Aged, 80 and over ; Cardiomegaly ; etiology ; pathology ; Cerebrovascular Disorders ; etiology ; Humans ; Hypertension ; complications ; pathology ; Proteinuria ; etiology ; pathology

PURPOSE: After a century, cheiro-oral syndrome (COS) was harangued and emphasized for its localizing value and benign course in recent two decades. However, an expanding body of case series challenged when COS may arise from an involvement of ascending sensory pathways between cortex and pons and terminate into poor outcome occasionally. MATERIALS AND METHODS: To analyze the location, underlying etiologies and prognosis in 76 patients presented with COS collected between 1989 and 2007. RESULTS: Four types of COS were categorized, namely unilateral (71.1%), typically bilateral (14.5%), atypically bilateral (7.9%) and crossed COS (6.5%). The most common site of COS occurrence was at pons (27.6%), following by thalamus (21.1%) and cortex (15.8%). Stroke with small infarctions or hemorrhage was the leading cause. Paroxysmal paresthesia was predicted for cortical involvement and bilateral paresthesia for pontine involvement, whereas crossed paresthesia for medullary involvement. However, the majority of lesions cannot be localized by clinical symptoms alone, and were demonstrated only by neuroimaging. Deterioration was ensued in 12% of patients, whose lesions were large cortical infarction, medullary infarction, and bilateral subdural hemorrhage. CONCLUSION: COS arises from varied sites between medulla and cortex, and is usually caused by small stroke lesion. Neurological deterioration occurs in 12% of patients and relates to large vessel occlusion, medullary involvement or cortical stroke. Since the location and deterioration of COS cannot be predicted by clinical symptoms alone, COS should be considered an emergent condition for aggressive investigation until fatal cause is substantially excluded.
Adult ; Aged ; Cerebrovascular Disorders/classification/complications/etiology/*pathology ; Female ; Humans ; Male ; Middle Aged ; Nervous System Diseases/pathology ; Prospective Studies ; Syndrome

Brain ; pathology ; Cerebrovascular Disorders ; diagnosis ; Child ; Diagnosis, Differential ; Female ; Humans ; Magnetic Resonance Imaging ; Neurofibromatosis 1 ; diagnosis ; therapy ; Treatment Outcome

Vascular dementia (VaD) is a history-laden disease entity that dates back to the 19th century when arteriosclerotic brain atrophy due to hardening of the arteries was perceived as the major cause of senile dementia. Its existence had been overshadowed by the emergence of Alzheimer's disease (AD) in the past century and research on AD dominated the field of dementia. Interest in VaD has been revived in recent years as vascular lesions have been shown to make great contributions to the development of dementia, particularly in the elderly. VaD has now evolved into the concept of vascular cognitive impairment (VCI), which encompasses not only VaD but also AD with cerebrovascular disorder and VCI with no dementia. The concept of VCI is intended to maximize the therapeutic potential in dementia management because the vascular component may be amenable to therapeutic intervention particularly in the early stages of cognitive impairment. Subcortical ischemic vascular dementia (SIVD) is pathologically driven by severe stenosis and the occlusion of small vessels that culminate into white matter ischemia and multiple lacunar infarctions in the subcortical structures. The relatively slow progression of symptoms and clinical manifestations associated with cholinergic deficits often make the differentiation of SIVD from AD difficult. The recent development of in vivo amyloid imaging enabled further pathological breakdown of SIVD into pure SIVD and mixed dementia with subcortical ischemia based on the absence or existence of amyloid pathology in the brain. In this article, the authors reviewed the emerging concepts of VaD/VCI and the clinical manifestations, biomarkers, treatments, and preclinical models of SIVD based on the pathophysiologic mechanisms of the disease.
Aged ; Alzheimer Disease ; Amyloid ; Arteries ; Atrophy ; Biomarkers ; Brain ; Cerebrovascular Disorders ; Constriction, Pathologic ; Dementia ; Dementia, Vascular* ; Humans ; Ischemia ; Pathology ; Stroke, Lacunar

BACKGROUND AND PURPOSE: MicroRNA (miRNA) expression has been examined in multiple conditions, including various cancers, neurological diseases, and cerebrovascular diseases, particularly stroke. Existing evidence indicates that miRNA biosynthesis and function play crucial roles in ischemic stroke physiology and pathology. In this study, we selected six known polymorphisms in miRNA-biogenesis genes; DICER rs13078A>T, rs3742330A>G; DROSHA rs10719T>C, rs6877842G>C; Ran GTPase (RAN) rs14035C>T; exportin 5 (XPO5) rs11077A>C. METHODS: We analyzed the associations between these polymorphisms and disease status and clinical factors in 585 ischemic stroke patients and 403 controls. Genotyping was performed with the polymerase chain reaction-restriction fragment length polymorphism method. RESULTS: The DICER rs3742330A>G (AA vs. AG+GG: adjusted odds ratio [AOR], 1.360; 95% confidence interval [CI], 1.024 to 1.807; P=0.034) and DROSHA rs10719T>C polymorphisms (TT vs. CC: AOR, 2.038; 95% CI, 1.113 to 3.730; P=0.021) were associated with ischemic stroke prevalence. During a mean follow-up of 4.80±2.11 years, 99 (5.91%) of the stroke patients died. In multivariate Cox proportional hazard regression models, a significant association was found between RAN rs14035 and survival of large artery disease patients with ischemic stroke (CC vs. TT: adjusted hazard ratio, 5.978; P=0.015). CONCLUSIONS: An association was identified between the DICER and DROSHA polymorphisms and ischemic stroke. Specifically, polymorphisms (rs3742330 and rs10719) were more common in stroke patients, suggesting that they may be associated with an increased risk of ischemic stroke.
Arteries ; Cerebrovascular Disorders ; Follow-Up Studies ; GTP Phosphohydrolases ; Humans ; Methods ; MicroRNAs ; Mortality ; Odds Ratio ; Pathology ; Physiology ; Polymorphism, Genetic ; Prevalence ; Stroke

Vascular dementia is a very frequent form of dementia. Debates over classification and diagnostic criteria, and controversy over identifiable treatment targets will continue until distinct pathophysiological mechanism of vascular dementia is found. Clinical diagnostic criteria are sufficiently strong to be useful for clinical trials, but need further refinement. Cognitive changes in vascular dementia are more variable than other disorders, and are dependent on the vascular pathology. Accurate diagnosis of vascular dementia is known to need the presence of reliable cerebrovascular disease on brain imaging. Although it seems obvious that cerebrovascular disease causes pathological damage and impaired cognition, it is very difficult to find the accurate contribution of cerebrovascular pathology to cognitive decline. Most studies have shown a small but significant benefit of cholinesterase inhibitors on cognition, the significance of this effect has been slight and benefits on global functioning, activities of daily living, and behaviour have not been consistently reported. Management of vascular dementia should focus on identifying and managing vascular risk factors.
Activities of Daily Living ; Cerebrovascular Disorders ; Cholinesterase Inhibitors ; Classification ; Cognition ; Dementia ; Dementia, Vascular* ; Diagnosis ; Neuroimaging ; Pathology ; Risk Factors

As a "Gold Standard" in clinical diagnosis of cerebrovascular disease, cerebral angiography also plays an important role in forensic postmortem examination. The key roles of identifying the site of cerebrovascular disease and the cause of death as well as providing guidance for autopsy and postmortem sampling and the broad future application of cerebral angiography in forensic practice are reviewed.
Autopsy ; Cause of Death ; Cerebral Angiography ; Cerebrovascular Disorders/diagnostic imaging* ; Forensic Pathology/methods* ; Humans ; Intracranial Hemorrhages/etiology* ; Postmortem Changes ; Wounds and Injuries/pathology*

BACKGROUNDRecent autopsy study showed a high incidence of cerebrovascular lesions in Alzheimer's disease (AD). To assess the impact of cerebrovascular pathology in AD, we used diffusion tensor imaging (DTI) to study AD patients with and without cerebrovascular lesions.

MATERIALS AND METHODSConventional and DTI scans were obtained from 10 patients with probable AD, 10 AD/V patients (probable AD with cerebrovascular lesions) and ten normal controls. Mean diffusivity (D) and fractional anisotropy (FA) values of some structures involved with AD pathology were measured.

RESULTSD value was higher in AD patients than in controls in hippocampus and the cingulate gyrus. In AD/V patients, increased D value was found in the same structures and also in the thalamus and basal ganglia compared to controls. There was a significant difference of D value between AD and AD/V patients. FA value reduced in the white matter of left inferior temporal gyrus and in the bilateral middle cingulate gyrus in patients with AD/V compared with controls. The MMSE (mini-mental state examination) score significantly correlated with FA value in the right hippocampus (r=0.639, P<0.019), in the right anterior cingulate gyrus (r=0.587, P<0.035) and in left parahippocampal gyrus (r=0.559, P<0.047).

CONCLUSIONCerebrovascular pathology had stronger impact on the D value than the AD pathology alone did. Elevated D value in thalamic and basal ganglia may contribute to cognitive decline in AD/V patients. Reduced FA values in AD/V patients may indicate that cerebrovascular pathology induced more severe white matter damage than the AD pathology alone did.

Aged ; Alzheimer Disease ; complications ; pathology ; Brain ; blood supply ; pathology ; Cerebral Cortex ; pathology ; Cerebrovascular Disorders ; complications ; pathology ; Cognition ; Corpus Callosum ; pathology ; Diffusion Magnetic Resonance Imaging ; Female ; Hippocampus ; pathology ; Humans ; Male ; Temporal Lobe ; pathology

Type 2 diabetes (T2D) increases the risk for cerebrovascular disease (CVD) and dementia. The underlying molecular mechanisms remain elusive, which hampers the development of treatment or/and effective prevention strategies. Recent studies suggest that dyshomeostasis of amylin, a satiety hormone that forms pancreatic amyloid in patients with T2D, promotes accumulation of amylin in cerebral small blood vessels and interaction with Alzheimer's disease (AD) pathology. Overexpression of human amylin in rodents (rodent amylin does not form amyloid) leads to late-life onset T2D and neurologic deficits. In this Review, we discuss clinical evidence of amylin pathology in CVD and AD and identify critical characteristics of animal models that could help to better understand molecular mechanisms underlying the increased risk of CVD and AD in patients with prediabetes or T2D.
Alzheimer Disease ; Amyloid ; Blood Vessels ; Cerebrovascular Disorders ; Dementia ; Diabetes Complications ; Diabetes Mellitus, Type 2 ; Humans ; Islet Amyloid Polypeptide ; Models, Animal ; Neurologic Manifestations ; Pathology ; Prediabetic State ; Rodentia

BACKGROUND: Asymptomatic coronary artery stenosis (ACAS) ≥50% is common in patients with acute ischemic cerebrovascular disease (AICVD), which portends a poor cardiovascular and cerebrovascular prognosis. Identifying ACAS ≥50% early may optimize the clinical management and improve the outcomes of these high-risk AICVD patients. This study aimed to investigate whether aortic arch plaque (AAP), an early atherosclerotic manifestation of brain blood-supplying arteries, could be a predictor for ACAS ≥50% in AICVD. METHODS: In this cross-sectional study, atherosclerosis of the coronary and brain blood-supplying arteries was simultaneously evaluated using one-step computed tomography angiography (CTA) in AICVD patients without coronary artery disease history. The patients were divided into ACAS ≥50% and non-ACAS ≥50% groups according to whether CTA showed stenosis ≥50% in at least one coronary arterial segment. The AAP characteristics of CTA were depicted from aspects of thickness, extent, and complexity. RESULTS: Among 118 analyzed patients with AICVD, 29/118 (24.6%) patients had ACAS ≥50%, while AAPs were observed in 86/118 (72.9%) patients. Increased AAP thickness per millimeter (adjusted odds ratio [OR]: 1.56, 95% confidence interval [CI]: 1.18-2.05), severe-extent AAP (adjusted OR: 13.66, 95% CI: 2.33-80.15), and presence of complex AAP (adjusted OR: 7.27, 95% CI: 2.30-23.03) were associated with ACAS ≥50% among patients with AICVD, independently of clinical demographics and cervicocephalic atherosclerotic stenosis. The combination of AAP thickness, extent, and complexity predicted ACAS ≥50% with an area under the receiver-operating characteristic curve of 0.78 (95% CI: 0.70-0.85, P < 0.001). All three AAP characteristics provided additional predictive power beyond cervical and intracranial atherosclerotic stenosis for ACAS ≥50% in AICVD (all P < 0.05). CONCLUSIONS Thicker, severe-extent, and complex AAP were significant markers of the concomitant ACAS ≥50% in AICVD, possibly superior to the indicative value of cervical and intracranial atherosclerotic stenosis. As an integral part of atherosclerosis of brain blood-supplying arteries, AAP should not be overlooked in predicting ACAS ≥50% for patients with AICVD.
Aged ; Aorta, Thoracic ; pathology ; Cerebrovascular Disorders ; diagnosis ; Coronary Stenosis ; diagnosis ; Cross-Sectional Studies ; Female ; Humans ; Male ; Middle Aged ; Odds Ratio ; Plaque, Atherosclerotic ; diagnosis ; Risk Factors