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

Jonas Feldheim; Cornelius Deuschl; Martin Glas; Christoph Kleinschnitz; Tim Hagenacker

doi:10.1186/s42466-021-00129-w

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.

Authors

Jonas Feldheim^{1

2

3

4}, Cornelius Deuschl⁵, Martin Glas^{1

2

3}, Christoph Kleinschnitz^{1

2

3}, Tim Hagenacker^{6

7}

Affiliations

¹ Department of Neurology, University Hospital Essen, Essen, Germany.
² Center for Translational Neuro- and Behavioral Sciences, University Hospital Essen, Essen, Germany.
³ Department of Neurology, Division of Clinical Neurooncology, University Hospital, Essen, Germany.
⁴ Tumorbiology Laboratory, Department of Neurosurgery, University of Würzburg, Würzburg, Germany.
⁵ Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany.
⁶ Department of Neurology, University Hospital Essen, Essen, Germany. tim.hagenacker@uk-essen.de.
⁷ Center for Translational Neuro- and Behavioral Sciences, University Hospital Essen, Essen, Germany. tim.hagenacker@uk-essen.de.

Abstract

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).

Publication types

Letter