Simultaneous paraneoplastic cerebellar degeneration, Lambert-Eaton syndrome and neuropathy associated with AGNA/anti-SOX1 and VGCC antibodies

Neurol Res Pract. 2021 May 24;3(1):30. doi: 10.1186/s42466-021-00129-w.


Anti-glial nuclear antibody (AGNA) is an onconeuroal antibody targeting the nuclei of Bergmann glia in the cerebellum and Anti-SRY-related HMG-box 1 (SOX1). It is highly specific for small cell lung cancer (SCLC) and correlates to the appearance of paraneoplastic neurological syndromes such as Lambert-Eaton myasthenic syndrome (pLEMS) and paraneoplastic cerebellar degeneration (PCD) amongst others. Herein, we present a SCLC patient with rapidly progressive PCD, LEMS and axonal polyneuropathy associated with AGNA/SOX1-antibodies, successfully treated with plasma-exchange (PLEX).

PMID:34030742 | DOI:10.1186/s42466-021-00129-w