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Dementia in cerebral amyloid angiopathy: a clinicopathological study
M Yoshimura1, H Yamanouchi, S Kuzuhara
1Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Japan.
Insights
Cerebral amyloid angiopathy (CAA) is linked to dementia and cerebral hemorrhage. This study identifies multiple factors contributing to dementia in CAA, including Alzheimer's-type degeneration and white matter damage.
Area of Science:
- Neuropathology
- Vascular Neurology
- Geriatric Medicine
Background:
- Cerebral amyloid angiopathy (CAA) is a significant cause of cerebral hemorrhage and dementia.
- Understanding the pathological basis of dementia in CAA is crucial for clinical management.
Purpose of the Study:
- To conduct a clinicopathological analysis of CAA, focusing on the diverse pathological bases for dementia.
- To classify CAA-related dementia into distinct subtypes based on clinical and pathological findings.
Main Methods:
- Clinicopathological analysis of 150 patients with autopsy-proven intracranial hemorrhage and 20 patients with CAA.
- Classification of CAA-related conditions into hemorrhagic, dementia-hemorrhagic, and dementia types.
- Examination of neuropathological features including senile plaques, neurofibrillary tangles, and white matter damage.
Main Results:
- CAA accounted for 8.0% of intracranial hemorrhages and 21.1% of lobar hemorrhages.
- Among 20 CAA patients, 75% had dementia, with subtypes including hemorrhagic, dementia-hemorrhagic, and dementia types.
- Dementia in CAA is associated with Alzheimer-type degeneration, diffuse leucoencephalopathy (like Binswanger's disease), and hypertension.
Conclusions:
- Dementia in CAA is multifactorial, involving not only Alzheimer-type changes but also diffuse white matter damage.
- CAA-related dementia can be categorized into distinct subtypes, aiding in understanding its varied presentations.
- Further research into these subtypes may lead to improved diagnostic and therapeutic strategies for CAA-associated cognitive decline.
Abstract:
Dementia is in addition to cerebral haemorrhage major symptom of cerebral amyloid angiopathy (CAa). In order to explore the pathological basis for dementia in CAa-related conditions, we made a clinicopathological analysis of CAa, with special attention to dementia. Among 150 patients (mean age 78.6 years) with autopsy-proven intracranial haemorrhage in Tokyo Metropolitan Geriatric Medical Center, CAa with cerebral haemorrhage accounted for 8.0% (12 cases), associated with hypertension and metastatic brain tumour. Among 38 patients with lobar haemorrhage, CAa represented the second most common cause (21.1%) of intracranial haemorrhage after hypertension. A total of 20 patients with CAa (mean age 82.5 years) were studies clinically and pathologically. Hypertension was present in 50%. Thirteen had a history of stroke and others had either ill-defined or no strokes. The average number of strokes 2.9. Fifteen patients (75%) had dementia. Based on the clinicopathological grounds for dementia, CAa-related conditions could be divided into three subtypes: "haemorrhagic", "dementia-haemorrhagic" and "dementia" type. Haemorrhagic type (30%, 6 cases) showed multiple recurrent lobar haemorrhages caused by CAa. Hypertension was present in only 1 patient. The incidence of senile plaques and neurofibrillary tangles was generally correlated with age. Only 1 patient had dementia. The dementia-haemorrhagic type (40%, 8 patients) had recurrent strokes with cerebral haemorrhage after preceding dementia. There were two different neuropathological subsets: CAa with atypical senile dementia of Alzheimer type (SDAT) and CAa with diffuse leucoencephalopathy. Patients with CAa with atypical SDAT had multiple cerebral haemorrhages caused by CAa combined with atypical Alzheimer-type pathology. Patients with CAa with diffuse leucoencephalopathy had cerebral haemorrhages in combination with diffuse white matter damage like Binswanger's subcortical vascular encephalopathy (BSVE). The incidence of senile changes correlated with age. Patients with the dementia type (30%, 6 patients) showed progressive dementia with or without haemorrhage. All had hypertension. They had a combined condition of Alzheimer-type pathology with conspicuous CAa with BSVE. Dementia in CAa-related conditions may be responsible for multiple factors including not Alzheimer-type degeneration, but also diffuse leucoencephalopathy like Binswanger's disease. We also found an asymptomatic type, an ischaemic type, a vasculitis type and an hereditary type in this condition.