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The following is a summary of “Anti-RGS8 paraneoplastic cerebellar ataxia is preferentially associated with a particular subtype of Hodgkin’s lymphoma,” published in the August 2024 issue of Neurology by Peter et al.
Ataxia with an anti-regulator of G-protein signaling 8 autoantibodies (RGS8-Abs) is an autoimmune disease first identified in just 4 patients.
Researchers conducted a retrospective study identifying additional cases of patients with RGS8-Abs, describing their clinical symptoms, including the association between RGS8-related autoimmune cerebellar ataxia (ACA) and cancer.
They identified RGS8-Abs from the biological collections at the French Reference Center for Paraneoplastic Neurological Syndrome and the University of California San Francisco Center for Encephalitis and Meningitis. Clinical information was gathered, and cerebrospinal fluid, serum, and tumor pathology samples were collected to analyze the autoantibodies and related malignancies.
The results showed that only 3 new patients with RGS8-Abs were identified. All patients experienced pure cerebellar ataxia, ranging from mild to severe, and did not respond to current immunotherapy for ACA. Also, 2 of these patients had a rare form of Hodgkin Lymphoma called nodular lymphocyte-predominant Hodgkin lymphoma, which showed very mild extension. The autoantibodies in all patients targeted the same area on the RGS8 protein, which is naturally present in Purkinje cells but was also found in lymphoma cells in patients with RGS8-related ACA.
Investigators concluded that RGS8-Abs indicate a rare neurological syndrome found primarily in middle-aged men, linked to cerebellar ataxia and a specific lymphoma type. As in other paraneoplastic ACA with intracellular antigen, the condition is severe, with poor response to immunotherapy.
Source: link.springer.com/article/10.1007/s00415-024-12618-4
