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2015 ; 12
(ä): 167
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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
#MMPMID26377184
Jarius S
; Wildemann B
J Neuroinflammation
2015[Sep]; 12
(ä): 167
PMID26377184
show ga
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.
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