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GABAB receptor antibodies in paraneoplastic cerebellar ataxia

Autoantibodies to the gamma-aminobutyric acid-B (GABAB) receptor were recently described in patients with limbic encephalitis presenting with early or prominent seizures. We report on a 64-year-old man with malignant melanoma who during adjuvant therapy with interferon (IFN)-alpha developed cerebell...

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Main Authors: Jarius, Sven (Author) , Steinmeyer, Florian (Author) , Knobel, Astrid (Author) , Streitberger, Kaspar (Author) , Hotter, Benjamin (Author) , Horn, Sigrun (Author) , Heuer, Heike (Author) , Schreiber, Stephan (Author) , Wilhelm, Tabea (Author) , Trefzer, Uwe (Author) , Wildemann, Brigitte (Author) , Ruprecht, Klemens (Author)
Format: Article (Journal)
Language:English
Published: 14 January 2013
In: Journal of neuroimmunology
Year: 2013, Volume: 256, Issue: 1/2, Pages: 94-96
ISSN:1872-8421
DOI:10.1016/j.jneuroim.2012.12.006
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.jneuroim.2012.12.006
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S0165572812003396
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Author Notes:Sven Jarius, Florian Steinmeyer, Astrid Knobel, Kaspar Streitberger, Benjamin Hotter, Sigrun Horn, Heike Heuer, Stephan J. Schreiber, Tabea Wilhelm, Uwe Trefzer, Brigitte Wildemann, Klemens Ruprecht

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520 |a Autoantibodies to the gamma-aminobutyric acid-B (GABAB) receptor were recently described in patients with limbic encephalitis presenting with early or prominent seizures. We report on a 64-year-old man with malignant melanoma who during adjuvant therapy with interferon (IFN)-alpha developed cerebellar ataxia. Indirect immunofluorescence on brain tissue sections revealed high-titer (1:20,000) IgG1 serum autoantibodies to the cerebellar molecular and granular layer, which were confirmed to be directed against GABAB receptor in a cell-based assay. This case highlights cerebellar ataxia in the absence of seizures as a clinical manifestation of GABAB receptor autoimmunity and extends the spectrum of tumors underlying this condition to malignant melanoma. IFN-alpha therapy may have contributed to the development of autoimmunity in this patient. 
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