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The most frequent clinical features(occurring in at least 75%of the patient)were early postural instability and falls,vertical supra-nuclear palsy,akinetic rigid predominant parkinsonian disorder characterized by symmetric bradykinesia and axial rigidity unrelieved by levodopa,pseudo- bulbar 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.Medical 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.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 emphasized,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. |
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dysphagia
falling
fracture,long bone
frontal lobe,pathologic signs of
gaze palsy,supranuclear
gaze palsy,vertical
misdiagnosis
neurologic disease,diagnoses of
neuropathology
prognosis
progressive supranuclear palsy
release phenomena
review article
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