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KMID : 1100220050040010001
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Dementia and Neurocognitive Disorders
2005 Volume.4 No. 1 p.1 ~ p.5
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Overview of Frontotemporal Lobar Degeneration
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Park Key-Chung
Yoon Soo-Jin
Na Duk-Lyul
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Abstract
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Frontotemporal lobar degeneration (FTLD) predominatly affects the frontal and anterior part of the temporal cortex in the early stage, a feature distinct from Alzheimer disease which primarily involves the temporal and the parietal lobes. FTLD is clinically classified into frontotemporal dementia (FTD), semantic dementia (SD) and progressive nonfluent aphasia (PA). FTD usually presents with personality change and frontal executive dysfunction with preservation of episodic memory and topographical orientation. SD and PA manifest language disturbances as intial symptoms; the former is characterized by fluent but decreased production and auditory comprehesion of words (especially noun) while the latter is characterized by non-fluent aphasia of Broca or transcortical motor type. CT or MRI in patients with FTD usually shows atrophy of the frontal lobe and the anterior portion of the temporal lobe. Functional imaging (SPECT or PET) studies, however, demonstrate more extensive lesion involving the uncus, the insula, the cingulate gyrus and the subcortical structures such as the basal ganglia and the medial region of the thalamus in addition to the frontal and the anterior temporal cortex. Atrophy in structural images and functional alteraltions in functional images usually demonstrate hemispheric asymmetry, and correlations exist between structural and functional changes. About 10% of FTD patients accompay motor neuron disease (MND) and it has been reported that MND combined cases show frontal dominant hypometabolism than FTD alone cases and the frontal involvement is relatively symmetric compared to FTD only group. Pathologically, FTD can be classified into Pick disease type, frontal lobe degeneration type and motor neuron disease type.
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KEYWORD
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Frontotemporal lobar degeneration, Frontotemporal dementia, Semantic dementia, Progressive non-fluent aphasia, Motor neuron disease, Pick disease
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