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KMID : 1100220050040020086
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Dementia and Neurocognitive Disorders
2005 Volume.4 No. 2 p.86 ~ p.92
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White Matter Change of the Temporopolar Area and Cognitive Impairment Pattern in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy(CADASIL)
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Ahn Hyun-Jung
Lee Jae-Hong
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Abstract
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Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a disease characterized by recurrent stroke, mood disturbance, and progressive cognitive impairment leading to subcortical vascular dementia. Typical MRI findings in CADASIL include multiple, focal lacunar infarcts and diffuse T2-weighted hyperintensities of the periventricular or deep subcortical white matter. Temporal pole hyperintensity, in particular, is considered a radiological hallmark of CADASIL, if present. This study was conducted to see if there is any difference in the neuropsychological profile between CADASIL patients with the involvement of white matter of temporal lobe and those without.
Methods: Ten CADASIL patients proved by the genetic testing were divided into two groups by the involvement of the temporal lobe on brain MRI. All patients underwent a comprehensive neuropsychological test including language, visuospatial function, memory, and frontal executive functions. We compared the neuropsychological findings and memory impairment pattern between the two groups.
Results: Five patients had white matter involvement of the temporal lobe (TI group, age: 51.8±6.3), whereas five patients had only multiple lacunes or ischemic change in the basal ganglia and periventricular or deep subfrontal white matter (nTI group age: 57.4± 8.7). There was no significant difference between the two groups in age, sex and education level. TI group performed poorer than nTI group on the following tests: digit span backward, praxis, calculation, visuospatial function, verbal memory, non-verbal memory, and frontal executive functions (fist-edge-palm, COWAT letter, Stroop test color reading). On memory test, both recall and recognition were significantly poorer in TI group than in nTI group.
Conclusions: CADASIL patients with involvement of the white matter of the temporal lobe have multiple cognitive dysfunctions. They also manifest a memory impairment produced by learning and retention failure. On the other hand, CADASIL patients without involvement of the white matter of the temporal lobe only have a mild memory decline and a retrieval deficit pattern. The temporal pole involvement seems to produce two neuropsychologically distinguishable subtypes of CADASIL patients. A further large study is needed to confirm these findings.
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KEYWORD
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CADASIL, White matter change, Neuropsychological test
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