Pathological evidence of encephalomyelitis in the stiff man syndrome with anti-GAD antibodies

Warren, J.
Scott, G.
Blumbergs, P.
Thompson, P.

Open link

Publication date

January 2002

DOI

10.1054/jocn.2001.1014

Publisher

Elsevier BV

ISSN

0967-5868

Journal

Journal of Clinical Neuroscience

Citation count (estimate)

Abstract

Copyright © 2002 Elsevier Science Ltd. All rights reserved.We report the case of a 57 year old woman with typical clinical features of the stiff man syndrome (SMS) and antibodies to glutamic acid decarboxylase (antiGADAb), who developed a supranuclear gaze palsy, ileus and died of bronchopneumonia eight years after the onset of illness. Post mortem examination revealed perivascular lymphocyte cuffing throughout the cerebral hemispheres, brainstem and spinal cord and neuronal loss in medial anterior horns of the cervical spinal cord. These findings support the notion that the SMS and progressive encephalomyelitis with rigidity (PER) form a clinical and pathologic continuum.Jason D. Warren, Grace Scott, Peter C. Blumbergs, and Philip D. Thomp...