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<p>Spinocerebellar ataxias (SCAs) are heterogeneous neurodegenerative disorders with variable phenotypes. Somatosensory evoked potentials (SSEPs) provide insights into the integrity of the dorsal-lemniscal pathway. This study aimed to evaluate SSEP findings in patients with SCA3 and SCA10 and compare their neurophysiological profiles. We conducted a cross-sectional study including 20 SCA3 patients and 20 SCA10 patients under follow-up at a tertiary ataxia clinic in Southern Brazil. Clinical assessment comprised demographic, genetic, and phenotypic characterization, including the Scale for the Assessment and Rating of Ataxia (SARA). SSEPs were recorded bilaterally after median and tibial nerve stimulation, assessing latencies and amplitudes of N9, N20, N21, and P40 potentials, as well as interpotential intervals. Examinations were classified as normal or abnormal, and abnormalities were categorized. Abnormal SSEPs were detected in 18 SCA3 patients (90%) and in 3 SCA10 patients (15%) (<i>p</i> &lt; 0.001). In SCA3, abnormalities predominantly involved central generators. In contrast, most SCA10 patients exhibited normal SSEPs, with only a few isolated cortical responses and interval abnormalities. No significant associations were found between SSEP abnormalities and demographic, clinical, imaging, or genetic variables in either group. SCA3 shows widespread somatosensory dysfunction, whereas SCA10 patients, particularly in Southern Brazil, show preserved SSEPs consistent with pure cerebellar ataxia. These findings highlight distinct neurophysiological signatures in SCAs, reinforcing their heterogeneity and supporting SSEPs as candidate markers for differential characterization, requiring validation.</p>

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Somatosensory Evoked Potentials in Spinocerebellar Ataxia Type 3 and Type 10

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年4月17日

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  • Léo Coutinho,
  • Otto Jesus Hernandez Fustes,
  • Carlos Henrique Ferreira Camargo,
  • Gabriel Abrahão Stoliar,
  • Francisco Manoel Branco Germiniani,
  • Salmo Raskin,
  • Tetsuo Ashizawa,
  • Cláudia Suemi Kamoi Kay,
  • Paulo José Lorenzoni,
  • Renata Dal-Prá Ducci,
  • Rosana Herminia Scola,
  • Hélio A. Ghizoni Teive

摘要

Spinocerebellar ataxias (SCAs) are heterogeneous neurodegenerative disorders with variable phenotypes. Somatosensory evoked potentials (SSEPs) provide insights into the integrity of the dorsal-lemniscal pathway. This study aimed to evaluate SSEP findings in patients with SCA3 and SCA10 and compare their neurophysiological profiles. We conducted a cross-sectional study including 20 SCA3 patients and 20 SCA10 patients under follow-up at a tertiary ataxia clinic in Southern Brazil. Clinical assessment comprised demographic, genetic, and phenotypic characterization, including the Scale for the Assessment and Rating of Ataxia (SARA). SSEPs were recorded bilaterally after median and tibial nerve stimulation, assessing latencies and amplitudes of N9, N20, N21, and P40 potentials, as well as interpotential intervals. Examinations were classified as normal or abnormal, and abnormalities were categorized. Abnormal SSEPs were detected in 18 SCA3 patients (90%) and in 3 SCA10 patients (15%) (p < 0.001). In SCA3, abnormalities predominantly involved central generators. In contrast, most SCA10 patients exhibited normal SSEPs, with only a few isolated cortical responses and interval abnormalities. No significant associations were found between SSEP abnormalities and demographic, clinical, imaging, or genetic variables in either group. SCA3 shows widespread somatosensory dysfunction, whereas SCA10 patients, particularly in Southern Brazil, show preserved SSEPs consistent with pure cerebellar ataxia. These findings highlight distinct neurophysiological signatures in SCAs, reinforcing their heterogeneity and supporting SSEPs as candidate markers for differential characterization, requiring validation.