cerebral amyloid angiopathy
Findings
No curated finding names cerebral amyloid angiopathy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hereditary cerebral hemorrhage with amyloidosis (HCHWA) describes a group of rare familial central nervous system disorders characterized by amyloid deposition in the cerebral blood vessels leading to hemorrhagic and non-hemorrhagic strokes, focal neurological deficits, and progressive cognitive decline eventually leading to dementia.
Definition from the Mondo Disease Ontology (MONDO:0005620), read 2026-09-29. CC BY 4.0.
Features
13 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Cerebral amyloid angiopathyHPOHP:0011970
- Very frequent (80% to 99% of cases)
- Cerebral cortical atrophyHPOHP:0002120
- Frequent (30% to 79% of cases)
- Cerebral hemorrhageHPOHP:0001342
- Frequent (30% to 79% of cases)
- Dilation of Virchow-Robin spacesHPOHP:0012520
- Frequent (30% to 79% of cases)
- HeadacheHPOHP:0002315
- Frequent (30% to 79% of cases)
- Progressive neurologic deteriorationHPOHP:0002344
- Frequent (30% to 79% of cases)
- SeizureHPOHP:0001250
- Frequent (30% to 79% of cases)
- Subarachnoid hemorrhageHPOHP:0002138
- Frequent (30% to 79% of cases)
- Cerebral cortical microinfarctHPOHP:0025714
- Occasional (5% to 29% of cases)
- CSF pleocytosisHPOHP:0012229
- Occasional (5% to 29% of cases)
- DementiaHPOHP:0000726
- Occasional (5% to 29% of cases)
- Hyperintensity of cerebral white matter on MRIHPOHP:0030890
- Occasional (5% to 29% of cases)
Show the remaining 1
- Increased CSF protein concentrationHPOHP:0002922
- Occasional (5% to 29% of cases)
Where it sits
Other names
4 names
Resolves to: cerebral amyloid angiopathy
- Also called
- dutch hereditary cerebral amyloid angiopathyHCHWAhereditary cerebral haemorrhage with amyloidosis - Dutch typehereditary cerebral hemorrhage with amyloidosis - Dutch type