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NEUROLOGY 2004;62:1357-1362
© 2004 American Academy of Neurology

Stiff-person syndromes

Motor cortex hyperexcitability correlates with anti-GAD autoimmunity

Claudia Koerner, MD, Bettina Wieland, Wiltrud Richter, PhD and Hans-Michael Meinck, PhD

From the Departments of Neurology (Drs. Koerner and Meinck, and B. Wieland) and Orthopedics (Dr. Richter), Ruprecht-Karls-University Heidelberg, FRG.

Address correspondence and reprint requests to Dr. Claudia Koerner, Central Institute of Mental Health, J 5, 68159 Mannheim, FRG; e-mail: koerner{at}zi-mannheim.de

Objectives: To investigate whether motor cortex excitability is enhanced in both stiff-person syndrome (SPS) and its "plus" variant, progressive encephalomyelitis with rigidity (PER), and related to autoimmunity against glutamic acid decarboxylase (GAD).

Methods: The authors compared 21 patients with SPS or PER (7 untreated, 14 treated) with 14 age-matched healthy controls and used transcranial magnetic stimulation (TMS, paired-pulse paradigm) to investigate intracortical inhibition (ICI) and intracortical facilitation (ICF). GAD autoantibody levels in serum and CSF were determined by radioimmunoassay.

Results: The authors found significantly enhanced motor cortex excitability in untreated SPS and PER patients. GABAmimetic medication significantly reduced ICF but did not affect ICI. Motor cortex excitability was more enhanced in patients with GAD antibodies than in patients without GAD antibodies and correlated positively with GAD antibody levels in CSF.

Conclusions: The motor cortex is hyperexcitable in SPS and PER patients. However, hyperexcitability is partly masked by GABAmimetic treatment. Correlation of elevated GAD antibody levels with enhanced ICF suggests that motor cortex hyperexcitability in SPS and PER is related to anti-GAD autoimmunity.

Received August 18, 2003. Accepted in final form December 18, 2003.






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