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Sharon Hassin-Baer, MD; Eilon D. Kirson, MD, PhD; Lester Shulman, PhD; Aron S. Buchman, MD; Hanna Bin, PhD; Musa Hindiyeh, PhD; Lea Markevich; Ella Mendelson, PhD

Arch Neurol. 2004;61:938-941.

Background  Stiff-person syndrome is a rare autoimmune disorder associated with antibodies against glutamic acid decarboxylase (GAD), the key enzyme in -aminobutyric acid synthesis. In most cases, a trigger cannot be identified.

Objective  To describe a 41-year-old man who developed stiff-person syndrome and antibodies to GAD following acute West Nile virus infection.

Design  A case report and a search in GenBank for common epitopes.

Result  The search revealed a stretch of 12 amino acids in the NS1 protein of West Nile virus with a high degree of homology to the GAD65 region (an isoform of GAD) containing the PEVKEK motif.

Conclusion  Cross-reactivity between antibodies directed against West Nile virus and GAD may have contributed to the development of stiff-person syndrome in this patient.

From the Department of Neurology, Chaim Sheba Medical Center, Tel Hashomer, Israel (affiliated with the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel) (Drs Hassin-Baer, Kirson, and Buchman); the Central Virology Laboratory, Public Health Laboratories, Ministry of Health, Tel-Hashomer (Drs Shulman, Bin, Hindiyeh, and Mendelson); the Institute of Endocrinology, Tel-Aviv Sourasky Medical Center, Tel-Aviv (Ms Markevich); the Faculty of Life Sciences, Bar-Ilan University, Ramat Gan, Israel (Dr Mendelson); and the Department of Neurological Sciences, Rush Medical College, Chicago, Ill (Dr Buchman).