eprintid: 19474
rev_number: 38
eprint_status: archive
userid: 1589
dir: disk0/00/01/94/74
datestamp: 2015-12-10 14:51:51
lastmod: 2024-03-29 20:04:58
status_changed: 2015-12-10 14:51:51
type: article
metadata_visibility: show
creators_name: Jarius, Sven
creators_name: Wildemann, Brigitte
title: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 2: Anti-PKC-gamma, anti-GluR-delta2, anti-Ca/ARHGAP26 and anti-VGCC
subjects: ddc-610
divisions: i-911100
abstract: Serological testing for anti-neural autoantibodies is important in patients presenting with idiopathic cerebellar ataxia, since these autoantibodies may indicate cancer, determine treatment and predict prognosis. While some of them target nuclear antigens present in all or most CNS neurons (e.g. anti-Hu, anti-Ri), others more specifically target antigens present in the cytoplasm or plasma membrane of Purkinje cells (PC). In this series of articles, we provide a detailed review of the clinical and paraclinical features, oncological, therapeutic and prognostic implications, pathogenetic relevance, and differential laboratory diagnosis of the 12 most common PC autoantibodies (often referred to as ‘Medusa head antibodies’ due their characteristic somatodendritic binding pattern when tested by immunohistochemistry). To assist immunologists and neurologists in diagnosing these disorders, typical high-resolution immunohistochemical images of all 12 reactivities are presented, diagnostic pitfalls discussed and all currently available assays reviewed. Of note, most of these antibodies target antigens involved in the mGluR1/calcium pathway essential for PC function and survival. Many of the antigens also play a role in spinocerebellar ataxia. Part 1 focuses on anti-metabotropic glutamate receptor 1-, anti-Homer protein homolog 3-, anti-Sj/inositol 1,4,5-trisphosphate receptor- and anti-carbonic anhydrase-related protein VIII-associated autoimmune cerebellar ataxia (ACA); part 2 covers anti-protein kinase C gamma-, anti-glutamate receptor delta-2-, anti-Ca/RhoGTPase-activating protein 26- and anti-voltage-gated calcium channel-associated ACA; and part 3 reviews the current knowledge on anti-Tr/delta notch-like epidermal growth factor-related receptor-, anti-Nb/AP3B2-, anti-Yo/cerebellar degeneration-related protein 2- and Purkinje cell antibody 2-associated ACA, discusses differential diagnostic aspects, and provides a summary and outlook. 
date: 2015
publisher: BioMed Central
id_scheme: DOI
ppn_swb: 1656159473
own_urn: urn:nbn:de:bsz:16-heidok-194740
language: eng
bibsort: JARIUSSVENMEDUSAHEAD2015
full_text_status: public
publication: Journal of neuroinflammation
volume: 12
number: 167
place_of_pub: London
pagerange: 1-21
issn: 1742-2094
citation:   Jarius, Sven ; Wildemann, Brigitte  (2015) ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 2: Anti-PKC-gamma, anti-GluR-delta2, anti-Ca/ARHGAP26 and anti-VGCC.  Journal of neuroinflammation, 12 (167).  pp. 1-21.  ISSN 1742-2094     
document_url: https://archiv.ub.uni-heidelberg.de/volltextserver/19474/1/12974_2015_Article_357.pdf