1/15. encephalomyelitis-associated antimyelin autoreactivity induced by streptococcal exotoxins.OBJECTIVE: After implicating streptococcus pyogenes as causing acute disseminated encephalomyelitis (ADEM) in a child, we wanted to prove that in vivo activation of autoreactive T lymphocytes by superantigens of this Streptococcus contributed to the dramatic demyelination. BACKGROUND: ADEM is a demyelinating disorder of the CNS sharing many similarities with MS. Demyelination in MS is considered to be the result of an autoimmune process mediated by autoreactive T lymphocytes with specificity for myelin antigens. methods: Phenotypic analysis and proliferation assays on blood monocytes, as well as isolation of myelin basic protein (MBP)-reactive T-cell lines/clones; and TCR repertorium analysis by PCR-ELISA and cytokine production. RESULTS: 1) The blood T-cell receptor (TCR) repertoire was compatible with in vivo expansion induced by S. pyogenes exotoxins. 2) TCR expression analysis indicated clonal expansion of CD8 MBP-reactive T cells, suggesting in vivo activation. MBP-reactive T cells showed crossreactivity to S. pyogenes supernatant and exotoxins. 3) Cytokine mRNA quantification of the mononuclear cells revealed a Th2-biased profile. CONCLUSION: In vivo exposure to S. pyogenes may have induced activation of pathogenic myelin reactive T cells, contributing to the dramatic inflammatory demyelination.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
2/15. Motor dominant neuropathy in sjogren's syndrome: report of two cases.Most of the peripheral neuropathies in sjogren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of guillain-barre syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.- - - - - - - - - - ranking = 10.168636484077keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/15. Spinal cord edema: unusual magnetic resonance imaging findings in cervical spondylosis.OBJECT: Spinal cord edema is a rare radiological finding in chronic degenerative disorders of the spine. Between 1997 and 2001, the authors treated six patients with cervical spondylotic myelopathy in whom postoperative spinal cord edema was demonstrated. The authors describe the radiological and clinical features of this unusual condition. methods: The six patients were all men, and ranged in age from 44 to 72 years. All patients presented with mild quadriparesis and underwent laminoplasty or anterior fusion. Preoperative magnetic resonance (MR) imaging revealed marked spinal cord compression with intramedullary hyperintensity on T2-weighted sequences and spinal cord enhancement at the compression level after administration of Gd. After surgery, spinal cord edema was observed in all patients; the spinal cord appeared swollen on the postoperative MR images. Preoperative and postoperative Gd-enhanced MR imaging demonstrated clear enhancement of the white matter at the compressed segment Neurologically, five of six patients experienced good improvement of symptoms; however, the spinal cord edema as documented on follow-up MR imaging persisted for several months after surgery. CONCLUSIONS: The radiological characterization of spinal cord edema was based on the reversible white matter lesion most likely caused by disturbed local venous circulation induced by chronic spinal cord compression. Such unusual MR findings in cervical spondylotic myelopathy should be differentiated from intramedullary spinal cord tumors, demyelinating disorders, or inflammatory processes.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
4/15. Acute flaccid paralysis and west nile virus infection.Acute weakness associated with west nile virus (WNV) infection has previously been attributed to a peripheral demyelinating process (guillain-barre syndrome); however, the exact etiology of this acute flaccid paralysis has not been systematically assessed. To thoroughly describe the clinical, laboratory, and electrodiagnostic features of this paralysis syndrome, we evaluated acute flaccid paralysis that developed in seven patients in the setting of acute WNV infection, consecutively identified in four hospitals in St. Tammany Parish and new orleans, louisiana, and Jackson, mississippi. All patients had acute onset of asymmetric weakness and areflexia but no sensory abnormalities. Clinical and electrodiagnostic data suggested the involvement of spinal anterior horn cells, resulting in a poliomyelitis-like syndrome. In areas in which transmission is occurring, WNV infection should be considered in patients with acute flaccid paralysis. Recognition that such weakness may be of spinal origin may prevent inappropriate treatment and diagnostic testing.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
5/15. mycoplasma pneumoniae causing nervous system lesion and SIADH in the absence of pneumonia.A patient was admitted for fever and acute respiratory failure (ARF), rapidly progressive tetraparesis, delirium, behavioral abnormalities, and diplopia. leukocytosis and a rise in c-reactive protein were present. A syndrome of inappropriate anti-diuretic hormone secretion (SIADH) was also diagnosed. Lumbar puncture yielded colorless CFS with mononuclear pleocytosis and protein rise. electrodiagnosis revealed demyelinating polyneuropathy and axonal degeneration. serum IgG and IgM for mycoplasma pneumoniae (MP) was consistent with acute infection, and erythromycin was started with rapid resolution of symptoms. Contrarily to most reports, an associated respiratory disease was not present and SIADH in association with MP has been reported only once, in a patient without direct central nervous system (CNS) involvement. Differential diagnosis and possible pathogenic mechanisms are discussed.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
6/15. guillain-barre syndrome in the course of dengue: case report.This case report describes the findings of a 45-year-old white woman from brazil, who developed myalgia, fever and macular rash. She was diagnosed as having dengue, based on clinical manifestations and specific IgM titers. One week after the first symptoms of dengue, the patient developed muscle weakness, followed by tetraplegia with areflexia, and respiratory insufficiency. The electromyography had evidence of demyelinating neuropathy and the cerebrospinal fluid showed albuminocytologic dissociation. These neurologic findings were consistent with the diagnosis of guillain-barre syndrome. The patient was treated with immunoglobulin and metylprednisolone. Mechanical ventilation was started one week after hospital admission and maintained for four weeks. After six weeks of hospitalization the patient was discharged from the hospital on wheel chair, presenting mild muscle weakness and loss of patellar and ankle reflexes. When the patient was seen at the outpatient service three weeks after hospital discharge she was able to walk with help. This case report suggests a possible association between dengue and guillain-barre syndrome.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
7/15. Sustained remission of CIDP associated with Evans syndrome.A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) developed Evans syndrome (hemolytic anemia/thrombocytopenia) 17 months after onset of symptoms despite different immunomodulatory treatments. A therapeutic approach with the chimeric monoclonal anti-CD20 antibody rituximab induced substantial improvement of CIDP and hematologic recovery. The patient remains in sustained remission 17 months after completion of therapy. Rituximab may represent a successful therapy in otherwise refractory CIDP.- - - - - - - - - - ranking = 36.707390126822keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
8/15. Encephalopathy and peripheral neuropathy following diethylene glycol ingestion.The authors report a 24-year-old man who developed encephalopathy and rapid quadriplegia following ingestion of a solution containing diethylene glycol (DEG). As quadriparesis evolved, motor response amplitudes were markedly reduced with preserved conduction velocities. Studies during clinical recovery revealed marked motor conduction velocity slowing and prolonged distal latencies. These data indicate that DEG intoxication may cause a primary acute axonal sensorimotor polyneuropathy with demyelinating physiology during recovery.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
9/15. A fulminant case of guillain-barre syndrome: topographic and fibre size related analysis of demyelinating changes.In a necropsy case of early fulminant guillain-barre syndrome, demyelinating changes were observed throughout the peripheral nervous system, most conspicuous in the spinal nerve roots. The central/peripheral nervous system transition regions and most proximal zones of the roots escaped damage. In some root areas there were widespread early changes in myelin sheaths in the absence of significant infiltrates of inflammatory cells. In the fibre size analytical study, small myelinated fibres were preferentially involved.- - - - - - - - - - ranking = 5keywords = demyelinating (Clic here for more details about this article) |
10/15. Motor neuropathy with proximal multifocal persistent conduction block, fasciculations and myokymia. Evolution to tetraplegia.We describe a patient with chronic asymmetric motor neuropathy, which began in the upper extremity. The paretic muscles showed abundant fasciculations and myokymia but only little amyotrophy. Electrophysiologic examination revealed proximal multifocal persistent conduction block (CB) not located at the usual entrapment sites, and arrhythmic isolated or grouped fasciculation potentials originating distally on blocked axons. Over the years, new CBs developed, which led to tetraplegia, and amyotrophy slowly increased with progressive denervation. This patient differs from the cases of chronic acquired demyelinating polyneuropathy described in the literature by the absence of sensory deficit and the proximal location of CB.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
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