Abstract for presentation at The Australian and New Zealand Association of Neurologists Annual Scientific Meeting 2007

Background: Despite established clinical diagnostic criteria for Progressive Supranuclear Palsy (PSP) cases resembling Parkinson's disease (PD) are well recognised. Atypical features for PD are however almost always present late in the clinical course. We report a case of pathologically proven PSP with a typical and prolonged clinical course for PD.
Case Report: In 1985 a 65 year old man was diagnosed with PD after presenting with rest tremor involving the left hand and foot, bradykinesia of finger movements, cogwheeling and reduced arm swing. Levodopa led to significant improvement and he remained clinically stable (modified Webster's score between 11 and 13; equates to mild disability) for the next 10 years. In 1997 he developed increasing levodopa-related motor fluctuations and dyskinesias, postural instability and gate freezing. He declined gradually but continued to respond well to levodopa (modified Webster's score "OFF" 19, "ON" 13 at levodopa challenge in 1998). In 2002 he developed hallucinations and mild delusions characteristic of levodopa-related neuropsychiatric toxicity. Despite occasional falls he remained independently mobile until 2005 when he died from pneumonia. At no time did he develop significant cognitive impairment, supra nuclear gaze palsy, bulbar dysfunction or axial rigidity. Histological examination revealed marked neuronal loss in substantia nigra and locus caeruleus. No Lewy bodies were found in brain stem nuclei, basal forebrain or cerebral cortex. Immunohistochemical staining showed extensive neuronal tau positive cytoplasmic neurofibrillary tangles in substantia nigra, globus pallidus, caudate nucleus, putamen, and medulla oblongata in a typical distribution for PSP. Alpha-synuclein staining was minimal.
Conclusions: Clinicopathological series have highlighted clinical features useful in distinguishing PSP from PD. This exceptional case is reported to highlight that rarely PSP can mimic the "full hand" of clinical features thought typical for PD.