Search

Background: Neuromyelitis optica spectrum disorders (NMOSD) typically lead to spastic paraparesis and spare the peripheral nervous system (PNS)., Case Report: Here, we describe an anti-aquaporin-4-seropositive NMOSD patient suffering from acute transverse myelitis with painful, flaccid paralysis and incontinence of urine and feces. Due to the involvement of the PNS as indicated by electrodiagnostic examination, we verified the expression of aquaporin-4-channels on the proximal dorsal spinal radix of rats by staining rat tissue with human NMOSD serum., Conclusion: This case suggests a manifestation of the proximal PNS in NMOSD. Thus, NMOSD should be considered as a differential diagnosis for patients presenting with signs of spinal cord disease and additional radicular involvement., (Copyright © 2018. Published by Elsevier B.V.)

We report a 76-year-old MS patient, treated with DMF for 3 years. Lymphocytes never showed values below 1240/µl. CSF analysis revealed 1,988,880 copies/ml of JCV-DNA, JCV-DNA was detectable in serum and anti-JCV-antibody in CSF and serum were highly positive. Stratify®-JCV-test was positive. CD8-positive T-lymphocytes were reduced. Therapy with mefloquine, mirtazapine and cidofovir resulted in complete elimination of the virus in serum and 90% reduction of viral load in CSF. This case shows that despite careful monitoring for lymphopenia JCV spreading to the CSF may occur during treatment with DMF.

Objective: Although statin pretreatment (SP) is associated with better outcomes in patients with acute cerebral ischemia after an ischemic stroke/transient ischemic attack, data on the underlying mechanism of this beneficial effect are limited., Approach and Results: We sought to evaluate the potential association between SP and microembolic signal (MES) burden in acute cerebral ischemia because of large artery atherosclerosis (LAA). We prospectively evaluated consecutive patients with first-ever acute cerebral ischemia because of LAA in 3 tertiary stroke centers over a 2-year period. All patients underwent continuous 1-hour transcranial Doppler monitoring of the relevant vessel at baseline (≤24 hours). SP was recorded and dichotomized as high dose or low-to-moderate dose. SP was documented in 43 (41%) of 106 LAA patients (mean age, 65.4±10.3 years; 72% men; low-to-moderate dose, 32%; high dose, 8%). There was a significant ( P =0.022) dose-dependent effect between SP and MES prevalence: no SP (37%), SP with low-to-moderate dose (18%), and SP with high dose (0%). Similarly, a significant ( P =0.045) dose-dependent effect was documented between SP and MES burden: no SP (1.1±1.8), SP with low-to-moderate dose (0.7±1.6), and SP with high dose (0±0). In multivariable logistic regression analysis adjusting for demographics, vascular risk factors, location of LAA, stroke severity, and other prevention therapies, SP was associated with lower likelihood of MES presence (odds ratio, 0.29; 95% confidence interval, 0.09-0.92; P =0.036). In addition, SP was found also to be independently related to higher odds of functional improvement (common odds ratio, 3.33; 95% confidence interval, 1.07-10.0; P =0.037)., Conclusions: We found that SP in patients with acute LAA is related with reduced MES presence and lower MES burden with an apparently dose-dependent association., (© 2017 American Heart Association, Inc.)

Fatigue is a usual reaction to prolonged performance but also a major symptom in various neuroimmunological diseases. In neurosarcoidosis fatigue is a core symptom, but little is known about the relevance of fatigue on cognitive functions in this disease. Previous results in healthy subjects suggest that fatigue strongly affects cognitive control processes. However, fatigue is not a uni-dimensional construct but consists of different facets. It is unknown which of these facets are most important for mechanisms of cognitive control. In the current study we investigate conflict monitoring and response selection processes in neurosarcoidosis patients as a 'model disease' of fatigue and healthy controls in relation to the impact of 'cognitive' and 'motor fatigue' on these processes using event-related potentials (ERPs). We focus on ERPs reflecting attentional selection (P1, N1) and conflict monitoring/response selection processes (N2). ERPs reflecting attentional selection processes were unchanged. The N2 on incompatible trials was reduced in neurosarcoidosis suggesting that response selection and conflict monitoring functions are dysfunctional. Of note, fatigue strongly modulates responses selection processes in conflicting situations (N2) in controls and neurosarcoidosis, but the effect of fatigue on these processes was stronger in neurosarcoidosis. Neuroimmunological parameters like TNF-α and soluble interleukin-2 receptor serum concentrations do not seem to modulate the pattern of results. Concerning fatigue it seems to be the 'cognitive' dimension and not the 'motor' dimension that is of relevance for the modulation of response selection in conflicting situations., (Copyright © 2015 Elsevier B.V. and ECNP. All rights reserved.)

Neurosarcoidosis is a rare central nervous system manifestation of sarcoidosis. T cell, T-helper cell and macrophage activation via the major histocompatibility complex (MHC) II-mediated pathway causes this disease. Little is known about the possible cognitive disturbances in this disease as most reported instances are case studies. Here, we provide the first in-depth analysis of psychomotor functions in a sample of 30 neurosarcoidosis patients. We investigated action control processes using a paradigm that is able to examine how different tasks are cascaded to achieve the task goal. We integrated electrophysiological (EEG) data with behavioural and neuroimmunological data. Our results show that there was no general cognitive decline in patients with neurosarcoidosis. Patients only presented deficits when two response options have to be prioritized. Patients apply an inefficient processing strategy where they try to processes different response options in parallel. The electrophysiological data show that the deficits are due to dysfunctions at the response selection stage. Behavioural and neurophysiological changes are predictable on the basis of soluble interleukin 2 receptor serum concentrations. The results show that neurosarcoidosis is not associated with nonspecific changes in cognitive functions but does lead to specific alterations in cognitive control that are strongly dependent on immunological parameters., (Copyright © 2014 Elsevier Inc. All rights reserved.)