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Olfactory dysfunction in familial parkinsonism
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Impaired olfactory function is commonly observed in idiopathic Parkinson's
disease (IPD).

However, it is unknown whether it is also found in familial parkinsonism.

To address this issue we administered a smell test to 12 affected, three
monosymptomatic, and 12 at-risk individuals from six large parkinsonian
kindreds.

Three kindreds exhibited an IPD phenotype and three exhibited a
parkinsonism-plus syndrome (PPS) phenotype.

All but one of the affected individuals had impaired olfactory function.

In contrast, only five of the 12 at-risk individuals had impaired olfactory
function.

The degree of olfactory impairment in the at-risk individuals was less
severe than in the affected individuals.

The difference in the degree of olfactory impairment in individuals
exhibiting the IPD and the PPS phenotypes was not statistically significant.

These findings suggest that olfactory dysfunction is a phenotypic
characteristic of familial parkinsonism and that it is independent of the
kindred phenotype.

The appearance of olfactory dysfunction soon after disease onset raises the
possibility that it is part of the neurodegenerative disease process.


Neurology 1997 Nov;49(5):1262-7
Markopoulou K, Larsen KW, Wszolek EK, Denson MA
Lang AE, Pfeiffer RF, Wszolek ZK
Section of Neurology, University of Nebraska Medical Center, Omaha, NE, USA.
PMID: 9371905   UI: 98039174
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