Parkinson's Disease with a Phenotype of Progressive Supranuclear Palsy - a Case Report

Current clinical diagnostic criteria for Parkinson's disease and progressive supranuclear palsy (PSP) are considered relatively safe tools for the clinical differentiation of the two neurodegenerative diseases; neither diagnosis includes the possibility that Parkinson's disease might duplicate the clinical genotype of PSP. We document the case of a male patient who first manifested parkinsonian signs at the age of 56 years. Parkinsonian syndrome developed over the course of the following three years without tremor, but with typical subcortical dementia, gaze palsy and impairment of conjugated gaze upwards, eyelid opening apraxia and gait apraxia. The disease then progressed to sudden falls, typical Lhermitte's frontal signs, utilizations and imitations. Response to L-DOPA therapy disappeared relatively quickly. The findings of paraclinical examinations (MRI, electrophysiology) also suggested a diagnosis of PSP. After approximately four years, the patient died of intercurrent disease. Pathological and histopathological examinations and evaluation of the results by two independent pathologists documented and confirmed changes typical of Parkinson's disease. This case indubitably raises the question of overlap of phenotypes of different types of neurodegenerative diseases with parkinsonian syndrome dominant.