Cases reported "Muscle Weakness"

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1/85. MRI of the spinal cord in myelopathy complicating vitamin B12 deficiency: two additional cases and a review of the literature.

    Focal spinal cord lesions have been present in all previously reported cases of MRI appearances in myelopathy complicating vitamin B12 deficiency. We describe two further cases showing mild atrophy only and review the salient features of the previous 11 publications. MRI findings reflect quite closely the known pathological changes in this condition.
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2/85. Neurological deficit following spinal anaesthesia: MRI and CT evidence of spinal cord gas embolism.

    A 62-year-old diabetic woman developed permanent neurological deficits in the legs following spinal anaesthesia. MRI showed oedema in the spinal cord and a small intramedullary focus of signal void at the T10 level, with negative density at CT. Intramedullary gas bubbles have not been reported previously among the possible neurological complications of spinal anaesthesia; a combined ischaemic/embolic mechanism is hypothesised.
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3/85. Cervical cord tethering mimicking focal muscular atrophy.

    Spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. skin lesion at the nuchal area should be carefully looked for.
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4/85. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis.

    We describe 4 patients with acute disseminated encephalomyelitis (ADEM) who were treated with intravenous immunoglobulins (IVIg) after getting no immediate response from a 3-5 day course of high dose intravenous methylprednisolone. All had clinical features to suggest poor prognosis and MRI findings to indicate extensive white matter changes in the brain. Two patients who had spinal cord involvement as well, required ventilatory support during acute phase of the illness. All the 4 patients recovered dramatically. Recovery pattern suggested that IVIg might be useful in fulminant ADEM. Further trials are needed to look for the efficacy of IVIg alone and in combination with methylprednisolone in the treatment of ADEM.
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5/85. electrodiagnosis in spinal cord injured persons with new weakness or sensory loss: central and peripheral etiologies.

    OBJECTIVE: To assess the prevalence and causes of late neurologic decline of persons with spinal cord injury (SCI). DESIGN: Retrospective review of persons with SCI over a 9-year period. Those with complaints of new weakness or sensory loss were grouped into three categories based on clinical examination, electrodiagnosis, and imaging: (1) central pathology (ie, brain, spinal cord, or nerve root); (2) peripheral pathology (plexus or peripheral nerve); or (3) no identifiable etiology. The specific diagnoses of late neurologic decline were identified. SETTING: Regional veterans Affairs Spinal Cord Injury Service. patients: Five hundred two inpatient and outpatient adults with SCI. RESULTS: Nineteen percent of the study population complained of new weakness and/or sensory loss. Neurologic abnormalities were noted in 13.5%, 7.2% with central and 6.4% with peripheral causes. The most common pathologies were posttraumatic syringomyelia (2.4%) and cervical (1.6%) and lumbosacral (1.2%) myelopathy/radiculopathy. A specific etiology was not determined in 6 cases (1.6%). Peripheral involvement was mostly from ulnar nerve entrapment (3.4%) and carpal tunnel syndrome (3.0%). CONCLUSIONS: Late-onset neurologic decline is common after SCI and can result from central or peripheral pathology. Regular neurologic monitoring of SCI patients is recommended, since many with neurologic decline respond favorably if diagnosed and treated early.
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6/85. Hereditary neuropathy and vocal cord paralysis in a man with childhood diphtheria.

    We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessitating nocturnal ventilatory support. His examination suggested a distal symmetric sensorimotor neuropathy, and his brother was similarly affected, although to a lesser degree. electromyography (EMG) and nerve conduction studies revealed this process to be purely axonal. A diagnosis of possible hereditary motor and sensory neuropathy (HMSN) type IIc, hereditary axonal polyneuropathy with vocal cord paralysis, is proposed, although the question of early diphtheritic involvement of the vocal cords and peripheral nerves is also considered.
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7/85. Irreversible spinal cord injury as a complication of subarachnoid ethanol neurolysis.

    ARTICLE ABSTRACT: Subarachnoid neurolysis using ethanol to destroy selectively the posterior roots of the spinal cord is a method for providing pain relief in patients with advanced cancer. Weakness of the extremities is a complication of the procedure that has been attributed to spread of the neurolytic agent to the anterior roots. The authors provide evidence of spinal cord injury as a cause of lower extremity weakness in a patient after subarachnoid ethanol neurolysis.
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8/85. Spinal intramedullary cavernoma: clinical presentation and surgical outcome.

    OBJECT: Improved neuroimaging techniques have led to an increase in the reported cases of intramedullary cavernomas. The purpose of this study was to define the spectrum of presenting signs and symptoms in patients with spinal intramedullary cavernomas and to analyze the role of surgery as a treatment for these lesions. methods: The authors reviewed the charts of 16 patients who underwent surgery for spinal intramedullary cavernomas. All patients underwent preoperative magnetic resonance imaging studies. Cavernomas represented 14 (5.0%) of 280 intramedullary lesions found in adults and two (1.1%) of 181 intramedullary lesions found in pediatric cases. A posterior laminectomy and surgical resection of the malformation were performed in all 16 patients. CONCLUSIONS: magnetic resonance imaging is virtually diagnostic for spinal cavernoma lesions. patients with spinal intramedullary cavernomas presented with either an acute onset of neurological compromise or a slowly progressive neurological decline. Acute neurological decline occurs secondary to hemorrhage within the spinal cord. Chronic progressive myelopathy occurs due to microhemorrhages and the resulting gliotic reaction to hemorrhagic products. There is no evidence that cavernomas increase in size. The rate of rebleeding is unknown, but spinal cavernomas appear to be clinically more aggressive than cranial cavernomas, probably because the spinal cord is less tolerant of mass lesions. Complete surgical removal of the cavernoma was possible in 15 of 16 of the authors' cases.
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9/85. A viable residual spinal hydatid cyst cured with albendazole. Case report.

    Spinal hydatid disease is a rare entity that frequently yields to severe, acute-onset neurological deficits. Although the gold standard treatment is total surgical removal of the cysts without inducing any spillage, it may not be possible to perform this in patients with multiple and fragile cysts. In such cases, the neural structures should be adequately decompressed and albendazole should be administered promptly. The authors describe the case of a 13-year-old girl who was admitted with a history of back pain and acute-onset lower-extremity weakness. magnetic resonance imaging scans demonstrated severe spinal cord compression caused by multiple cysts involving T-4 and the mediastinum. The patient underwent surgery, and the cysts were removed, except for one cyst that was hardly exposed. Following histopathological confirmation of spinal hydatid disease, she was treated with albendazole for 1 year. One year postoperatively, the residual cyst had gradually shrunk and had almost disappeared. Although a single case is not sufficiently promising, we believe that administration of albendazole is efficient to prevent recurrences in cases in which it is not possible to obtain total removal of the cysts without inducing spillage.
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10/85. Clinical predominance of proximal upper limb weakness in CMT1A syndrome.

    We report an Austrian family with proximal muscle weakness and wasting predominantly of the shoulder girdle musculature, normal or slightly reduced distal muscle power, mild foot deformity, absent or reduced tendon reflexes in the lower limbs, and normal or slightly diminished sensation. Electrophysiologically, motor nerve conduction velocities were slowed to less than 33 m/s, distal latencies were prolonged, and compound motor action potentials were low. Sensory nerve conduction velocities were extremely reduced or no sensory potentials were recordable. genetic testing in three affected individuals revealed a duplication of the chromosomal region 17p11.2. In addition, genetic testing for facioscapulohumeral muscular dystrophy (FSHD) revealed a 33 kb EcoRI fragment on chromosome 4q35 in one affected individual and in the clinically normal parent, whereas in a second affected person normal dna-sizes were observed. These clinical findings define a new phenotypic variant associated with the Charcot-Marie-tooth 1A duplication. This may be due to a mutation in another gene contained in the 1.5 Mb duplication although mutations in the peripheral myelin protein 22 gene have been excluded. Alternatively, the genetic background of other genes in the family may modify the phenotypic expression, as found in other inherited diseases.The unusual phenotype cannot be explained by the concomitant presence of FSHD despite some evidence for coexistance in one individual.
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