神经领域-神经系统自身免疫性疾病方向

METHODS::Although generally presenting as a chronic, progressive peripheral neuropathy, we present a case of acute alcoholic neuropathy initially mistaken for acute Guillain-Barré syndrome. Electrodiagnostic evaluation of alcoholic neuropathy reveals an axonal neuropathy and may be complicated by demyelination if comorbid nutritional deficiencies are present. This is in contrast to the significant demyelination classically associated with Guillain-Barré syndrome. F waves, which are absent or prolonged in Guillain-Barré syndrome, are unaffected in alcoholic neuropathy. Further evaluation with lumbar puncture demonstrates normal protein and white blood cell counts in alcoholic neuropathy, as opposed to albuminocytologic dissociation characteristically present in acute Guillain-Barré syndrome.

METHODS:OBJECTIVES:Walking impairments are common in individuals with multiple sclerosis. Trunk control is a prerequisite for walking; however, knowledge regarding whether core stability and balance training influence walking is limited. This study aimed to investigate the immediate and long-term effects of a group-based, individualized, comprehensive core stability and balance intervention (GroupCoreDIST) compared with those of standard care on walking. METHODS:This assessor-blinded, prospective randomized controlled trial included 80 participants (Expanded Disability Status Scale scores 1-6.5) randomly allocated to GroupCoreDIST, conducted in groups of three for 60 min three times per week for 6 weeks (18 sessions) or standard care (n = 40/40). One participant attended no posttests, leaving 79 subjects for intention-to-treat analysis. The assessments were performed at baseline and at Weeks 7, 18, and 30. Outcomes included the 2-min walk test (2MWT), 10-m walk test-preferred/fast/slow speed (10MWT), Multiple Sclerosis Walking Scale-12 (MSWS-12), Patient Global Impression of Change-walking (PGIC-walking), Rivermead Visual Gait Assessment (RVGA), and ActiGraphsWgt3X-BT activity monitors (ActiGraph). The statistical analyses included repeated-measures mixed models performed in IBM SPSS Version 24. RESULTS:There were no significant between-group differences in the outcome measurements at baseline. The mean differences between groups were significant at all follow-up time points in favour of GroupCoreDIST for the 2MWT, 16.7 m at 7 weeks (95% CI [8.15, 25.25], 15.08 m at 18 weeks (95% CI [6.39, 23.77]) and 16.38 m at 30 weeks (95% CI [7.65, 25.12]; and the PGIC-walking, 0.89 points at 7 weeks (95% CI [1.34, 0.45]), 0.97 points at 18 weeks (95% CI [1.42, 0.52]), and 0.93 points at 30 weeks (95% CI [1.39, 0.48]; all p ≤ .001). The 10MWT-fast speed and the MSWS-12 showed significant between-group differences at 7 and 18 weeks and the RVGA at 7 weeks. No between-group differences were found regarding activity level (ActiGraph) or the 10MWT-preferred or slow speed. CONCLUSION:Compared with standard care, GroupCoreDIST significantly improved walking immediately after the intervention for up to 24 weeks of follow-up.

METHODS:OBJECTIVE:To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective inception cohort of systemic lupus erythematosus (SLE) patients. METHODS:Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate. RESULTS:Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow-up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 [41.0%]), mononeuropathy (44 of 161 [27.3%]), and cranial neuropathy (39 of 161 [24.2%]), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF-36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF-36 summary scores for peripheral neuropathy and mononeuropathy. CONCLUSION:PNS disease is an important component of total NPSLE and has a significant negative impact on health-related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution.

METHODS:OBJECTIVE:To assess how postural sway deficits during eyes open and closed relate to the integrity of cerebellar peduncles in individuals with multiple sclerosis (MS). DESIGN:Cross-sectional study. SETTING:Laboratory based setting. PARTICIPANTS:Twenty-nine adults with MS (Expanded Disability Status Scale: 2-4) and 15 adults without MS were recruited (N=44). Inclusion criteria for all participants were ability to maintain balance independently by standing on toes for 3 seconds, and no known biomechanical conditions affecting balance. INTERVENTIONS:Not applicable. MAIN OUTCOME MEASURES:Postural sway using body-worn, inertial sensors during quiet standing, integrity of cerebellar peduncles quantified using diffusion-tensor imaging and clinical assessment scales for ataxia and balance. RESULTS:Radial diffusivity of the inferior cerebellar peduncle was related to postural sway measures during both eyes open and closed. In contrast, radial diffusivity of the superior cerebellar peduncle was related to postural sway only in stance with eyes open. CONCLUSIONS:The inferior cerebellar peduncle, which carries somatosensory information to the cerebellum, contributes to control of standing balance with or without visual inputs, consistent with the high dependence on somatosensory information for posture. The superior cerebellar peduncle, which carries cortical information to the cerebellum, contributes to control of standing posture only when vision is available. Radial diffusivity of the inferior cerebellar peduncle was related to reactive balance control, whereas radial diffusivity of the superior cerebellar peduncle was related to the kinetic component of the ataxia rating scale.

METHODS:PURPOSE:To propose a new method of identifying clusters in multifocal electrophysiology (multifocal electroretinogram: mfERG; multifocal visual-evoked potential: mfVEP) that conserve the maximum capacity to discriminate between patients and control subjects. METHODS:The theoretical framework proposed creates arbitrary N-size clusters of sectors. The capacity to discriminate between patients and control subjects is assessed by analysing the area under the receiver operator characteristic curve (AUC). As proof of concept, the method is validated using mfERG recordings taken from both eyes of control subjects (n = 6) and from patients with multiple sclerosis (n = 15). RESULTS:Considering the amplitude of wave P1 as the analysis parameter, the maximum value of AUC = 0.7042 is obtained with N = 9 sectors. Taking into account the AUC of the amplitudes and latencies of waves N1 and P1, the maximum value of the AUC = 0.6917 with N = 8 clustered sectors. The greatest discriminant capacity is obtained by analysing the latency of wave P1: AUC = 0.8854 with a cluster of N = 12 sectors. CONCLUSION:This paper demonstrates the effectiveness of a method able to determine the arbitrary clustering of multifocal responses that possesses the greatest capacity to discriminate between control subjects and patients when applied to the visual field of mfERG or mfVEP recordings. The method may prove helpful in diagnosing any disease that is identifiable in patients' mfERG or mfVEP recordings and is extensible to other clinical tests, such as optical coherence tomography.

METHODS:BACKGROUND:The revised 15-item Myasthenia Gravis (MG) Quality of Life Questionnaire (MGQoL15R) is a validated scale of quality of life in patients with MG. We aimed to study the factors causing the variability within the Arabic version of the MGQoL15R (MGQoL15R-A). METHOD:A standardized questionnaire was completed by 118 patients. Correlations and hierarchical regression analyses were used to assess the contribution of sociodemographic variables, clinical factors, Patient Health Questionnaire-9 (PHQ9-A), and Generalized Anxiety Disorder-7 (GAD7-A) to the variability in the MGQoL15R-A. RESULTS:The MGQoL15R-A was highly correlated with PHQ9-A (r = 0.76), and moderately correlated with GAD7-A (r = 0.52). Clinical factors and PHQ9-A independently explained 30.4% and 34.5% of the variability, respectively. Among the clinical factors, uncontrolled MG status, relapse within the past year, and a higher number of current MG therapies were significantly associated with a higher MGQoL15R-A score. CONCLUSIONS:MG severity and depressive symptoms (measured by PHQ9-A) can affect the MGQoL15R-A score.

METHODS::Thymic small cell cancer is a very rare type of thymic epithelial tumor. Lambert-Eaton myasthenic syndrome is a rare paraneoplastic syndrome associated with thymic epithelial tumors. We report an extremely rare case of Lambert-Eaton myasthenic syndrome associated with thymic small cell carcinoma. A 71-year-old man was referred to our institution for a mediastinal tumor and a 2-month history of ptosis, fatigue, and gait disorder. Based on radiologic findings thymoma associated with Lambert-Eaton myasthenic syndrome was diagnosed, and extended thymectomy was performed. After surgery the patient's symptoms had not improved. Anticholinesterase treatment alleviated his symptoms.

METHODS:BACKGROUND:Multiple sclerosis (MS) is a chronic, inflammatory, central nervous system demyelinating disease that requires long-term use of disease-modifying therapies (DMT). Patient adherence to DMT is key in reducing the inflammation that leads to relapses and neurodegeneration. Dimethyl fumarate (DMF) poses unique challenges to adherence including being the only twice-daily dosing DMT. Previous research suggests there are direct roles that providers play on improving their patients' adherence rates, such as focusing on the patient-provider relationship, helping put the patient at ease so that they feel understood and respected. Also, route of administration affects adherence in other chronic healthcare conditions. However, the issue of adherence to DMT in MS is more complex than just route of administration, with adverse effects being the main predictor of adherence. OBJECTIVES:(1) To define various patient specific factors (e.g. fatigue and mood disorders) that affect adherence with DMF and (2) to understand how patients' perceptions of treatment satisfaction (such as effectiveness, convenience, side effects and global satisfaction) and DMFs impact on quality of life (such as social support, activities of daily living, coping) influence adherence. METHODS:Our study was a prospective, observational measurement of adherence to treatment with DMF in MS patients over 52 weeks. Twenty-five out of thirty-five patients enrolled completed the study. Adverse event (AE) data was reviewed on all participants. RESULTS:Adherence rates correlated with patient's perceived effectiveness (0.25, p < 0.023) and the level of bothersome symptoms the patient experienced (0.45, p < 0.0001). The majority of new AE onset was reported within 12 weeks of DMF initiation. This is consistent with previously published data with DMF use. CONCLUSION:Adherence rates are an important factor to be considered when starting patients on DMT. DMF creates its own barriers to adherence with our study highlighting some, including twice-daily dosing and AEs experienced following treatment initiation. Healthcare providers should be aware of these barriers prior to treatment initiation and counsel patients appropriately.

METHODS:OBJECTIVE:To compare the presence of head, neck and upper extremity symptoms in patients with Takayasu's (TAK) and giant cell arteritis (GCA) and their association with vascular inflammation assessed by 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) or arterial damage assessed by magnetic resonance angiography (MRA). METHODS:Patients with TAK and GCA underwent clinical and imaging assessments within 24 hours, blinded to each other. Vascular inflammation was defined as arterial FDG-PET uptake greater than liver by visual assessment. Arterial damage was defined as stenosis, occlusion, or aneurysm by MRA. Clinically reported symptoms were compared with corresponding imaging findings using generalised mixed model regression. Cranial symptoms were studied in association with burden of arterial disease in the neck using ordinal regression. RESULTS:Participants with TAK (n=56) and GCA (n=54) contributed data from 270 visits. Carotidynia was reported only in patients with TAK (21%) and was associated with vascular inflammation (p<0.01) but not damage (p=0.33) in the corresponding carotid artery. Posterior headache was reported in TAK (16%) and GCA (20%) but was only associated with corresponding vertebral artery inflammation and damage in GCA (p<0.01). Arm claudication was associated with subclavian artery damage (p<0.01) and inflammation (p=0.04) in TAK and with damage in GCA (p<0.01). Patients with an increased burden of damaged neck arteries were more likely to experience positional lightheadedness (p<0.01) or a major central nervous system event (p=0.01). CONCLUSION:The distribution of symptoms and association with imaging abnormalities differs in patients with TAK and GCA. These findings may help clinicians predict associated FDG-PET and MRA findings based on a specific clinical symptom. CLINICAL TRIAL REGISTRATION NUMBER:NCT02257866.

METHODS:OBJECTIVE:To provide a meta-analysis of articles that have included the timed 25-foot walk (T25FW) in persons with multiple sclerosis (MS), quantify differences in T25FW scores between those with MS and controls without MS, and quantify differences between categories of disability status and clinical disease courses within MS. DATA SOURCES:The literature search was conducted using 4 databases (Google Scholar, PubMed, Cumulative Index to Nursing and Allied Health, EBSCO Host). We searched reference lists of published articles to identify additional articles. STUDY SELECTION:A systematic literature search identified articles reporting average T25FW performance in seconds between those with MS and controls without MS, between those with MS who had mild and moderate and/or severe disability status, and between relapsing-remitting and progressive clinical courses of MS. DATA EXTRACTION:Information was extracted and categorized based on reported data: comparisons of controls without MS and MS, comparisons of mild and moderate and/or severe MS based on study-defined Expanded Disability Status Scale groups, and comparisons of relapsing-remitting and progressive MS clinical courses. DATA SYNTHESIS:We performed a random effects meta-analysis to quantify differences between groups as estimated by effect sizes (ESs). We expressed the differences in Cohen d as well as the original units of the T25FW (ie, seconds). CONCLUSIONS:There was a large difference in T25FW performance in MS compared with controls without MS (ES=-0.93, mean difference=2.4s, P<.01). Persons with moderate and/or severe disability walked substantially slower compared with mild disability (ES=-1.02, mean difference=5.4s, P<.01), and persons with progressive courses of MS walked substantially slower than relapsing-remitting MS (ES=-1.4, mean difference=13.4s, P<.01).