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NEUROLOGY 2006;66:932-934
© 2006 American Academy of Neurology
Brief Communications

Electrophysiologic characterization in spinocerebellar ataxia 17

F. Manganelli, MD, A. Perretti, MD, M. Nolano, MD, B. Lanzillo, MD, A. C. Bruni, MD, G. De Michele, MD, A. Filla, MD and L. Santoro, MD

From Department of Neurologic Sciences (F.M., A.P., G.D., A.F., L.S.), Federico II University, Naples, Italy; Salvatore Maugeri Foundation (M.N., B.L.), IRCCS, Center of Telese Terme, Telese Terme, Italy; Regional Neurogenetic Center (A.C.B.), Lamezia Terme, Lamezia Terme, Italy.

Address correspondence and reprint requests to Dr. Lucio Santoro, Dipartimento di Scienze Neurologiche, Università degli Studi di Napoli "Federico II", via Sergio Pansini 5, 80131 Naples, Italy; e-mail: lusantor{at}unina.it

The authors performed a multimodal electrophysiologic evaluation in nine patients belonging to four SCA17 (spinocerebellar ataxia type 17) families. Peripheral nerve and visual system were not involved. Brainstem auditory evoked potentials were constantly abnormal with central type lesions. Magnetic motor evoked potentials were abnormal only in the lower limbs, suggesting a length-dependent involvement of the pyramidal tract. Somatosensory evoked potentials were abnormal in almost all our patients, and abnormalities were consistent with a somatosensory pathway involvement along the brainstem.

Disclosure: The authors report no conflicts of interest.

Received September 20, 2005. Accepted in final form December 19, 2005.






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