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NEUROLOGY 2006;67:1068-1070
© 2006 American Academy of Neurology
Brief Communications

Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies

Goran Rakocevic, MD, Raghavan Raju, PhD, Cristina Semino-Mora, MD, PhD and Marinos C. Dalakas, MD

From Neuromuscular Diseases Section (G.R., R.R., M.C.D.), National Institutes of Neurological Disorders and Stroke, NIH, Bethesda, MD; and Laboratory of Gastrointestinal and Liver Studies (C.S.-M.), Uniformed Services University of the Health Sciences, Bethesda, MD.

We report five of 38 patients with stiff person syndrome (SPS), who also had cerebellar disease, gait ataxia, dysarthria, and oculomotor dysfunction (SPS-Cer). Cerebellar manifestations either preceded SPS or occurred concurrently. Brain MRI was normal. The intrathecal production of glutamic acid decarboxylase antibodies was elevated. {gamma}-Aminobutyric acid–enhancing drugs and immunotherapies improved only the stiffness. SPS-Cer is a distinct subset of SPS causing a more severe and complex clinical phenotype.

Supported by the Intramural Research Program of the NIH, National Institute of Neurological Disorders and Stroke.

Disclosure: The authors report no conflicts of interest.

Received November 21, 2005. Accepted in final form May 23, 2006.

Address correspondence and reprint request to Marinos C. Dalakas, MD, Chief, Neuromuscular Diseases Section, NINDS, Bldg.10, Room 4N248, 10 Center Drive, MSC 1382, Bethesda, MD 20892; e-mail: dalakasm{at}ninds.nih.gov.




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C. R. Gordon, A. Z. Zivotofsky, T. Siman-Tov, N. Gadoth, and M. C. Dalakas
Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies
Neurology, April 3, 2007; 68(14): 1161 - 1161.
[Full Text] [PDF]

Correspondence:

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Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies
Carlos R. Gordon, et al.
Neurology Online, 4 Dec 2006 [Full text]
Reply from the Authors
Marinos C. Dalakas
Neurology Online, 4 Dec 2006 [Full text]

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