카다실 환자에서 뇌졸중발생 유무에 따른 임상증상과 뇌자기공명영상소견의 차이
Clinical and MRI Profiles Predicting Clinical Overt Stroke in Patients with CADASIL
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초록
Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)is an inherited arteriopathy caused by mutation in the Notch 3 gene. Cognitive impairment, which is the second most frequent clinical manifestation, worsens with recurrent stroke. Comparison studies between the pre- and poststroke phases in CADASIL have not yet been performed in Asia. Here we describe the clinical characteristics of both the pre-and poststroke phases and identified the risk factors for stroke in CADASIL. Methods: Fifty-three consecutive patients were investigated. Patients were divided into two groups depending on the presence (poststroke group; n=31) or absence (prestroke group; n=22) of clinically overt stroke. All patients underwent an MRI scan with the same protocol. Cognition was assessed by applying detailed neuropsychological tests. Results: The poststroke group exhibited an increase in lacunae and cerebral microbleeds. The memory scores on the Alzheimer’s Dementia Assessment Scale cognitive subscale and the number of correct Stroop color-naming scores were lower in the poststroke group than in the prestroke group. Hypertension was more prevalent in the poststroke group,while chronic headache was more prevalent in the prestroke group. Conclusions: These findings suggest that cerebral microbleeds as well as lacunae predict the risk of clinically overt stroke, which leads to a worsening of frontal-lobe function in CADASIL.
Abstract
Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)is an inherited arteriopathy caused by mutation in the Notch 3 gene. Cognitive impairment, which is the second most frequent clinical manifestation, worsens with recurrent stroke. Comparison studies between the pre- and poststroke phases in CADASIL have not yet been performed in Asia. Here we describe the clinical characteristics of both the pre-and poststroke phases and identified the risk factors for stroke in CADASIL. Methods: Fifty-three consecutive patients were investigated. Patients were divided into two groups depending on the presence (poststroke group; n=31) or absence (prestroke group; n=22) of clinically overt stroke. All patients underwent an MRI scan with the same protocol. Cognition was assessed by applying detailed neuropsychological tests. Results: The poststroke group exhibited an increase in lacunae and cerebral microbleeds. The memory scores on the Alzheimer’s Dementia Assessment Scale cognitive subscale and the number of correct Stroop color-naming scores were lower in the poststroke group than in the prestroke group. Hypertension was more prevalent in the poststroke group,while chronic headache was more prevalent in the prestroke group. Conclusions: These findings suggest that cerebral microbleeds as well as lacunae predict the risk of clinically overt stroke, which leads to a worsening of frontal-lobe function in CADASIL.
참고문헌 (32)
- Joutel A, "Notch 3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia", Nature, 383, pp.707~710 (1996)
- Baudrimont M, "Autosomal dominant leukoencephalopathy and subcortical ischemic stroke. A clinicopathologic study", Stroke, 24, pp.122~125 (1993)
- O’Sullivan M, "MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL", Neurology, 56, pp.628~634 (2001)
- Viswanathan A, "Lacunar lesions are independently associated with disability and cognitive impairment in CADASIL", Neurology, 69, pp.172~179 (2007)
- Liem MK, "Lacunar infarcts are the main correlate with cognitive dysfunction in CADASIL", Stroke, 38, pp.923~928 (2007)
- Leim MK, "MR correlates of cognitive decline in CADASIL: a 7-year follow-up study", Neurology, 72, pp.143~148 (2009)
- Viswanathan A, "Blood pressure and haemoglobin A1C are associated with microhemorrhage in CADASIL: a two-centre cohort study", Brain, 129, pp.2375~2383 (2006)
- Jouvent E, "Brain atrophy is related to lacunar lesions and tissue microstructural change in CADASIL", Stroke, 38, pp.1786~1790 (2007)
- Chabriat H, "Clinical spectrum of CADASIL: a study of 7 families", Lancet, 346, pp.934~939 (1995)
- Chabriat H, "CADASIL", Lancet Neurol, 8, pp.643~653 (2009)
- Obserstein SA, "Diagnostic Notch3 sequence analysis in CADASIL: three new mutations in Dutch patients", Neurology, 52, pp.1913~1915 (1999)
- Choi JC, "Intracranial hemorrhages in CADASIL", Neurology, 67, pp.2042~2044 (2006)
- Lee JS, "Olfactory identification deficits in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy", Eur Neurol, 64, pp.280~285 (2010)
- Kang SY, "Nerve conduction studies in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy", J Neurol, 256, pp.1724~1727 (2009)
- Kang YW, "A validity study in the Korean mini-mental state examination (K-MMSE) in dementia patients", J Korean Neuro Assoc, 15, pp.300~307 (1997)
- Ferris S, "General measures of cognition", Int Psychogeriatr, 15, pp.215~217 (2003)
- Golden C, "Stroop color and word test" (2002), Stoelting
- 이한승, "한국판 노인형 기호잇기검사의 개발과 타당화 연구", Dementia and Neurocognitive Disorders(대한치매학회지), 6(2), pp.54~67 (2007), 대한치매학회
- Suh GH, "Development of the Korean version of the Alzheimer’s Disease Assessment Scale", J Korean Geriatr Soc, 7, pp.267~277 (2003)
- Lesnik Oberstein SA, "Cerebral microbleeds in CADASIL", Neurology, 57, pp.1066~1070 (2001)
- Dichgans M, "Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study", Stroke, 33, pp.67~71 (2002)
- Peters N, "A two-year clinical follow-up study in 80 CADASIL subjects: progression patterns and implications for clinical trials", Stroke, 35, pp.1603~1608 (2004)
- Dichgans M, "The phenotypic spectrum of CADASIL: clinical findings in 102 cases", Ann Neurol, 44, pp.731~739 (1998)
- Lee YC, "Population-specific spectrum of NOTCH3 mutations, MRI features and founder effect of CADASIL in Chinese", J Neurol, 256, pp.249~255 (2009)
- Kim Y, "Characteristics of CADASIL in Korea: a novel cysteine-sparing Notch3 mutation", Neurology, 66, pp.1511~1516 (2006)
- Amberla K, "Insiduous cognitive decline in CADASIL", Stroke, 35, pp.1598~1602 (2004)
- Charlton RA, "The cognitive profiles of CADASIL and sporadic small vessel disease", Neurology, 66, pp.1523~1526 (2006)
- Buffon F, "Cognitive profile in CADASIL", J Neurol Neurosurg Psychiatry, 77, pp.175~180 (2006)
- Peters N, "The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia", Am J Psychiatry, 162, pp.2078~2085 (2005)
- Dichgans M., "Cognition in CADASIL", Stroke, 41, pp.45~57 (2009)
- 이정석, "R544C Mutation을 가진 CADASIL 환자의 혈관성 위험인자", Dementia and Neurocognitive Disorders(대한치매학회지), 8(2), pp.98~103 (2009), 대한치매학회
- Kang Y, "Seoul Neuropsychological Screening Battery" (2003), Human Brain Resarch & Consulting Co