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Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder, in which antibodies against voltage-gated calcium channels located at nerve terminals cause muscle weakness and autonomic dysfunction. In approximately half of the patients the autoimmune process is initiated by a tumor. In the other half of patients no tumor is found and the etiology is unknown. The aims of this study were to investigate the strength of HLA-associations with nontumor LEMS (NT-LEMS) and to study the relation of HLA-haplotypes with age at onset of LEMS and other clinical features. Therefore, typing of FILA class I and II was performed in 19 patients with NT-LEMS, who were clinically evaluated. NT-LEMS was significantly associated with alleles of both HLA-class I (i.e. HLA-BS) as well as -class II (i.e. HLA-DR3 and -DQ2). HLA-B8+ patients had significantly younger age at onset of LEMS and tended to be female. This study shows that HLA-class I haplotype is associated with a distinct phenotype in NT-LEMS. Human Immunology 62 809-813 (2001). (C) American Society for Histocompatibility and Immunogenetics, 2001. Published by Elsevier Science Inc.

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33 percent) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37 percent, myopathic in 40 percent, and a combination in 23 percent of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40 percent and 60 percent of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58 percent, 53 percent and 79 percent respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFR75 were also produced locally (IL-1 in 71 percent, IFN- in 40 percent, IL-12 in 73 percent, TNFR75 in 90 percent). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96 percent of the biopsies. HLA-DR, TNFR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

Contains fulltext : 138256.pdf (Publisher’s version ) (Open Access) BACKGROUND: For persons with multiple sclerosis (MS) it is important to preserve their autonomy, in spite of increasing disability. A major factor mediating autonomy is self-efficacy. According to the social cognitive theory stressors are crucial determinants of self-efficacy, as well as the interaction with partners. METHODS: In an explorative observational study we assessed in 47 persons with MS (PwMS) the effect of an intense, multidisciplinary, 3-day, social cognitive wellness program with the participation of support partners, after 1, 3 and 6 months. Primary outcomes: self-efficacy-control and -function (Multiple Sclerosis Self-Efficacy Scale [MSSES]),limitations to and problems with participation and autonomy (Impact on Participation and Autonomy [IPA] scale). Secondary outcomes: health-related quality of life (HRQoL) (MS Quality of Life-54 Items [MSQoL-54] questionnaire), anxiety, depression (Hospital Anxiety and Depression Scale [HADS]), and fatigue (Modified Fatigue Impact Scale-5 Items [MFIS-5]). Disability was measured with the Expanded Disability Status Scale (EDSS). Percentage changes from baseline were tested with T-tests, level of significance 0.05. RESULTS: In the whole group the MSQoL-54 Mental score was increased at 1, 3 and 6 months (+16.0%, +13.2%, +12.2%), and the MSQoL-54 Physical (+10.2%) at 6 months, with no changes in other outcomes. The relapsing remitting (RR) subgroup (n = 20) had at 6 months an increase in the MSSES-Control score (+24.8%) and in the MSQoL54 Mental and Physical scores (+22.3%, +17.6%). Progressive patients (n = 22) only showed an increase in the MSQoL-54 Mental score (+11.5%) at 1 month. In the low-disability (EDSS < 4.0) subgroup the MSSES-Control score was increased (+23.8%) at 6 months, and the IPA-Limitations and -Problems scores decreased at 3 months (-6.1%, -8.8%); the MSQoL-54 Mental score had increased at 1, 3 and 6 months (+19.3%, +21.5%, +19.3%). In the high-disability (EDSS > =4.0) subgroup no significant changes occurred. CONCLUSIONS: Results from this observational study suggest that 6 months after an intense, 3-day, multidisciplinary, social cognitive wellness program with support partners, PwMS with a RR course or low disability may experience an improved self-efficacy-control and HRQoL.

We present a 42-year-old woman without a known connective tissue disorder, who sought treatment at the emergency department because of sudden right-sided deafness, vertigo, and tendency to fall to the right. She also reported right-sided neck pain for 3 days. Neurologic examination, performed 1 day after the onset of symptoms, revealed total deafness on the right side. There were no other abnormalities, including no nystagmus (also not present after use of Frenzel glasses) and no ataxia. An MRI scan showed a small cerebellar infarction in the territory of the right posterior inferior cerebellar artery (PICA) on the T2-weighted image (figure, …

Objective: To determine the influence of clinical, laboratory, and electrodiagnostic factors on the prognosis of Guillain-Barre syndrome (GBS). Background: Identification of prognostic factors may lead to better selection of patients with a poor prognosis for new therapeutic trials. Methods: The authors studied 147 patients with GBS who participated in the Dutch GBS trial comparing the effect of IV immunoglobulins with plasma exchange (PE). Outcome was measured at 8 weeks because half of the patients had recovered independent locomotion by then and at 6 months, the endpoint of the study. Results: Multivariate logistic regression revealed the following factors predicting outcome (inability to walk independently) at 8 weeks: a preceding gastrointestinal illness (yes, no), age (≥50, Conclusions: The main predictors of outcome in GBS are clinical factors. Diarrhea is an important poor predictor of outcome, especially for the PE-treated group, and a recent CMV infection predicts delayed early recovery.

Reply from the Authors: Our primary aim of the study was not to confirm the association of a recent cytomegalovirus (CMV) infection with the Guillain-Barre syndrome (GBS), which has already been established7-9 and confirmed by us using age, sex, and seasonal matched neurologic controls. Virus-specific antibodies against CMV were found in 20 (15%) of 134 GBS patients and in only three(