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Vascular chorea: case report with pathology
K P Bhatia1, G Lera, P J Luthert
1University Department of Clinical Neurology, Institute of Neurology, London, England.
Insights
This study details a rare case of late-onset chorea and dementia caused by vascular pathology, specifically hypertension-related brain changes, mimicking Huntington's disease.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Systemic hypertension is a common condition with known neurological complications.
- Late-onset chorea and dementia can present diagnostic challenges, sometimes mimicking neurodegenerative disorders like Huntington's disease.
Observation:
- A 70-year-old patient with a history of hypertension developed progressive chorea and dementia.
- Clinical presentation lacked typical features of Huntington's disease or other hereditary conditions.
Findings:
- Postmortem examination revealed congophilic angiopathy and atherosclerosis affecting the neostriatum, leading to neuronal loss and gliosis.
- Cortical examination showed Alzheimer's-type plaques and neurofibrillary tangles.
Implications:
- This case highlights a rare vascular etiology for late-onset chorea and dementia.
- It underscores the importance of considering vascular contributions in atypical dementia presentations.
- The findings suggest a complex interplay between vascular disease and Alzheimer's-type pathology in the aging brain.
Abstract:
We report a patient with long-standing systemic hypertension who developed progressive generalized chorea and dementia beginning at 70 years of age with no family history or other features to suggest Huntington's disease. At postmortem examination, congophilic angiopathy and atherosclerosis causing neostriatal neuronal loss and gliosis were found, in addition to plaques and neurofibrillary tangles in the cortex. This case is a rare demonstration of a vascular pathology causing late onset generalized chorea in association with dementia due to Alzheimer's-type cortical changes.