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KMID : 1100220030020020159
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Dementia and Neurocognitive Disorders
2003 Volume.2 No. 2 p.159 ~ p.163
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A Case of Young-Age Onset Vascular Dementia: CADASIL and Its Clinical Progression
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Yoon Soo-Jin
Lee Seung-Hwan
Ryu Hui-Jin
Kim Man-Ho
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Abstract
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A variety of disorders should be included in the differential diagnosis of young-age onset dementia, such as Alzheimer’s disease, vascular dementia, frontotemporal dementia, alcoholrelated dementia, prion disease, and hypoxic brain injury. A 34-year-old man visited our hospital with complaints of memory disturbance and personality change. He had a strong family history of stroke or dementia and also had a few previous stroke episodes. We performed brain imaging, neuropsychological test, and gene study. Brain MRI showed multiple lacunar infarcs and periventricular white matter ischemic changes, which were compatible with lacunar state. Brain MRA was normal. On neuropsychological test, memory disturbance, visuospatial dysfunction, dyscalculia, and frontal dysfunction were noted. The gene study revealed a missense mutation of Notch3 gene, a result consistent with the diagnosis of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). We report a case of youngage onset vascular dementia and its clinical progression.
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KEYWORD
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CADASIL, Young-age onset vascular dementia
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