Clinical features and outcome of patients with autoimmune cerebellar ataxia evaluated with the Scale for Assessment and Rating of Ataxia

2021 ◽  
Author(s):  
Valentina Damato ◽  
Claudia Papi ◽  
Gregorio Spagni ◽  
Amelia Evoli ◽  
Gabriella Silvestri ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Clinical Features ◽  
Autoimmune Cerebellar Ataxia

Autoimmune Cerebellar Ataxia due to Inflammation of the Inferior Olivary Nuclei

2021 ◽  
Author(s):  
Jae-Gyum Kim ◽  
Young-Bin Park ◽  
Sun-Uk Lee ◽  
Sung-Wook Yu ◽  
Kun-Woo Park ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Autoimmune Cerebellar Ataxia ◽  
Inferior Olivary

Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS): a review of the clinical features and video-oculographic diagnosis

2011 ◽  
Vol 1233 (1) ◽  
pp. 139-147 ◽  
Author(s):  
David J. Szmulewicz ◽  
John A. Waterston ◽  
Hamish G. MacDougall ◽  
Stuart Mossman ◽  
Andrew M. Chancellor ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Clinical Features

scholarly journals Autoantibody to the Rab6A/Rab6B in Autoimmune Cerebellar Ataxia Associated with Sjogren’s Syndrome

2020 ◽  
Author(s):  
Liyuan Guo ◽  
Haitao Ren ◽  
Siyuan Fan ◽  
Hongzhi Guan ◽  
Jing Wang
Keyword(s):  
Purkinje Cell ◽  
Cerebellar Ataxia ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Hek293 Cells ◽  
Rat Cerebellum ◽  
Autoimmune Cerebellar Ataxia ◽  
Sjögren‘S Syndrome

Abstract Background To report a novel autoantibody against Purkinje cell in a patient with autoimmune cerebellar ataxia (ACA) associated to Sjogren’s syndrome (SS).Methods The Patients on one centre with cerebellar ataxia of unknown cause, who were tested positive with tissue-based indirect immunofluorescence assay (TBA) on rat cerebellum sections and negative for comprehensive anti-neural autoantibodies panel, were investigated for novel autoantibody identification. Among them, one patient with comorbid ACA and SS was qualified for further exploration. His-immunoprecipitation (HIP) combined with mass spectrometric (MS) analysis was used to identify the target antigen, which was confirmed by recombinant cell based assay (CBA) and antibody neutralization experiments. Results TBA of the patient’s serum and cerebrospinal fluid (CSF) for autoantibody testing revealed binding of IgG antibody, mainly IgG1, to Purkinje cell and granular layer of rat cerebellum. Rab6A was identified as the autoantigen by MS and Western blot, subsequently verified by CBA with HEK293 cells expressing human Rab6A/Rab6B. Furthermore, recombinant human Rab6A/Rab6B protein to neutralize the autoantibodies’ tissue reaction was performed by a parallel confirmed approach.Conclusion Autoantibody against Rab6A/Rab6B may be a novel biomarker in diagnosis of ACA, especially in patients with comorbid ACA and SS. The role of the antibody in mechanism of ACA warrants further study.

scholarly journals Diagnostic Criteria for Primary Autoimmune Cerebellar Ataxia—Guidelines from an International Task Force on Immune-Mediated Cerebellar Ataxias

2020 ◽  
Vol 19 (4) ◽  
pp. 605-610 ◽  
Author(s):  
Marios Hadjivassiliou ◽  
Francesc Graus ◽  
Jerome Honnorat ◽  
Sven Jarius ◽  
Maarten Titulaer ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cerebellar Ataxia ◽  
Task Force ◽  
Immune Mediated ◽  
Autoimmune Cerebellar Ataxia ◽  
Cerebellar Ataxias

scholarly journals Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies

2017 ◽  
Vol 75 (3) ◽  
pp. 142-146 ◽  
Author(s):  
Tiago Silva Aguiar ◽  
Andrea Fragoso ◽  
Carolina Rouanet de Albuquerque ◽  
Patrícia de Fátima Teixeira ◽  
Marcus Vinícius Leitão de Souza ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Pancreatic Beta Cells ◽  
Clinical Characteristics ◽  
Immunoglobulin Therapy ◽  
Acid Decarboxylase ◽  
Partial Recovery ◽  
Gamma Aminobutyric Acid ◽  
Intravenous Immunoglobulin Therapy ◽  
Autoimmune Cerebellar Ataxia ◽  
Insidious Onset

ABSTRACT The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia.

scholarly journals Autosomal dominant cerebellar ataxia type I Clinical features and MRI in families with SCA1, SCA2 and SCA3

Brain ◽  
1996 ◽  
Vol 119 (5) ◽  
pp. 1497-1505 ◽  
Author(s):  
K. Bürk ◽  
M. Abele ◽  
M. Fetter ◽  
J. Dichgans ◽  
M. Skalej ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Clinical Features ◽  
Autosomal Dominant ◽  
Autosomal Dominant Cerebellar Ataxia ◽  
Type I

Neurochondrin Antibody Serum Positivity in Three Cases of Autoimmune Cerebellar Ataxia

2019 ◽  
Vol 18 (6) ◽  
pp. 1137-1142 ◽  
Author(s):  
Zhang Weihua ◽  
Ren Haitao ◽  
Fang Fang ◽  
Yang Xunzhe ◽  
Wang Jing ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Autoimmune Cerebellar Ataxia

scholarly journals Prevalence of Autoantibodies and the Efficacy of Immunotherapy for Autoimmune Cerebellar Ataxia

2016 ◽  
Vol 55 (5) ◽  
pp. 449-454 ◽  
Author(s):  
Kazunori Nanri ◽  
Misaki Okuma ◽  
Saki Sato ◽  
Makoto Yoneda ◽  
Takeshi Taguchi ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Autoimmune Cerebellar Ataxia
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