Pathological evidence of encephalomyelitis in the stiff man syndrome with anti-GAD antibodies.
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.
Warren
Jason D
JD
Department of Neurology, Royal Adelaide Hospital, and University Department of Medicine, University of Adelaide, South Australia.
Scott
Grace
G
Blumbergs
Peter C
PC
Thompson
Philip D
PD
D000906:Antibodies / Q000032:analysis*; D002490:Central Nervous System / Q000473:pathology; D004679:Encephalomyelitis / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008875:Middle Aged; D016750:Stiff-Person Syndrome / Q000209:etiology*
D000906:Antibodies; D005968:Glutamate Decarboxylase