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Intracerebral hemorrhage in two patients with Down's syndrome and cerebral amyloid angiopathy
J E Donahue1, J S Khurana, L S Adelman
1Department of Neurology, Tufts University School of Medicine, Boston, MA, USA.
Insights
Cerebral amyloid angiopathy (CAA) is a common cause of hemorrhage in the elderly. This study highlights that CAA-related hemorrhage risk may be independent of underlying conditions like Down syndrome.
Area of Science:
- Neurology
- Pathology
- Genetics
Background:
- Cerebral amyloid angiopathy (CAA) is a significant cause of spontaneous intracerebral hemorrhage (ICH) in older adults.
- CAA is frequently observed in Alzheimer's disease, Down syndrome (DS), and hereditary cerebral hemorrhage with amyloidosis (Dutch type).
Observation:
- Two cases of Down syndrome patients with extensive cerebral amyloid angiopathy (CAA) who died from intracerebral hemorrhage are presented.
- Literature review revealed only two prior case reports of this specific combination.
Findings:
- The incidence of intracerebral hemorrhage in patients with Down syndrome and extensive CAA may be underestimated.
- The risk of hemorrhage associated with CAA appears to be independent of the patient's specific underlying disease, including Down syndrome.
Implications:
- Increased awareness of CAA-related hemorrhage risk in Down syndrome patients is warranted.
- These findings suggest that CAA is a critical factor in ICH pathogenesis, irrespective of associated conditions.
- Further research is needed to elucidate the precise mechanisms and prevalence of CAA-induced hemorrhage across different patient populations.
Abstract:
Cerebral amyloid angiopathy (CAA) is an important cause of spontaneous intracerebral hemorrhages in the elderly and is often seen in the brains of patients with Alzheimer's disease, Down's syndrome (DS), and hereditary cerebral hemorrhage with amyloidosis of the Dutch type. We report two patients with DS and extensive CAA who died of intracerebral hemorrhage; only two other such case reports exist in the literature. We believe the incidence of such cases is higher than is reported and that the likelihood of hemorrhage in the setting of CAA is independent of the patient's underlying disease.