Identification of RIMKLA-IgG Paraneoplastic Neurological Syndrome With Cerebellar Ataxia.

RIMKLA-IgG autoantibodies were identified in 8 of 35 patients with inflammatory cerebellar ataxia, most linked to small cell lung cancer (6 of 8).

• Why it matters: Early detection of autoimmune cerebellar ataxias is difficult in seronegative cases because specific autoantibodies are often unknown.
• What they did: A multicenter case series (2015–2020, UCSF) used blinded cell-based and tissue-based assays at a French PNS/AE reference center to screen sera and CSF, then validated candidates.
• The result: RIMKLA-IgG showed a specific CSF staining pattern in cerebellar deep nuclei and brainstem, and was associated with progressive cerebellar ataxia, diplopia, cranial VI paresis, inflammatory CSF, and intrathecal immunoglobulin synthesis.

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