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...
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
In this study, we emphasize exercise training and the consumption of probiotics. Our results showed that in addition to the effect of exercise training and consumption of probiotics on demyelination, both of these interventions have a greater effect on demyelination. AbstractObjectiveMultiple sclerosis (MS) is a central nervous system disorder characterized by inflammation, demyelination and neurodegeneration. It has been suggested that exercise and intake of probiotics might influence MS pathology and, thereby, slow down the disease process in MS patients. Therefore, this study aimed to investigate the effect of exercise ...
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 e...
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 inc...
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

Glial cell line ‐derived neurotrophic factor and basic fibroblast growth factor derived from skeletal muscle pericytes increase the barrier function of endothelial cells in the endomysium
ConclusionGDNF and bFGF released from HSMPCTs increase the barrier function of endomysial endothelium. These HSMPCTs and TSM15 cells, which are recognized as anin vitro model of the endomysial capillary, might facilitate the analysis of the pathophysiology of the microvasculature in human skeletal muscle. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 6, 2021 Category: Neurology Authors: Eri Ishiguchi, Yasuteru Sano, Toshihiko Maeda, Fumitaka Shimizu, Miwako Fujisawa, Masaya Honda, Yukio Takeshita, Michiaki Koga, Takashi Kanda Tags: ORIGINAL ARTICLE Source Type: research

Radial linear perivascular emphasis in coronavirus disease 2019 ‐associated acute disseminated encephalomyelitis
A 62-year-old woman with coronavirus disease (COVID-19) developed coma on day 19 after her pneumonia was ameliorated. Brain magnetic resonance imaging with gadolinium showed radial linear perivascular enhancement, typically seen in glial fibrillary acidic protein (GFAP) astrocytopathy, although anti-GFAP antibody results were negative. Her consciousness recovered with high-dose steroid administration. We diagnosed the patient with COVID-19-associated acute disseminated encephalomyelopathy (ADEM) with radial linear perivascular emphasis. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 3, 2021 Category: Neurology Authors: Ryo Tanaka, Takamasa Nukui, Ratna Dini Haryuni, Makoto Mori, Shigeru Hayashi, Kyo Noguchi, Yuji Nakatsuji Tags: CASE IMAGE Source Type: research

Anti ‐GM2 antibodies‐associated meningoradiculitis in the context of HIV immune reconstitution inflammatory syndrome
ConclusionWe postulate that meningoradiculitis in this patient was a consequence of anti-GM2 antibodies produced as part of an anti-HIV-1 immune response, and that this case falls within the scope of autoimmune manifestations linked to HIV IRIS. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - September 1, 2021 Category: Neurology Authors: Nathalie Forgeard, Edouard Januel, Anne Blancher, Laurent Fonquernie, Agn ès Gautheret‐Dejean, Jean Neil, Marie‐Caroline Meyohas Tags: CASE REPORT Source Type: research

Issue Information
(Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 27, 2021 Category: Neurology Tags: Issue Information Source Type: research

Emerging topics in Guillain –Barré syndrome
(Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 27, 2021 Category: Neurology Authors: Atsuro Chiba Tags: EDITORIAL Source Type: research

Immune dysregulation syndrome with CTLA4 mutation showing multiple central nervous system lesions
ConclusionsA patient withCTLA4 variant may develop multiple CNS lesions including longitudinally-extended spinal cord lesions. Since there is effective targeted immunotherapy, a complete immunological workup is needed for early diagnosis when a patient presents with CNS lesions and multi-organ symptoms. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 24, 2021 Category: Neurology Authors: Makoto Eriguchi, Hiroo Katsuya, Keisuke Kidoguchi, Hiroshi Ureshino, Nishi Masanori, Masashi Nishihara, Motoshi Sonoda, Masataka Ishimura, Shinya Kimura, Hideo Hara Tags: CASE REPORT Source Type: research

Effects of exercise training with Lactobacillus plantarum intake on Iba ‐1, GFAP, IL‐6, and IL‐1β in cuprizone‐induced demyelination mouse model of multiple sclerosis
AbstractObjectiveMultiple sclerosis (MS) is a central nervous system (CNS) disorder characterized by inflammation, demyelination, and neurodegeneration. It has been suggested that exercise and intake of probiotics might influence MS pathology and, thereby, slow down the disease process in MS patients. Therefore, this study aimed to investigate the effect of exercise training withLactobacillus plantarum intake on Ionized calcium-binding adaptor molecule 1 (Iba1), glial fibrillary acidic protein (GFAP), interleukin (IL)-6, and IL-1 β in cuprizone (CPZ)-induced demyelination mouse model of MS.MethodsMice were exposed to ...
Source: Clinical and Experimental Neuroimmunology - August 23, 2021 Category: Neurology Authors: Donya Sajedi, Ramin Shabani, Alireza Elmieh Tags: ORIGINAL ARTICLE Source Type: research

The role of B cells in the pathogenesis of multiple sclerosis
AbstractMultiple sclerosis (MS) is a complex immune-mediated disease of the central nervous system (CNS). 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-targting 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 cytoki...
Source: Clinical and Experimental Neuroimmunology - August 23, 2021 Category: Neurology Authors: Hirofumi Ochi Tags: INVITED REVIEW Source Type: research

Anti ‐aquaporin‐4 positive‐to‐negative seroconversion in Latin American patients with neuromyelitis optica spectrum disorder treated with rituximab
ConclusionsTreatment with RTX in patients with seropositive AQP4-IgG NMOsd can lead to negative conversion, but our findings do not support a clinical reflection from this change. The role of monitoring for AQP4-IgG is yet to be defined in the Mexican NMOsd population. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 21, 2021 Category: Neurology Authors: Indhira Zabala ‐Ángeles, Christian García‐Estrada, Enrique Gómez‐Figueroa, Roberto Rodriguez‐Rivas, Edgar Higuera‐Gonzalez, Adriana Casallas‐Vanegas, Lucinda Aguirre‐Cruz, Teresa Corona‐Vázquez, José Flores‐Rivera, Verónic Tags: ORIGINAL ARTICLE Source Type: research

B cells in experimental autoimmune encephalomyelitis
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 until recently, mainly owing to the technical difficulty of studying this cell population in humans. In this context, experimental autoimmune encephalomyelitis (EAE), a putative animal model of MS, has profoundly contributed to our understanding of B cells in this disease. B cells are now recognized to contribute to MS pathology through the produ...
Source: Clinical and Experimental Neuroimmunology - August 19, 2021 Category: Neurology Authors: Yusei Miyazaki, Masaaki Niino Tags: INVITED REVIEW Source Type: research

Strongyloidiasis prophylaxis: is there room for ivermectin in neurological patients?
AbstractWith the advent of monoclonal therapies, there is currently an array of treatment options for autoimmune neurological disorders involving the use of immunosuppressive and immunomodulatory drugs; nevertheless, these therapies may predispose patients to the hyperinfection syndrome (HS) caused by strongyloidiasis, leading to severe manifestations1. For this reason, a prophylactic treatment with ivermectin has been proposed for patients from endemic regions undergoing immunosuppressive treatment2,3. However, there is no evidence in the literature that supports that recommendation. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 18, 2021 Category: Neurology Authors: Marcelo Henrique De Moura Campos, Ariel Jos é Gomes Marques Neto, Rodrigo Kleinpaul, Marcus Vinicius Magno Gonçalves Tags: LETTER TO THE EDITOR Source Type: research

The radial linear perivascular emphasis in COVID ‐19‐associated acute disseminated encephalomyelitis (ADEM)
AbstractA 62-year-old woman who has a history of chronic hepatitis B and hypertension, presented to a local hospital with cough and fever and was admitted on day 3. Since her chest CT revealed bilateral ground-glass opacities, and SARS-Cov-2 saliva PCR was positive, she was diagnosed with COVID-19 pneumonia and was treated with favipiravir. On day 7, she was in a relatively good condition, as she was afebrile with a little cough. However, on day 18, she became coma which was followed with convulsive seizures, prompting administration of diazepam and levetiracetam. Afterwards, she was then transferred to our hospital, prese...
Source: Clinical and Experimental Neuroimmunology - August 9, 2021 Category: Neurology Authors: Ryo Tanaka, Takamasa Nukui, Ratna Dini Haryuni, Makoto Mori, Shigeru Hayashi, Kyo Noguchi, Yuji Nakatsuji Tags: CASE REPORT Source Type: research

Safety and efficacy of Mycophenolate Mofetil in Myelin oligodendrocyte glycoprotein ‐immunoglobulin (MOG‐IgG) associated disorder among adults: A meta‐analysis
AbstractIntroductionMyelin oligodendrocyte glycoprotein-immunoglobulin (MOG-IgG) associated disorder (MOGAD) is an autoimmune demyelinating disorder of the Central Nervous System having distinct clinical phenotypes and epidemiological distribution than Multiple Sclerosis (MS) and Neuromyelitis Optica Spectrum Disorder (NMOSD). Mycophenolate Mofetil (MMF) has shown good safety and efficacy in MS and NMOSD patients, but the published case series and observational studies of MOGAD showed contrasting results. Thus, we ought to perform meta-analysis.MethodsWe searched PubMed, Embase, Cochrane Library, Google scholar, and additi...
Source: Clinical and Experimental Neuroimmunology - August 7, 2021 Category: Neurology Authors: Sanjeev Kharel, Rajeev Ojha, Rakesh Ghimire, Neha Guragain, Anish Mudvari Tags: REVIEW ARTICLE Source Type: research

Unusual concomitant small and large fiber neuropathy related to hypereosinophilic syndrome
ConclusionsIn this 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 - August 6, 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

Anti ‐GM2 antibodies associated meningoradiculitis in the context of HIV immune reconstitution inflammatory syndrome
AbstractA 71 year-old patient diagnosed with HIV-1 infection developed neurological symptoms congruent with meningoradiculitis one month after initiation of antiretroviral therapy (ART). Concurrent syphilitic seroconversion raised the hypothesis of neurosyphilis, which was later infirmed. Serum antiganglioside IgM antibodies were highly positive. Previous studies have demonstrated that GM2 is overexpressed on HIV-1 infected T lymphocytes. Treatment with intravenous immunoglobulins was effective. We postulate that meningoradiculitis in this patient was a consequence of anti-GM2 antibodies produced as part of an anti HIV-1 i...
Source: Clinical and Experimental Neuroimmunology - August 5, 2021 Category: Neurology Authors: N Forgeard, E Januel, A Blancher, L Fonquernie, A Gautheret ‐Dejean, J Neil, M‐C Meyohas Tags: CASE REPORT Source Type: research

Wernicke ’s encephalopathy precipitated by neuromyelitis optica spectrum disorder and Graves’ disease: A tale of clinical and radiological dilemmas
ConclusionOur case establishes the importance of considering NMOSD variants in metabolic encephalopathy, especially if neuroimaging is suggestive and in the backdrop of another autoimmune disorder. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - August 4, 2021 Category: Neurology Authors: Ritwik Ghosh, Souvik Dubey, Adrija Ray, Dipayan Roy, Kaustav De, Arpan Mandal, Dinobandhu Naga, Bikash Chandra Swaika, Alak Pandit, Juli án Benito‐León Tags: CASE REPORT Source Type: research

Antiaquaporin ‐4 positive‐to‐negative seroconversion in Latin American patients with NMOSD treated with rituximab
ConclusionTreatment with RTX in patients with seropositive AQP4-IgG NMOsd can lead to negative conversion, but our findings do not support a clinical reflection from this change. The role of monitoring for AQP4-IgG is yet to be defined in the Mexican NMOsd population. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 28, 2021 Category: Neurology Authors: Indhira Zabala ‐Ángeles, Christian García‐Estrada, Enrique Gómez‐Figueroa, Roberto Rodriguez‐Rivas, Edgar Higuera‐Gonzalez, Adriana Casallas‐Vanegas, Lucinda Aguirre‐Cruz, Teresa Corona‐Vázquez, José Flores‐Rivera, Verónic Tags: ORIGINAL ARTICLE Source Type: research

Anti ‐GQ1b antibody: Recent topics
AbstractResearches on antibodies against glycolipids, mainly gangliosides, have advanced the understanding of the pathophysiology of Guillain-Barr é syndrome. Glycolipid antibody research began with the study of isolated ganglioside antigens and further expanded that area with the discovery of antibodies against complex antigens of two glycolipids. In Fisher syndrome-related disorders, IgG anti-GQ1b antibody is detected at a high rate, but 10 –30% of the cases are antibody-negative. Recently, an antibody that requires Ca2+ to react with ganglioside GQ1b (Ca2+-dependent GQ1b antibody) was discovered in the anti...
Source: Clinical and Experimental Neuroimmunology - July 24, 2021 Category: Neurology Authors: Ayumi Uchibori Tags: REVIEW ARTICLE Source Type: research

Unilateral basal ganglia restricted diffusion in anti ‐leucine‐rich glioma‐inactivated 1 encephalitis mimicking cerebral infarction: A report on two cases and literature review
ConclusionOur report adds to the heterogeneity of anti-leucine-rich glioma-inactivated  1 encephalitis and highlights the need to consider this diagnosis in patients who present atypically. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 23, 2021 Category: Neurology Authors: Mervyn Q. W. Poh, Hui Jin Chiew, Zheyu Xu, Kevin Tan, Tchoyoson C. C. Lim, Tianrong Yeo Tags: CASE REPORT Source Type: research

Good response to rituximab in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids presenting as tumor ‐like infratentorial lesion
ConclusionsTumor-like onset was found to be an extremely rare variant of CLIPPERS, which expands the spectrum of possible magnetic resonance imaging manifestation, which can be observed during the course of this disease. The efficacy of rituximab in the treatment of CLIPPERS syndrome supports the role of B  cells as important regulators of T-cell activity. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 23, 2021 Category: Neurology Authors: Antonio Marangi, Luigi Zuliani, Valerio Vitale, Mariam Mansour, Lisa Federle, Mariangela Novello, Emanuele Stefano Giovanni d ’Amore, Gianola Stenta, Francesco Perini Tags: CASE REPORT Source Type: research

Case of autoimmune glial fibrillary acidic protein astrocytopathy associated with Epstein –Barr virus reactivation
ConclusionsThe MRI findings characteristic of autoimmune GFAP astrocytopathy might not be present at the illness onset when triggered by Epstein –Barr virus reactivation. Our results suggest that MRS might show astrocytic dysfunction and be useful in evaluating treatment progress. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 21, 2021 Category: Neurology Authors: Hayato So, Takashi Ohashi, Sae Yamagishi, Harushi Mori, Jun ‐ichi Takanashi Tags: CASE REPORT Source Type: research

Wernicke ’s encephalopathy Precipitated by Neuromyelitis Optica Spectrum Disorder and Grave’s Disease: A Tale of Clinical and Radiological Dilemmas
ConclusionOur case establishes the importance of considering NMOSD variants in metabolic encephalopathy, especially if neuroimaging is suggestive, and in the backdrop of another autoimmune disorder. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 16, 2021 Category: Neurology Authors: Ritwik Ghosh, Souvik Dubey, Adrija Ray, Dipayan Roy, Kaustav De, Arpan Mandal, Dinobandhu Naga, Bikash Chandra Swaika, Alak Pandit, Juli án Benito‐León Tags: CASE REPORT Source Type: research

Immunotherapy ‐responsive brainstem lesion accompanied with anti‐galactocerebroside antibody
ConclusionsOur case highlights that an anti-Gal-C positivity can cause brainstem inflammatory lesion which should be differentiated from ADEM, BBE, and MS. Her symptoms improved with intense immunotherapy and antibody measurement led to the discontinuation of unnecessary disease-modifying drugs. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 11, 2021 Category: Neurology Authors: Wataru Shiraishi Tags: CASE REPORT Source Type: research

Cognition and psychopathology in autoimmune encephalitides: A focus on risk factors and patient outcomes
Neurocognitive compromise, neuropsychiatric symptoms and psychopathology are all evident in autoimmune encephalitides. This review provides detailed analysis of the various cognitive deficits and psychopathology that occur in different subsets of autoimmune encephalitides . It also delves into the implication of the aforementioned in regard to patient quality of life and overall prognosis. AbstractNeurocognitive compromise, neuropsychiatric symptoms and psychopathology are all evident in the acute stages of autoimmune encephalitides. These factors considerably affect functional independence after discharge. Drawing on psyc...
Source: Clinical and Experimental Neuroimmunology - July 10, 2021 Category: Neurology Authors: Sarah P. Griffith, Charles B. Malpas, Chris Kyndt, Rubina Alpitsis, Terence J. O ’Brien, Mastura Monif Tags: REVIEW ARTICLE Source Type: research

Multifaceted features of immunoglobulin  G anti‐GM1 antibodies in Guillain–Barré syndrome
AbstractImmunoglobulin  G (IgG) anti-GM1 antibodies, the most common autoantibodies in Guillain–Barré syndrome (GBS), have diverse characteristics regarding IgG subclass, complement activation, affinity and fine specificity. In this review, an update on multifaceted aspects of IgG anti-GM1 antibodies is provided, emph asizing their roles in creating a varying clinical picture of GBS and in developing pure motor neuropathy after antecedent infection. Diversity in IgG subclass and cross-reactivity, both of which are closely related to the type of antecedent infection, appears to cause the variation in clini...
Source: Clinical and Experimental Neuroimmunology - July 8, 2021 Category: Neurology Authors: Michiaki Koga Tags: INVITED REVIEW Source Type: research

Istradefylline, an adenosine A2a receptor antagonist, ameliorates neutrophilic airway inflammation and psoriasis in mice.
ConclusionThese results indicate that adenosine-mediated IL-17A production plays a role in neutrophilic inflammation models, and moreover, istradefylline, ARL67156, and AMP-CP are effective in animal models of neutrophilic inflammation. Some clinical relevancies in COVID-19 are discussed. (248 words) (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 6, 2021 Category: Neurology Authors: Mieko Tokano, Masaaki Kawano, Rie Takagi, Sho Matsushita Tags: ORIGINAL ARTICLE Source Type: research

A case of autoimmune glial fibrillary acidic protein astrocytopathy associated with Epstein ‐Barr virus reactivation
ConclusionsThe MRI findings characteristic of autoimmune GFAP astrocytopathy may not be present at the illness onset when triggered by EBV reactivation. Our results suggest that MRS may show astrocytic dysfunction and be useful in evaluating treatment progress. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 6, 2021 Category: Neurology Authors: Hayato So, Takashi Ohashi, Sae Yamagishi, Harushi Mori, Jun ‐ichi Takanashi Tags: CASE REPORT Source Type: research

Good response to rituximab in CLIPPERS presenting as tumor ‐like infratentorial lesion
Conclusionstumor-like onset turns out to be an extremely rare variant of CLIPPERS, which expands the spectrum of possible MRI manifestation which can be observed during the course of this disease. The efficacy of rituximab in the treatment of CLIPPERS syndrome supports the role of B cells as important regulators of T-cell activity. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 6, 2021 Category: Neurology Authors: Antonio Marangi, Luigi Zuliani, Valerio Vitale, Mariam Mansour, Lisa Federle, Mariangela Novello, Emanuele Stefano Giovanni d ’Amore, Gianola Stenta, Francesco Perini Tags: CASE REPORT Source Type: research

Primary diffuse large B ‐cell lymphoma of the central nervous system with rapidly progressing lesions following dimethyl fumarate treatment, showing relapsing and remitting symptoms: A case report
ConclusionPatients with rapidly progressing white matter lesions following DMF administration should be suspected for B-cell type PCNSL and pathologically diagnosed using brain biopsy. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 4, 2021 Category: Neurology Authors: Yuki Kitazaki, Asako Ueno, Kenichiro Maeda, Rei Asano, Go Aoki, Ayumu Katsuki, Norimichi Shirafuji, Takahiro Yamauchi, Makoto Isozaki, Takahiko Saida, Yasunari Nakamoto, Tadanori Hamano Tags: CASE REPORT Source Type: research

Unilateral basal ganglia restricted diffusion in anti ‐LGI1 encephalitis mimicking cerebral infarction: A report on two cases and literature review
ConclusionOur report adds to the heterogeneity of anti-LGI1 encephalitis and highlights the need to consider this diagnosis in patients who present atypically. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - July 1, 2021 Category: Neurology Authors: Mervyn Qi Wei Poh, Hui Jin Chiew, Zheyu Xu, Kevin Tan, Tchoyoson Choie Chioe Lim, Tianrong Yeo Tags: CASE REPORT Source Type: research

A patient with probable neuro ‐Behçet's disease presenting a unique linear pontine trigeminal root lesion and a linear subcortical white matter lesion.
ConclusionThe case of a patient with NBD presenting with atypical brain lesions is reported. This NBD in this case may have a pathogenetic mechanism that is different from that of typical NBD. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - June 28, 2021 Category: Neurology Authors: Akane Suzuki, Kouichi Miyazawa, Juichi Fujimori, Kazuhiro Murakami, Ichiro Nakashima Tags: CASE REPORT Source Type: research

Multifaceted features of IgG anti ‐GM1 antibodies in Guillain‐Barré syndrome
AbstractIgG anti-GM1 antibodies, the most common autoantibodies in Guillain-Barr é syndrome (GBS), have diverse characteristics regarding IgG subclass, complement activation, affinity, and fine specificity. In this review, I provide an update on multifaceted aspects of IgG anti-GM1 antibodies, emphasizing their roles in creating a varying clinical picture of GBS and in developi ng pure motor neuropathy after antecedent infection. Diversity in IgG subclass and cross-reactivity, both of which are closely related to the type of antecedent infection, appears to cause the variation in clinical features and severity of th...
Source: Clinical and Experimental Neuroimmunology - June 10, 2021 Category: Neurology Authors: Michiaki Koga Tags: REVIEW ARTICLE Source Type: research

Cortical adult neurogenesis and its biological implication
AbstractIt is well established that adult neurogenesis occurs in the subventricular zone and the subgranular zone, but a controversy remains about adult neurogenesis in other regions. The cerebral cortex is the most controversial site and has long been of interest to not only neuroscientists but also scientists with broad backgrounds and medical doctors since the late nineteenth century. However, recent studies are gradually clarifying that neurogenesis in the adult cerebral cortex occurs, especially under pathological conditions. These studies suggest that endogenous neural stem cells and/or progenitor cells may exist in ...
Source: Clinical and Experimental Neuroimmunology - June 8, 2021 Category: Neurology Authors: Koji Ohira Tags: REVIEW ARTICLE Source Type: research

Cognition and Psychopathology in Autoimmune Encephalitides – a focus on risk factors and patient outcomes.
AbstractNeurocognitive compromise, neuropsychiatric symptoms, and psychopathology are all evident in the acute stages of autoimmune encephalitides (AE). These factors considerably affect functional independence after discharge. Drawing on psychometric assessments and qualitative descriptions, this review will explore the nature, extent, and diagnosis of cognitive disorder in AE. Potential pathophysiological and neuroanatomical architecture related to neurocognitive compromise in the acute and chronic stages of this illness is examined. In regards to outcomes, the review highlights clinicodemographic factors currently known...
Source: Clinical and Experimental Neuroimmunology - June 7, 2021 Category: Neurology Authors: Sarah P. Griffith, Charles B. Malpas, Chris Kyndt, Rubina Alpitsis, Terence J. O ’Brien, Mastura Monif Tags: REVIEW ARTICLE Source Type: research

Differential production of interleukin ‐6 and tumor necrosis factor‐α in primary rat astrocyte cultures using two distinct methods of microglia elimination
In conclusion, we have demonstrated that HE-relevant factors evoke oxidative stress and cytokine production, albeit depending on the elimination method used. (Source: Clinical and Experimental Neuroimmunology)
Source: Clinical and Experimental Neuroimmunology - May 26, 2021 Category: Neurology Authors: Zoltan Barany, Istvan Toth, Gergely Jocsak, Laszlo V. Frenyo, Tibor Bartha, Agnes Sterczer, David S. Kiss Tags: ORIGINAL ARTICLE Source Type: research