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[Juvenile arteriosclerotic leukoencephalopathy: anatomoclinical study of a case]
E Berthier1, E Broussolle, M Garcia-Jacquier
1Service de Neurologie, Hôpital de l'Antiquaille, Lyon.
Insights
This case report details arteriosclerotic leukoencephalopathy in a young adult, presenting with recurrent cerebro-vascular insults and dementia. Pathological findings revealed significant arteriosclerotic lesions, consistent with Binswanger
Area of Science:
- Neurology
- Vascular Neurology
- Pathology
Background:
- Binswanger's disease, a form of arteriosclerotic leukoencephalopathy, typically affects older adults.
- Understanding its presentation in younger individuals is crucial for early diagnosis and management.
- Recurrent cerebro-vascular insults can indicate underlying progressive vascular pathology.
Observation:
- A 34-year-old male presented with recurrent cerebro-vascular insults, lacunar infarcts, and leukoaraiosis.
- Clinical course included tardive hypertension and dementia.
- Arteriography showed no significant vascular lesions.
Findings:
- Autopsy revealed numerous ischemic lacunar infarcts and significant arteriosclerotic lesions in cerebral vasculature.
- Histological examination confirmed arteriosclerotic leukoencephalopathy with white matter edema, demyelination, and intimal arterial wall changes.
- Absence of inflammatory or amyloid findings ruled out other causes.
Implications:
- This case highlights that Binswanger's type arteriosclerotic encephalopathy can manifest in younger adults.
- Early recognition of vascular risk factors, even in young patients, is essential.
- Further research into the pathogenesis of early-onset arteriosclerotic leukoencephalopathy is warranted.
Abstract:
A 34-year old right-handed man was suffering from recurrent cerebro-vascular insults. CT-scans revealed several subcortical lacunar infarcts, and leukoaraïosis. Arteriography of the left and the right carotid arteries was performed respectively on the 4th and the 9th year of the disease, and did not elicit significant extracranial and intracranial vascular lesions. There were no arguments in favor of infectious, inflammatory, or auto-immune vascular diseases. The patient had tardive hypertension and dementia, and died at the age of 44. Pathological findings, limited to the brain and cervical spinal cord, revealed numerous ischemic lacunar infarcts. Histological lesions were consistent with the diagnosis of arteriosclerotic leukoencephalopathy. There were oedema, palor, and loss of myelin in the white matter, and nonspecific diffuse arteriosclerotic lesions that were particularly pronounced in the intimal part of the arterial wall. No inflammatory process nor amyloid deposits were found. Despite the onset of the disease in a young adult and the late occurrence of hypertension, our case report shares most of the pathological features of the Binswanger's type of arteriosclerotic encephalopathy.