Dandy–Walker malformation is a rare cause of syringomyelia in adults

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G. Yu. Evzikov

2017-01-01

Full Text Available Neural tube defects are the most common central nervous system malformation. Dandy–Walker malformation (DWM is a rare abnormality of the posterior cranial fossa, which is generally diagnosed in the prenatal period or early infancy.The paper describes a case of the late clinical manifestation of DWM, which has caused syringomyelia. All variants of pathological changes within the Dandy–Walker complex very rarely become a cause of syringomyelia. Only four cases of DWM, in which syringomyelia was found in adults, are known.The authors believe that formation of syringomyelia cysts in these patients is associated with the impaired circulation of normal cerebrospinal fluid between the superior cistern and the subarachnoid spaces of the spinal cord due to the caudal dislocation of the cyst formed in the fourth ventricle.Decompression of the craniovertebral junction, by resecting the caudal portion of the cyst in the foramen magnum, and repair of the free communication between the superior cistern and the cerebrospinal fluid spaces of the spinal cord, which is complemented by duroplasty, are pointed out to be an optimal treatment in these patients.

Exercise Concepts for Individuals with Syringomyelia

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... 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 ...

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.

Pan, Syrinx and syringomyelia.

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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.

Symptomatic arrhythmias due to syringomyelia-induced severe autonomic dysfunction.

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Riedlbauchová, Lucie; Nedělka, Tomáš; Schlenker, Jakub

2014-10-01

Syringomyelia is characterized by cavity formation in the spinal cord, most often at C2-Th9 level. Clinical manifestation reflects extent and localization of the spinal cord injury. 20-year old woman was admitted for recurrent rest-related presyncopes with sudden manifestation. Paroxysms of sinus bradycardia with SA and AV blocks were repeatedly documented during symptoms. There was normal echocardiographic finding, (para) infectious etiology was not proved. Character of the ECG findings raised suspicion on neurogenic cause. Autonomic nervous system testing demonstrated abnormalities reflecting predominant sympathetic dysfunction. Suspicion on incipient myelopathy was subsequently confirmed by MRI, which discovered syringomyelia at Th5 level as the only pathology. A 52-year old man with hypotrophic quadruparesis resulting from perinatal brain injury was sent for 2-years lasting symptoms (sudden palpitation, sweating, muscle tightness, shaking) with progressive worsening. Symptoms occurred in association with sudden increase of sinus rhythm rate and blood pressure that were provoked by minimal physical activity. Presence of significant autonomic dysregulation with baroreflex hyperreactivity in orthostatic test and symptomatic postural orthostatic tachycardia with verticalization-associated hypertension were proved. MRI revealed syringomyelia at C7 and Th7 level affecting sympathetic centers at these levels. Sympathetic fibers dysfunction at C-Th spinal level may cause significant autonomic dysfunction with arrhythmic manifestation.

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

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Isu, Toyohiko; Iwasaki, Yoshinobu; Nunomura, Mitsuru; Akino, Minoru; Koyanagi, Izumi; Abe, Hiroshi [Hokkaido Univ., Sapporo (Japan). School of Medicine; 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.).

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

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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.)

Increased detection rate of syringomyelia by whole spine sagittal magnetic resonance images: Based on the data from military conscription of Korean young males

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Lee, Myoung Seok; Oh, Chang Hyun [Seoul Regional Military Manpower Administration, Seoul (Korea, Republic of); Yoon, Seung Hwan; Park, Hyeong Chun; Park, Chong Oon; Kim, Yeo Ju [Inha Univ. Hospital/College of Medicine/Inha Univ., Incheon (Korea, Republic of)

2012-09-15

We compared the detection rate of syringomyelia according to the type of magnetic resonance (MR) images among the Korean military conscription. Among the total of 238910 examinees (males aged 18 to 32 years old) from January 2008 to December 2011, the examinees with conventional single lesion MR images (cervical, thoracic, and lumbar) with and without whole spine sagittal T2 weighted MR images (WSST2I) totaled 1206 cases, and syringomyelia was observed in 24 cases. The detection rate of syringomyelia according to the MR protocol (the presence of WSST2I or not) was done through analysis by annually and the clinical characters of syringomyelia was reviewed. The estimated prevalence of syringomyelia was approximately 10.0 cases per 100000 people. The detection rate was increased annually when the WSST2I proportion was increased (from 3.4 to 14.9 cases per 100000 persons, r = 0.939, p = 0.018). Clinical character of syringomyelia was ambiguous with other spinal diseases. The most affected spinal level was C5 to C7 (83%), and most cases were non communicating syringomyelia with benign central canal widening (79%). Whole spine sagittal MR image is useful to detect coexisting spinal diseases such as syringomyelia, and most syringomyelia in young males was benign hydromyelia. A whole spine sagittal MR image is recommended to increase the detection of syringomyelia.

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

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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

Pan, Syrinx and syringomyelia

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Leonardo Palacios-Sánchez

Full Text Available ABSTRACT 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.

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)

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

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

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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...

Posttraumatic syringomyelia associated with heavy weightlifting exercises: case report.

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Balmaseda, M T; Wunder, J A; Gordon, C; Cannell, C D

1988-11-01

Posttraumatic syringomyelia is a well-recognized late sequel to spinal trauma occurring in 1% to 3.2% of spinal cord injured patients. Its clinical presentation is usually marked by pain, ascending sensory loss, increased muscle weakness, and depressed deep tendon reflexes. The case of a 25-year-old man with C8 complete quadriplegia, who developed a syrinx five years after his initial injury, is presented. This patient kept a log of his daily physical workout which consisted of lifting weights of 50 to 60 pounds with his neck extensors and biceps. The diagnosis was made clinically and confirmed by magnetic resonance imaging. Repeated valsalva maneuvers from daily heavy weightlifting exercises most likely predisposed this patient to the development and extension of his syringomyelia. Dramatic improvement followed surgical placement of a subarachnoid shunt.

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

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

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.

Are Established Methods of Physiotherapeutic Management for Long-term Neurological Conditions Applicable to 'Orphan' Conditions such as Syringomyelia?

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Smith, Rebecca; Jones, Gareth; Curtis, Alexandra; Murphy, Hollie; Flint, Graham

2016-03-01

Syringomyelia is a rare or 'orphan' condition with the potential to cause significant disability and detrimental effects to quality of life. Syringomyelia shares similar symptoms to those common in other long-term neurological conditions, including spinal cord injury and multiple sclerosis. In these more prevalent conditions, physiotherapy is utilized widely and is effective in optimizing physical, psychological and social parameters. Therefore, we theorized that physiotherapy might be transferable to, and beneficial to syringomyelia patients. As a paucity of literature exists in this area, we aimed to evaluate the existing uptake and perceived efficacy of physiotherapy. An exploratory, mixed methodology was selected to derive sufficient qualitative data for analysis. Specifically designed questionnaires and semi-structured interviews yielded data on uptake and perceived physiotherapy efficacy. One hundred patients from a National Health Service tertiary syringomyelia service were invited to participate. The questionnaire and interviews were completed by 49 and 20 patients, respectively. Of the small number of patients receiving physiotherapy, the majority reported beneficial effects on pain modulation and quality of life. Stretching and hydrotherapy were deemed effective for relief of pain and stiffness. Additionally, physiotherapy was reported to provide similar benefits to surgical intervention. Syringomyelia patients report physiotherapy to provide benefits for symptom management and quality of life. Such findings suggest that established rehabilitation techniques in more common conditions may be transferable to those less prevalent. Uptake of physiotherapy was limited, seemingly because of inadequate information, knowledge and resources. To address these deficiencies, further studies should be planned investigating the effectiveness of physiotherapy modalities, such as hydrotherapy, in parallel or in conjunction with surgery and/or pharmacology. Additionally

Syringomyelia and Craniocervical Junction Abnormalities in Chihuahuas

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Kiviranta, A.‐M.; Rusbridge, C.; Laitinen‐Vapaavuori, O.; Hielm‐Björkman, A.; Lappalainen, A.K.; Knowler, S.P.; Jokinen, T.S.

2017-01-01

Background: Chiari-like malformation (CM) and syringomyelia (SM) are widely reported in Cavalier King Charles Spaniels and Griffon Bruxellois dogs. Increasing evidence indicates that CM and SM also occur in other small and toy breed dogs, such as Chihuahuas. Objectives: To describe the presence of SM and craniocervical junction (CCJ) abnormalities in Chihuahuas and to evaluate the possible association of CCJ abnormalities with SM. To describe CM/SM-related clinical signs and neuro...

Magnetic resonance tomography in syringomyelia

International Nuclear Information System (INIS)

Koehler, D.; Treisch, J.; Hertel, G.; Schoerner, W.; Fiegler, W.; Staedtisches Rudolf-Virchow Krankenhaus, Berlin

1985-01-01

Thirteen patients with a clinical diagnosis of syringomyelia were examined by nuclear tomography (0.35 T magnet) in the spin-echo mode. In all thirteen patients, the T1 images (Se 400/35) showed a longitudinal cavity with a signal intensity of CSF. The shape and extent of the syrinx could be adequately demonstrated in 12 of the 13 examinations. Downward displacement of the cerebellar tonsils was seen in eight cases. The examination took between half and one hour. Advantages of magnetic resonance tomography (nuclear tomography) include the absence of artifacts, images in the line of the lesion and its non-invasiveness. (orig.) [de

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

Chiari-like malformation and syringomyelia in cavalier King Charles spaniels

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Rusbridge, C.

2007-01-01

This thesis is the cumulation of over a decade of study into the pathogenesis and treatment Chiari-like malformation and syringomyelia (CM/SM) in the cavalier King Charles spaniel (CKCS). Chiari-like malformation is a condition where the brain is too big for the skull and is crushed and pushed out

Treatment of selected syringomyelias with syringo-pleural shunt: the experience with a consecutive 26 cases.

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Fan, Tao; Zhao, XinGang; Zhao, HaiJun; Liang, Cong; Wang, YinQian; Gai, QiFei; Zhang, Fangyi

2015-10-01

It is well established that syringomyelia can cause neurological symptoms and deficit by accumulation of fluid within syrinx cavities that lead to internal compression within the spinal cord. When other intervention treating the underlying etiology failed to yield any improvement, the next option would be a procedure to divert the fluid from the syrinx cavity, such as syringo-subarachnoid, syringo-peritoneal or syringo-pleural shunting. The indications and long term efficacy of these direct shunting procedures are still questionable and controversial. To investigate the clinical indication, outcome and complication of syringe-pleural shunt (SPS) as an alternative for treatment of syringomyelia. We reported a retrospective 26 cases of syringomyelia were found to have indication for a diversion procedure. SPS was offered. Patients' symptoms, mJOA score, and MRI were collected to evaluate the change of the syringomyelia and prognosis of the patients. 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. All 26 patients underwent SPS. The clinical information was collected, the mean follow-up time was 27.4 months, 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. The key surgical technique, outcome and complications of SPS were reported in detail. No mortality and severe complications occurred. Postoperative MRIs revealed near-complete resolution of syrinx in 14 patients, significant shrinkage of syrinx in 10 patients, no obvious reduction or unchanged in remaining 2 patient. Postoperatively, the symptoms improved in 24 cases (92.3%). Statistical analysis of the mJOA scores showed a statistical significance (P<0.001) between the preoperative group and the 2-week postoperative group. No further significant improvement between 2 weeks to the final follow up at 27 months. Collapse or remarkable shrinkage of the syrinx by SPS could ameliorate or at least stabilize the symptoms

Quantitative analysis of Chiari-like malformation and syringomyelia in the Griffon Bruxellois dog.

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Susan P Knowler

Full Text Available This study aimed to develop a system of quantitative analysis of canine Chiari-like malformation and syringomyelia on variable quality MRI. We made a series of measurements from magnetic resonance DICOM images from Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables. We found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes.

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

A feline case of isolated fourth ventricle with syringomyelia suspected to be related with feline infectious peritonitis.

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Kitagawa, Masato; Okada, Midori; Sato, Tsuneo; Kanayama, Kiichi; Sakai, Takeo

2007-07-01

A one-year-old female cat was unable to stand. Magnetic resonance imaging was performed, and an enlargement of the lateral, third, and fourth ventricles and syringomyelia were detected. The cat was diagnosed with an isolated fourth ventricle (IFV) with syringomyelia. The serum isoantibody test for the feline infectious peritonitis (FIP) virus was 1:3,200. After the cat died, a pathological examination revealed nonsuppurative encephalomyelitis. We suspected that the IFV, detected in the cat, was associated with FIP encephalomyelitis. To our knowledge, there has been no report on IFV in veterinary medicine.

The nature, meanings, and dynamics of lived experiences of a person with syringomyelia: a phenomenological study.

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Hilton, Edith L; Henderson, Lesley J

2003-01-01

Syringomyelia, considered a rare neurological disease, is relatively uninvestigated in the nursing literature. The aims of this qualitative phenomenological case study were to discover the nature, meanings, and dynamics of lived experiences of a 52-year-old Caucasian male with syringomyelia. Using van Manen's Method of Phenomenological inquiry (van Manen, 1990), data were collected, checked, and analyzed according to the philosophy, approach, and methodological procedures of phenomenology. Findings revealed an overarching theme of engulfment by disease. Essential themes included loss of abilities, struggles to adapt to changes, and life as a person who was disabled. Eleven sub-themes were also identified. Implications for nursing practice are discussed.

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.

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.

Subarachnoid pouches of the posterior fossa with syringomyelia

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Williams, B [Midland Centre for Neurosurgery and Neurology, Smethwick (UK)

1979-01-01

Three men are presented, all of whom were born with difficulty and later developed severe syringomyelia with collapsing cords on air myelography. Each had hydrocephalus and a sizable subarachnoid pouch in the posterior fossa. The clinical features and investigations are presented with a discussion of the aetiology and possible mechanisms concerned in pathogenesis and treatment. All three men had posterior fossa exploration over fifty years after birth, and the first two were also treated by valved ventriculo-atrial shunts. Each patient has improved a little since operation, but no certainly that this was due to surgery has been proved in any case; none has progressed since the last operation.

Hydrodynamic study of syringomyelia by MRI and intraoperative ultrasonography

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Moritake, Kouzo; Takaya, Mikio; Minamikawa, Jun; Ishikawa, Masatsune; Kikuchi, Haruhiko; Minami, Shunsuke

1989-01-01

Syringomyelic cavities were studied with magnetic resonance imaging (MRI) in eleven patients with special reference to the hemodynamic contribution to the pathogenesis of syringomyelia. They were also studied intraoperatively with ultrasonography (USG) in five patients. Syrinx cavities combined with lumbosacral lipomyelomeningocele or with spinal stenosis did not present a flow-void phenomenon reflecting pulsatile movements of syrinx fluid. On serial MRI study in those the patients, enlargement of the syrinx cavity was not observed. Syrinx cavities occupying the caudal part of the spinal cord did not develop either. These cases were not treated surgically but followed conservatively. On the other hand, the flow-void sign in the syrinx cavities was present on MRI in patients who also had Chiari type I or type II malformations. They were treated with a syringo-subarachnoid shunt. In these cases, intraoperative USG disclosed marked fluctuation of syrinx cavity size synchronous with the motions of pulmonary ventilation. In all of them, clinical signs and symptoms improved postoperatively to various degrees. These results suggest that both the flow-void sign in the syrinx cavity on MRI and marked fluctaution of cavity size on intraoperative USG are indications for the shunt operation and support William's revised theory (1987). Fluctuation of cystic cavity size synchronous with ventilation suggests that venous pressure in the spinal subarachnoid space contributes to the pathogenesis of syringomyelic cavities. Further analysis of the fluctuation of cystic cavities by video monitoring will provide further information on the etiology and other clinical problems of syringomyelia. (author)

Role of Cysternography in the management of Chiari I malformation related syringomyelia

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Das, B.K.; Pradhan, P.K.; Arora, P.; Bihari, S.; Arya, A.; Jain, V.K.; Banerjee, D.; Chhabra, D.K.

2002-01-01

Aim: In syringomyelia related to Chiari I malformation there is significant alteration of CSF flow dynamics . However, no systematic study has been reported revealing the changes of flow dynamics and its role in assessing the outcome of treatment. The aim of this prospective study was to characterize the changes of CSF flow dynamics in MRI proven Chiari I malformation and correlate the findings with the outcome of posterior decompression and duraplasty. Material and Method : Fifteen patients with MRI proven Chiari I malformation without associated hydrocephalus or fixed AAD requiring trans oral decompression were included in this study. 185 MBq ( 5 mCi ) Tc99m-DTPA was instilled in lumbar region and the ascent of the tracer was followed at 2 hrs, 4 hrs, 6 hrs and 24 hrs by Gamma Camera imaging. The time and pattern of ascent to Foramen magnum(FM), cranial subarachnoidal spaces, delay or hold up of tracer or filling of syrinx were noted. Results: Out of 15 patients six (40%) showed timely tracer ascent with normal CSF flow to the cerebral convexities(group I) whereas 9 patients (60%) had delayed ascent and abnormal intra cranial CSF flow. In these 9 patients two distinctive groups could be identified. Six patients (group II) had mild delay whereas three patients (group III) showed almost complete block of CSF flow at the FM level. Comparison of clinical status as measured by modified JOAS disability grading system before and six months after surgical intervention showed significant improvement in group III and moderate improvement in group II. Group I patients had a low disability grade before and showed minor improvement after intervention. Conclusions: (i) Cysternography can be helpful in predicting the outcome of surgical intervention in Chiari I malformation related syringomyelia. (ii) Pre and post operative clinical scores correlate with the cysternography findings. (iii) The traditional belief that FM block causes Chiari I malformation related syringomyelia

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

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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.

Clinical study of syringomyelia. Relation of neurological symptoms and imaging diagnosis

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Ohga, Ritsu; Konishi, Yoshihiro; Higashi, Yasuto; Kawai, Kingo; Yasuda, Takeshi; Terao, Akira (Kawasaki Medical School, Kurashiki, Okayama (Japan))

1988-12-01

We discussed the relationship between neurological symptoms and the locations of syringes observed by CT and MRI (imaging diagnosis) in six cases of syringomyelia admitted to our department during the past five years. Neurological symptoms of the upper cervical and thoracic cords were found in six cases and five cases of them had symmetric distribution. Syringes were found in all cases by delayed CT (D-CT) and MRI. Five cases had laterality. The sites in the spinal cord exhibiting severe involvement of neurological symptoms corresponded with the sites of syringes in imaging diagnosis. The main asymmetric lesions of the syringes were located in the posterior horn. They indicated the relationship with the appearance of the neurological symptoms of the lesion. We compared with the width of the longitudinal level from neurological findings and imaging diagnosis. The rostral level of both corresponded in all cases, but the caudal level corresponded in only one case and neurological symptoms were broader than syringes in imaging diagnosis. It was difficult to identify small syringes when there was complicated scoliosis. The diagnosis of typical cases of syringomyelia is mainly based on such neurological symptoms as a bilateral segmental pattern of dissociated sensory impairment in the past, but imaging diagnosis has recently come to be regarded as very important. (J.P.N.).

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

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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

Dumb-bell sarcoma of the foramen jugulare with syringomyelia. A radio-induced tumour

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Sieben, G; Sieben-Praet, M; de Reuck, J; de Coster, W; Remouchamps, A; Roels, H; van der Eecken, H [Ghent Rijksuniversiteit (Belgium). Dept. of. Neurology and Pathology

1980-01-01

The clinicopathological findings of a 58-year-old man, who developed cervicothoracic syringomyelia at the age of 25 are presented. He was given radiation therapy at the age of 33. At the age of 57 he developed a foramen jugulare syndrome on the left, caused by a low grade leiomyosarcoma. Etiologically, the most attractive hypothesis appears to be that the tumour was induced by radiation therapy administered 24 years previously.

Radiographic studies of the ventricles in syringomyelia

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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.)

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

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.

Foraminal syringomyelia: suggestion for a grading system.

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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.

Clinical and neuroradiological features of syringomyelia associated with Chiari malformation

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Isu, Toyohiko; Iwasaki, Yoshinobu; Akino, Minoru; Abe, Hiroshi; Tashiro, Kunio; Sudo, Kazumasa; Miyasaka, Kazuo [Hokkaido Univ., Sapporo (Japan). School of Medicine; Saito, Hisatoshi

1990-01-01

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scolisis (12 cases) or pescavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. The authors think that the syrinx which originates from the unilateral posterolateral portion, extends to involve the gray matter around the central canal and the posterolateral portion of the other side with progression of the syrinx to cause bilateral disturbance. As a result, unilateral neurological symptoms also become bilateral with progression of the syrinx. (author).

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

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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

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.

Scoliosis and Syringomyelia With Chiari Malformation After Lumbar Shunting

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Hsin-Hung Chen

2010-07-01

Full Text Available Unsteady gait was noted in a 2-year-old boy with a lumboperitoneal (LP shunt that had been inserted 1 year earlier for increased head circumference caused by communicating hydrocephalus. Scoliosis was also noted during postoperative follow-up. Magnetic resonance imaging revealed new hindbrain tonsillar herniation and an extensive syrinx from C3 to L1. The malfunctioning LP shunt was removed and posterior fossa decompression with ventriculoperitoneal shunt insertion was performed. The unsteady gait recovered completely and scoliosis improved. Magnetic resonance imaging demonstrated resolution of the syrinx and tonsillar herniation. Acquired Chiari I malformation after LP shunt is well documented; usually, patients have no symptoms. This is the first report to have all the cause and effect mechanisms among syringomyelia, scoliosis and Chiari I malformation in 1 patient. We review the literature and discuss the possible mechanisms.

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

Three-dimensional constructive interference in steady-state magnetic resonance imaging in syringomyelia: advantages over conventional imaging.

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Roser, Florian; Ebner, Florian H; Danz, Søren; Riether, Felix; Ritz, Rainer; Dietz, Klaus; Naegele, Thomas; Tatagiba, Marcos S

2008-05-01

Neuroradiology has become indispensable in detecting the pathophysiology in syringomyelia. Constructive interference in steady-state (CISS) magnetic resonance (MR) imaging can provide superior contrast at the sub-arachnoid tissue borders. As this region is critical in preoperative evaluation, the authors hypothesized that CISS imaging would provide superior assessment of syrinx pathology and surgical planning. Based on records collected from a database of 130 patients with syringomyelia treated at the authors' institution, 59 patients were prospectively evaluated with complete neuroradiological examinations. In addition to routine acquisitions with FLAIR, T1- and T2-weighted, and contrast-enhanced MR imaging series, the authors obtained sagittal cardiac-gated sequences to visualize cerebrospinal fluid (CSF) pulsations and axial 3D CISS MR sequences to detect focal arachnoid webs. Statistical qualitative and quantitative evaluations of spinal cord/CSF contrast, spinal cord/CSF delineation, motion artifacts, and artifacts induced by pulsatile CSF flow were performed. The 3D CISS MR sequences demonstrated a contrast-to-noise ratio significantly better than any other routine imaging sequence (p CSF flow voids. Constructive interference in steady-state MR imaging enables the neurosurgeon to accurately identify cases requiring decompression for obstructed CSF. Motion artifacts can be eliminated with technical variations.

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

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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

The analysis of the pathophysiology of syringomyelia by means of magnetic resonance imaging

International Nuclear Information System (INIS)

Abe, Toshiaki

1992-01-01

The pathophysiology of syringomyelia was examined with magnetic resonance imaging (MRI) in 70 patients. According to complications, syringomyelia was classified as being associated with (1) Chiari malformation, (2) both Chiari malformation and basal arachnoiditis, (3) basal arachnoiditis, (4) an obstruction of the foramen Magendie, and (5) spinal arachnoiditis. MRI features in Type 1 included: wedge-shaped herniated tonsils with the cisterna magna, pencil-shaped rostral end of the intraspinal syrinx, and obliterated caudal part of the fourth ventricle. These findings suggested that the herniated tonsils might result in the disturbance of cerebro-spinal fluid (CSF) circulation. In Type 2, the tip of the herniated tonsils was round; a triangular space was noted between the herniated tonsils and the upper spinal cord; the rostral end of the intraspinal syrinx was round; and the caudal part of the fourth ventricle was well visualized. These findings revealed that the brain stem and spinal cord were less pressed, suggesting that CSF circulatory disturbance resulted from basal arachnoiditis around the foramen magnum. In Type 3, the cisterna magna was narrowed by a thickened arachnoid membrane. In type 4, the intraspinal syrinx was directly connected with the fourth ventricle via the patent central canal associated with hydrocephalus. The position of the cerebellar tonsils and the size of the cisterna magna were both normal. In Type 5, a partial adhesion of the arachnoid membrane around the spinal cord was shown, and the syrinx was found extended toward the rostral as well as the caudal direction of the arachnoid adhesion. The rostral end of the syrinx was sometimes changed in size during MRI examinatin. This phenomenon sustained William's theory of 'suck and slosh mechanism' of intraspinal syrinx extension. (N.K.)

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

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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.

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

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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

Post-traumatic syringomyelia: CSF hydrodynamic changes following spinal cord injury are the driving force in the development of PTSM.

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Shields, Christopher B; Zhang, Yi Ping; Shields, Lisa B E

2012-01-01

Post-traumatic syringomyelia (PTSM) is a disorder that occurs infrequently following spinal cord injury (SCI), characterized by progressive neurological deterioration resulting from syrinx expansion originating in proximity to the traumatic epicenter. Several pathogenetic factors are associated with this disorder, however, the precise mechanism of the development of PTSM is controversial. Combined anatomical alterations and molecular changes following trauma to the spinal cord and arachnoid participate in the development of this condition. These factors include narrowing or obstruction of the subarachnoid space (SAS), central canal occlusion, myelomalacia, and alterations in intramedullary water permeability. If a patient sustains a SCI with delayed progressive deterioration in neurological function, in association with the MRI appearance of syringomyelia (SM), the diagnosis of PTSM is straightforward. The treatment of PTSM has not undergone any significant changes recently. The surgical treatment of PTSM consists of reconstructing the SAS or shunting fluid away from the syrinx to other locations. The advantages and disadvantages of each procedure will be discussed. With greater understanding of the mechanisms contributing to the development of SM, including advanced diagnostic methods and further advances in the development of artificial dural and shunting tubing, future therapies of PTSM will be more effective and long-lasting. Incorporation of alterations of AQP4 expression provides an intriguing possibility for future treatment of PTSM. Copyright © 2012 Elsevier B.V. All rights reserved.

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

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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.

Triple A (Allgrove) syndrome: an unusual association with syringomyelia

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2013-01-01

Triple A (Allgrove) syndrome was first described by Allgrove in 1978 in two pairs of siblings. Since then, about 100 cases have been reported, all of them displaying an autosomal recessive pattern of inheritance. Clinical picture is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure. A progressive neurological syndrome including central, peripheral and autonomic nervous system impairment, and mild mental retardation is often associated. The triple A syndrome gene, designated AAAS, is localized on chromosome 12q13. It consists of 16 exons, encoding for a 546 aminoacid protein called ALADIN (Alacrimia-Achalasia-aDrenal Insufficiency Neurologic disorder). We report on a 13 year-old boy presenting with Addison’s disease, dysphagia, muscle weakness, excessive fatigue and recent onset gait ataxia. The analysis of the AAAS gene revealed a homozygous missense mutation in exon 12. It was a T > G transversion at nucleotide position 1224, resulting in a change of leucine at amino acid position 381 into arginine (Leu381Arg or L381R). Brain appearance was found normal at magnetic resonance imaging (MRI) and functional spectroscopy analysis showed normal levels of the main metabolites. Spine MRI showed a cystic cavity within the spinal cord (syringomyelia), localized between the sixth cervical vertebra and the first thoracic vertebra. Cerebellar tonsils descended 7 mm caudal to foramen magnum, consistently with a mild type 1 Chiari malformation. Mild posterior inter-vertebral disk protrusions were evident between T9 and T10 and between L4 and L5. To our knowledge, this is the first report describing type 1 Chiari malformation and multiple spinal cord abnormalities in a patient with Allgrove syndrome. PMID:23800107

Inherited occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel: experiences in setting up a worldwide DNA collection.

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Rusbridge, Clare; Knowler, Penny; Rouleau, Guy A; Minassian, Berge A; Rothuizen, Jan

2005-01-01

Inherited diseases commonly emerge within pedigree dog populations, often due to use of repeatedly bred carrier sire(s) within a small gene pool. Accurate family records are usually available making linkage analysis possible. However, there are many factors that are intrinsically difficult about collecting DNA and collating pedigree information from a large canine population. The keys to a successful DNA collection program include (1) the need to establish and maintain support from the pedigree breed clubs and pet owners; (2) committed individual(s) who can devote the considerable amount of time and energy to coordinating sample collection and communicating with breeders and clubs; and (3) providing means by which genotypic and phenotypic information can be easily collected and stored. In this article we described the clinical characteristics of inherited occipital hypoplasia/syringomyelia (Chiari type I malformation) in the cavalier King Charles spaniel and our experiences in establishing a pedigree and DNA database to study the disease.

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

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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.

Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment

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García-Ramos Rocío

2009-12-01

Full Text Available Abstract Chiari disease (or malformation is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years. Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation. It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms. Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition. It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.

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

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Schmidt, Martin Jürgen; Ondreka, Nele; Sauerbrey, Maren; Volk, Holger Andreas; Rummel, Christoph; Kramer, Martin

2012-09-06

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. 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 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.

International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions.

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Singhal, Ash; Cheong, Alexander; Steinbok, Paul

2018-03-12

In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)-an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

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.

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

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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

Syringomyelia

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... Institutes of Health (NIH), the leading supporter of biomedical research in the world. Genetics and birth defects Studies ... Testimony Legislative Updates Impact NINDS Contributions to Approved Therapies ... Director, Division of Intramural Research

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

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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

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.

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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.

Symptomatic Chiari malformation in infancy and adolescence; Cine-MRI and clinical evaluation

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Fukushima, Takeo; Matsuda, Toshihiro; Tsugu, Hitoshi; Sakamoto, Seisaburou; Tomonaga, Masamichi; Asakawa, Koji; Mitsudome, Akihisa (Fukuoka Univ. (Japan). School of Medicine); Hashimoto, Takahisa

1994-04-01

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).

Symptomatic Chiari malformation in infancy and adolescence

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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)

Neuropathic arthropathy of the shoulder interpreted as neoplasic lesion (reports of two cases)

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Galvis Ramirez, Javier Fernando; Soto, Camilo; Escandon, Santiago

2006-01-01

The neuropathic arthropathy is an illness that destroys the articulation, associated to loss of the propiocepcion. Inside the main causes they are the syringomyelia, tertiary syphilis and diabetes mellitus. In the superior member the frequently affected articulation is the glen humeral. In this report two patients are presented with neuropathic arthropathy from the shoulder secondary to syringomyelia and remitted to orthopedic oncology with diagnosis of condrosarcoma. The clinical radiological presentation and the differential diagnosis are analyzed

A genome-wide association study identifies candidate loci associated to syringomyelia secondary to Chiari-like malformation in Cavalier King Charles Spaniels.

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Ancot, Frédéric; Lemay, Philippe; Knowler, Susan P; Kennedy, Karen; Griffiths, Sandra; Cherubini, Giunio Bruto; Sykes, Jane; Mandigers, Paul J J; Rouleau, Guy A; Rusbridge, Clare; Kibar, Zoha

2018-03-22

Syringomyelia (SM) is a common condition affecting brachycephalic toy breed dogs and is characterized by the development of fluid-filled cavities within the spinal cord. It is often concurrent with a complex developmental malformation of the skull and craniocervical vertebrae called Chiari-like malformation (CM) characterized by a conformational change and overcrowding of the brain and cervical spinal cord particularly at the craniocervical junction. CM and SM have a polygenic mode of inheritance with variable penetrance. We identified six cranial T1-weighted sagittal MRI measurements that were associated to maximum transverse diameter of the syrinx cavity. Increased syrinx transverse diameter has been correlated previously with increased likelihood of behavioral signs of pain. We next conducted a whole genome association study of these traits in 65 Cavalier King Charles Spaniel (CKCS) dogs (33 controls, 32 with extreme phenotypes). Two loci on CFA22 and CFA26 were found to be significantly associated to two traits associated with a reduced volume and altered orientation of the caudal cranial fossa. Their reconstructed haplotypes defined two associated regions that harbor only two genes: PCDH17 on CFA22 and ZWINT on CFA26. PCDH17 codes for a cell adhesion molecule expressed specifically in the brain and spinal cord. ZWINT plays a role in chromosome segregation and its expression is increased with the onset of neuropathic pain. Targeted genomic sequencing of these regions identified respectively 37 and 339 SNPs with significantly associated P values. Genotyping of tagSNPs selected from these 2 candidate loci in an extended cohort of 461 CKCS (187 unaffected, 274 SM affected) identified 2 SNPs on CFA22 that were significantly associated to SM strengthening the candidacy of this locus in SM development. We identified 2 loci on CFA22 and CFA26 that contained only 2 genes, PCDH17 and ZWINT, significantly associated to two traits associated with syrinx transverse

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.

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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.

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

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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

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

Siringomielia idiopática a propósito de un caso

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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.

Sleep Disordered Breathing in Children and Adolescents with Chiari Malformation Type I

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Losurdo, Anna; Dittoni, Serena; Testani, Elisa; Di Blasi, Chiara; Scarano, Emanuele; Mariotti, Paolo; Paternoster, Giovanna; Di Rocco, Concezio; Massimi, Luca; Della Marca, Giacomo

2013-01-01

Study Objectives: Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. Methods: Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. Results: Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). Conclusions: In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB. Citation: Losurdo A; Dittoni S; Testani E; Di Blasi C; Scarano E; Mariotti P; Paternoster G; Di Rocco C; Massimi L; Della Marca G. Sleep disordered breathing in children and adolescents with Chiari malformation Type I. J Clin Sleep Med 2013;9(4):371-377. PMID:23585753

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

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Morais, Barbara Albuquerque; Cardeal, Daniel Dante; Ribeiro E Ribeiro, Renan; Frassetto, Fernando Pereira; Andrade, Fernanda Goncalves; Matushita, Hamilton; Teixeira, Manoel Jacobsen

2017-08-01

Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

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

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

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

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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.

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

Clinical and radiological findings in arnold chiari malformation

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Khan, A A; Bhatti, S N; Ahmed, E; Aurangzeb, A; Ali, A; Khan, A; Afzal, S; Khan, G [Ayub Medical College, Abbottabad (Pakistan). Department of Neurosurgery

2010-04-15

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

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

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.)

Walter Reed Army Medical Center, Washington, D. C. Annual Progress Report FY-89. Volume 2. Part 2

Science.gov (United States)

1990-01-02

equipment. Patients are divided into 5 groups Spinal Cord Tumor, Syringomyelia, Spondylosis Demyelinating disorders and miscellaneous. Central condition...form original protocol. A small piece of skeletal muscle is removed from the paraspinal muscles in a patient undergoing lumbar laminecomy. This muscle

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

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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

Horner Syndrome

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... of the protective sheath on neurons (myelin) Neck trauma Cyst or cavity in the spinal column (syringomyelia) Second-order neurons This neuron path extends from the spinal column, across the upper part of the chest and into the side of the neck. Causes ...

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

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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.

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)

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

International Nuclear Information System (INIS)

Solomou, E.K.; Kotsarini, C.; Badra, F.A.; Krepis, A.; Papanastasiou, D.; Patriarcheas, G.

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.)

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

Usefulness of MRI in the diagnosis of atlanto-axial dislocation

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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.

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.

Charcot joint-like changes following ankle fracture in a patient with no underlying disease: report of a rare case.

Science.gov (United States)

Kumagai, Masaru; Yokota, Kiyoshi; Endoh, Toshiya; Takemoto, Hitoshi; Nagata, Kensei

2002-01-01

Charcot joint is a disease that often occurs in patients with diabetes mellitus, tabes dorsalis, syringomyelia, chronic alcoholism, leprosy, trauma, or infection after fractures and dislocations. The treatment for Charcot joint has various complications, such as skin lesions, infections, and delayed union. We present our experience with a male patient who developed Charcot joint-like changes without diabetes mellitus or any other disease after an ankle fracture due to minor trauma.

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

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

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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. .

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

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.

Magnetic resonance imaging in patients with progressive myelopathy following spinal surgery.

OpenAIRE

Avrahami, E; Tadmor, R; Cohn, D F

1989-01-01

Thirty one patients with insidious progressive myelopathy 2 to 8 years following surgery of the cervical spine were subjected to magnetic resonance imaging (MRI). In 15 patients operated on for vascular malformations or intramedullary tumours, syringomyelia and cystic lesions of the spinal cord were shown. Seven of these patients also showed a combination of a recurrent tumour and spinal atrophy. Out of 16 patients who had surgery for herniated disc or spinal stenosis of the cervical spine, f...

The tragedy of syrinx.

Science.gov (United States)

Demerdash, Amin; Loukas, Marios; Tubbs, R Shane

2014-03-01

Today, the term "syrinx" is used by many clinicians. Most users of the term, however, are not familiar with the origin of the word. The present paper reviews the history of the word syrinx, which is based in Greek mythology. Many modern terms have a derivation that is based on a rich history that has often been forgotten. The myth of Syrinx lives on in modern times by the continued use of syrinx in various words such as syringomyelia and syringe.

Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

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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.

A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation.

Science.gov (United States)

Botelho, Ricardo Vieira; Bittencourt, Lia Rita Azeredo; Rotta, José Marcos; Tufik, Sergio

2003-12-01

Craniovertebral junction malformation (CVJM) or Chiari malformation in adults, with or without syringomyelia and basilar invagination, produces neuronal dysfunction of the brainstem, cerebellum, cranial nerves, and upper spinal cord. The respiratory center and some of its afferent and efferent components can be altered in these diseases. The authors studied patients with and without CVJM to determine whether this physical feature contributed to sleep disturbances. Respiratory manifestations during sleep were studied prospectively, by using whole-night polysomnography, in 32 symptomatic patients (CVJM group) and 16 healthy volunteers (control group). Patients with CVJM presented with more sleep disturbances (reports of snoring and apnea) than those in the control group. The apnea/hypopnea index values were higher in patients with CVJMs than in the control group (13 +/- 15 compared with 3 +/- 6; p = 0.007) and the rate of central sleep apneas was higher in the CVJM than in the control group (22 +/- 30 compared with 4 +/- 8%; p = 0.009). The apnea/hypopnea index was highest in the subgroup with basilar invagination than in the other subgroups. The central apneic episodes were more frequent in the patients with basilar invagination (35 +/- 40%; p = 0.001) and in those with syringomyelia (17.6 +/- 24.6%; p = 0.003) than in the control group (4 +/- 8%). Patients with symptomatic CVJM, especially those with basilar invagination, presented with more sleep respiratory compromise than did those in the control group. The incidence of sleep apnea/hypopnea syndrome is significantly higher in patients with CVJM.

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

Richard Bright and his neurological studies.

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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.

Magnetic resonance imaging of cerebrospinal fluid flow in pediatrics

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Heroux, R. [Children' s Hospital of Eastern Ontario, Magnetic Resonance Imaging Dept., Ottawa, Ontario (Canada)

2000-06-30

Magnetic Resonance Imaging of flowing protons in cerebrospinal fluid is useful for demonstrating areas of obstruction or stenosis of the ventricular system causing hydrocephalus. This is used in pediatric patients to assess the circulation of the cerebrospinal fluid. This article discusses two studies. In the first, the cerebrospinal fluid flow study helped the neurosurgeon assess the patency after a third ventriculocisternostomy. The second study evaluated the cerebrospinal fluid flowing through the foramen magnum in a patient with cerebellar tonsilar descent (Chiari malformation) and a syringomyelia. Different techniques to evaluate the flow studies are also discussed. (author)

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

A New Classification for Pathologies of Spinal Meninges-Part 2: Primary and Secondary Intradural Arachnoid Cysts.

Science.gov (United States)

Klekamp, Jörg

2017-08-01

Spinal intradural arachnoid cysts are rare causes of radiculopathy or myelopathy. Treatment options include resection, fenestration, or cyst drainage. To classify intradural spinal arachnoid cysts and present results of their treatment. Among 1519 patients with spinal space occupying lesions, 130 patients demonstrated intradural arachnoid cysts. Neuroradiological and surgical features were reviewed and clinical data analyzed. Twenty-one patients presented arachnoid cysts as a result of an inflammatory leptomeningeal reaction related to meningitis, subarachnoid hemorrhage, intrathecal injections, intradural surgery, or trauma, ie, secondary cysts. For the remaining 109 patients, no such history could be elucidated, ie, primary cysts. Forty-six percent of primary and 86% of secondary cysts were associated with syringomyelia. Patients presented after an average history of 53 ± 88 months. There were 122 thoracic and 7 lumbar cysts plus 1 cervical cyst. Fifty-nine patients with primary and 15 patients with secondary cysts underwent laminotomies with complete or partial cyst resection and duraplasty. Mean follow-up was 57 ± 52 months. In the first postoperative year, profound improvements for primary cysts were noted, in contrast to marginal changes for secondary cysts. Progression-free survival for 10 years following surgery was determined as 83% for primary compared to 15% for secondary cysts. Despite differences in clinical presentation, progression-free survival was almost identical for patients with or without syringomyelia. Complete or partial resection leads to favorable short- and long-term results for primary arachnoid cysts. For secondary cysts, surgery can only provide clinical stabilization for a limited time due to the often extensive arachnoiditis. Copyright © 2017 by the Congress of Neurological Surgeons

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

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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.

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

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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.).

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

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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.

Prominent central spinal canal on MRI - normal variant or pathology

International Nuclear Information System (INIS)

Dugal, T.P.; Brazier, D.; Roche, J.

2002-01-01

Full text: The sensitivity of MRI can make differentiation of normal from abnormal challenging.The study investigates whether a visible central spinal canal is pathological or a normal variant. We review eight MRI (mostly on a 1.5 Tesla unit) cases where there is a visible central cavity in keeping with a central canal and review the literature. The central canal is a space in the medial part of the grey-matter commissure between the anterior and posterior horns. Histopathological studies show that the canal is present at birth with the majority showing subsequent involution but is uncommonly imaged on MRI. The main differential diagnosis is syringomyelia which usually presents with deficits in pain and sensation corresponding to the appropriate level often with a demonstrable aetiology. Two thirds of our patients were female with an average age of thirty-six years (range 26-45). The patients were largely asymptomatic or their symptoms appeared unrelated to the imaging findings. Three patients had minor previous trauma and two others had non-bacterial meningitis up to twenty years earlier. No patient had known spinal surgery or trauma.The cavity corresponded tomographically to the expected site of the central canal. The canal was in the thoracic location. The canal diameter ranged from one to five millimetres and its length varied from one half a vertebral body height to extending over the entire thoracic region. Its configuration was either filiform or fusiform, with smooth contours. No predisposing features to suggest syringomyelia or other structural abnormalities were noted. Where Gadolinium was given no abnormal enhancement was observed. These cases add to the literature and suggest that these prominent canals are largely asymptomatic and should be viewed as normal variants. Copyright (2002) Blackwell Science Pty Ltd

An Unusual Case of Post-Traumatic Headache Complicated by Intracranial Hypotension

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Sara Siavoshi

2016-12-01

Full Text Available We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension. In addition, the delayed onset of upper motor neuron symptoms along with initially normal head computerized tomography scan (CT findings, beg the question of whether or not a post-traumatic headache warrants earlier magnetic resonance imaging (MRI.

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

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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.

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)

Cerebrospinal fluid flow. Pt. 3

International Nuclear Information System (INIS)

Schroth, G.; Klose, U.

1992-01-01

Cardiac- and respiration-related movements of the cerebrospinal fluid (CSF) were investigated by MRI in 71 patients. In most patients with arteriosclerotic occlusive vascular disease CSF pulsations are normal. Decreased pulsatile flow is detectable in those with arteriovenous malformations, intracranial air and following lumbar puncture and withdrawal of CSF. Increased pulsatile flow in the cerebral aqueduct was found in 2 patients with large aneurysms, idiopathic communicating syringomyelia and in most cases of normal pressure hydrocephalus (NPH). CSF flow in the cervical spinal canal is, however, reduced or normal in NPH, indicating reduction of the unfolding ability of the surface of the brain and/or inhibition of rapid CSF movements in the subrachnoid space over its convexity. (orig.)

Cervical myelopathy: magnetic imaging findings

International Nuclear Information System (INIS)

Kholin, A.V.; Makarov, A.Yu.; Gurevich, D.V.

1996-01-01

69 patients with clinical signs of cervical myelopathy were examined using magnetic imaging (T1- and T2-suspended tomograms of the sagittal and transverse section using a device with 0.04 T field intensity). Vertebral disk hernias were revealed in 35 patients, compression of the spinal cord with metastases into vertebral body in 2, extramedullary tumor in 11, intramedullary tumor in 9, and syringomyelia in 12 patients. T2-suspended tomograms proved to be more informative due to their higher sensitivity to aqueous content. T1-suspended tomograms help assess the degree of spinal cord compression and the direction of the disk protrusion. Magnetic imaging is an informative method used for objective identification of the cases of myelopathy of cervical localization [ru

[Cervical myelopathy from an aspect of a neurological surgeon].

Science.gov (United States)

Koyama, T

1994-12-01

More than 65 per cent of intramedurally tumors and intradural extramedurally tumors were located in cervical region. They enact a cause of cervical myelopathy. A special attention should be paid to solitaly intramedurally vascular malformations, when the patients show an acute progressive myelopathy. In very rare cases of syringomyelia, intramedurally tumors such as hemangioblastoma or ependymoma could be found, so that enhanced MRI or angiography should be done if necessary. In cases of a herniated disc, cervical spondylosis and OPLL, the distance between articulo-pillar line and spinolaminal line must be measured. If the distance of both lines is near or the same, that means narrow cervical canal. In rare cases the form of the vertebral body is round. This is a rare cause of a type of narrow canal.

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

International Nuclear Information System (INIS)

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

1983-01-01

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

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

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

Magnetic resonance imaging of traumatic cervical injury

Energy Technology Data Exchange (ETDEWEB)

Juhng, S. K.; Lee, K. S.; Sohn, K. J.; Choi, S. S.; Won, J. J. [Wonkwang University School of Medicine, Iri (Korea, Republic of)

1994-04-15

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.

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

Magnetic resonance tomography for trauma of the cervical spine

Energy Technology Data Exchange (ETDEWEB)

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

1986-12-01

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.

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

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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.

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

Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation.

Science.gov (United States)

Udani, Vikram; Holly, Langston T; Chow, Daniel; Batzdorf, Ulrich

2014-01-01

We describe our use of a perforated titanium plate to perform a partial posterior fossa cranioplasty in the treatment of cerebellar ptosis and dural ectasia after posterior fossa decompression (PFD). Twelve patients who had undergone PFD underwent posterior fossa reconstruction using a titanium plate. Symptoms were related to either descent of the cerebellum into the decompression or to dural ectasia into the craniectomy defect. Twelve patients who had undergone large suboccipital craniectomies and who presented with persistent headaches and some with neurological symptoms related to syringomyelia, underwent reoperation with placement of a small titanium plate. Ten of 12 patients showed symptomatic improvement after reoperation. Placement of a titanium plate appears to be an effective method of treatment of cerebellar ptosis and dural ectasia after PFD for Chiari malformation. Copyright © 2014 Elsevier Inc. All rights reserved.

Assessing the relative importance of health and conformation traits in the cavalier king Charles spaniel.

Science.gov (United States)

Wijnrocx, Katrien; François, Liesbeth; Goos, Peter; Buys, Nadine; Janssens, Steven

2018-01-01

The selection of a future breeding dog is a complicated task, in which disease characteristics and different traits have to be combined and weighed against one another. Truncation selection, that is the exclusion of affected animals, may be very inefficient when selecting on a large number of traits, and may result in a reduction of the genetic diversity in a population or breed. Selection could be facilitated by the use of a selection index that combines multiple traits or breeding values into one score. This however requires a consideration of their relative value according to their economic weight, which is difficult to express in monetary units for health traits. The use of a choice experiment to derive non-market values might be a solution to this problem. This is a pilot study to assess the potential use of choice experiments to ascertain the public preference and relative importance attached to health- and conformation traits in the selection of a Cavalier King Charles spaniel. The focus was on two prevalent disorders, mitral valve disease and syringomyelia, and on several important conformation traits such as muzzle length and eye shape. Based on available prior information, a Bayesian D-optimal design approach was used to develop a choice experiment and the resulting choice sets. Every participant (breeder or owner) in the choice experiment was presented with a total of 17 choice sets, in which at most four traits could vary to reduce the cognitive burden. A total of 114 respondents participated in the choice experiment and results showed that respondents (breeders/owners) current attitudes were directed towards health (syringomyelia and mitral valve disease), followed by eye shape and level of inbreeding. This approach identifies the value breeders and owners attach to certain traits in the breeding objective. The resulting relative weights, represented as the logworths obtained from the choice experiment, could be an alternative to economic weights. They

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.

A case report of congenital sensory neuropathy with anhidrosis

International Nuclear Information System (INIS)

Lee, Won Hyong; Chang, Hae Soon; Han, Man Chung; Lee, Suck Hyun; Lee, Duk Yong

1974-01-01

Congenital sensory neuropathy with anhidrosis is rare disease and may be confused with other cause of pain insensitivity or indifference. Other cause of pain insensitivity include congenital indifference to pain, congenital sensory neuropathy, hereditary sensory radicular neuropathy, nonprogressive sensory radicular neuropathy, syringomyelia, and hysterical analgesia. It is hereditary disease which is transmitted with autosomal recessive trait. The patient is 8 years old Korean male with complaint of swelling and local heat on right knee joint. Generalized analgesia is noted on physical examination. The skin is dry and coarse with no evidence of sweating. Delayed motor development was noted on early children. Mental development is retarded. On past history, patient showed unpredictable rises of temperature, though the general condition remained good. Multiple painless fracture on right humerus and right metatasal bone was occurred. Rt.knee radiograms show marked swelling of soft tissue and periosteal calcification on distal femru,which are resemble with neurotrophic joint

Nuclear magnetic resonance tomography of the cervical canal

International Nuclear Information System (INIS)

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

1985-01-01

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. (orig.) [de

A case report of congenital sensory neuropathy with anhidrosis

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Lee, Won Hyong; Chang, Hae Soon; Han, Man Chung; Lee, Suck Hyun; Lee, Duk Yong [Seoul National University College of Medicine, Seoul (Korea, Republic of)

1974-10-15

Congenital sensory neuropathy with anhidrosis is rare disease and may be confused with other cause of pain insensitivity or indifference. Other cause of pain insensitivity include congenital indifference to pain, congenital sensory neuropathy, hereditary sensory radicular neuropathy, nonprogressive sensory radicular neuropathy, syringomyelia, and hysterical analgesia. It is hereditary disease which is transmitted with autosomal recessive trait. The patient is 8 years old Korean male with complaint of swelling and local heat on right knee joint. Generalized analgesia is noted on physical examination. The skin is dry and coarse with no evidence of sweating. Delayed motor development was noted on early children. Mental development is retarded. On past history, patient showed unpredictable rises of temperature, though the general condition remained good. Multiple painless fracture on right humerus and right metatasal bone was occurred. Rt.knee radiograms show marked swelling of soft tissue and periosteal calcification on distal femru,which are resemble with neurotrophic joint.

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

Directory of Open Access Journals (Sweden)

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.

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.

Visibility of the central canal on MRI

Energy Technology Data Exchange (ETDEWEB)

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.)

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.)

Anophthalmia-plus syndrome: a clinical report and review of the literature.

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Makhoul, Imad R; Soudack, Michalle; Kochavi, Orna; Guilburd, Joseph N; Maimon, Shimon; Gershoni-Baruch, Ruth

2007-01-01

We describe a term male infant of healthy non-consanguineous parents, born with congenital malformations, including bilateral cleft palate and lip, mild microphthalmia with iris coloboma and glaucoma of the right eye, and blepharophimosis with severe microphthalmia of the left eye. Spine radiograph and MRI showed first sacral hemivertebra with spina bifida, and agenesis of the 2nd, 3rd, 4th, and 5th sacral vertebrae and coccyx. Spine MRI showed caudal tethering of spinal cord at L(3) level, filum terminalis lipoma and a syringomyelia. Brain ultrasound and MRI showed hypoplasia of corpus callosum with mild dilatation of the lateral ventricles. Orbital MRI showed bilateral microphthalmia-distorted small left eyeball with posteriorly located lens, and a split vitreous body in the right eye, suggestive of primary hyperplastic vitreous. The karyotype was normal. Summary of the findings in nine cases (our case and eight published cases) support the notion that anophthalmia-plus syndrome (APS) is a distinct syndrome. Gene locus of APS is yet to be identified. (c) 2006 Wiley-Liss, Inc.

September 2012 critical care case of the month

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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 …

Habitual biting of a finger in a child

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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.

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

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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.

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.

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.

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

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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.

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.

Treatment planning in severe scoliosis: the role of MRI

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Freund, M. [Dept. of Clinical Radiology, Univ. of Muenster (Germany); Dept. of Neuroradiology, Univ. of Heidelberg Medical School (Germany); Haehnel, S.; Sartor, K. [Dept. of Neuroradiology, Univ. of Heidelberg Medical School (Germany); Thomsen, M. [Dept. of Orthopaedic Surgery, University of Heidelberg, Medical School (Germany)

2001-06-01

The use of magnetic resonance imaging (MRI) in the preoperative investigation of children with idiopathic scoliosis is controversial. Syringomyelia and other intraspinal lesions may be risk factors for neurological injury during surgical correction. Our purpose was to investigate whether pathology of the neuraxis is associated with scoliosis and to detect lesions which may threaten neurological sequelae during distraction and instrumented correction. We obtained T1- and T2-weighted images of 40 children (28 girls, 12 boys), mean age 12.7 years with severe idiopathic scoliosis (Cobb angle 50-70 ) obtained in coronal, sagittal and axial planes from the posterior cranial fossa to the sacrum, and these were assessed by two neuroradiologists and an orthopaedic surgeon prior to further treatment planning. Abnormalities of the neuraxis were found in 24 patients (60 %); five (12 %) had two or more lesions. No abnormalities of the neuraxis were found in 16 patients (40 %). There were 15 patients (38 %) with intraspinal abnormalities who deteriorated clinically and nine (22 %) who showed no clinical changes. We transferred 16 patients (40 %) from the orthopaedic to the neurosurgical department for further assessment. Our results suggest that one should investigate the neuraxis with MRI before contemplating orthopaedic surgical correction of severe idiopathic scoliosis, because the findings may lead to a change of procedure. (orig.)

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...

Lumbosacral lipoma : gadolinium-enhanced fat saturation T1 weighted MR image is necessary?

International Nuclear Information System (INIS)

Yoon, Man Won; Kim, Hyun Chul; Chung, Tae Woong; Seo, Jeong Jin; Chung, Gwang Woo; Kim, Yun Hyeon; Kim, Jae Kyu; Park, Jin Gyoon; Kang, Heoung Keun

1999-01-01

To evaluate the usefulness of contrast-enhanced fat saturation T1-weighted imaging for the evaluation of spinal lipoma, compared with clinical symptoms and surgical findings. Ten patients with lipomyelomeningocele, confirmed by surgery, were included in this study. In all cases, conventional spin echo T1-and T2-weighted MR imaging, and contrast-enhanced fat saturation T1-weighted imaging was performed to evaluate clinical symptoms, the position of the conus medullaris, the presence of cord tethering, and associated anomalies, and to compare the relative usefulness of the techniques. All ten patients were suffering from lipomyelomeningocele without filum terminale fibrolipoma or intradural lipoma. All cases were associated with cord tethering. As associated anomalies, there were seven cases of syringomyelia without hydrocephalus or anorectal anomaly. To evaluate the position of the spinal conus and the presence of cord tethering, conventional T1-weighted imaging was more useful than the contrast-enhanced fat saturation equivalent. In patients with early-stage spinal lipoma, MRI is useful for evaluation of the causes and position of cord tethering and associated anomalies Our results suggest that contrast-enhanced fat saturation T1-weighted images do not provide additional information concerning spinal lipoma, and that for the diagnosis of this condition, conventional T1 and T2-weighted images are more useful than those obtained by contrast-enhanced fat saturation T1-weighted imaging

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.

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.

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.

Spinal cord involvement in tuberculous meningitis.

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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.

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)

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)

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

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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.

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

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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.

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

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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.

Imaging findings of charcot joint

International Nuclear Information System (INIS)

Meng Quanfei; Zhou Chunxiang; Chen Yingming; Jiang Bo

2003-01-01

Objective: To analyze the MRI characters of Charcot joint, and to evaluate the diagnostic value of X-ray, CT, and MRI on Charcot joint. Methods: Eight patients with 8 Charcot joints underwent X-ray, CT, and MR examinations. 6 of them had syringomyelia, 1 patient had injury of the spinal cord, and 1 case had diabetes. All 8 patients had sensory reduction or deficit in the sick extremities. Results: There were two types of Charcot joint, hypertrophic and atrophic. Radiographic and CT features of hypertrophic joint (n=3) showed hyperostotic osteosclerosis and mammoth osteophytes in the sick bones, periarticular ossification, and articular disorganization. Radiographic and CT features of atrophic joint (n=5) showed extensive bone resorption (destruction), periarticular debris, and articular disorganization. Main MRI features of Charcot joint included hydrarthrosis within joint capsule, thickened, loose, and elongated joint capsule with para-joint, peri-diaphysis, and inter-muscular extension in a pseudopodia pattern. The irregular joint capsule wall was presented as mild hypointensity on T 1 WI, slight hyper-intensity on T 2 WI, and was markedly enhanced after Gd-DTPA was administrated, which was considered as a characteristic manifestation of the lesion. Soft tissue mass containing hypo-intense stripes on both T 1 WI and T 2 WI was commonly noted adjacent to the involved joint. Conclusion: X-rays plain film is the first choice for the diagnosis of Charcot joint, and MRI is pretty useful in the diagnosis of Charcot joint

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.

Stereological and Morphometric Analysis of MRI Chiari Malformation Type-1

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Alkoç, Ozan Alper; Songur, Ahmet; Eser, Olcay; Toktas, Muhsin; Esi, Ertap; Haktanir, Alpay

2015-01-01

Objective In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements. Methods A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development. Results Tonsillar herniation length was measured 9.09±3.39 mm below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased. Conclusion Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition. PMID:26713146

Pregnancy in spinal cord-injured women, a cohort study of 37 pregnancies in 25 women.

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Le Liepvre, H; Dinh, A; Idiard-Chamois, B; Chartier-Kastler, E; Phé, V; Even, A; Robain, G; Denys, P

2017-02-01

A retrospective observational study. To describe specificities of pregnancy in a traumatic spinal cord-injured (SCI) population managed by a coordinated medical care team involving physical medicine and rehabilitation (PMR) physicians, urologists, infectious diseases' physicians, obstetricians and anaesthesiologists. NeuroUrology Department in a University Hospital, France. All consecutive SCI pregnant women managed between 2001 and 2014 were included. A preconceptional consultation was proposed whenever possible. Obstetrical and urological outcomes, delivery mode and complications were reported. Overall, thirty-seven pregnancies in 25 women, of a mean age of 32±4 years, were included. Thirty-five children were born alive (three miscarriages, a twin pregnancy) without complications except for a case of neonatal respiratory distress in premature twins born at 33 weeks. The mean birth weight was 2979±599 g. Twenty-one (57%) pregnancies benefited from preconceptional care. A weekly oral cyclic antibiotic programme was prescribed in 28 (75%) pregnancies. The main complications during pregnancy included pyelonephritis (30%), lower urinary tract infections (UTI) (32%), pressure sores (8.8%) and prematurity (12% deliveries before 37 weeks, with only one delivery before 36 weeks). Two patients suffered from autonomic dysreflexia, one with serious complication (brain haematoma). Caesarean sections were performed for 68% of deliveries (23/34) to prevent syringomyelia deterioration (n=10), stress urinary incontinence aggravation (n=3) or for obstetrical reasons (n=7). Mothers' and infants' outcomes were satisfying after pregnancy in SCI women, but required many adjustments. Pregnancy must be prepared by a preconceptional consultation, and managed by a multidisciplinary team involving specialists of neurological disability and pregnancy.

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.

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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.

Magnetic resonance imaging of spinal cord syndromes

International Nuclear Information System (INIS)

Einsiedel, H. von; Stepan, R.

1985-01-01

Thirty-four patients with intramedullary space-occupying lesions or cord compression syndromes were examined with a resistive and two different superconductive magnetic resonance (MR) imaging units. Studies were done primarily by the spin-echo (SE) technique and in the majority of patients different pulse sequences were used. Images with short echo-time (TE) and short recovery-time (TR) were best for demonstration of spinal cord anatomy, for depicting cystic portions in intramedullary tumours and for showing syringomyelia. Solid intramedullary tumours showed normal cord signal intensity. Images with prolonged TE and TR predominantly enhanced CSF signal intensity and, to a more considerable extent, solid intramedullary tumours. Thus, the diameter of the subarachnoid space and the presence of a solid intramedullary tumour, not concomittant with a significant enlargement of the spinal cord, could only be recognized on these prolonged SE images. Major advantages of MR in comparison to CT are that the spinal cord can be imaged in the sagittal plane and that beam hardening artifacts do not occur; in comparison to myelography the cord can be imaged directly by MR. Partial volume is a major limitation of MR, not only in the preferably applied sagittal plane. The choice of slice thickness adequate to the diameter of the lesion and straight positioning of the patient for sagittal single slice midline images are fundamental for reliable MR investigations. Another limitation to MR is that cortical bone gives no signal. The actual diameter of the spinal canal therefore cannot be correctly appreciated and consequently it was difficult or impossible to assess spinal stenosis. (orig.)

Childhood angular kyphosis: a plea for involvement of the pediatric neurosurgeon.

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Cornips, E; Koudijs, S; Vles, J; van Rhijn, L

2017-06-01

Childhood angular kyphosis is rare, as most children are affected by a mixed kyphotic and scoliotic deformity. Published series involving a mix of kyphosis and kyphoscoliosis, pediatric and adult, congenital and acquired cases are almost exclusively authored by orthopedic surgeons, suggesting that (pediatric) neurosurgeons are not involved. We present five cases that illustrate the spectrum of angular kyphosis, and these were treated by a multidisciplinary team including child neurologist, orthopedic surgeon, and pediatric neurosurgeon as complementary partners. Angular kyphosis is a cosmetic problem but above all a serious threat to the spinal cord and as such to the child's ambulatory, sphincter, and genito-urinary functions. Spinal cord stretch over the internal kyphosis may cause pain and/or neurological deficit, often accompanied by myelomalacia or even segmental cord atrophy. Spinal cord function may be additionally affected by associated disorders such as syringomyelia or tethered cord, an orthopedic surgeon may be less familiar with. The decision when and how to proceed surgically should be made by a multidisciplinary team, including a pediatric neurosurgeon who actively participates in the operation and helps to safely achieve adequate spinal cord decompression and stabilization. Childhood angular kyphosis is a complex, heterogeneous disorder that should be managed by a multidisciplinary team in specialized pediatric spine centers. While every case is truly unique, the spinal cord is always at risk, especially during decompression, stabilization, and eventual correction of deformity. Pediatric neurosurgeons have an important role to play in preoperative work-up, actual operation, and follow-up.

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.

Ogilvie′s syndrome following posterior spinal arthrodesis for scoliosis

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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.

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.

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

Science.gov (United States)

Plaum, Pål-Erik; Riemer, Gunnar; Frøslie, Kathrine Frey

2006-01-01

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. PMID:17196099

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

Science.gov (United States)

Plaum, Pål-Erik; Riemer, Gunnar; Frøslie, Kathrine Frey

2006-12-29

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. 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. 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. 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.

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)

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.)

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

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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.)

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.

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)

Acute Presentation of Chiari I Malformation with Hemiparesis in a Pediatric Patient.

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Miranda, Stephen P; Kimmell, Kristopher T; Silberstein, Howard J

2016-01-01

Chiari I malformation (CM-I) is defined by cerebellar tonsillar herniation through the foramen magnum. Patients typically present with chronic complaints, including headache, dizziness, and numbness, although there are few reports in the literature of pediatric patients presenting acutely with neurological deficit caused by CM-I. We report a child who presented acutely with hemiparesis and magnetic resonance imaging findings consistent with CM-I and spinal cord edema. A 3-year old boy with normal development presented with difficulty walking and increased drooling. His mother stated he was running into objects and had balance issues for several days. Neurological examination showed ataxia with falling to the right after a few steps and weakness of the right arm and leg. His medical history was remarkable only for mild asthma, although he had recently been treated for an upper respiratory viral infection. Computed tomography of the head demonstrated no brainstem mass. Magnetic resonance imaging of the head and cervical spine showed tonsillar ectopia approximately 2 cm below the craniocervical junction with increased T2 signal in the spinal cord from C1 to C3 consistent with syringomyelia and cord edema. The patient underwent suboccipital craniectomy with removal of the posterior arch of C1 and dural patch graft. His postoperative course was unremarkable, with complete resolution of his symptoms at his 1-month follow-up visit. This case highlights an unusual presentation of CM-I with neurological deficit related to spinal cord edema, possibly precipitated by the "water-hammer" effect of this patient's coughing fits. Providers should be aware of the acute presentations of CM-I. Copyright © 2016 Elsevier Inc. All rights reserved.

Clinicopathologic Features and Magnetic Resonance Imaging Findings in 24 Cats With Histopathologically Confirmed Neurologic Feline Infectious Peritonitis.

Science.gov (United States)

Crawford, A H; Stoll, A L; Sanchez-Masian, D; Shea, A; Michaels, J; Fraser, A R; Beltran, E

2017-09-01

Feline infectious peritonitis (FIP) is the most common infectious central nervous system (CNS) disease in the cat and is invariably fatal. Improved means of antemortem diagnosis is required to facilitate clinical decision making. Information regarding the magnetic resonance imaging (MRI) findings of neurologic FIP currently is limited, resulting in the need for better descriptions to optimize its use as a diagnostic tool. To describe the clinicopathologic features and MRI findings in cases of confirmed neurologic FIP. Twenty-four client-owned cats with histopathologic confirmation of neurologic FIP. Archived records from 5 institutions were retrospectively reviewed to identify cases with confirmed neurologic FIP that had undergone antemortem MRI of the CNS. Signalment, clinicopathologic, MRI, and histopathologic findings were evaluated. Three distinct clinical syndromes were identified: T3-L3 myelopathy (3), central vestibular syndrome (7), and multifocal CNS disease (14). Magnetic resonance imaging abnormalities were detected in all cases, including meningeal contrast enhancement (22), ependymal contrast enhancement (20), ventriculomegaly (20), syringomyelia (17), and foramen magnum herniation (14). Cerebrospinal fluid was analysed in 11 cases; all demonstrated a marked increase in total protein concentration and total nucleated cell count. All 24 cats were euthanized with a median survival time of 14 days (range, 2-115) from onset of clinical signs. Histopathologic analysis identified perivascular pyogranulomatous infiltrates, lymphoplasmacytic infiltrates, or both affecting the leptomeninges (16), choroid plexuses (16), and periventricular parenchyma (13). Magnetic resonance imaging is a sensitive means of detecting neurologic FIP, particularly in combination with a compatible signalment, clinical presentation, and CSF analysis. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American

Asymptomatic Chiari Type I malformation: should patients be advised against participation in contact sports?

Science.gov (United States)

Spencer, Robert; Leach, Paul

2017-08-01

Chiari type I malformation (CM-I) is characterised by caudal displacement of the cerebellar tonsils through the foramen magnum, crowding the craniocervical junction. It is being increasingly diagnosed in asymptomatic patients due to the widespread availability of MRI, and there are case reports of these patients suffering sudden death or neurological injury following head or neck trauma, raising the issue of whether they should be prohibited from contact sport participation, given the likelihood of frequent trauma. General neurosurgical opinion is that patients who are symptomatic and those with an associated spinal cord syrinx should be offered foramen magnum decompression, however asymptomatic patients without syringomyelia are not offered this in the majority of cases. The authors performed a full review of the published literature, including all case reports, case series, studies and literature reviews regarding CM-I and either contact sports or trauma, excluding patients that had undergone surgical intervention and those becoming symptomatic in circumstances other than head or neck trauma. 21 case reports of CM-I patients deteriorating following trauma were identified, including four cases of sudden death following head or neck injury. However, studies of large samples of CM-I patients are yet to capture an incident of sudden death or acute neurological deterioration, suggesting that the risk is very low. CM-I patients may have an increased risk of concussion and post-concussion syndrome compared to the background population however. Overall, the authors feel that there should be no restriction of sports participation for CM-I patients, but a discussion to make them and their families aware of the possible increased risks is important.

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

Suitability of foramen magnum measurements in sex determination and their clinical significance.

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Tellioglu, A Metin; Durum, Y; Gok, M; Karakas, S; Polat, A G; Karaman, C Z

2018-01-01

The foramen magnum provides a transition between fossa cranii posterior and canalis vertebralis. Medulla oblongata, arteria vertebralis and nervus accessorius spinal part pass through the foramen magnum. In this study, we aimed to make the morphometric measurements of the foramen magnum on computed tomography (CT) and to determine the feasibility of sex determination based on these measurements. Besides sex determination, from a clinical aspect, it is important to know the measurements of the foramen magnum in the normal population in terms of diseases characterised by displacement of the posterior fossa structures through foramen magnum to upper cervical spinal canal such as Chiari malformations and syringomyelia. All the data for our study was obtained retrospectively from 100 patients (50 males, 50 females) who had a CT scan of the head and neck region in Adnan Menderes University Hospital, Department of Radiology. To examine the foramen magnum in each and every occipital bone, we measured the foramen magnum's anteroposterior diameter, transverse diameter, the area of the foramen magnum and its circumference. We found that men have a higher average value than women in our study. According to Student's t-test results; in all measured parameters, there is significant difference between the genders (p discriminant function test is performed for all four measurements, the discrimination rate is 64% for all women, 70% for all men and 67% for both genders. As a result of our study, the metric data we obtained will be useful in cases where the skeletons' sex could not be determined by any other methods. We believe that, our study may be useful for other studies in determining of sex from foramen magnum. Our measurements could give some information of the normal ranges of the foramen magnum in normal population, so that this can contribute to the diagnosis process of some diseases by imaging. (Folia Morphol 2018; 77, 1: 99-104).

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

One stage surgical treatment for scoliosis associated with intraspinal abnormalities

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Kai WANG

2017-09-01

Full Text Available Objective To evaluate the effectiveness and safety of one stage surgical treatment for scoliosis and coexisting intraspinal abnormalities. Methods The data of 6 patients who underwent one stage surgical treatment for scoliosis and coexisting intraspinal abnormalities from October 2016 to January 2017 were retrospectively analyzed. Treatment for intraspinal abnormalities, posterior correction, osteotomy and internal fixation were performed simultaneously. The clinical and radiologic presentations, operative details, complications and postoperative outcomes were evaluated. Results The success rate was 100%. The operating time was (470.83 ± 136.20 min and intraoperative bleeding amount was 1350 (625, 2150 ml. Total fusion segments were 11.00 ± 2.76. Both Cobb angle of scoliosis [postoperation (19.60 ± 5.94° vs. preoperation (59.40 ± 14.31°, P = 0.007] and kyphosis [postoperation (25.80 ± 10.87° vs. preoperation (62.40 ± 21.04°, P = 0.005] were improved after operation. Tethered cords were released and epidermoid cyst, ganglioglioma and lipoma were excised. Syringomyelia was left untreated. No neurological functional defect or worsening was found. Muscle strength of all patients was improved. Muscular tone of 4 patients and difficulty in urination of 5 patients were also improved. The mean hospital stay was (8.83 ± 3.31 d. No severe complications, such as infection, cerebrospinal fluid (CSF leakage, failed internal fixation, fractured pedicle screws or rods occurred after operation. None of the patients died, or experienced deterioration of neurological function, delayed infection, pseudoarthrosis, or loss correction during the (7.50 ± 1.22 months follow - up. Conclusions The one stage surgical treatment for scoliosis and intraspinal abnormalities seems to be a safe and effective approach. Neurological functional defect can be improved after operation. Osteotomy can improve correction result. DOI: 10.3969/j.issn.1672-6731.2017.09.011

Giant encephalocele: a study of 14 patients.

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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.

Shape of the feline cerebellum and occipital bone related to breed on MRI of 200 cats.

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Huizing, Xander; Sparkes, Andy; Dennis, Ruth

2017-10-01

Objectives The MRI features of the feline cerebellum and occipital bone have not previously been described in the literature. The aims of this study were three-fold. Firstly, to document variations in cerebellar shape on MRI in neurologically normal cats to support our hypothesis that crowding of the contents of the caudal fossa or herniation of the cerebellar vermis through the foramen magnum occurs frequently as an anatomical variant. Secondly, to document variations in the morphology of the occipital bone. Thirdly, to see whether these variations in shape of the feline cerebellum and occipital bone could be associated with head conformation, such as brachycephaly. Methods The imaging records of the small animal clinic at the Animal Health Trust between 2000 and 2013 were searched retrospectively to identify adult cats that had undergone high-field (1.5 T) MRI investigation which included the brain. Exclusion criteria included evidence of intracranial disease or the presence of cervical syringomyelia. Midline sagittal T2-weighted and transverse images were used to assess the occipital bone morphology and cerebellar shape, and to measure the width to length ratio of the cranial cavity. Results Fourteen different breeds were represented. A cerebellar shape consistent with crowding of the contents of the caudal fossa, or herniation through the foramen magnum was present in 40% of the entire population. Persians (recognised as a brachycephalic breed) had a higher proportion of cerebellar crowding or herniation than all other breeds. There was no significant difference in the distribution of occipital bone morphology between these breed groups. Conclusions and relevance It is important to recognise morphological variations of the feline cerebellum and occipital bone in order to avoid false-positive diagnoses of raised intracranial pressure and pathological herniation on MRI.

Relationship between Cough-Associated Changes in CSF Flow and Disease Severity in Chiari I Malformation: An Exploratory Study Using Real-Time MRI.

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Bezuidenhout, A F; Khatami, D; Heilman, C B; Kasper, E M; Patz, S; Madan, N; Zhao, Y; Bhadelia, R A

2018-05-10

Currently no quantitative objective test exists to determine disease severity in a patient with Chiari I malformation. Our aim was to correlate disease severity in symptomatic patients with Chiari I malformation with cough-associated changes in CSF flow as measured with real-time MR imaging. Thirteen symptomatic patients with Chiari I malformation (tonsillar herniation of ≥5 mm) were prospectively studied. A real-time, flow-sensitized pencil-beam MR imaging scan was used to measure CSF stroke volume during rest and immediately following coughing and relaxation periods (total scan time, 90 seconds). Multiple posterior fossa and craniocervical anatomic measurements were also obtained. Patients were classified into 2 groups by neurosurgeons blinded to MR imaging measurements: 1) nonspecific Chiari I malformation (5/13)-Chiari I malformation with nonspecific symptoms like non-cough-related or mild occasional cough-related headache, neck pain, dizziness, paresthesias, and/or trouble swallowing; 2) specific Chiari I malformation (8/13)-patients with Chiari I malformation with specific symptoms and/or objective findings like severe cough-related headache, myelopathy, syringomyelia, and muscle atrophy. The Spearman correlation was used to determine correlations between MR imaging measurements and disease severity, and both groups were also compared using a Mann-Whitney U test. There was a significant negative correlation between the percentage change in CSF stroke volume (resting to postcoughing) and Chiari I malformation disease severity ( R = 0.59; P = .03). Mann-Whitney comparisons showed the percentage change in CSF stroke volume (resting to postcoughing) to be significantly different between patient groups ( P = .04). No other CSF flow measurement or anatomic measure was significantly different between the groups. Our exploratory study suggests that assessment of CSF flow response to a coughing challenge has the potential to become a valuable objective noninvasive

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

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

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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.

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

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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 (Pregression analysis of total resection-related factors 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 Mc

Intraspinal neural axis abnormalities in severe spinal deformity: a 10-year MRI review.

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Zhang, Ying; Xie, Jingming; Wang, Yingsong; Bi, Ni; Li, Tao; Zhang, Jie; Zhao, Zhi; Ou, Hua; Liu, Siyuan

2018-02-14

Documents indicated that the average prevalence of intraspinal neural axis abnormalities (INAAs) in presumed idiopathic scoliosis (PIS) patients was about 17.7%. However, paucity study focuses on the incidence of INAAs in severe spinal deformity (SSDs). In this study, we investigate the incidence of intraspinal neural axis abnormalities (IINAAs) and the clinical relevance in SSD at a single center. All the patients with SSDs admitted for spinal surgery were evaluated from 2003 to 2014. patients who present with coronal Cobb over 90° (and/or the sagittal Cobb ≥ 90°); patients with whole spine magnetic resonance imaging (MRI) done preoperatively; and patients with documented clinical findings preoperatively. ankylosing spondylitis, adult onset scoliosis, scoliosis secondary to bone destruction, and spinal dysraphism. 101 patients fulfilled the criteria were included. 43 patients were detected with INAAs (42.6%, 43/101). The most common INAAs was syrinx (S) (16/43, 37.2%). Of which, 43.7% (7/16), 37.5% (6/16), and 18.7% (3/16) were spindle, slit, and swelling types, respectively. Most of them were located in thoracic (6/16, 37.5%) and cervical (5/16, 31.3%) region. MRI revealed Chiari malformation with syringomyelia (C + S) in ten patients (10/43, 23.2%), Chiari malformation (C) in 6 patients (6/43, 13.9%) and others in 11 patients (11/43, 25.6%). As to the etiology, most patients with INAAs were PIS (34/43, 79.1%). On clinical examination, 16 of 101 patients (16/101, 15.8%) had abnormal neurologic signs. 15 of 16 patients (15/16, 93.7%) with abnormal neurologic signs had INAAs on MRI. On the other hand, 28 of 43 patients (28/43, 65.1%) with INAAs on MRI presented neurologically intact. 28 of 85 patients (28/85, 32.9%) with neurologically intact were detected with INAAs on MRI. The incidence of INAAs in SSDs was 42.6%. 65.1% of them present intact neurologic status. The most common neural anomaly was syrinx. Preoperative whole spine MRI must be beneficial for

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

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

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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

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

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

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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

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-thoracic level (1

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

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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

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

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.

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