Human Prion Disease
AbstractHuman prion diseases (HPDs) are progressive and fatal neurodegenerative diseases caused by abnormal prion protein (PrPSc). They can be sporadic, genetic, or acquired. Sporadic HPDs include sporadic Creutzfeldt –Jakob disease (CJD) and sporadic fatal insomnia. Genetic HPDs include genetic CJD, Gerstman–Sträussler–Scheinker disease and fatal familial insomnia. Acquired HPDs include Kuru, variant CJD, and iatrogenic CJD. The World Health Organization clinical diagnostic criteria for HPDs include clini cal findings, cerebrospinal fluid (CSF) protein markers, and electroencephalography, and the UK and European cl...
Source: Clinical and Experimental Neuroimmunology - November 25, 2021 Category: Neurology Authors: Katsuya Satoh, Tatsufumi Nakamura Tags: INVITED REVIEW Source Type: research

Issue Information
(Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - November 18, 2021 Category: Neurology Tags: Issue Information Source Type: research

Time to revisit B cells in multiple sclerosis
(Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - November 18, 2021 Category: Neurology Authors: Jin Nakahara Tags: EDITORIAL Source Type: research

Optic neuritis after Covid ‐19 vaccine application
ABSTRACTAs the COVID-19 pandemic continues to rise, the development of effective vaccines is of crucial importance to prevent further morbidity and mortality. In parallel, some rare adverse events related to COVID-19 vaccines, have been reported, most of them mild. Here we report the case of a previous healthy 19-year-old woman who developed optic neuritis 1 week after single dose of Ad26.COV2.S vaccine with marked improvement after management with steroids.Although causality cannot be confirmed due to lack of a biological marker, this case may help to guide further research for potential pathogenic mechanism. (Source: Cli...
Source: Clinical and Experimental Neuroimmunology - November 12, 2021 Category: Neurology Authors: Christian Garc ía‐Estrada, Enrique Gómez‐Figueroa, Lennyn Alban, Alejandro Arias Tags: CASE REPORT Source Type: research

A patient with generalized myasthenia gravis facing an impending crisis triggered by early fast ‐acting treatment
ConclusionsSuch acute exacerbation after only 250 mg/day of IVMP for 2 days is rare. However, caution is needed when using IVMP for EFT of MG because the risk factors for exacerbation after IVMP use, and the best approach for treating the exacerbation, are not yet known. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - November 5, 2021 Category: Neurology Authors: Kana Tamaki, Yoichiro Nishida, Nobuo Sanjo, Takanori Yokota Tags: CASE REPORT Source Type: research

Guillain –Barré syndrome after coronavirus disease 2019 vaccine: A temporal association
ConclusionsIn the absence of other causes, the diagnosis of GBS was made, with evidence of a clear temporal association with COVID-19 vaccine. However, a cautious position is important when assigning a particular side-effect directly to a vaccine. It is important to emphasize that it is a temporal association only and the benefits of COVID-19 vaccination continue to outweigh the possible consequences. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - October 28, 2021 Category: Neurology Authors: Gustavo Figueiredo Silva, Caroline Figueiredo Silva, Raddib Eduardo Noleto da Nobrega Oliveira, Fabiana Romancini, Rafael Marques Mendes, Amanda Locks, Maria Francisca Moro Longo, Carla Heloisa Cabral Moro, Alexandre Luiz Longo, Vera Lucia Braatz Tags: CASE REPORT Source Type: research

Anti ‐B cell therapies in multiple sclerosis
AbstractMultiple Sclerosis (MS) has long been considered a T-cell-mediated autoimmune disease of the central nervous system. However, newer evidence shows that B cells play a crucial role in the pathogenesis of MS via antibody production, antigen presentation, T cell stimulation and activation, and production of pro-inflammatory cytokines. Developing B cells express the CD20 molecule from the pre-B cell to the plasmablast stage, and the anti-CD20 monoclonal antibodies rituximab, ocrelizumab, ofatumumab and ublituximab are either in use or are being developed for treatment of MS as B cell-depleting therapies. They have show...
Source: Clinical and Experimental Neuroimmunology - October 19, 2021 Category: Neurology Authors: Kenji Kufukihara Tags: INVITED REVIEW Source Type: research

Guillain ‐Barré syndrome after COVID‐19 vaccine: A temporal association
ConclusionsThen, in the absence of other causes, the diagnosis of GBS was made, with evidence of a clear temporal association with COVID-19 vaccine. However a cautious position is important when assigning a particular side effect directly to a vaccine. It is important to emphasize that it is a temporal association only and the benefits of vaccination continue to outweigh the possible consequences. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - October 16, 2021 Category: Neurology Authors: Gustavo Figueiredo da Silva, Caroline Figueiredo da Silva, Raddib Eduardo Noleto da Nobrega Oliveira, Fabiana Romancini, Rafael Marques Mendes, Amanda Locks, Maria Francisca Moro Longo, Carla Heloisa Cabral Moro, Alexandre Luiz Longo, Vera Lucia Tags: CASE REPORT Source Type: research

Stiff Person Syndrome and Prior Temporal Lobectomy: Is It a Case of Misplaced Autoimmunity?
ConclusionThe diagnosis of SPS can get delayed in a patient with history of epilepsy. Although SPS and epilepsy are often linked by anti-GAD antibodies as common etiology, it is possible that epilepsy surgery might be a trigger for development of SPS in a small subset. Development of autoimmunity to neuronal GAD65 protein, exposed during surgery, is suspected in pathogenesis. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 14, 2021 Category: Neurology Authors: Ahmed Babiker, Sohum Trivedi, Klepper Alfredo Garcia, Manan Shah Tags: CASE REPORT Source Type: research

Neuro ‐COVID‐19
AbstractNeuromuscular manifestations of new coronavirus infection (COVID-19) are frequent and include dizziness, headache, myopathy, and olfactory and gustatory disturbances. Patients with acute central nervous system (CNS) disorders, such as delirium, impaired consciousness, stroke, and convulsive seizures, have a high mortality rate. The encephalitis/encephalopathy that causes consciousness disturbance and seizures can be classified into three conditions, including direct infection with the SARS-CoV-2 virus, encephalopathy caused by CNS damage secondary to systemic hypercytokinemia (cytokine storm), and autoimmune-mediat...
Source: Clinical and Experimental Neuroimmunology - September 12, 2021 Category: Neurology Authors: Takayoshi Shimohata Tags: REVIEW ARTICLE Source Type: research

Effects of exercise training with Lactobacillus plantarum intake on ionized calcium ‐binding adaptor molecule 1, glial fibrillary acidic protein, interleukin‐6 and interleukin‐1β in cuprizone‐induced demyelination mouse model of multiple sclerosis
ConclusionsExercise with the intake of probiotics improved demyelination in the cuprizone-induced demyelination mouse model of MS. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 10, 2021 Category: Neurology Authors: Donya Sajedi, Ramin Shabani, Alireza Elmieh Tags: ORIGINAL ARTICLE Source Type: research

B  cells in experimental autoimmune encephalomyelitis
Studies using experimental autoimmune encephalomyelitis have highlighted the activities of B  cells in multiple sclerosis, including the production of autoantibodies, antigen presentation to T cells, secretion of effector cytokines, recognition of microbe-associated molecules and the induction of ectopic lymphoid follicles in the meninges. AbstractB  cells have been implicated in the pathology of multiple sclerosis (MS) since initial observations in the 1940s of elevated immunoglobulin concentrations in the cerebrospinal fluid of MS patients. However, the precise mechanism of their involvement has not been elucidated un...
Source: Clinical and Experimental Neuroimmunology - September 9, 2021 Category: Neurology Authors: Yusei Miyazaki, Masaaki Niino Tags: INVITED REVIEW Source Type: research

Immune dysregulation syndrome with cytotoxic T ‐lymphocyte antigen 4 mutation showing multiple central nervous system lesions
ConclusionsA patient withCTLA4 variant might develop multiple central nervous system lesions including longitudinally-extended spinal cord lesions. As there is effective targeted immunotherapy, a complete immunological work-up is required for early diagnosis when a patient presents with central nervous system lesions and multi-organ symptoms. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 8, 2021 Category: Neurology Authors: Makoto Eriguchi, Hiroo Katsuya, Keisuke Kidoguchi, Hiroshi Ureshino, Masanori Nishi, Masashi Nishihara, Motoshi Sonoda, Masataka Ishimura, Shinya Kimura, Hideo Hara Tags: CASE REPORT Source Type: research

Role of B  cells in the pathogenesis of multiple sclerosis
AbstractMultiple sclerosis (MS) is a complex immune-mediated disease of the central nervous system. Traditionally, it has been considered to be mediated primarily by T  cells, and the contribution of B cells in the pathogenesis of MS has long been debated. However, clinical success of B-cell depletion therapies targeting CD20 has established the essential role of B cells in the pathogenesis of MS. Because CD20-targeting B-cell depletion therapies primarily have their effect on non-antibody secreting cells, emerging studies have dedicated intense focus on B-cell functions beyond antibody production, which include cytokin...
Source: Clinical and Experimental Neuroimmunology - September 8, 2021 Category: Neurology Authors: Hirofumi Ochi Tags: INVITED REVIEW Source Type: research

Unusual concomitant small ‐ and large‐fiber neuropathy related to hypereosinophilic syndrome
ConclusionsIn the present case, we hypothesized that idiopathic hypereosinophilic syndrome was the likely cause of the atopic dermatitis and small-fiber neuropathy, consistent with pain and dysautonomic symptoms. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 7, 2021 Category: Neurology Authors: Elena Merico, Alessandra Govoni, Erika Schirinzi, Lucia Chico, Giulia Ricci, Rosanna Calabrese, Chiara Baldini, Gaetano La Rocca, Raffaella Lombardi, Valentina Dini, Gabriele Buda, Barbara Loggini, Gabriele Siciliano Tags: CASE REPORT Source Type: research