Cases reported "Syringomyelia"

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1/413. Post-meningitic hydrocephalus and syringomyelia treated with a ventriculoperitoneal shunt.

    Following cryptococcal meningitis, symptoms of cervical syringomyelia developed in a young heroin addict. myelography confirmed syringomyelia and angiography demonstrated severe hydrocephalus. Ventriculoperitoneal shunting resulted in complete resolution of signs and symptoms of both hydrocephalus and syringomyelia.
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keywords = syringomyelia
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2/413. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.

    BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. methods: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.
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ranking = 1.7142857142857
keywords = syringomyelia
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3/413. The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.

    OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.
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ranking = 1.4473359164622
keywords = syringomyelia, hydrosyringomyelia
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4/413. shoulder enlargement as the presenting sign in syringomyelia. Report of two cases and review of the literature.

    Two adult patients had painful enlargement of the shoulder and complete destruction of the humeral head. Angiograms of the shoulder showed highly vascular lesions in both patients. Both patients had syringomyelia, for which the swollen shoulder was the first manifestation.
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ranking = 0.71428571428571
keywords = syringomyelia
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5/413. A neurodystrophic syndrome resembling carbohydrate-deficient glycoprotein syndrome type III.

    A 10-month old girl is described with a serum transferrin isoform abnormality of the same kind as in two previously reported girls with carbohydrate-deficient glycoprotein syndrome type III. This patient presented with joint abnormalities and rapidly developing hypsarrhythmia, hypotonia, psychomotor delay and growth retardation. fingers, toes, nails and local skin were dysmorphic. She had pale optic discs, thoracic syringomyelia and frontal lobe atrophy at three months. The CDT value in serum was greatly elevated. Several carbohydrate-deficient isoforms were found in transferrin (four), alpha1-antitrypsin (three), antithrombin (two) and thyroxine-binding globulin (four). Mutations in the CDGS 1-gene were excluded. The CDGS III glycoprotein abnormality most probably represents a distinct disorder of glycoprotein metabolism, and needs to be considered in unclear hypsarrhythmia with developmental delay. Dysmorphic features may be added to this syndrome.
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ranking = 0.14285714285714
keywords = syringomyelia
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6/413. Syringomyelic dystonia and athetosis.

    Two patients with movement disorders associated with syringomyelia are described, one of whom developed unusual torticollis, and the other had choreoathetoid-dystonic movements of the hand and arm. In each case, the movements resolved with decompression of the syrinx. The literature is reviewed and possible mechanisms explored.
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ranking = 0.14285714285714
keywords = syringomyelia
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7/413. syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.

    OBJECTIVE: To describe a patient from Southeast asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.
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ranking = 0.28571428571429
keywords = syringomyelia
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8/413. arnold-chiari malformation with syringomyelia in an elderly woman.

    PRESENTATION: A 76-year-old woman, complaining of leg pain and unsteady gait for 3 years, presented with a spastic paraparetic gait, severe spasticity and touch, thermal and pain sensory loss limited to arms, lower thorax and upper abdomen. brain and spinal cord magnetic resonance imaging showed a large loculated syrinx. Cerebellar tonsillar herniation into the foramen magnum was also seen (arnold-chiari malformation, type I). OUTCOME: The patient had successful cervico-spinal surgical decompression which resulted in marked reduction in hypertonia and weakness, normal gait and normal joint movement at 6 months. CONCLUSION: This unusual, late clinical presentation of a congenital disease underlines the importance of a comprehensive diagnostic work-up in the elderly patients with complex neurological signs.
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ranking = 0.57142857142857
keywords = syringomyelia
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9/413. Extensive arachnoid ossification with associated syringomyelia presenting as thoracic myelopathy. Case report and review of the literature.

    The authors present the case of progressive thoracic myelopathy caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and results of the literature search, they describe a pathological basis for this rare condition, discuss its incidence and symptomatology, and suggest a simple classification for various types of the arachnoid ossification. They also discuss the magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. The particular value of plain computerized tomography, which is highly sensitive in revealing intraspinal calcifications and ossifications, in the diagnostic evaluation of patients with a clinical picture of progressive myelopathy is emphasized.
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ranking = 0.57142857142857
keywords = syringomyelia
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10/413. multiple sclerosis and non-communicating syringomyelia: a casual association or linked diseases?

    Non-communicating syringomyelia (NCS) can be associated with different disease processes such as arachnoidytis, trauma or tumor. Approximately 12 cases, documented radiographically, of the association of non-communicating syringomyelia and multiple sclerosis (MS) have been described but their relationship remains obscure. In 3 patients with laboratory supported MS spinal magnetic resonance imaging (MRI) revealed a central cystic cavity. In 2 patients lesions on the spinal cord above the cavity were demonstrated. At 3-year follow-up in 1 patient, no change in the cavity was detected. Although 3 cases are insufficient for providing a definitive conclusion on the relationship between these two diseases, we suggest that demyelinating lesions have to be regarded as possible causes of spinal, asymptomatic cavities.
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ranking = 0.85714285714286
keywords = syringomyelia
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