Effects of Lacunar Infarctions on Cognitive Impairment in Patients with Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Lee, Jung Seok; Choi, Jay Chol; Kang, Sa Yoon; Kang, Ji Hoon; Na, Hae Ri; Park, Ji Kang

doi:10.3988/jcn.2011.7.4.210

Cited by 27 publications

(24 citation statements)

References 19 publications

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“…Cognitive impairment in CADASIL is similar to that reported for sporadic subcortical ischemic vascular dementia and is thought to the result of dysfunction within the subcortical/frontal network due to the leukoaraiosis, although a multi‐infarction origin based on the accumulation of cerebral infarcts was also proposed . Cognitive decline preceding strokes is unusual in CADASIL, but several cases were reported .…”

Section: Discussionsupporting

confidence: 63%

“…Our report suggest to consider CADASIL in those cases with recurrent TGA, especially when MRI shows multifocal hyperintensities affecting the cerebral white matter or when it is followed by cognitive decline. Cognitive impairment in CADASIL is similar to that reported for sporadic subcortical ischemic vascular dementia and is thought to the result of dysfunction within the subcortical/frontal network due to the leukoaraiosis, although a multi-infarction origin based on the accumulation of cerebral infarcts was also proposed [5,13,14]. Cognitive decline preceding strokes is unusual in CADASIL, but several cases were reported [1,3,4].…”

Section: Discussionsupporting

confidence: 61%

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Recurrent transient global amnesia as presenting symptoms of CADASIL

Pradotto

Orsi

Mencarelli

et al. 2016

Clinical Case Reports

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Key Clinical MessageDespite transient global amnesia is considered unusual in Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and causal relation is still unclear, this report suggests to consider CADASIL in those patients with recurrent transient global amnesia, especially when MRI shows multifocal hyperintensities affecting the cerebral white matter or when it is followed by cognitive decline.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionsupporting

confidence: 61%

Recurrent transient global amnesia as presenting symptoms of CADASIL

Pradotto

Orsi

Mencarelli

et al. 2016

Clinical Case Reports

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“…Previous studies that examined the relationship between CMB and cognitive impairment showed mixed results; with some reporting significant associations [36, 37] and others were not significant [38, 39]. Our sample had a lower prevalence of CMB compared to studies in developed countries (18–38%) [40], which might reflect regional or lifestyle differences that need to be further examined.…”

Section: Discussionmentioning

confidence: 50%

Cerebral Small Vessel Disease and Motoric Cognitive Risk Syndrome: Results from the Kerala-Einstein Study

Wang

Allali

Kesavadas

et al. 2016

JAD

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Background The contribution of cerebral small vessel disease to cognitive decline, especially in non-Caucasian populations, is not well established. Objective We examined the relationship between cerebral small vessel disease and motoric cognitive risk syndrome (MCR), a recently described pre-dementia syndrome, in Indian seniors. Methods 139 participants (mean age 66.6 ± 5.4 y, 33.1% female) participating in the Kerala-Einstein study in Southern India were examined in a cross-sectional study. The presence of cerebral small vessel disease (lacunar infarcts and cerebral microbleeds (CMB)) and white matter hyperintensities on MRI was ascertained by raters blinded to clinical information. MCR was defined by the presence of cognitive complaints and slow gait in older adults without dementia or mobility disability. Results Thirty-eight (27.3%) participants met MCR criteria. The overall prevalence of lacunar infarcts and CMB was 49.6% and 9.4%, respectively. Lacunar infarcts in the frontal lobe, but no other brain regions, were associated with MCR even after adjusting for vascular risk factors and presence of white matter hyperintensities (adjusted Odds Ratio (aOR): 4.67, 95% CI: 1.69–12.94). Frontal lacunar infarcts were associated with slow gait (aOR: 3.98, 95% CI: 1.46–10.79) and poor performance on memory test (β: −1.24, 95% CI: −2.42 to −0.05), but not with cognitive complaints or non-memory tests. No association of CMB was found with MCR, individual MCR criterion or cognitive tests. Conclusions Frontal lacunar infarcts are associated with MCR in Indian seniors, perhaps, by contributing to slow gait and poor memory function.

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“…[ 2 ] The main clinical manifestations are recurrent stroke, cognitive decline, chronic headache, mood disturbances, and seizure. [ 3 , 4 ] Magnetic resonance imaging (MRI) is crucial in the diagnosis of CADASIL. Typical MRI findings include multiple subcortical lacunes, extensive white matter change, and multiple cerebral microbleeds (CMBs).…”

Section: Introductionmentioning

confidence: 99%

Clinical Significance of Cerebral Microbleeds Locations in CADASIL with R544C NOTCH3 Mutation

et al. 2015

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Background and PurposeAlthough cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common single-gene disorder of cerebral small blood vessels caused by NOTCH3 mutations, little has been described about the variation in the clinical findings between its underlying types of mutations. In particular, the presence of cerebral microbleeds (CMBs) has been an increasingly recognized magnetic resonance imaging finding in CADASIL, but their clinical significance is not clear. The purpose of this study is to assess whether CMBs are associated with symptomatic stroke in the CADASIL patients with R544C mutation and to compare the cerebral distribution of CMBs between CADASIL patients with and without symptomatic stroke.MethodsThis is a cohort study of patients who were diagnosed with genotype-confirmed R544C-mutation CADASIL. Primary neurologic symptoms were recorded. Symptomatic strokes were defined as transient ischemic attack, ischemic strokes and hemorrhagic strokes. CMBs were defined as focal areas of round signal loss on T2*-weighted gradient echo planar images with a diameter of less than 10 mm. The locations of CMBs were divided into lobar, basal ganglia, thalamus, brain stem and cerebellum. Multiple logistic regressions were performed to identify the epidemiologic or vascular risk factors associated with symptomatic stroke in patients with CADASIL.ResultsAmong total of 51 subjects in this cohort, CMBs were present in 20 of 32 patients (64.5%) in the symptomatic stroke-group and in 8 of 19 patients (42.1%) in the non-stroke group (p = 0.16). CMBs were observed more frequently in the basal ganglia (p<0.001) and the cerebellum (p<0.018) in the symptomatic stoke group compared to the non-stroke group. The mean number of CMBs was significantly higher in the symptomatic stroke group (15.4±18.0 lesions per patients with CMBs) versus those without symptomatic stroke (3.3±3.0 lesions per patients with CMBs) (p = 0.003). Hypertension was an independent risk factor for symptomatic stroke in CADASIL (p = 0.014). It was independently associated with CMBs locations as basal ganglia (p = 0.016), thalamus (p = 0.010), brainstem (p = 0.044), and cerebellum (p = 0.049). However, It was not independently associated with CMBs on lobar lesion (p = 0.152).ConclusionsIn this study hypertension was an independent predictor of CMBs presence in specific brain locations, as well as symptomatic stroke in the CADASIL patients. The distribution and burden of CMBs might be a clinically useful marker for the risk of symptomatic stroke. However, further prospective studies on the relationship between CMBs distribution and symptomatic stroke are required in order to support these preliminary findings.

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Effects of Lacunar Infarctions on Cognitive Impairment in Patients with Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Cited by 27 publications

References 19 publications

Recurrent transient global amnesia as presenting symptoms of CADASIL

Recurrent transient global amnesia as presenting symptoms of CADASIL

Cerebral Small Vessel Disease and Motoric Cognitive Risk Syndrome: Results from the Kerala-Einstein Study

Clinical Significance of Cerebral Microbleeds Locations in CADASIL with R544C NOTCH3 Mutation

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