Electrophysiological features in patients and presymptomatic relatives with spinocerebellar ataxia type 2

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• 作者：Luis Velá ; zquez Pé ; rez ; Gilberto Sá ; nchez Cruz ; Nalia Canales Ochoa ; Roberto Rodrí ; guez Labrada ; Julio Rodrí ; guez Dí ; az ; Luis Almaguer Mederos ; José ; Laffita Mesa
• 关键词：Spinocerebellar ataxia type 2 (SCA2) ; Cuban ataxia ; Electrophysiological disorders ; Sensory axonopathy ; Nerve conduction studies ; Evoked potentials
• 刊名：Journal of the Neurological Sciences
• 出版年：2007
• 出版时间：15 December 2007
• 年：2007
• 卷：263
• 期：1-2
• 页码：158-164
• 全文大小：179 K

文摘

Motor and sensitive nerve conduction studies, visual (VEP), brainstem auditory (BAEP) and somatosensory (SSEP) evoked potentials in 82 patients with spinocerebellar ataxia type 2 (SCA2), 62 presymptomatics relatives and 80 controls, correlating it with CAG repeat, disease duration and ataxia score were assessed. All the groups showed differences in the amplitude of sensory action potentials in median and sural nerves. Sural amplitude was negatively correlated with disease duration and ataxia score. Differences among patients and controls in the mean latency and conduction velocity of sensory action potentials were found. Abnormal morphology and replicability of SSEPs and BSAEPs were found in patients and presymptomatics. Moreover, consistent increase in latencies of P40 component of SSEPs, III and V peaks and I–III interpeak of BSAEPs was found in patients. A positive correlation was found between latency of III and V waves, I–III and III–V interpeak latencies of BSAEPs and disease duration. CAG repeat and electrophysiological markers assessed were not correlative. Electrophysiological alterations in the SCA2 appeared in presymptomatic stages. These alterations are consistent markers which could be used to evaluate the progression of the disease.