Cases reported "Demyelinating Diseases"

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1/9. Childhood vertigo: a case report and review of the literature.

    vertigo is a relatively uncommon pediatric complaint, with correct diagnosis made even more challenging by the patient's inherent difficulty in describing his or her symptoms. confusion may exist among vertigo, dizziness, dysequilibrium, ataxia, pre-syncope or syncope, and seizure activity by both the pediatric patient and his or her family. A complete history and physical examination, as well as appropriate ancillary studies, may assist in clarifying the diagnosis. This paper attempts, via case report and literature review, to discuss the various etiologies of acute vertigo in the pediatric population, provide diagnostic clues, and evaluate some of the available diagnostic modalities.
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2/9. Monofocal acute inflammatory Demyelination (MAID): a unique disorder simulating brain neoplasm.

    BACKGROUND: Monofocal acute inflammatory demyelinating (MAID) lesions present as large masses that mimic brain tumors. The natural history and nosologic classification are not well defined. methods: Five patients with MAID from our files and 4 from the literature are presented. We defined MAID as a mass-like lesion, >4 cm in diameter. Demyelination was pathologically proven in all patients. RESULTS: Headaches and motor deficits were the most common presenting symptoms. language problems and motor deficits were the most common findings on physical examination. After a median follow-up of 44 months, none of our 5 patients had multiple sclerosis. In similar cases, additional lesions indicative of multiple sclerosis developed within an average of 8 months. CONCLUSIONS: It may be difficult to distinguish MAID from brain tumor. It may be that MAID represents a unique form of isolated demyelinating disease, or it may represent transition to multiple sclerosis.
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3/9. A misleading case of central pontine myelinolysis. risk factors for psychiatric patients.

    Central pontine myelinolysis (CPM) is an uncommon disorder initially described in alcoholic or malnourished patients. Recent reports suggest an aetiological association with abnormalities of serum sodium. A physically unwell non-alcoholic chronic schizophrenic patient, whose symptoms led to psychiatric referral, died of CPM. A review of the literature reveals that psychiatric patients may indeed be at risk for CPM.
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4/9. Monofocal acute inflammatory demyelination manifesting as open ring sign. Case report.

    A 48-year-old woman with a history of viral influenza infection was admitted with rapidly progressive numbness and weakness of the right extremities. On admission, general physical examination revealed no abnormality. cerebrospinal fluid analysis showed no abnormal findings. brain computed tomography and magnetic resonance imaging showed an open ring-like enhanced lesion in the white matter of the right parietal lobe with massive perifocal edema. cerebral angiography showed no tumor staining and thallium-201 single photon emission computed tomography showed no abnormal uptake. The preoperative diagnosis was malignant glioma and partial resection was performed. Histological examination showed perivascular accumulation of small lymphocytes and a large number of macrophages with reactive astrocytes. phagocytosis of myelin was observed in the macrophages and nuclear fragmentation in the reactive astrocytes. The histological diagnosis was acute inflammatory demyelinating disease. After therapy with methylprednisolone, her neurological symptoms improved gradually and no relapse occurred during 18 months of follow up. Tumor-like masses of demyelination may occupy an intermediate position between acute multiple sclerosis and postinfectious encephalitis. Open ring sign may be a pathognomonic feature of these lesions.
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5/9. Serial evaluation of neuromuscular function in management of chronic inflammatory demyelinating polyneuropathy. A case report.

    In this case report, we describe the serial evaluation of neuromuscular function of a woman with chronic inflammatory demyelinating polyneuropathy and document the return of limb strength and hand function after plasmapheresis and immunosuppressive therapy. The assessment of muscle strength and functional abilities by physical therapists can provide timely, objective, and quantitative information that is important to the medical management of patients with neurologic disease.
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6/9. Neuronal and vascular disorders of the brain and spinal cord in Menkes kinky hair disease.

    A 3 2/12-year-old boy had recurrent seizures, chronic respiratory infection, and delayed physical and mental development. He also had low plasma copper content typical of Menkes syndrome. autopsy showed marked neuronal loss and gliosis in most areas of the cerebral and cerebellar cortices, midbrain, pons, and medulla. The spinal cord showed severe demyelination in both ascending (spinocerebellar) and descending (lateral corticospinal) tracts from the cervical to the sacral level. In addition to these neuronal lesions, both the meningeal and parenchymal arterial and venous branches were remarkably dilated in the brain and spinal cord. Our previous study of this case showed abnormal perivascular innervation and abnormal axonal swelling of the postganglionic adrenergic fibers elsewhere in the body. The metabolic disorder caused by copper deficiency induces severe neuronal degeneration that is apparently exaggerated by extensive and progressive vascular abnormality.
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7/9. Lack of a correlation between demyelinating plaques on MRI scan and clinical recovery in multiple sclerosis by treatment with electromagnetic fields.

    A 50 year-old woman presented in January of 1995 with a prolonged history of symptoms of multiple sclerosis (MS) and was classified at the time with a remitting-progressive course. Her chief symptoms included slurring of speech, impairment of vision with intermittent diplopia, difficulties with gait and balance with spastic-ataxic gait, mental depression, insomnia, fatigue, impaired cognitive functions notably poor short term memory and recurrent urinary tract and sinus infections. An MRI scan showed multiple nodular demyelinating lesions scattered in the subcortical white matter and periventricularly of both cerebral hemispheres. Over the following 18 months, while receiving three treatment sessions per week with picotesla electro-magnetic fields (EMFs) which were applied extracranially, she showed a significant recovery in both physical and mental symptoms and additionally experienced decreased susceptibility to infections. In addition, the course of her disease appeared to have stabilized as opposed to the preceding 5 years during which time she experienced insidious, steady deterioration in her functioning. Despite this remarkable clinical recovery through the application of EMFs, and MRI scan obtained at the same diagnostic center 18 months after initiation of treatment with EMFs showed no changes in the number and size of the demyelinating plaques. These findings demonstrate lack of a correlation between recovery of symptoms and the number and extent of demyelinating plaques on MRI scan. It has been known since the days of Charcot in the latter half of the 19th century that in MS there is a great disparity between the histopathological changes of the disease and neurologic deficits. This report enhances the notion that demyelination may reflect an epiphenomenon of the disease.
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8/9. Peripheral neuropathy in the nondiabetic patient.

    The differential diagnosis includes an array of acquired and hereditary disorders. The history often offers the richest source of information, which can then direct the physical examination and electrodiagnostic testing. Further studies may include nerve biopsy and, most recently, blood testing for genetic disorders. Some neuropathies may respond to treatment; indeed, the response to treatment may be diagnostic.
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9/9. marchiafava-bignami disease with widespread extracallosal lesions and favourable course.

    We report a 61-year-old alcoholic man who presented with subacute physical deterioration and severe dysarthria. MRI, suggestive of corpus callosum demyelination with associated white matter involvement in both cerebral hemispheres, indicated the diagnosis of marchiafava-bignami disease. During his stay in hospital the patient showed remarkable improvement, and was discharged 22 days after admission. On MRI 2 months later, the extracallosal lesions had disappeared. This case raises questions about some previous ideas on this disease, such as the prognosis of its acute forms and the significance of the extracallosal lesions seen on neuroimaging.
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