Parkinsonian features of Steele-Richardson-Olszewski syndrome
Fenelon, G.; Guillard, A.; Romatet, S.; Feve, A.; Mahieux, F.
Revue Neurologique (Paris) 149(1): 30-36
1993
ISSN/ISBN: 0035-3787 PMID: 8337559 Document Number: 406413
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. Although the parkinsonian syndrome is, at least in part, probably due to lesions of the nigro-striatal dopaminergic pathway, other lesions, in particular of the pallidum, might play a part in its genesis. It is suggested that forms of Steele-Richardson-Olszewski syndrome mimicking Parkinson's disease at the onset are initially associated with lesions predominating in the substantia nigra. However, in most cases the parkinsonian signs differ from those of Parkinson's disease. This could be due to associated lesions of the pallidum and/of the subthalamic nucleus. Finally, the primary or secondary resistance to dopaminergic agents might be due to a loss of striatal dopaminergic receptors, to lesions situated downstream (eg in the pallidum) or to other non-dopaminergic lesions.