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KMID : 0960920030020010036
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Dementia and Neurocognitive Disorders
2003 Volume.2 No. 1 p.36 ~ p.41
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Clinical and Neuropsychological Findings in Frontotemporal Dementia with Motor Neuron Disease
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Park Key-Chung
Jeong Yong
Kim Eun-Joo
Chin Ju-Hee
Kang Sue-Jin
Na Duk-Lyul
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Abstract
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Backgrounds: Frontotemporal dementia (FTD) is rarely associated with motor neuron disease (MND). This comorbidity (FTD-MND), a subtype of FTD, results in progressive dementia and muscle weakness. Among the few available reports of series of patients, however, there have been controversies about the clinical course of FTD-MND. This study, the first report of a series of FTD-MND patients in Korea, investigated demographic and clinical features, clinical course, and neuropsychological findings of nine patients with FTD-MND.
Methods: Nine FTDMND patients (2 men and 7 women with mean age 55.6+/-7.2 years) were selected among 45 FTD patients who met the FTD criteria proposed by the Lund and Manchester Groups. Their clinical and neuropsychological findings were analyzed retrospectively.
Results: Mean age of onset was 54.3+/-8.0 years. The time interval from onset to death was 27+/-11.3 months. Presenting symptoms were personality change, hyperphagia, nonfluent speech or motor weakness. In most cases (8/9), dementia preceded the muscle weakness; in only one patient the muscle weakness preceded the cognitive decline. Regarding the muscle weakness, bulbar symptoms occurred earlier than limb weakness in four patients; bulbar and limb weakness occurred simultaneously in another four patients; in the remaining one patient limb weakness preceded bulbar weakness. Neuropsychologic tests showed no specific patterns but diffuse dysfunction in all cognitive domains.
Conclusions: Our study suggests that FTD-MND predominantly affects the bulbar muscles rather than the limb muscles in early stage and symptoms related to MND occurs later than those related to FTD. The time from diagnosis to death was 10.6+/-6.5 months, suggesting that FTD-MND is a rapidly progressive disease. This rapid clinical course may account for our neuropsychological findings that showed general cognitive deficits rather than predominant frontal dysfunctions.
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KEYWORD
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Frontotemporal dementia, Motor neuron disease, Dementia, Bulbar weakness
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