Early clinical features of patients with progressive supranuclear palsy with predominant cerebellar ataxia

Parkinsonism Relat Disord. 2013 Dec;19(12):1149-51. doi: 10.1016/j.parkreldis.2013.07.019. Epub 2013 Aug 3.

Abstract

Background: Patients who develop progressive supranuclear palsy with predominant cerebellar ataxia (PSP-C) develop cerebellar ataxia as the initial and principal symptom, may be misdiagnosed as having multiple system atrophy with predominant cerebellar features (MSA-C). Therefore, we investigated the clinical signs and symptoms between PSP-C and MSA-C early in their disease course.

Methods: We reviewed the medical records of 15 consecutive patients with pathologically proven PSP-C (4) and MSA-C (11). We recorded the presence or absence of clinical features that developed within 2 years of disease onset.

Results: The age at onset of PSP-C patients was older than that of MSA-C patients (p = 0.009). The frequencies of falls were higher in PSP-C patients than in MSA-C patients (p = 0.026). Additionally, the development of supranuclear vertical gaze palsy was higher in PSP-C patients than in MSA-C patients (p = 0.011), whereas the frequency of dysautonomia was lower in PSP-C patients than in MSA-C patients (p = 0.035).

Conclusions: Older onset, early falls, and supranuclear vertical gaze palsy without dysautonomia may predict the diagnosis of PSP-C in patients with late-onset sporadic cerebellar ataxia.

Keywords: Cerebellar ataxia; Falls; MSA-C; Multiple system atrophy; NINDS-SPSP; National Institute for Neurological Diseases and Stroke – the Society for Progressive Supranuclear Palsy; PSP; PSP-C; Progressive supranuclear palsy; Supranuclear vertical gaze palsy; multiple system atrophy with predominant cerebellar features; progressive supranuclear palsy; progressive supranuclear palsy with predominant cerebellar ataxia.

MeSH terms

  • Accidental Falls
  • Age of Onset
  • Aged
  • Cerebellar Ataxia / etiology*
  • Diagnosis, Differential
  • Female
  • Humans
  • Male
  • Middle Aged
  • Multiple System Atrophy / complications*
  • Multiple System Atrophy / diagnosis*
  • Retrospective Studies
  • Supranuclear Palsy, Progressive / complications*
  • Supranuclear Palsy, Progressive / diagnosis*