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Dementia in hereditary cerebral hemorrhage with amyloidosis-Dutch type
J Haan1, J B Lanser, I Zijderveld
1Department of neurology, University Hospital, Leiden, The Netherlands.
Insights
Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) frequently causes dementia. Neuropsychological testing revealed progressive intellectual decline and memory issues in affected individuals, suggesting a link between HCHWA-D and cognitive impairment.
Area of Science:
- Neurology
- Neuroscience
- Genetics
Background:
- Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) is a rare genetic disorder.
- It is characterized by the deposition of amyloid in cerebral blood vessels, leading to recurrent hemorrhages.
- The long-term cognitive consequences of HCHWA-D are not fully understood.
Purpose of the Study:
- To investigate the neuropsychological profile of patients with HCHWA-D.
- To assess the prevalence and progression of cognitive impairment in HCHWA-D.
- To explore the potential mechanisms underlying dementia in HCHWA-D.
Main Methods:
- Neuropsychological examination of 16 patients diagnosed with HCHWA-D.
- Repeated testing over several years for a subset of patients.
- Clinical assessment for intercurrent cerebrovascular events.
Main Results:
- All 16 patients exhibited neuropsychological abnormalities.
- Dementia was diagnosed in 12 out of 16 patients.
- Three patients showed progressive intellectual deterioration and memory disturbances over time, even without evident strokes.
Conclusions:
- Cerebral amyloid angiopathy, a hallmark of HCHWA-D, can cause dementia through multiple focal cerebral lesions.
- Chronic ischemic or metabolic effects on brain parenchyma may also contribute to cognitive decline in HCHWA-D.
- HCHWA-D is a significant cause of dementia, necessitating further research into its pathogenesis and management.
Abstract:
Sixteen patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type were examined neuropsychologically. Abnormalities were found in all patients, and dementia was present in 12 of them. Three patients were tested twice at an interval of some years. All three exhibited a progressive intellectual deterioration and memory disturbance; in two of them there was no evidence of intercurrent strokes. Cerebral amyloid angiopathy can lead to dementia because it produces multiple focal cerebral lesions, but a chronic ischemic or metabolic effect on brain parenchyma may play a contributing role.