The Clinical Features of 67 Patients with Clinically Definite Steele-Richardson-Olszewski Syndrome

Abstract

The clinical features of 67 patients with probable Steele-Richardson-Olszewski syndrome have been analysed. Bradykinesia was present in 92.5% of cases at the time of initial assessment and the two commonest presenting complaints were walking difficulties and stiffness. Only 11 patients had a supranuclear gaze palsy when first seen. Forty-nine patients developed cognitive impairment in the course of their illness based on neuropsychological testing. In addition to the classical clinical findings occasional other findings included focal dystonia, tonic inhibition of levator palpebrae and blepharospasm, respiratory disturbances and impaired hearing. Only nine cases responded at all to levodopa therapy.

Pathologic correlates of supranuclear gaze palsy with parkinsonism

“…11 SGP is often absent early in the course of the disease with one study reporting supranuclear gaze palsy in only 11/67 patients at initial evaluation and ultimately clinically diagnosed with PSP. 12 Our findings are consistent with the clinical mantra that SGP develops by the time of death in the majority of patients with PSP although we found that a significant minority retained normal eye movements, similar to previous reports in the literature. 13,14 The retrospective nature of our data does not allow us to assess the differential involvement of horizontal vs vertical gaze or of up- vs down-gaze.…”

Neuropsychiatry of the basal ganglia

“…74 The pattern of deficits is characteristic of the so called "subcortical dementia": with bradyphrenia, executive deficits, forgetfulness (related to retrieval difficulties), visuospatial problems, and frontal lobetype behavioural abnormalities (inertia, stereotyped behaviour, apathy, disinterest, environmental dependency, etc) which are most likely related to denervation of the prefrontal cortex. Mood symptoms of depression and lability have also been described.…”

A case presenting with a major depressive episode with palilalia and difficulty opening eyes as prodromal symptoms of progressive supranuclear palsy

“…Of 67 patients with clinically diagnosed PSP, one (1%) had lid levator inhibition at the first assessment with a disease duration of 2–4 years, whereas 17 (25%) had lid levator inhibition at the final assessment with a disease duration of 3–9 years. 20 In our previous study, difficulty in opening eyes was observed in 0 of 22 (0%) patients with PSP, 1 of 9 (11%) patients with pallido‐nigro‐luysian atrophy (PNLA) Type 1, and 0 of 9 (0%) patients with PNLA Type 2 during the first 2 years of the disease. 21 Moreover, the prevalence of difficulty in opening eyes was significantly higher in patients with PNLA Type 1 than in those with PSP and PNLA Type 2 (5/9 [55.6%] vs. 1/22 [4.5%] and 0/9 [0%], respectively) during the entire disease course.…”

“…The prevalence of difficulty in opening eyes may be relatively rare during the early stage of the disease and may vary among pathological subtypes of the PSP spectrum. Of 67 patients with clinically diagnosed PSP, one (1%) had lid levator inhibition at the first assessment with a disease duration of 2–4 years, whereas 17 (25%) had lid levator inhibition at the final assessment with a disease duration of 3–9 years 20 . In our previous study, difficulty in opening eyes was observed in 0 of 22 (0%) patients with PSP, 1 of 9 (11%) patients with pallido‐nigro‐luysian atrophy (PNLA) Type 1, and 0 of 9 (0%) patients with PNLA Type 2 during the first 2 years of the disease 21 .…”