RESEARCH: Presynaptic parkinsonism in multiple system atrophy mimicking Parkinson's disease

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Mov Disord 2002 Jul;17(4):812-816

Presynaptic parkinsonism in multiple system atrophy mimicking Parkinson's

disease: A clinicopathological case study.

Berciano J, Valldeoriola F, Ferrer I, Rumia J, Pascual J, Marin C, Rey MJ,

Tolosa E.

Service of Neurology, University Hospital Marques de Valdecilla, University

of Cantabria, Santander, Spain.

We describe the clinicopathological findings in a patient aged 63 years at

death who, at age 55 years, developed levodopa-responsive parkinsonism with

no atypical features. A diagnosis of idiopathic Parkinson's disease (PD) was

made. During the clinical course, fluctuations and dyskinesias appeared.

Eight years after onset, he was successfully treated with subthalamic

nucleus stimulation but died 3 weeks postoperatively from pulmonary embolus.

Brain autopsy showed marked neuronal loss and gliosis in the substantia

nigra and locus coeruleus, and, to a much lesser extent, in the basis

pontis, inferior olivary nuclei, and cerebellar cortex. Striatum was normal.

There were numerous oligodendroglial and neuronal cytoplasmic inclusions and

neuropil threads, the highest density being localized in the pons and

cerebellar white matter. No Lewy bodies were observed. We conclude that

nigral, presynaptic parkinsonism may occur in multiple system atrophy, which

even in the long run can be indistinguishable from PD. Putaminal

preservation accounts for good response to both levodopa therapy and

subthalamic nucleus stimulation. Copyright 2002 Movement Disorder Society

PMID: 12210882 [PubMed - as supplied by publisher]