Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A form of hereditary cerebral hemorrhage with amyloidosis characterized by an age of onset of 45 years of age, progressive Alzheimer's disease-like dementia, and lobar intracerebral hemorrhage in some patients. This subtype is due to a mutation in the <i>APP</i> gene (21q21.2), encoding the beta-amyloid precursor protein. This mutation causes an increased accumulation of amyloid-beta protein in the walls of the arteries and capillaries of the meninges, cerebellar cortex and cerebral cortex, leading to the weakening and eventual rupture of these vessels. Disease data Classification Clinical subtype Synonyms ABeta amyloidosis, Flemish type Amyloidoza zależna od ABetaA21G Beta amyloidoza, typ flamandzki Dziedziczny krwotok mózgowy z amyloidozą, typ flamandzki HCHWA, typ flamandzki ABetaA21G-related amyloidosis HCHWA, Flemish type Hereditary cerebral hemorrhage with amyloidosis, Flemish type ORPHA code 324718 OMIM code 605714 ICD10 code I68.0* ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl