1/10. A case of the subacute brainstem encephalitis.A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/10. Recurrent miller fisher syndrome: clinical and laboratory features and hla antigens.In rare cases, miller fisher syndrome (MFS) has been known to recur. However, clinical features of recurrent MFS have not been well analyzed, and the precipitating factors relating to recurrence remain unknown. From 1981 to 1996, we examined four patients with recurrent MFS among 28 Japanese MFS patients. In the four patients, the recurrent episodes occurred after long asymptomatic intervals, ranging from 2.5 to 12.5 years. The clinical and laboratory features of recurrent episodes were similar either to those of the initial episodes or to those of the 24 non-recurrent patients. Of the two patients tested for serum IgG anti-GQ1b antibody, both were positive. Serological HLA typing showed that all recurrent patients were both HLA-Cw3 and -DR2 positive. However, out of 13 non-recurrent patients examined, six had HLA-Cw3, and four had HLA-DR2. The frequency of HLA-DR2 among the recurrent patients was significantly higher than among healthy controls (corrected P = 0.038), and was also higher than among the non-recurrent patients but not significantly. These findings suggest that recurrent MFS is clinically the same as typical MFS and that HLA-DR2 is possibly associated with recurrence.- - - - - - - - - - ranking = 4keywords = antigen (Clic here for more details about this article) |
3/10. Anti-Ri-associated paraneoplastic opsoclonus-ataxia syndrome in a man with transitional cell carcinoma.BACKGROUND: There are several case reports describing paraneoplastic syndromes in patients with various forms of bladder carcinoma. Current immunologic analyses have enabled the identification of the antineuronal autoantibodies associated with specific syndromes. methods: A patient with a history of bladder carcinoma presented with opsoclonus and myoclonus. RESULTS: Workup confirmed the presence of anti-Ri antibodies in the patient's serum and cerebrospinal fluid. The target Ri antigen was found to be expressed by the tumor. CONCLUSIONS: To the authors' knowledge, there are few reports in the literature describing the long-term clinical follow-up and postmortem evaluation in a patient with this form of paraneoplastic syndrome. More important, the authors believe the current study represents the first time that the presence of anti-Ri antibodies has been noted in a paraneoplastic syndrome associated with transitional cell carcinoma of the bladder.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/10. Quantification of circulating anti-Hu antibody in serial samples from patients with paraneoplastic neurological syndromes: possible correlation of antibody concentration and course of neurological symptoms.Serial samples, spanning an observation period of 4 to 10 years, from five patients with anti-Hu associated paraneoplastic neurological syndromes (PNS) were investigated with an enzyme linked immunosorbent assay (ELISA) employing recombinant HuD protein as antigen. In one patient the anti-Hu antibody concentration converted from negative to highly positive levels after the onset of neurological symptoms. These findings argue in favour of the idea that an autoimmune process, which is generated at the beginning of the neurological disease, causes the anti-Hu associated PNS. serum samples obtained shortly after the beginning of the PNS were available from two other patients. The anti-Hu antibody levels in these latter patients increased from modest to highly positive anti-Hu antibody in follow up samples. In two patients a clear decrease of the anti-HuD antibody concentration together with an improvement of paraneoplastic neurological symptoms after successful tumour treatment were seen. overall these data suggest that there might be a correlation between the anti-HuD antibody level and the clinical course of paraneoplastic neurological symptoms which supports further the hypothesis that an autoimmune process is responsible for the anti-Hu associated paraneoplastic neurological symptoms.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/10. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments.We identified the IgG autoantibody ANNA-2 ("anti-Ri") in 34 patients in a 12-year period by immunofluorescence screening of sera from approximately 75000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality. Cancer anteceded neurological symptoms in 4 of 28 patients. Cancer detection frequency increased with continued surveillance. Neurological disorders, in decreasing frequency, were brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, peripheral neuropathy, cranial neuropathy, movement disorder, encephalopathy, Lambert-Eaton syndrome, and seizures. Four patients had laryngospasm and four had jaw opening dystonia (two with neck dystonia). Nine (32%) were wheelchair-bound 1 month after neurological symptom onset. Most improved neurologically after immunomodulatory or tumor-directed therapy. Accompanying autoantibodies, found in 73% of sera, included ANNA-1, ANNA-3, CRMP-5-IgG, P/Q-type and N-type Ca(2 ) channel antibodies, and muscle-type acetylcholine receptor antibody. Some neurological accompaniments of ANNA-2 may reflect potentially pathogenic humoral or cell-mediated responses to coimmunogenic tumor antigens, for example, Lambert-Eaton syndrome (P/Q-type Ca(2 ) channel antibody) and peripheral neuropathy (ANNA-1 effector T cells).- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/10. Ataxic polyneuropathy and anti-Pr2 IgM kappa M proteinemia.A case of ataxic neuropathy associated with IgM kappa M proteinemia is reported. Double filtration plasmapheresis effectively treated the neuropathy. The IgM kappa antibody had anti-Pr2 cold agglutinin activity. We demonstrated reactivities of the IgM kappa antibody to sialosyl paragloboside, sialosyl lactosaminyl paragloboside, GT1b, GD1a, GD1b, GM3 and GD3 on high-performance thin-layer chromatography immunostaining and enzyme-linked immunosorbent assay, which is previously unreported antigenic specificity of the M proteins in cases of paraproteinemic neuropathy. IgM M protein with anti-Pr2 cold agglutinin activity may play a pathogenetic role in peripheral nerve demyelination, because the target antigens of the M protein are present in myelin and possibly in endothelial cells of the peripheral nervous system.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/10. Treatable gait disorder and polyneuropathy associated with high titer serum IgM binding to antigens that copurify with myelin-associated glycoprotein.We studied clinical and electrodiagnostic features of 9 patients with very high titers (> 1:10,000) of serum IgM binding to a CNS myelin antigen (CMA) preparation that copurified with myelin-associated glycoprotein (MAG). We found that 8 of the 9 patients had a combined syndrome of gait ataxia and polyneuropathy (GAPN) with late-age onset (mean = 70 years of age). In the 8 GAPN patients progressive difficulty with ambulation led to significant functional disability and frequent falling. Examination showed a wide-based unsteady gait, especially when standing still or turning. There was mild-to-moderate distal sensory loss with involvement of joint position sense only in the toes. Motor changes, when present, were mild and mainly involved distal leg musculature. Treatment of 5 GAPN patients resulted in clear improvement of 2 after intravenous human immunoglobulin and of 3 others after other immunodulating agents. Immune-mediated GAPN syndromes with high titers of serum IgM binding to CMA appear to be treatable causes of gait disorders in older patients.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
8/10. Anti-gabaergic neuron autoantibodies in a patient with stiff-man syndrome and ataxia.We describe a patient with autoimmune insulin-dependent diabetes whose original symptoms of trunk stiffness and rigidity of the abdomen were followed three years later by a pancerebellar syndrome. An autoantibody (autoAb) against gabaergic neurons was found in the patient's serum and cerebrospinal fluid (CSF); on Western blot, this autoAb recognized a 64-kDa antigen of cerebellar protein. The detection of this antibody in a case with ataxia suggests that a spectrum of different neurological diseases may be observed in patients harbouring anti-GABAergic neuron autoAb and supports the concept that factors other than autoAb contribute to the clinical presentation of these disorders.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/10. Antineuronal antibody in a patient with neuroblastoma and opsoclonus-myoclonus-ataxia: a case report.We describe a case of a patient with neuroblastoma and opsoclonus- myoclonus ataxia displaying serum and CSF anti-Hu antibodies that were able to recognize antigens of the patient's own tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/10. Intraventricular perineurioma: case report.OBJECTIVE AND IMPORTANCE: Perineurioma, a rare benign nerve sheath neoplasm occurring in either an intraneural or soft tissue form, has never been reported to arise in the central nervous system. CLINICAL PRESENTATION: We describe the clinical, radiological, and pathological features of a perineurioma arising in the choroid plexus of the third ventricle in a 65-year-old woman and causing obstructive hydrocephalus. INTERVENTION: The lesion, apparently unassociated with a nerve, was gross totally resected by frontal craniotomy using a left-sided transcallosal approach. Short-term follow-up showed no evidence of recurrence. CONCLUSION: Perineurioma of the variety found in soft tissue may occur in the central nervous system, wherein it shows the typical light microscopic, immunohistochemical (epithelial membrane antigen- and collagen IV-positive, S-100 protein-negative), and ultrastructural (pinocytotic vesicles, discontinuous basement membrane) features.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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