Two new cases of anti-Ca (anti-ARHGAP26/GRAF) autoantibody-associated cerebellar ataxia

Jarius, Sven; Martínez-García, Pedro J.; Hernandez, Adelaida Leon; Brase, Jan C.; Borowski, Kathrin; Regula, Jens Ulrich; Meinck, H.-M.; Stöcker, W.; Wildemann, Brigitte; Wandinger, Klaus‐Peter

doi:10.1186/1742-2094-10-7

Cited by 42 publications

(29 citation statements)

References 21 publications

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“…New antibodies associated with PCD and idiopathic cerebellitis have been described in single cases and small case series, but their relevance and tumour association is currently unclear [anti‐protein kinase Cγ in PCD and adenocarcinoma , anti‐RhoGTPase‐activating protein 26 (ARHGAP26) in PCD with ovarian cancer ; voltage‐gated potassium channel (VGKC)‐complex antibodies, GAD, mGluR1 and Homer‐3 in idiopathic cerebellitis].…”

Section: Paraneoplastic Cerebellar Degenerationmentioning

confidence: 99%

Paraneoplastic neurological syndromes

Leypoldt

Wandinger

2014

Clinical and Experimental Immunology

133

113

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OTHER ARTICLES PUBLISHED IN THIS SERIESDiagnosis, pathogenesis and treatment of myositis: recent advances. Clinical and Experimental Immunology 2014, 175: 349-58. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies. Clinical and Experimental Immunology 2014, 175: 359-72 Monoclonal antibodies in treatment of multiple sclerosis. Clinical and Experimental Immunology 2014, 175: 373-84

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Section: Paraneoplastic Cerebellar Degenerationmentioning

confidence: 99%

Paraneoplastic neurological syndromes

Leypoldt

Wandinger

2014

Clinical and Experimental Immunology

133

113

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“…Antibodies to ARHGAP26 (also termed anti-Ca) were first described in 2010 [ 22 ]. So far, four patients with Ca/ARHGAP26 antibodies have been published [ 22 , 86 , 87 ]. Age at diagnosis was 24, 33, 38 and 68 years.…”

Section: Anti-ca/arhgap26mentioning

confidence: 99%

‘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

Jarius

Wildemann

2015

J Neuroinflammation

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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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“…No tumor was detected ( 1 ). Two more patients with ARHGAP26 autoantibodies and cerebellar ataxia were reported 3 years later ( 2 ). In one of these patients, antibody detection lead to the discovery of ovarian cancer, suggesting ARHGAP26 autoantibodies as a potential marker of a paraneoplastic neurological syndrome (PNS).…”

Section: Introductionmentioning

confidence: 99%

Anti-ARHGAP26 Autoantibodies Are Associated With Isolated Cognitive Impairment

2018

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Autoantibodies against the RhoGTPase-activating protein 26 (ARHGAP26) were originally identified in the context of subacute autoimmune cerebellar ataxia. Further studies identified a wider clinical spectrum including psychotic, affective, and cognitive symptoms. Only a few patients reported so far had evidence of a tumor association. A prospective analysis between January 2015 and December 2017 at the Dept. of Neurology at Charité—Universitätsmedizin Berlin identified 14 patients with ARHGAP26 autoantibodies on a cell-based assay, of which three patients had additional brain immunohistochemistry staining of cerebellar molecular layer and Purkinje cells, who were therefore considered antibody-positive. In all three patients, ARHGAP26 autoantibodies were associated with tumors. In two patients, an isolated cognitive impairment without additional neurological deficits was observed. These cases thus further extend the clinical spectrum associated with ARHGAP26 autoantibodies and strengthen a potential paraneoplastic context.

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Two new cases of anti-Ca (anti-ARHGAP26/GRAF) autoantibody-associated cerebellar ataxia

Cited by 42 publications

References 21 publications

Paraneoplastic neurological syndromes

Paraneoplastic neurological syndromes

‘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

Anti-ARHGAP26 Autoantibodies Are Associated With Isolated Cognitive Impairment

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