title: Early clinical features of patients with progressive supranuclear palsy with predominant cerebellar ataxia.
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.
authors.affiliation: Department of Neurology, Brain Research Institute, Niigata University, 1-757 Asahimachi-dori, Chuoku, Niigata 951-8585, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Kanazawa
authors.forename: Mari
authors.initials: M
authors.lastname: Tada
authors.forename: Osamu
authors.initials: O
authors.lastname: Onodera
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Shimohata
mesh_terms: D000058:Accidental Falls; D017668:Age of Onset; D000368:Aged; D002524:Cerebellar Ataxia / Q000209:etiology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000150:complications* / Q000175:diagnosis*; D012189:Retrospective Studies; D013494:Supranuclear Palsy, Progressive / Q000150:complications* / Q000175:diagnosis*
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