Suh, Jeong Soo; Lim, Hyo Keun; Lee, Seoung Ro; Lee, Young Seok

    1994-01-01

    Tethered cord syndrome(TCS)is defined as low position of the conus medullaris by the abnormally fixed spinal cord with progressive neurologic deficit. To evaluate the findings of TCS at MRI and its diagnostic value, we performed a retrospective analysis of MRI of 30 patients with emphasis on clinical manifestation, level of conus medullaris, cause of tethering, and associated findings. Clinical presentation included back mass(26 cases), neurogenic bladder(5 cases), urinary incontinences(5 cases), progressive constipation(2 cases), skin dimpling(1 case), gait disturbance(1 case) and club foot (1 case). Neurologic deficit was developed 11 cases(40%) and mean age of these patients at the time of diagnosis was 8.6 years. The most common cause of tethering was lipoma(63%). The tips of conus medullaris were below the level of the second lumbar spine ia all patients. The causes of tethering were lipomatous components(spinal lipoma and lipomyelomenigocele) in 67% myelomeningocele in 20%, presacral mass in 7%, thickened filum terminale in 3% and postoperative change in 3%. Associated anomalies included syringomyelia(20%) and hydrocephalus was associated in 3 out 5 patients who underwent brain MRI. MRI clearly delineated the location of conus, tethering of the filum terminate with their causes and associated abnormalities. MRI examination is a very useful diagnostic tool for the early evaluation of TCS and the postoperative follow up

  3. Expanding the phenotype of DST-related disorder: A case report suggesting a genotype/phenotype correlation.

    Science.gov (United States)

    Cappuccio, Gerarda; Pinelli, Michele; Torella, Annalaura; Alagia, Marianna; Auricchio, Renata; Staiano, Annamaria; Nigro, Vincenzo; Brunetti-Pierri, Nicola

    2017-10-01

    The gene DST encodes for the large protein BPAG1 involved in hemidesmosomes. Its alternative splicing gives rise to tissue-enriched isoforms in brain, muscle, and skin. The few patients described so far with bi-allelic mutations in the DST gene have either a skin phenotype of epidermolysis bullosa simplex or a neurological phenotype. Here, we report a 17-year-old female individual presenting with a more complex phenotype consisting of both skin and neuronal involvement, in addition to several previously unreported findings, such as iris heterochromia, cataract, hearing impairment, syringomyelia, behavioral, and gastrointestinal issues, osteoporosis, and growth hormone deficiency. Family-trio whole exome sequencing revealed that she was a compound heterozygous for two variants in the DST gene with highly-predicted functional impact, c.3886A>G (p.R1296X) in exon 29 and c.806C>T (p.H269R) in exon 7. Interestingly, exon 7 is included in the neuronal isoform whereas exon 29 is expressed in both skin and neuronal isoforms. The patient we described is the first case with a mutation affecting an exon expressed in both the neuronal and skin isoforms that can explain the more complex phenotype compared to previously reported cases. © 2017 Wiley Periodicals, Inc.

  4. Magnetic resonance imaging in neurologic diseases

    International Nuclear Information System (INIS)

    Chang, Kee Hyun; Han, Man Chung; Wan, Chu Wan; Myung, Ho Jin; Choi, Kil Soo; Ahn, Chang Beom; Oh, Chang Hyun; Cho, Zang Hee

    1985-01-01

    Magnetic resonance (MR) imaging with 0.15 Tesla resistive magnet developed by Korea Advanced Institute of Science were performed in 27 patients with various neurologic diseases and compared with x-ray computed tomography (CT). The purpose of the paper is to evaluate the image quality, the diagnostic value and limitation, and the optimal pulse sequence of MR imagings with a resistive magnet. The MR images were obtained by using a variety of pulse sequence with spin echo technique including saturation recovery. T2-weighted spin echo, and/or inversion recovery with various pulse repetition (TR) and echo delay (TE) times. The MR imaging demonstrated the capability of detecting the lesions shown on CT in al cases and also detected an additional finding in one case (multiple sclerosis) which was not seen on CT. The MR imaging appeared to be more useful than CT in the evaluation of syringomyelia of spinal cord and white matter disease, while it failed to demonstrated small calcific lesion or inflammatory nodule (less than 1 cm) shown on CT and has shown somewhat poor contrast resolution in the case of meingloma. The spatial resolution of saturation recovery images was similar or superior to CT, whereas the contrast resolution of saturation recovery was inferior to CT. While the saturation recovery images have shown false negative findings in 5 patients (19%), the inversion recovery and T2-weighted spin echo have shown consistently positive findings. The inversive recovery and T2-weighted spin echo images demonstrated better contrast discrimination between normal and pathologic conditions than the saturation recovery images, but somewhat poorer spatial resolution. Authors suggest that the MR images of both the saturation recovery with 300/30 and T2-weighted spin echo with 1000/90 be used as a routine procedure and additional inversion recovery of 1300/300/30 sequence as a option if white matter disease is suspected

  5. Georges Marinesco (1863-1938): neurologist, neurohistologist and neuropathologist.

    Science.gov (United States)

    Catala, M; Poirier, J

    2012-01-01

    Gheorghe Marinescu (Georges Marinesco, in French) is a Romanian physician, founder of the School of Neurology in that country. He begins his medical studies in Bucharest, then has the opportunity to reach Paris and join the School of Neurology in La Salpêtrière Hospital, lead by Jean-Martin Charcot. This trip will forever imprint the mind of Marinescu, a great friend of France, a respectful student of Charcot and a friendly colleague of many Parisian neurologists. Marinescu's works are multiple and very important. He describes the succulent hand in syringomyelia and the palmar-jaw reflex. Marinescu is also one of the first to use the cinema for medical purposes. His work as an anatomo-clinician, a method developed by Charcot, is important. We denote the description of the locus niger affected by tuberculosis in a case of parkinsonism (this description paving Etienne Brissaud's way to highlight the anatomical origin of Parkinson's disease), the original clinical description of Marinesco-Sjögren syndrome, and that of medullomyoblastoma. Marinescu is also a famous neurocytologist as evidenced by his work, La Cellule Nerveuse, published in 1909. The first volume of the book is devoted to the aspects of the normal nervous tissue: the neurofibrillar network, the chromatophilic elements, and the paranucleolar corpuscles (now known as Marinescu's bodies). The second volume of the book is almost related to features revealed by experimental lesions: chromatolysis and neuronophagia. Furthermore, Marinescu describes with Oscar Blocq, small nodules than are now regarded as the first description of senile plaques.

  6. MRI characteristics of spinal ependymoma in WHO grade II: a review of 59 cases.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Kato, Fumihiko; Kanemura, Tokumi; Sato, Koji; Kamiya, Mitsuhiro; Ando, Kei; Ito, Kenyu; Tsushima, Mikito; Matsumoto, Akiyuki; Morozumi, Masayoshi; Tanaka, Satoshi; Machino, Masaaki; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-11-20

    Retrospective multicenter study. The goal of this study is to determine the characteristic imaging features of spinal ependymoma in a review of MRI data for a large series of surgically proven cases. Common spinal intramedullary neoplasms are mostly ependymomas and comprise 50-60% of spinal neuroepithelial tumors in adults. Preoperative prediction of the pathological diagnosis could enhance surgical planning and explanation of the procedure to patients. However, these types of tumors exhibit a variety of MRI findings. Records were examined for 59 patients who underwent surgery for spinal cord ependymoma and had a pathological diagnosis of cellular ependymoma of WHO classification grade II. The ependymomas included 28 in the cervical spine, 34 in the thoracic spine, and 3 conus lesions. All cases were isointense or hypointense on T1-weighted MRI, and 55 (93%) were hyperintense on T2-weighted MRI. Tumors were located centrally in all cases; 50 (85%) showed surrounding cord edema; and 52 (88%) had associated cysts, including 36 (61%) rostral or caudal cysts, 10 (17%) intratumoral cysts, and 6 (10%) with syringomyelia. Of the 59 tumors, 17 (29%) showed the "cap sign", a rim of extreme hypointensity seen around the tumor on T2-weighted images, due to hemosiderin. In gadolinium-enhanced MRI, all cases were enhanced, and 27 (46%), 16 (27%), 11 (19%), and 5 (8%) cases showed homogeneous, heterogeneous, rim and nodular enhancement, respectively. Hypointense changes on T2-weighted MRI and hemosiderin deposition reflect easy bleeding. Tumors are associated with various types of cysts, and gadolinium-enhancement patterns reflect a variety of intratumor cellular components. In cases in which the whole tumor shows gadolinium enhancement on MRI, rostral or caudal cyst and a cap sign with hemorrhage are characteristics of grade II classical ependymoma. 3.

  7. Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology

    Energy Technology Data Exchange (ETDEWEB)

    Thomas, B.; Krishnamoorthy, T.; Kesavadas, C. [Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Imaging Sciences and Interventional Radiology, Kerala (India); Radhakrishnan, V.V. [Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Pathology, Kerala (India)

    2007-09-15

    Lhermitte-Duclos disease (LDD, dysplastic gangliocytoma) is an extremely rare cerebellar lesion of uncertain etiology. The debate as to whether it constitutes a neoplastic, malformative, or hamartomatous lesion is still continuing. In this report we explore the usefulness of susceptibility-weighted imaging (SWI), diffusion weighted imaging (DWI), perfusion imaging, and chemical shift imaging (CSI) in demonstrating the pathology and pathophysiology in two patients with LDD. MR imaging of the brain and the cervicodorsal spine was performed on a 1.5-T scanner in a 47-year-old woman presenting with numbness and paresthesia of both upper and lower limbs, and in a 17-year-old male with right frontal headache associated with neck pain. Routine imaging in the first patient showed a left-side cerebellar mass with characteristic 'tiger-striped' thick folia associated with Chiari I malformation, tonsillar herniation and cervicodorsal syringomyelia and in the second patient a right cerebellar mass with similar findings. The SWI demonstrated the characteristic deep running veins between the folia, which is thought to be the cause for vascular contrast enhancement. Diffusion showed a T2 shine-through effect with mild increased diffusivity, and perfusion showed increase in relative cerebral blood volume, relative cerebral blood flow, and mean transit time in the lesion. MR spectroscopy demonstrated reduction in metabolites and a prominent lactate peak in both the patients. The pathological and pathophysiological significance of these findings is discussed. MRI with the newer imaging capabilities can demonstrate the pathology and pathophysiology in Lhermitte-Duclos disease better. SWI helps in detecting the veins around the thickened folia. (orig.)

  8. Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology

    International Nuclear Information System (INIS)

    Thomas, B.; Krishnamoorthy, T.; Kesavadas, C.; Radhakrishnan, V.V.

    2007-01-01

    Lhermitte-Duclos disease (LDD, dysplastic gangliocytoma) is an extremely rare cerebellar lesion of uncertain etiology. The debate as to whether it constitutes a neoplastic, malformative, or hamartomatous lesion is still continuing. In this report we explore the usefulness of susceptibility-weighted imaging (SWI), diffusion weighted imaging (DWI), perfusion imaging, and chemical shift imaging (CSI) in demonstrating the pathology and pathophysiology in two patients with LDD. MR imaging of the brain and the cervicodorsal spine was performed on a 1.5-T scanner in a 47-year-old woman presenting with numbness and paresthesia of both upper and lower limbs, and in a 17-year-old male with right frontal headache associated with neck pain. Routine imaging in the first patient showed a left-side cerebellar mass with characteristic 'tiger-striped' thick folia associated with Chiari I malformation, tonsillar herniation and cervicodorsal syringomyelia and in the second patient a right cerebellar mass with similar findings. The SWI demonstrated the characteristic deep running veins between the folia, which is thought to be the cause for vascular contrast enhancement. Diffusion showed a T2 shine-through effect with mild increased diffusivity, and perfusion showed increase in relative cerebral blood volume, relative cerebral blood flow, and mean transit time in the lesion. MR spectroscopy demonstrated reduction in metabolites and a prominent lactate peak in both the patients. The pathological and pathophysiological significance of these findings is discussed. MRI with the newer imaging capabilities can demonstrate the pathology and pathophysiology in Lhermitte-Duclos disease better. SWI helps in detecting the veins around the thickened folia. (orig.)

  9. Pattern of spinal compression (retrospective and prospective clinical study)

    International Nuclear Information System (INIS)

    Ahmed, Abubakr Darrag Salim

    1997-04-01

    Seventy two patients with spinal cord compressions were admitted to the national centre for neurological sciences in the period between january 1995 and december 1996. Male female ratio was 2.5:1 and the mean age was 40.5 years, myelogram was found to be the most helpful investigation in (90.3%) of patients and plain x-ray was abnormal in (43%) of patients. Tumors were found as the cause of compression in (26.4%), disc prolapse in (26.4%) of patients, spinal injuries in 13.8%, arachnoid cysts in (8.3%) of patients and tuberculosis in (8.3%). Other causes like spinal osteopathy, syringomyelia, spinal hematomas, spinal canal stenosis and spinal haemangiomas were also encountered. Thick ligamentum flavum was found in (25%) of cases, mostly in association with other pathologies, and as the sole compressing pathology in only two patients. The patients were followed up for a period from one month to two years, 41.6% of them were cured completely, while 37.5% were partially improved, 5.6% showed no improvement, 5.6% were died, 9.7% lost their follow up. The factors affecting the outcome were found to be, the duration of the condition before presentation for treatment, presence of blocks in myelograms, and the type of the pathology, disc and benign tumors gave the best outcome. Urinary complications like urine incontinence, urinary tract infection, and urine retention, were observed in (26.4%, 30.6%, 11.1%) of patients respectively, D.V.T. occurred in (15.3%) of patients and the mortality rate was (5.6%) and the major cause of death was pulmonary embolism. (Author)

  10. The impact of spinal cord nerve roots and denticulate ligaments on cerebrospinal fluid dynamics in the cervical spine.

    Science.gov (United States)

    Heidari Pahlavian, Soroush; Yiallourou, Theresia; Tubbs, R Shane; Bunck, Alexander C; Loth, Francis; Goodin, Mark; Raisee, Mehrdad; Martin, Bryn A

    2014-01-01

    Cerebrospinal fluid (CSF) dynamics in the spinal subarachnoid space (SSS) have been thought to play an important pathophysiological role in syringomyelia, Chiari I malformation (CM), and a role in intrathecal drug delivery. Yet, the impact that fine anatomical structures, including nerve roots and denticulate ligaments (NRDL), have on SSS CSF dynamics is not clear. In the present study we assessed the impact of NRDL on CSF dynamics in the cervical SSS. The 3D geometry of the cervical SSS was reconstructed based on manual segmentation of MRI images of a healthy volunteer and a patient with CM. Idealized NRDL were designed and added to each of the geometries based on in vivo measurments in the literature and confirmation by a neuroanatomist. CFD simulations were performed for the healthy and patient case with and without NRDL included. Our results showed that the NRDL had an important impact on CSF dynamics in terms of velocity field and flow patterns. However, pressure distribution was not altered greatly although the NRDL cases required a larger pressure gradient to maintain the same flow. Also, the NRDL did not alter CSF dynamics to a great degree in the SSS from the foramen magnum to the C1 level for the healthy subject and CM patient with mild tonsillar herniation (∼ 6 mm). Overall, the NRDL increased fluid mixing phenomena and resulted in a more complex flow field. Comparison of the streamlines of CSF flow revealed that the presence of NRDL lead to the formation of vortical structures and remarkably increased the local mixing of the CSF throughout the SSS.

  11. Giant encephalocele: a study of 14 patients.

    Science.gov (United States)

    Mahapatra, A K

    2011-01-01

    Giant encephalocele is a rare condition and few published reports are available in the English literature. It is a challenge to neurosurgeons, even today. This series consists of 14 patients with giant encephaloceles treated at our institute. Over a period of 8 years, from 2002 to 2009, 110 patients with encephaloceles were managed at our institute. Amongst them, 14 were children with giant encephaloceles. All patients had CT/MRI or both prior to surgery, and all were operated upon. Four patients were neonates, under 1 month of age, and 9/14 patients (64%) were under 3 months. The youngest child was a newborn baby aged 2 days. Except for 1 with an anterior encephalocele, the rest were patients with occipital encephaloceles. A CT scan was performed on 5 and an MRI on 1 patient. Both CT and MRI scans were performed on the other 8 patients. MRI/CT showed hydrocephalus in 10/14 patients. Of these, 7 required ventriculoperitoneal (VP) shunt, and the remaining 3 with mild to moderate hydrocephalus did not. Of the 7 patients who underwent VP shunt, 5 had a shunt during the encephalocele repair and 2 had a postoperative shunt for increasing hydrocephalus. Other associated anomalies recorded were acquired Chiari malformation in 3 patients, secondary craniostenosis with microcephaly in 5, and syringomyelia in 1 patient. All the patients underwent repair of encephalocele and 4 had suturectomy of coronal suture for the secondary craniostenosis. There were 2 postoperative deaths due to hypothermia. Among the 12 surviving patients, 9 had a good outcome and 3 had poor mental development. The present study shows overall good outcomes in 9/14 (66%) patients. Copyright © 2012 S. Karger AG, Basel.

  12. Endoscopic third ventriculostomy

    Directory of Open Access Journals (Sweden)

    Yad Ram Yadav

    2012-01-01

    Full Text Available Endoscopic third ventriculostomy (ETV is considered as a treatment of choice for obstructive hydrocephalus. It is indicated in hydrocephalus secondary to congenital aqueductal stenosis, posterior third ventricle tumor, cerebellar infarct, Dandy-Walker malformation, vein of Galen aneurism, syringomyelia with or without Chiari malformation type I, intraventricular hematoma, post infective, normal pressure hydrocephalus, myelomeningocele, multiloculated hydrocephalus, encephalocele, posterior fossa tumor and craniosynostosis. It is also indicated in block shunt or slit ventricle syndrome. Proper Pre-operative imaging for detailed assessment of the posterior communicating arteries distance from mid line, presence or absence of Liliequist membrane or other membranes, located in the prepontine cistern is useful. Measurement of lumbar elastance and resistance can predict patency of cranial subarachnoid space and complex hydrocephalus, which decides an ultimate outcome. Water jet dissection is an effective technique of ETV in thick floor. Ultrasonic contact probe can be useful in selected patients. Intra-operative ventriculo-stomography could help in confirming the adequacy of endoscopic procedure, thereby facilitating the need for shunt. Intraoperative observations of the patent aqueduct and prepontine cistern scarring are predictors of the risk of ETV failure. Such patients may be considered for shunt surgery. Magnetic resonance ventriculography and cine phase contrast magnetic resonance imaging are effective in assessing subarachnoid space and stoma patency after ETV. Proper case selection, post-operative care including monitoring of ICP and need for external ventricular drain, repeated lumbar puncture and CSF drainage, Ommaya reservoir in selected patients could help to increase success rate and reduce complications. Most of the complications develop in an early post-operative, but fatal complications can develop late which indicate an importance of

  13. Interventional management of intractable sympathetically mediated pain by computed tomography-guided catheter implantation for block and neuroablation of the thoracic sympathetic chain: technical approach and review of 322 procedures.

    Science.gov (United States)

    Agarwal-Kozlowski, K; Lorke, D E; Habermann, C R; Schulte am Esch, J; Beck, H

    2011-08-01

    We retrospectively evaluated the safety and efficacy of computed tomography-guided placement of percutaneous catheters in close proximity to the thoracic sympathetic chain by rating pain intensity and systematically reviewing charts and computed tomography scans. Interventions were performed 322 times in 293 patients of mean (SD) age 59.4 (17.0) years, and male to female ratio 105:188, with postherpetic neuralgia (n = 103, 35.1%), various neuralgias (n = 88, 30.0%), complex regional pain syndrome (n = 69, 23.6%), facial pain (n = 17, 5.8%), ischaemic limb pain (n = 7, 2.4%), phantom limb pain (n = 4, 1.4%), pain following cerebrovascular accident (n = 2, 0.7%), syringomyelia (n = 2, 0.7%) and palmar hyperhidrosis (n = 1, 0.3%). The interventions were associated with a total of 23 adverse events (7.1% of all procedures): catheter dislocation (n = 9, 2.8%); increase in pain intensity (n = 8, 2.5%); pneumothorax (n = 3, 0.9%); local infection (n = 2, 0.6%); and puncture of the spinal cord (n = 1, 0.3%). Continuous infusion of 10 ml.h(-1) ropivacaine 0.2% through the catheters decreased median (IQR [range]) pain scores from 8 (6-9 [2-10]) to 2 (1-3 [0-10]) (p < 0.0001). Chemical neuroablation was necessary in 137 patients (46.8%). We conclude that this procedure leads to a significant reduction of pain intensity in otherwise obstinate burning or stabbing pain and is associated with few hazards. © 2011 The Authors. Anaesthesia © 2011 The Association of Anaesthetists of Great Britain and Ireland.

  14. MR imaging of compressive cervical myelopathy after surgery; High signal intensity of the spinal cord on T2 weighted images

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    Mimura, Fumitoshi; Fujiwara, Kazuhisa; Otake, Shoichiro (Tenri Hospital, Nara (Japan)) (and others)

    1990-06-01

    We reviewed the MR images of 32 patients with cervical myelopathy, showing lesions of high signal intensity in the spinal cord on the sagittal T2 weighted images (T2WI) after surgery: 16 with ossification of posterior longitudinal ligament (OPLL); 9 with spondylosis; 4 with disc herniation and 3 with trauma. All images were obtained on a superconducting 1.5 Tesla system. The lesions were classified into five groups, according to the shape and grade of signal intensity on the sagittal T2WI: (I) oval-shaped lesion of signal intensity less brighter than CSF with blurred margin, (II) longitudinal linear-shaped lesion of signal intensity similar to CSF, (III) spindle-shaped lesion of signal intensity similar to CSF, (IV) round-shaped lesion of signal intensity similar to CSF, and (V) mixed-types lesions which consisted of group I and II. The present study was summarized as follows: Oval-shaped lesions were seen in the cases of disc herniation and spondylosis with relatively short duration of the symptom, presumptively with relatively short duration of the symptom, presumptively indicative of edema. Most cases of OPLL and spondylosis showed linear-shaped lesions, suggesting necrosis and/or cavitations of the central gray matter. One case of spondylosis developed a spindle-shaped lesion, implicating syringomyelia. Round-shaped lesions were seen in the cases of spinal trauma, suggesting postraumatic cyst. In a case of mixed-typed lesion examined pre- and postoperatively, only an oval-shaped lesion decreased in size after surgery. (author).

  15. MR imaging of compressive cervical myelopathy after surgery

    International Nuclear Information System (INIS)

    Mimura, Fumitoshi; Fujiwara, Kazuhisa; Otake, Shoichiro

    1990-01-01

    We reviewed the MR images of 32 patients with cervical myelopathy, showing lesions of high signal intensity in the spinal cord on the sagittal T2 weighted images (T2WI) after surgery: 16 with ossification of posterior longitudinal ligament (OPLL); 9 with spondylosis; 4 with disc herniation and 3 with trauma. All images were obtained on a superconducting 1.5 Tesla system. The lesions were classified into five groups, according to the shape and grade of signal intensity on the sagittal T2WI: (I) oval-shaped lesion of signal intensity less brighter than CSF with blurred margin, (II) longitudinal linear-shaped lesion of signal intensity similar to CSF, (III) spindle-shaped lesion of signal intensity similar to CSF, (IV) round-shaped lesion of signal intensity similar to CSF, and (V) mixed-types lesions which consisted of group I and II. The present study was summarized as follows: Oval-shaped lesions were seen in the cases of disc herniation and spondylosis with relatively short duration of the symptom, presumptively with relatively short duration of the symptom, presumptively indicative of edema. Most cases of OPLL and spondylosis showed linear-shaped lesions, suggesting necrosis and/or cavitations of the central gray matter. One case of spondylosis developed a spindle-shaped lesion, implicating syringomyelia. Round-shaped lesions were seen in the cases of spinal trauma, suggesting postraumatic cyst. In a case of mixed-typed lesion examined pre- and postoperatively, only an oval-shaped lesion decreased in size after surgery. (author)

  16. Closing the dura: dural hitching versus surgicel and tisseel overlay graft in craniocervicaldecompression for Chiari 1 malformation.

    Science.gov (United States)

    Tonkins, Michael; Farooqi, Naeem; Ahmed, Rohan; Sinha, Saurabh; Bhattacharyya, Debapriya

    2017-08-01

    This study compares dural hitching to surgicel and tisseel overlay graft following craniocervical decompression and C1 laminectomy with simple durotomy for Chiari I malformation. Outcome measures were syrinx decompression, headache resolution and complication rates. A retrospective analysis of case notes was conducted. Patients who had undergone craniocervical decompression (CCD) were grouped by method of dural closure. Outcomes compared were rates of syrinx decompression, headache resolution, and post-operative complications. Statistical analysis was conducted using SPSS v20. We identified 32 adult patients for inclusion in this study. 53.1% (n = 17) had asyrinx, and 78.1% (n = 25) had a pre-operative headache. All were treated with suboccipital craniectomy, C1 laminectomy (with or without C2 laminectomy), and durotomy. The dura was either left open by dural hitching (n = 23) or closed with surgicel and tisseel overlay graft (n = 9). We found a statistically significant association between the method of dural closure and the rate of syrinx resolution. Resolution occurred in 91.7% (n = 11) of the hitching group, compared to 20.0% (n = 1) of the overlay graft group: Χ 2 (1) = 5.6, p = .018. There were no statistically significant differences between the two groups in the rates of headache resolution or other complications. In patients with symptomatic Chiari I malformation and associated syringomyelia, syrinx resolution is more likely if the dura is hitched open rather than closed bysurgicel and tisseel overlay graft after durotomy.

  17. MRI assessment program. Consensus statement on clinical efficacy of MRI

    International Nuclear Information System (INIS)

    1998-05-01

    This consensus statement is largely based on the experience gained at the MRI units at the four hospitals which have operated scanners in the MRI program. It reflects the considered opinion of the radiologists responsible for the MRI services at those hospitals. Account has also been taken of relevant overseas data. This collection of opinion relates particularly to comparison with other imaging modalities. The specific comments will require further consideration as technical developments with MRI become available, additional experience is gained with gadolinium contrast material and additional data are obtained on the influence of MRI on patient management. MRI, at present, is used either to improve diagnostic accuracy when other tests are negative or equivocal, when there is strong clinical suspicion of disease, or to improve surgical or other management planning when the diagnosis known. In some situations (eg syringomyelia, congenital spinal disease, posterior fossa/cerebello-pontine angle tumours) it may entirely replace other tests (eg myelography, air contrast, CT) which are substantially less accurate and/or more invasive. In other situations (eg hemispheric brain tumours, lumbar disc protrusions) when other tests, such as CT, can be as accurate, MRI is not usually or initially indicated because it is currently more expensive and of limited availability. However, balanced against this is the fact that it does not expose the patient to potentially harmful ionising radiation. It is also stressed that MRI images depend on complex, widely variable and, as yet, incompletely understood parameters. There is concern that this may result in false positive diagnoses, especially where MRI is used alone as a screening test, or used as the initial test. For several reasons (availability, cost, medical and diagnostic efficacy), the specific comments on indications for MRI presented are based upon the assumption that MRI is a tertiary and complementary imaging examination

  18. Proximal tibia reconstruction with a porous tantalum cone in a patient with Charcot arthropathy.

    Science.gov (United States)

    Troyer, Joshua; Levine, Brett R

    2009-05-01

    Neuropathic arthritis, or Charcot arthropathy, is a rapidly destructive process associated with nerve damage and periarticular insensitivity. Most commonly it is associated with diabetes mellitus, tabes dorsalis, syringomyelia, or other peripheral nerve disorders. Clinical manifestations of this disorder classically include gross deformity, crepitus, lack of proprioception, joint effusion, calor, and decreased awareness of pain in the affected joint. Often the clinical picture is difficult to distinguish from septic arthritis. Radiographic findings are typically worse than portrayed during clinical examination. Rapid destruction of the articular surface with bony fragmentation is commonly found. The incidence of neuropathic arthritis among all diabetic patients is relatively small, ranging between 0.15% and 0.4%. Historically, there have been conflicting reports as to the efficacy of total knee arthroplasty (TKA) in patients with neuropathic arthritis. However, recent literature on the use of modern implants has been more optimistic as to the success of TKA. Difficulties associated with severe bone loss, poor bone quality, and ligamentous laxity may necessitate the use of structural allografts/augments, as well as stemmed and/or constrained TKA components. This article presents a case of a patient with Charcot arthropathy of the knee secondary to diabetes mellitus (type 2) treated successfully with a minimally constrained TKA and a porous tantalum cone (Zimmer, Warsaw, Indiana) as an "internal plate," for reconstruction of a combined segmental/cavitary defect of the proximal tibia. At 2-year follow-up, radiographs revealed stable TKA components with reconstitution of the fragmented proximal tibia around an osseointegrated porous cone.

  19. Risk factors for pressure sores in adult patients with myelomeningocele – a questionnaire-based study

    Directory of Open Access Journals (Sweden)

    Frøslie Kathrine

    2006-12-01

    Full Text Available Abstract Background Myelomeningocele (MMC is a part of a complex neural tube defect and a disorder of the cerebrospinal fluid system. Pressure sores are a frequent complication for patients with MMC. Little is known about the risk factors for pressure sores in adults with MMC. The aim of this study was to investigate an association between the presence of pressure sores and other patient characteristics, in order to develop an improved strategy for the management of sores. Methods A structured questionnaire regarding sores, medical condition, function and living factors was designed and sent to the 193 patients with MMC registered in the year 2003 at TRS, a National Centre for Rare Disorders in Norway. Results Out of 193 total, 87 patients participated and 71 patients (82% reported sores; 26 (30% at the time of the interview and 45 (52% during the last 5 years. Sores were mostly localized on toes and feet and occurred exclusively in regions with reduced or missing sensibility. A significant association was found between sores and memory deficit (p = 0.02, Arnold Chiari malformation (p = 0.02 and a record of previous sores (p = 0.004. Sores were not significantly associated with hydrocephalus, syringomyelia, nutrition, body mass index, smoking, physical activity, employment or living together with other persons. Some patients (18, 21% reported skin inspection by others and the remainder relied on self-inspection. Conclusion Patients with sensory deficit, memory problems, and Arnold Chiari malformation had a higher risk of having pressure sores. This patient group needs improved skin inspection routines and sore treatment.

  20. Comparative review of computed tomography of the spinal column and conventional x-ray films

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    Shin, H.; Yamaura, A.; Horie, T.; Makino, H. (Chiba Univ. (Japan). School of Medicine)

    1982-04-01

    Computerized tomography (CT) of the cervical spinal column was carried out in 39 patients using a GE.CT/T or Toshiba TCT60A scanner. There were 22 cervical disk lesions, 4 spinal neoplasms, 5 narrow spinal canals with or without ossification of the posterior longitudinal ligament, 2 syringomyelias, 5 traumas, and one Arnold-Chiari malformation. In all the patients, tomography was done after conventional spinal X-ray studies. The correlation between the CT findings and conventional X-ray films revealed the excellent capability of the CT. The measurement of the midline sagittal diameter of the spinal canal in the patient with the narrowest canal in this series showed 7.4 mm when done by CT and 9.6 mm when done by the conventional plain film at the C/sub 5/ level. To ascertain the precise sagittal diameter of the cord itself, CT myelography is indispensable after the intrathecal injection of metrizamide A; metrizamide CT myelogram is useful in determining the nature of the disease, the risk of and best approach to surgery, and the evaluation after a surgical procedure. Although the range of motion of cervical joints and intervertebral foramen are visible with the conventional films, the size of the spinal tumors, the degree of bony change, and the tumor extension to the paraspinal connective tissue can be precisely demonstrated only by CT. A CT study of the spine is a simple procedure and is less likely to produce complication, even with a metrizamide CT myelogram, though there are certain limitations in the examination.

  1. Quantitative trait loci (QTL study identifies novel genomic regions associated to Chiari-like malformation in Griffon Bruxellois dogs.

    Directory of Open Access Journals (Sweden)

    Philippe Lemay

    Full Text Available Chiari-like malformation (CM is a developmental abnormality of the craniocervical junction that is common in the Griffon Bruxellois (GB breed with an estimated prevalence of 65%. This disease is characterized by overcrowding of the neural parenchyma at the craniocervical junction and disturbance of cerebrospinal fluid (CSF flow. The most common clinical sign is pain either as a direct consequence of CM or neuropathic pain as a consequence of secondary syringomyelia. The etiology of CM remains unknown but genetic factors play an important role. To investigate the genetic complexity of the disease, a quantitative trait locus (QTL approach was adopted. A total of 14 quantitative skull and atlas measurements were taken and were tested for association to CM. Six traits were found to be associated to CM and were subjected to a whole-genome association study using the Illumina canine high density bead chip in 74 GB dogs (50 affected and 24 controls. Linear and mixed regression analyses identified associated single nucleotide polymorphisms (SNPs on 5 Canis Familiaris Autosomes (CFAs: CFA2, CFA9, CFA12, CFA14 and CFA24. A reconstructed haplotype of 0.53 Mb on CFA2 strongly associated to the height of the cranial fossa (diameter F and an haplotype of 2.5 Mb on CFA14 associated to both the height of the rostral part of the caudal cranial fossa (AE and the height of the brain (FG were significantly associated to CM after 10 000 permutations strengthening their candidacy for this disease (P = 0.0421, P = 0.0094 respectively. The CFA2 QTL harbours the Sall-1 gene which is an excellent candidate since its orthologue in humans is mutated in Townes-Brocks syndrome which has previously been associated to Chiari malformation I. Our study demonstrates the implication of multiple traits in the etiology of CM and has successfully identified two new QTL associated to CM and a potential candidate gene.

  2. Amelioration of motor/sensory dysfunction and spasticity in a rat model of acute lumbar spinal cord injury by human neural stem cell transplantation

    Science.gov (United States)

    2013-01-01

    Introduction Intraspinal grafting of human neural stem cells represents a promising approach to promote recovery of function after spinal trauma. Such a treatment may serve to: I) provide trophic support to improve survival of host neurons; II) improve the structural integrity of the spinal parenchyma by reducing syringomyelia and scarring in trauma-injured regions; and III) provide neuronal populations to potentially form relays with host axons, segmental interneurons, and/or α-motoneurons. Here we characterized the effect of intraspinal grafting of clinical grade human fetal spinal cord-derived neural stem cells (HSSC) on the recovery of neurological function in a rat model of acute lumbar (L3) compression injury. Methods Three-month-old female Sprague–Dawley rats received L3 spinal compression injury. Three days post-injury, animals were randomized and received intraspinal injections of either HSSC, media-only, or no injections. All animals were immunosuppressed with tacrolimus, mycophenolate mofetil, and methylprednisolone acetate from the day of cell grafting and survived for eight weeks. Motor and sensory dysfunction were periodically assessed using open field locomotion scoring, thermal/tactile pain/escape thresholds and myogenic motor evoked potentials. The presence of spasticity was measured by gastrocnemius muscle resistance and electromyography response during computer-controlled ankle rotation. At the end-point, gait (CatWalk), ladder climbing, and single frame analyses were also assessed. Syrinx size, spinal cord dimensions, and extent of scarring were measured by magnetic resonance imaging. Differentiation and integration of grafted cells in the host tissue were validated with immunofluorescence staining using human-specific antibodies. Results Intraspinal grafting of HSSC led to a progressive and significant improvement in lower extremity paw placement, amelioration of spasticity, and normalization in thermal and tactile pain/escape thresholds at

  3. Radiographic characteristics in congenital scoliosis associated with split cord malformation: a retrospective study of 266 surgical cases.

    Science.gov (United States)

    Feng, Fan; Tan, Haining; Li, Xingye; Chen, Chong; Li, Zheng; Zhang, Jianguo; Shen, Jianxiong

    2017-10-23

    Vertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM). A total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified. The demographic distribution and radiographic data were collected to investigate the characteristics of spine curve, vertebral, rib, and intraspinal anomalies. According to Pang's classification, all patients were divided into two groups: type I group is defined as two hemicords, each within a separate dural tube separated by a bony or cartilaginous medial spur, while type II group is defined as two hemicords within a single dural tube separated by a nonrigid fibrous septum. There were 104 patients (39.1%) in Type I group and 162 patients (60.9%) in Type II group. SCM was most commonly found in the lower thoracic and lumbar regions. The mean length of the septum in Type I SCM was significantly shorter than Type II SCM (2.7 vs. 5.2 segments). Patients in Type I group had a higher proportion of kyphotic deformity (22.1%). The vertebral deformities were simple in only 16.5% and multiple in 83.5% of 266 cases. Patients in Type I group presented higher prevalence of multiple (90.4%) and extensive (5.1 segments) malformation of vertebrae. In addition, hypertrophic lamina and bulbous spinous processes were more frequent in Type I group (29.7%), even developing into the "volcano-shape" deformities. Rib anomalies occurred in 62.8% of all patients and 46.1% of them were complex anomalies. The overall prevalence of other intraspinal anomalies was 42.9%. The most common coexisting intraspinal anomalies was syringomyelia (30.5%). The current study, with the largest cohort to date, demonstrated that

  4. Logistic regression analysis of risk factors for postoperative recurrence of spinal tumors and analysis of prognostic factors.

    Science.gov (United States)

    Zhang, Shanyong; Yang, Lili; Peng, Chuangang; Wu, Minfei

    2018-02-01

    The aim of the present study was to investigate the risk factors for postoperative recurrence of spinal tumors by logistic regression analysis and analysis of prognostic factors. In total, 77 male and 48 female patients with spinal tumor were selected in our hospital from January, 2010 to December, 2015 and divided into the benign (n=76) and malignant groups (n=49). All the patients underwent microsurgical resection of spinal tumors and were reviewed regularly 3 months after operation. The McCormick grading system was used to evaluate the postoperative spinal cord function. Data were subjected to statistical analysis. Of the 125 cases, 63 cases showed improvement after operation, 50 cases were stable, and deterioration was found in 12 cases. The improvement rate of patients with cervical spine tumor, which reached 56.3%, was the highest. Fifty-two cases of sensory disturbance, 34 cases of pain, 30 cases of inability to exercise, 26 cases of ataxia, and 12 cases of sphincter disorders were found after operation. Seventy-two cases (57.6%) underwent total resection, 18 cases (14.4%) received subtotal resection, 23 cases (18.4%) received partial resection, and 12 cases (9.6%) were only treated with biopsy/decompression. Postoperative recurrence was found in 57 cases (45.6%). The mean recurrence time of patients in the malignant group was 27.49±6.09 months, and the mean recurrence time of patients in the benign group was 40.62±4.34. The results were significantly different (Pfactors showed that total resection should be the preferred treatment for patients with benign tumors, thoracic and lumbosacral tumors, and lower McCormick grade, as well as patients without syringomyelia and intramedullary tumors. Logistic regression analysis of recurrence-related factors revealed that the recurrence rate was relatively higher in patients with malignant, cervical, thoracic and lumbosacral, intramedullary tumors, and higher McCormick grade and patient received partial resection or

  5. Evaluation of patients with headache due to spontaneous intracranial hypotension by RN cisternography

    International Nuclear Information System (INIS)

    Kim, Su Zy; Park, Chan Hee; Soo, Joo In; Pai, Moon Sun; Yoon, Suk Nam; Kim, Jang Sung

    1997-01-01

    Spontaneous intracranial hypotension (SIH) typically occurs without an obvious cause. However, it can be seen following the lumbar puncture, craniotomy, or spinal surgery. Also listed are contributing factors such as sneezing, coughing, intercourse or minor fall. Spontaneous spinal CSF leaks are not common, but are now increasingly recognized as a cause of postural headache associated with intracranial hypotension. The purpose of our study was to evaluate the role of RN cisternography in the diagnosis of SIH cuased by spinal CSF leakage. Four patients with clinical suspicion of SIH (Group I) and six patients as normal control (Group II) underwent RN cisternography. RN cisternography in Group II was done for various reasons, such as hydrocephalus, syringomyelia and memory loss. Group I consisted of the patients who presented with postural headache, as well as additional symptoms, such as nausea, vomiting, dizziness, tinnitus and eyeball pain. The age range of these patients was 27 - 67 years. Lumbar puncture and CSF examinations were performed in Group I more than once and showed typical findings of low CSF pressure and slightly elevated protein level. Brain MRI (4/4), Cervico-thoracic spine MRI (3/4) were also performed. On gadolinium-enhanced brain MRI, enhancement of the meninges which is the most characteristic radiographic finding in intracranial hypotension was found in all patients of Group I. But, cervico-thoracic spin MRI was nonspecific. None of Group I had contrast myelography because of the patient's refusal. Group I and Group II underwent radionuclide cisternography following lumbar subarachnoid injection of 99mTc-DTPA (1-2mCi). The scans were taken in 2, 5, 24 hours later using single head gamma camera equipped with LEAP. Entire spinal region in posterior view and head in frontal and lateral views were obtained. The cisternography of Group I showed the CSF leakage or diverticulum at the level of cervico-thoracic junction(3/4) and mid-thoracic level (1

  6. Evaluation of patients with headache due to spontaneous intracranial hypotension by RN cisternography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Su Zy; Park, Chan Hee; Soo, Joo In; Pai, Moon Sun; Yoon, Suk Nam; Kim, Jang Sung [College of Medicine, Ajou Univ., Suwon (Korea, Republic of)

    1997-07-01

    Spontaneous intracranial hypotension (SIH) typically occurs without an obvious cause. However, it can be seen following the lumbar puncture, craniotomy, or spinal surgery. Also listed are contributing factors such as sneezing, coughing, intercourse or minor fall. Spontaneous spinal CSF leaks are not common, but are now increasingly recognized as a cause of postural headache associated with intracranial hypotension. The purpose of our study was to evaluate the role of RN cisternography in the diagnosis of SIH cuased by spinal CSF leakage. Four patients with clinical suspicion of SIH (Group I) and six patients as normal control (Group II) underwent RN cisternography. RN cisternography in Group II was done for various reasons, such as hydrocephalus, syringomyelia and memory loss. Group I consisted of the patients who presented with postural headache, as well as additional symptoms, such as nausea, vomiting, dizziness, tinnitus and eyeball pain. The age range of these patients was 27 - 67 years. Lumbar puncture and CSF examinations were performed in Group I more than once and showed typical findings of low CSF pressure and slightly elevated protein level. Brain MRI (4/4), Cervico-thoracic spine MRI (3/4) were also performed. On gadolinium-enhanced brain MRI, enhancement of the meninges which is the most characteristic radiographic finding in intracranial hypotension was found in all patients of Group I. But, cervico-thoracic spin MRI was nonspecific. None of Group I had contrast myelography because of the patient's refusal. Group I and Group II underwent radionuclide cisternography following lumbar subarachnoid injection of 99mTc-DTPA (1-2mCi). The scans were taken in 2, 5, 24 hours later using single head gamma camera equipped with LEAP. Entire spinal region in posterior view and head in frontal and lateral views were obtained. The cisternography of Group I showed the CSF leakage or diverticulum at the level of cervico-thoracic junction(3/4) and mid

  7. Analysis and evaluation of the radiological aspects of scoliosis

    International Nuclear Information System (INIS)

    Vargas Gonzalez, Paula

    2012-01-01

    surgical drastic measures, before that the others vital organs such as lungs and abdomen are affected by the curve. The use of specialized imaging is limited in patients with suspected of condition by soft tissues, neurological, suspected of medullary condition, such as syringomyelia, and suspected tumor. The corset is used in scoliosis under 40 degrees, otherwise it is recommended surgical treatment. The success of the use of the King-Lenke classification has proved for surgical management. The surgery is chosen as appropriate, taking into account the type of scoliosis, measurement and classification. The monitoring has been important in the case of patients that have used corset, to prevent progression; as those that have been surgically treated for possible complications [es

  8. Spina bifida occulta: is it a predictor of underlying spinal cord abnormality in patients with lower urinary tract dysfunction?

    Science.gov (United States)

    Nejat, Farideh; Radmanesh, Farid; Ansari, Saeed; Tajik, Parvin; Kajbafzadeh, Abdolmohammad; El Khashab, Mostafa

    2008-02-01

    The purpose of this study was to evaluate the importance of spina bifida occulta in radiographs of children with lower urinary tract or bowel dysfunction. The authors prospectively investigated the presence of spinal cord abnormalities in 176 patients with functional urinary and bowel problems: 88 children with radiographic evidence of spina bifida occulta (SBO) and 88 age-and sex-matched controls. Each group included 46 boys and 42 girls (age range 5-14 years). Nocturnal enuresis, isolated diurnal enuresis, enuresis during both day and night, urinary tract infection, urinary frequency, encopresis, intractable constipation, and vesicoureteral reflux were assessed in all patients. Magnetic resonance (MR) images were obtained in all patients and evaluated for spinal cord abnormalities. Sacral ratios (SRs) were calculated on the basis of plain radiographs. There was no statistically significant difference between the 2 groups on any of the clinical measures. The most common sites of SBO on radiographs were the S-1 (47%) and L-5 and S-1 (25%). The mean SRs (+/-standard deviations) in the SBO and control groups, respectively, were 0.64+/-0.45 and 0.68+/-0.51 (no statistically significant difference). Sacral agenesis was found in 17 children (7 in the SBO group and 10 in the control group, p=0.44). Abnormal MR imaging findings were observed in 9 children (10.22%) in the SBO group and 3 (3.4%) in the control group. Abnormalities included tethered spinal cord in 5 children, syringomyelia in 4, and club-shaped conus in 2. No significant association was found between the presence of SBO and spinal cord abnormalities identified on MR images (p=0.13, paired t-test). Among children with functional bowel and urinary problems, there was no statistically significant difference in the prevalence of abnormal spinal MR imaging findings in those with radiographic SBO and an age- and sex-matched control group. Spina bifida occulta was not shown to be a reliable indicator of spinal cord

  9. The Clinical Features and Surgical Outcomes of Spinal Cord Tanycytic Ependymomas: A Report of 40 Cases.

    Science.gov (United States)

    Tao, Xiaogang; Hou, Zonggang; Hao, Shuyu; Zhang, Qi; Wu, Zhen; Zhang, Junting; Liu, Baiyun

    2017-10-01

    Spinal cord tanycytic ependymomas (TEs) rarely are reported because of extremely low incidence. Understanding of this disease is therefore poor. The aim of this study was to analyze the incidence and clinical, radiologic, pathologic, and prognostic features of spinal cord TEs. Approximately 4000 spinal cord tumors were resected surgically in Beijing Tiantan Hospital between April 2009 and May 2016. We identified all cases of pathologically proved TEs among these patients. TEs accounted for approximately 1% of spinal cord tumors (40 of an estimated 4000). Patients with TE were a mean age of 40.0 years and had no significant sex preference (21 male and 19 female). The median diameter of the maximal tumor was 54.2 mm (range, 16-153 mm). The mean preoperative Japanese Orthopedic Association (JOA) score was 13.0. Radiologically, 47.5% (19/40) cases showed poorly defined tumor border, and 40% (16/40) of them showed preoperative syringomyelia. Gross total resection was achieved in 30 cases, subtotal resection in 8, and partial resection in 2. At the time of discharge, JOA score and neurologic function showed improvement in 26 cases (65%), no change in 12 cases (30%), and worsening in 2 cases (5%). At a median follow-up of 43 months (range, 7-101 months), 1- and 5-year progression-free survival rates were 100.0% and 97.5%, respectively. Only one patient had tumor recurrence which was found 30 months after surgery. No patient died of tumor recurrence. At the latest follow-up, JOA score showed improvement in 30 cases (75%), no change in 8 cases (20%), and worsening in 2 cases (5%). Univariate analysis revealed that tumor size and preoperative JOA score were significantly associated with the short-term outcomes. Meanwhile, age ≥40 years, tumor size ≥50 mm, non-gross total resection, and preoperative JOA score Spinal cord TE is a rare subtype of ependymomas with low recurrence. Long-term survival can be expected, although poorly defined tumor border is an independent

  10. Intraspinal anomalies in scoliosis: An MRI analysis of 177 consecutive scoliosis patients

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    Rajasekaran S

    2010-01-01

    Full Text Available Background : The association of intraspinal neural anomalies with scoliosis is known for more than six decades. However, there are no studies documenting the incidence of association of intraspinal anomalies in scoliotic patients in the Indian population. The guide lines to obtain an magnetic resonance imaging (MRI scan to rule out neuro-axial abnormalities in presumed adolescent idiopathic scoliosis are also not clear. We conducted a prospective study (a to document and analyze the incidence and types of intraspinal anomalies in different types of scoliosis in Indian patients. (b to identify clinico-radiological ′indicators′ that best predict the findings of neuro-axial abnormalities in patients with presumed adolescent idiopathic scoliosis, which will alert the physician to the possible presence of intraspinal anomalies and optimize the use of MRI in this sub group of patients. Materials and Methods : The data from 177 consecutive scoliotic patients aged less than 21 years were analyzed. Patients were categorized into three groups; Group A - congenital scoliosis (n=60, group B -presumed idiopathic scoliosis (n=94 and group C - scoliosis secondary to neurofibromatosis, neuromuscular and connective tissue disorders (n=23. The presence and type of anomaly in the MRI was correlated to patient symptoms, clinical signs and curve characteristics. Results : The incidence of intraspinal anomalies in congenital scoliosis was 35% (21/60, with tethered cord due to filum terminale being the commonest anomaly (10/21. Patients with multiple vertebral anomalies had the highest incidence (48% of neural anomalies and isolated hemi vertebrae had none. In presumed ′idiopathic′ scoliosis patients the incidence was higher (16% than previously reported. Arnold Chiari-I malformation (AC-I with syringomyelia was the most common neural anomaly (9/15 and the incidence was higher in the presence of neurological findings (100%, apical kyphosis (66.6% and early

  11. Spinal cord hemangioblastomas: significance of intraoperative neurophysiological monitoring for resection and long-term outcome.

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    Siller, Sebastian; Szelényi, Andrea; Herlitz, Lisa; Tonn, Joerg Christian; Zausinger, Stefan

    2017-04-01

    OBJECTIVE Spinal cord hemangioblastomas are rare benign tumors developing either sporadically or as part of von Hippel-Lindau (VHL) disease. Generally, resection is the treatment of choice. However, the significance of intraoperative neurophysiological monitoring (IONM) for resection and postoperative outcome is still controversial. The authors analyzed the surgical and clinical courses of patients who had undergone resection of spinal cord hemangioblastoma, with special attention to preoperative imaging, the use of IONM, and short- and long-term outcomes. METHODS A series of 24 patients (male/female 1:1, lesion sporadic/associated with VHL 2.4:1) who had undergone 26 operations for the resection of 27 spinal cord hemangioblastomas was analyzed. All patients had undergone pre- and postoperative contrast-enhanced MRI. In all cases, microsurgical tumor removal had been performed under continuous IONM of both somatosensory and transcranial motor evoked potentials as well as electromyographic recording. Clinical characteristics, imaging findings, and operative records were retrospectively analyzed. Outcome parameters included short- and long-term status as regards sensorimotor deficits and a questionnaire on general performance, patient satisfaction, and Oswestry Disability Index (ODI) at the end of the follow-up period. The impact of IONM findings on postoperative deficits and outcome parameters as well as risk factors affecting functional prognosis was statistically assessed. RESULTS Preoperative symptoms (mean duration 16.2 ± 22.0 months) included sensory changes (100.0%), pain (66.7%), spinal ataxia (66.7%), motor deficit (41.7%), and bladder/bowel dysfunction (12.5%). Average age at the first operation was 36.8 ± 12.8 years. Most tumors (21 intramedullary, 6 intra- and/or extramedullary) were located dorsally (92.6%) and cervically (77.8%) and were accompanied by peritumoral edema and/or syringomyelia (81.5%). Tumor resection was achieved via laminectomy for 15

  12. Contribuição ao estudo das malformações occipito-cervical, particularmente da impressão basilar

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    Horacio M. Canelas

    1952-12-01

    nuclei of oculomotor and facial nerves. In case 4 there were occipitalization of atlas and Chiari deformity. In case 5 vertebral reduction in the cervical region was evidenced but bony coalescence could not be proved. In the last four cases the constitutional morphologic type was determined. Electroencephalograms of cases 3, 4 and 5 showed no abnormalities; in case 1 slow bilateral occipital waves were recorded. The authors call attention to the importance of the diagnosis of these anomalies susceptible of successful surgical correction, which can simulate many nervous diseases of untoward course, like lateral amyotrophic sclerosis, syringomyelia (which can be actually associated to the bony anomaly, as in case 2, sclerosis of dorsal funiculi of the spinal cord, tabes, multiple sclerosis, heredocerebellar ataxia, etc. The authors also stress the clinico-radiological differences between basilar impression and platybasia, frequently taken as synonyms. Although both anomalies may coexist (as in cases 4 and 5 their association is not an absolute rule; on the contrary, in case 2 the basal angle was in the inferior limit of normality and in case 3 it was much lower. Emphasis is put on the value of the Chamberlain's line and chiefly in the McGregor basal line for the correct diagnosis of basilar invagination of the occipital bone.

  13. Validation of the ovine fetus as an experimental model for the human myelomeningocele defect Validação do feto de ovino como modelo experimental de meningomielocele

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    Denise Araújo Lapa Pedreira

    2007-06-01

    Full Text Available PURPOSE: To produce a myelomeningocele-like human defect in the ovine fetus and validate this experimental model in our population. METHODS: A prospective study on 12 pregnant sheep of a crossed Hampshire/Down breed where a spinal defect was surgically created between Day 75 and Day 77 after conception. The technique consisted of a hysterotomy with exposure of fetal hind limbs and tail up to the mid spine. Fetal skin, paravertebral muscles, and 4 posterior spinal arches were excised, exposing the spinal cord. Duramater was opened and the medulla was incised until the medullar canal. Animals were euthanized at 139 days of gestation for fetal evaluation. The central nervous system was submitted to post-mortem magnetic resonance imaging (MRI and the spine was submitted to pathological examination. RESULTS: The defect was created in 13 fetuses and 5 survived. Mean gestational age at necropsy was 121.6 days (varying from 93 to 145 days. Macroscopically, the defect was present in 4 cases. Microscopy revealed a flattened medulla with disappearance of the medullar canal and disruption of normal medullar architecture with neuronal apoptosis and/or fusion of the piamater and duramater. The MRI showed herniation of the cerebellum into the cervical canal and syringomyelia. CONCLUSIONS: The surgically produced defect mimics the defect found in the human fetus, including the Arnold-Chiari malformation. Post-mortem MRI was used for the first time in our study and proved an excellent alternative for demonstrating the cerebellar herniation. We standardized the technique for creating the defect in our population.OBJETIVO: Produzir um defeito semelhante a meningomielocele humana em feto de ovinos, validando este modelo experimental, em nosso meio. MÉTODOS: Estudo prospectivo com 12 ovelhas de cruzamento das raças Hampshire e Down, onde um defeito na coluna foi criado cirurgicamente com 75 a 77 dias de gestação. A técnica consistiu em histerotomia com exposi