Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study.

Abstract

Objective-To analyse the natural history ofprogressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) and clinical predictors of survival in 24 patients with PSP confirmed by necropsy, who fulfilled the NINDS criteria for a neuropathological diagnosis of typical PSP. Methods-Patients were selected from the research and clinical files of seven medical centres involving tertiary centres of Austria, England, France, and the United States. Clinical features were analysed in detail. The patients' mean age at onset of PSP was 63 (range 45-73) years. Results-The most frequent clinical features (occurring in at least 75% of the patients) were early postural instability and falls, vertical supranuclear palsy, akinetic-rigid predominant parkinsonian disorder characterised by symmetric bradykinesia and axial rigidity unrelieved by levodopa, pseudobulbar palsy, and frontal release signs. Occasionally, segmental dystonia or myoclonus were described, but neither aphasia nor alien limb syndrome was reported. Fractures occurred in 25% of the patients but were unrelated to the severity of the gait or to the presence of falls. Median survival time was 5*6 (range 2-16-6) years. Onset of falls during the first year, early dysphagia, and incontinence predicted a shorter survival time. Age at onset, sex, early onset of dementia, vertical supranuclear palsy, or axial rigidity had no effect on prognosis of survival. Pneumonia was the most common immediate cause of death. PSP was most often clinically misdiagnosed as Parkinson's disease. Errors in diagnosis suggest that PSP is underdiagnosed.Conclusion-Progressive onset of early postural instability with falls or supranuclear vertical palsy in the fifth decade, should suggest the diagnosis of PSP. Onset of falls during the first year are emphasised, as they could lead to an early diagnosis and influence the prognosis of patients with PSP. Whether appropriate treatment of the dysphagia could prolong the survival of PSP patients needs to be explored.(7 Neurol Neurosurg Psychiatry 1996;61:615-620)

PSP as Distinguished from CBD, MSA-P and PD by Clinical and Imaging Differences at an Early Stage

“…PD patients (80-100%) showed a much better response to L-DOPA than PSP (24%), CBD (18%) and MSA-P (19%) patients. As compared with previous reports on PSP, the present results on clinical findings (Table 1) were compatible with previous studies (11)(12)(13)(14)(15)(16). Of importance regarding the clinical characteristics of PSP is supranuclear gaze disturbance, OKN impairment and falls at the first visit, while CBD and MSA-P show much less frequent supranuclear gaze disturbance, OKN impairment and falls and the same response to L-DOPA, and PD shows much less frequent supranuclear gaze disturbance, OKN impairment and falls and a much better response to L-DOPA.…”

ISIS Survey: an international study on the diagnostic process and its implications in amyotrophic lateral sclerosis

“…The PSP and CBD patients had been previously independently reported [17,19]. The patients' records were abstracted on standardized forms by neurologists who followed strict instructions.…”

Diagnostic Accuracy of Combined FP-CIT, IBZM, and MIBG Scintigraphy in the Differential Diagnosis of Degenerative Parkinsonism: A Multidimensional Statistical Approach

“…Brain Bank criteria (17). Further, 11 patients were diagnosed as probable MSA-P according to established criteria (18,19), and 6 patients fulfilled the National Institute of Neurologic Disorders and Stroke criteria for probable PSP (20,21).…”