Distinguishing spinocerebellar ataxia with pure cerebellar manifestation from multiple system atrophy (MSA-C) through saccade profiles

Clin Neurophysiol. 2017 Jan;128(1):31-43. doi: 10.1016/j.clinph.2016.10.012. Epub 2016 Oct 29.

Abstract

Objective: Patients with spinocerebellar ataxia with pure cerebellar presentation (SCD) and multiple system atrophy (MSA-C) show similar symptoms at early stages, although cerebellofugal pathology predominates in SCD, and cerebellopetal pathology in MSA-C. We studied whether saccade velocity profiles, which reflect the accelerating and braking functions of the cerebellum, can differentiate these two disorders.

Methods: We recorded visually guided (VGS) and memory guided saccades (MGS) in 29 MSA-C patients, 12 SCD patients, and 92 age-matched normal subjects, and compared their amplitude, peak velocity and duration (accelerating and decelerating phases).

Results: Hypometria predominated in VGS and MGS of MSA-C, whereas hypometria was less marked in SCD, with hypermetria frequently noted in MGS. Peak velocity was reduced, and deteriorated with advancing disease both in SCD and MSA-C groups at smaller target eccentricities. The deceleration phase was prolonged in SCD compared to MSA-C and normal groups at larger target eccentricities, which deteriorated with advancing disease.

Conclusion: Saccades in MSA-C were characterized by a more prominent acceleration deficit and those in SCD by a more prominent braking defect, possibly caused by the cerebellopetal and cerebellofugal pathologies, respectively.

Significance: Saccade profiles provide important information regarding the accelerating and braking signals of the cerebellum in spinocerebellar ataxia.

Keywords: Cerebellum; Fastigial nucleus; Multiple system atrophy; Peak velocity; Saccade metrics; Spinocerebellar ataxia.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Cerebellum / physiopathology*
  • Electrooculography / methods*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Multiple System Atrophy / diagnosis
  • Multiple System Atrophy / physiopathology*
  • Photic Stimulation / methods*
  • Reaction Time / physiology
  • Saccades* / physiology
  • Spinocerebellar Ataxias / diagnosis
  • Spinocerebellar Ataxias / physiopathology*