1/51. 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) |
2/51. 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 = 1keywords = nervous system (Clic here for more details about this article) |
3/51. 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) |
4/51. 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) |
5/51. 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) |
6/51. 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 = 5keywords = nervous system (Clic here for more details about this article) |
7/51. Acute polyradiculoneuropathy occurring after hymenoptera stings: a clinical case study.hymenoptera stings may be responsible for both local and systemic reactions; these can be immediate or delayed, depending on the time between the sting and the development of signs or symptoms. Delayed clinical reactions have been reported, although unusual, due to serum sickness and/or affecting organs or systems generally not involved in the immediate reaction, such as heart, kidneys, central and peripheral nervous systems. This paper describes the clinical and immunological findings in a 51-year-old subject, who, after two stings of paper wasps, the second one after the third venom immunotherapy (VIT) injection, presented immediate large local and systemic allergic reactions which quickly improved after e.v. methylprednisolone administration. About 40 hours later, he developed acute polyradiculoneuropathy with muscle weakness, paresthesia, difficulties in standing up and walking. skin tests and specific IgE determination showed allergy to paper wasp. The activation, by wasp venom, of peripheral blood mononuclear cells in primary culture, evaluated by tritiated thymidine incorporation proliferation assay, showed an important hypersensitivity to wasp venom. Therefore our results suggest the hypothesis that the polyradiculoneuritis causative etiopathogenetic mechanism might be a delayed immunological response to wasp antigens followed by an allergy-triggered autoimmune reaction, as previously suggested by other authors; they found lymphocytic infiltrates in demyelinization areas and at perivascular levels, by histologic examination of autoptical and bioptical material of patients with nervous system lesions after hymenoptera stings.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
8/51. Acute inflammatory demyelinating polyneuropathy following bone marrow transplantation.Acute inflammatory demyelinating polyneuropathy (AIDP) appeared in two patients following allogeneic bone marrow transplantation (BMT). In one transplanted patient (but not in the donor) T cells were sensitized against peripheral nervous system myelin. This could reflect a change in the T cell repertoire in a different milieu. In this context, AIDP might be part of graft-versus-host disease or another associated autoimmune disorder following BMT.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/51. guillain-barre syndrome during childhood.guillain-barre syndrome is an acquired disease of the peripheral nervous system. The etiology appears to be autoimmune in nature. The major clinical features are progressive weakness and loss of reflexes, with respiratory failure being a serious complication. The prognosis for guillain-barre syndrome in children is good with proper intensive care and early detection of the disease.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/51. A clinicopathological study of the guillain-barre syndrome. Nine cases and literature review.The postmortem findings are reported from 9 cases of the guillain-barre syndrome with survival between 10 days and 1 yr. In 8 cases there was multifocal loss of myelin throughout the peripheral nervous system with relative preservation of axons. In 1 case there was predominant loss of axons. Inflammatory mononuclear cell infiltration was present in the peripheral nervous system of all cases except 1 case surviving a year. The extent and severity of cell infiltration was variable, usually being less prominent than in previous reports, and sometimes sparing nerves in which myelin destruction was severe. Vesicular dissolution of myelin noted by electron microscopy was considered to be a postmortem artefact. In cases examined within 30 days after the onset, immunohistochemical studies with monoclonal antibodies identified more leucocytes (PD7/2B11 ) and T cells (UCHL1 ) in the endoneurium than in cases examined later or control cases. These findings and recent single case reports indicate that the pathology of the Guillian-Barre syndrome is variable. This variability may reflect differences in pathogenesis, with greater cell-mediated immunity in some cases and greater antibody targeted macrophage-mediated demyelination in others.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
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