[Parkinsonian features of Steele-Richardson-Olszewski syndrome].

Abstract

A study of parkinsonian signs was conducted in 29 patients fulfilling Lees' criteria for progressive supranuclear palsy, or Steele-Richardson-Olszewski syndrome, and 2 patients with incomplete ophthalmoplegia but 4 other cardinal features. The patients comprised 17 men and 14 women and their ages at onset ranged from 52 to 77 yrs (mean, 65.1). Parkinsonian features: akinesia, rigidity or resting tremor were present in 29 cases and appeared a mean 0.5 yrs (range 0-3) after onset. In 9 cases (29 p. 100), the parkinsonian features were similar to those of Parkinson's disease, including a resting tremor in 6 cases. The parkinsonian syndrome was initially the sole manifestation in 7 patients, who were diagnosed as having Parkinson's disease. Levodopa was effective in 6 cases, for more than a year in 3. In the other 20 cases (64 p. 100) parkinsonian signs were atypical, usually with a predominantly axial distribution. Levodopa or dopamine agonists showed transient efficacy in 6 cases. In most patients, the treatment with levodopa (n = 27) or bromocriptine (n = 15) was well tolerated. Only two disclosed akinetic fluctuations. The other main clinical features included postural instability with falls (n = 30; mean time after onset 1.8 yrs, range 0-7 yrs); characteristic ophthalmoplegia (n = 29; mean interval 4.4., range 0-18 yrs); intellectual decline and/or frontal signs (n = 22; mean interval 2.4, range 0-8 yrs); axial rigidity (n = 18; mean interval 4.2, range 1-11 yrs); pseudo-bulbar palsy (n = 18; mean interval 3, range 0-8 yrs). An upper limb dystonia was present in an autopsy-proven case.(ABSTRACT TRUNCATED AT 250 WORDS)