• Title/Summary/Keyword: Subcortical vascular dementia

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Diagnosis, Treatment and Prevention of Subcortical Vascular Dementia with a Case Report (피질하 혈관성치매의 진단, 치료 및 예방 : 1례 보고와 함께)

  • Choi, Seong-Hun;Ku, Sae-Kwang;Cheon, Woo-Hyun;Baek, Kyung-Min;Han, Chang-Hyun;Kyung, Jeon-Won;Lee, Young-Joon
    • Journal of Society of Preventive Korean Medicine
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    • v.14 no.3
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    • pp.63-75
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    • 2010
  • Vascular dementia(VaD) is currently considered to be the second most common type of dementia. VaD is not a single diagnostic entity, but a heterogeneous syndrome which encompasses several clinicopathological forms of dementia resulting from cerebrovascular diseases. A common form of VaD is subcortical VaD which is characterized by lacunar infarcts and deep white matter changes, leading to a progressive decline in memory and cognitive function. The neuropsychological and cognitive profiles of subcortical VaD have been reported relatively homogeneous. At present, subcortical vascular dementia is regarded as the most important subtype of VaD with getting the attention of vascular dementia. The aims of this study are to discuss the concept of subcortical VaD and its importance focusing on diagnosis, prevention and treatment with a case report.

Differences of Tc-99m HMPAO SPECT Imaging in the Early Stage of Subcortical Vascular Dementia Compared with Alzheimer's Disease (초기 단계의 피질하 혈관성 치매와 알쯔하이머병에서 Tc-99m HMPAO SPECT 영상 소견 차이)

  • Park, Kyung-Won;Kang, Do-Young;Park, Min-Jeong;Cheon, Sang-Myung;Cha, Jae-Kwan;Kim, Sang-Ho;Kim, Jae-Woo
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.6
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    • pp.530-537
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    • 2007
  • Purpose: The aim of this study is to assess the specific patterns of regional cerebral blood flow (rCBF) in patients with the early stage of subcortical vascular dementia (SVaD) and Alzheimer's disease (AD) using Tc-99m HMPAO SPECT, and to compare the differences between the two conditions. Materials and Methods: Sixteen SVaD, 46 AD and 12 control subjects participated in this study. We included the patients with SVaD and AD according to NINCDS-ADRDA and NINDS-AIREN criteria. They were all matched for age, education and clinical dementia rating scores. Three groups were evaluated by Tc-99m HMPAO SPECT using statistical parametric mapping (SPM) for measuring rCBF. The SPECT data of patients with SVaD and AD were compared with those of normal control subjects and then compared with each other. Results: SPM analysis of the SPECT image showed significant perfusion deficits on the right temporal region and thalamus, left insula and superior temporal gyrus, both cingulate gyri and frontal subgyri in patients with SVaD and on the left supramarginal gyrus, superior temporal gyrus, postcentral gyrus and inferior parietal lobule, right fugiform gyrus and both cingulate gyri in AD compared with control subjects (uncorrected p<0.01). SVaD patients revealed significant hypoperfusion in the right parahippocampal gyrus with cingulated gyrus, left insula and both frontal subgyral regions compared with AD (uncorrected p<0.01). Conclusion: Our study shows characteristic and different pattern of perfusion deficits in patients with SVaD and AD, and these results may be helpful to discriminate the two conditions in the early stage of illness.

Importance of CADASIL research in Jeju: a review and update on epidemiology, diagnosis, and clinical spectrum (제주도에서 CADASIL 연구의 중요성: 역학, 진단 및 임상양상에 대한 고찰)

  • Choi, Jay Chol;Lee, Jung Seok;Kim, Kitae
    • Journal of Medicine and Life Science
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    • v.17 no.3
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    • pp.65-73
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    • 2020
  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a single-gene disease of the cerebral small blood vessels caused by mutations in the NOTCH3 gene on chromosome 19. Although CADASIL was known as a rare disease, recent research has suggested that the NOTCH variants could be found frequently even in the general population. The main clinical features included recurrent stroke, migraine, psychiatric symptoms, and progressive cognitive decline. On brain magnetic resonance imaging, patients with CADASIL showed multifocal white matter hyperintensity lesions, lacunar infarcts, microbleeds, and brain atrophy. Among them, lacunar infarcts and brain atrophy are important in predicting the clinical outcomes of patients with CADASIL. In the Jeju National University Hospital, we have diagnosed 213 CADASIL patients from 2004 to 2020. Most NOTCH3 mutations were located in exon 11 (94.4%), and p.Arg544Cys was the most common mutation. The mean age at diagnosis was 61.0±12.8 years. The most common presenting symptoms were ischemic stroke (24.4%), followed by cognitive impairment(15.0%), headache (8.9%), and dizziness(8.0%). Although the exact prevalence of CADASIL in Jeju is still unknown, the disease prevalence could be as high as 1% of the population considering the prevalence reported in Taiwan. Therefore, it is necessary to discover efficient biomarkers and genetic tests that can accurately screen and diagnose patients suspected of having CADASIL in this region. Ultimately, it is urgent to explore the exact pathogenesis of the disease to identify leading substances of treatment potential, and for this, multi-disciplinary research through active support from the Jeju provincial government as well as the national government is essential.