Subject(s)
Electric Stimulation Therapy/methods , Glucosylceramidase/metabolism , Parkinson Disease/enzymology , Parkinson Disease/therapy , Subthalamic Nucleus/physiology , Adult , DNA Mutational Analysis , Female , Glucosylceramidase/genetics , Humans , Longitudinal Studies , Middle Aged , Mutation/genetics , Parkinson Disease/genetics , Retrospective Studies , Severity of Illness IndexABSTRACT
BACKGROUND: We hypothesized that postural instability and cognitive decline in patients with Richardson's syndrome could be a consequence of reduced thalamic and frontal metabolism. Severe Parkinsonian signs in patients with progressive supranuclear palsy-parkinsonism may be reflected by alterations in putaminal metabolism. METHODS: Eleven patients with Richardson's syndrome, 8 patients with progressive supranuclear palsy-parkinsonism, 12 with Parkinson's disease, and 10 controls underwent clinical assessment and fluorodeoxyglucose positron emission tomography (PET). RESULTS: Richardson's syndrome patients showed pronounced thalamic hypometabolism, and patients with progressive supranuclear palsy-parkinsonism pronounced putaminal hypometabolism, compared to all other investigated groups. The putamen/thalamus uptake ratio differentiated progressive supranuclear palsy-parkinsonism from Richardson's syndrome (area under the curve 5 0.86) and from Parkinson's disease (area under the curve 5 0.80) with acceptable accuracy. Frontal hypometabolism was predominantly found in Richardson's syndrome patients. CONCLUSIONS: Richardson's syndrome, progressive supranuclear palsy-parkinsonism and Parkinson's disease showed different metabolic patterns in fluorodeoxyglucose PET.