1/80. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/80. polyradiculopathy in sarcoidosis.We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, except a single case of cervical polyradiculopathy. Of 14 treated patients, nine improved with corticosteroids, laminectomy, or both. polyradiculopathy complicating sarcoidosis: (1) is uncommon; (2) primarily involves thoracic and lumbar roots; (3) may arise from contiguous, hematogenous, or gravitational nerve root sleeve seeding; (4) may be asymptomatic; and (5) may improve with corticosteroids. Differential diagnosis of weakness in patients with sarcoidosis should include nerve root involvement from the primary process by direct sarcoid involvement.- - - - - - - - - - ranking = 1.6459061466108keywords = nervous system, central nervous system (Clic here for more details about this article) |
3/80. Chronic hepatitis B exacerbated by guillain-barre syndrome: a report of two cases.Several neurologic complications involving both the central and peripheral nervous systems due to acute viral hepatitis have been described, but Guillain-Barre (G-B) syndrome occurring as a complication of chronic viral hepatitis is extremely rare. Although it is generally agreed that G-B syndrome develops as an immune-mediated reaction, its exact pathogenesis remains obscure. We report the cases of two patients with chronic hepatitis b virus (HBV) infection acutely exacerbated by the development of G-B syndrome. G-B syndrome was diagnosed by nerve conduction velocity studies, electromyographic studies and a rise in acellular total protein in the cerebrospinal fluid (albumino-cytologic dissociation). In these two patients, we were able to accurately define the relationship between the onset of acute exacerbation of chronic HBV infection and G-B syndrome. The neurologic symptoms of G-B syndrome resolved with the return of liver enzymes to normal. Interferon therapy may be beneficial in relieving neurologic symptoms in patients with HB infection-related G-B syndrome.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/80. Encephalomyeloradiculoneuropathy following exposure to an industrial solvent.A 19-year-old male developed complaints including weakness of the lower extremities and right hand, numbness, dysphagia and urinary difficulties following a 2 month exposure to an industrial solvent constituted mainly of 1-bromopropane, but also containing butylene oxide, 1,3 dioxolane, nitromethane, and other components. Nerve conduction studies revealed evidence of a primary, symmetric demyelinating polyneuropathy. Evidence of CNS involvement came from gadolinium enhanced MRI scans of the brain, showing patchy areas of increased T2 signal in the periventricular white matter, similar scans of the spinal cord revealing root enhancement at several lumbar levels, and SSEP studies. The patient's symptoms had started to resolve following the discontinuation of the exposure, before he was lost to follow-up. Similar findings have been reported following 1-bromopropane exposure in rats. I hypothesize that this patient's symptoms may have been due to 1-bromopropane-induced neurotoxicity.- - - - - - - - - - ranking = 0.033946413223357keywords = brain (Clic here for more details about this article) |
5/80. magnetic resonance imaging of meningoradiculomyelitis in early disseminated Lyme disease.lyme disease, a multisystem illness caused by the spirochete borrelia burgdorferi, is the most common vector-borne disease in the united states. There are 3 clinical stages of lyme disease: early localized, early disseminated, and late persistent disease. Neuroborreliosis, infection of the nervous system by B. burgdorferi, may occur during early disseminated or late persistent disease. spinal cord involvement in early disseminated disease is extremely rare. In patients with early disseminated neuroborreliosis, treatment with antibiotics often leads to rapid recovery and may prevent further complications of lyme disease. The authors present the clinical and radiographic findings, both before and after treatment, in a patient with meningoradiculomyelitis due to early disseminated lyme disease.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/80. The differentiation of peripheral effector neuron failure from acute brain stem dysfunction in a critically ill patient.A patient appeared to be in coma following pneumococcal meningitis, an intracerebral haemorrhage, and a cardiac arrest. Late in the course of his illness neurophysiological investigations confirmed a proposal that he also had a fulminant acute demyelinating polyneuropathy which, for a period of five days, rendered the patient completely unresponsive. The patient recovered and is now working without any disability.- - - - - - - - - - ranking = 0.13578565289343keywords = brain (Clic here for more details about this article) |
7/80. Polyneuritis cranialis: clinical and electrophysiological findings.A 13 year old boy, developed bilateral facial weakness, dysphonia and dysphagia acutely after a febrile illness. Neurological examination and MRI of the brain were normal. The CSF protein level increased. Blink reflex monitoring during clinical recovery was consistent with demyelination of the lower cranial nerves innervating the branchial arch musculature, a rare variant of guillain-barre syndrome.- - - - - - - - - - ranking = 0.033946413223357keywords = brain (Clic here for more details about this article) |
8/80. Acute severe combined demyelination.We present a second case in which guillain-barre syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) appeared simultaneously, both in acute and fulminant form. The patient, a 10-year-old girl, presented with acute onset of coma and flaccid, are-flexic quadriparesis. The elevated CSF protein levels and delayed F waves fulfilled the criteria of GBS and an MRI study revealed extensive multifocal demyelination compatible with a diagnosis of ADEM. Prompt clinical response followed by complete recovery was achieved by treatment with corticosteroids. It is suggested that acute severe combined demyelination might constitute a separate entity in which the demyelinating process, involving simultaneously the central and the peripheral nervous systems, indicates immune response against a component of the myelin of one system carrying cross-antigenicity with the other.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/80. central nervous system expression of a monoclonal paraprotein in a chronic lymphocytic leukemia patient.An unusual complication of chronic lymphocytic leukemia (CLL) is reported. The patient, a 79-year-old man, had a long standing history of CLL, that had been complicated by the development of a Guillain-Barre-like syndrome and a peripheral biclonal gammopathy. The biclonal immunoglobulins identified in the serum were IgM lambda and IgG lambda. The patient's condition progressed and he eventually developed ophthalmologic complications. cerebrospinal fluid (CSF) obtained during evaluation of his visual dysfunction contained numerous small, mature lymphocytes consistent with the presence of CLL cells in the central nervous system (CNS); immunoperoxidase staining of these cells revealed a monoclonal population. Protein electrophoretic evaluation of the patient's CSF showed a single monoclonal band and immunofixation electrophoresis of the CSF revealed that the immunoglobulin present was IgG lambda. No evidence for the monoclonal IgM paraprotein identified in serum could be appreciated in the CSF by immunofixation. Taken together, these findings strongly implied that there was CNS involvement by the leukemia and this process caused the patient's neurologic symptoms. Furthermore, this study demonstrates that chronic lymphocytic leukemia should also be considered as one of the hematopoietic malignancies associated with monoclonal gammopathies involving the CNS.- - - - - - - - - - ranking = 5.6459061466108keywords = nervous system, central nervous system (Clic here for more details about this article) |
10/80. Neurologist as expert witness.Prevailing liberal rules of evidence permit qualified medical and scientific experts to offer opinions designed to help courts decide issues to which their expertise relates. The opinions can be based on direct examinations, review of data assembled by others and data or inferences of a type relied on by other experts in the field. Application of these rules is illustrated through analysis of expert testimony in litigation involving a neurologic syndrome allegedly caused by an immunization and in a case involving controversy over the extent and outcome of major brain injury. Concerns about misuse of expert medical and scientific testimony in litigation are addressed. The article closes with a consideration of approaches designed to improve the reliability of expert testimony.- - - - - - - - - - ranking = 0.033946413223357keywords = brain (Clic here for more details about this article) |
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