Cerebral Amyloid Angiopathy, British TypeICD-10: E85.4+ I68.0*; ICD-11: 8B22.3

Last updated on: 12.08.2023

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DefinitionThis section has been translated automatically.

Abri/Dan amyloidosis, also called cerebral amyloid angiopathy or hereditary cerebral hemorrhage with amyloidosis, Brtian/Danish type, is an autosomal dominant form of dementia caused by mutations in the integral membrane protein 2Bgene (ITM2B gene, also known as BRI2).

ManifestationThis section has been translated automatically.

Onset around the fifth decade of life

Clinical featuresThis section has been translated automatically.

The disease is characterized by progressive dementia, spasticity, and cerebellar ataxia Cerebral amyloid angiopathy, non-neuritic and perivascular plaques, and neurofibrillary tangles are the predominant pathologic lesions.

LiteratureThis section has been translated automatically.

Michno W et al (2022) Chemical traits of cerebral amyloid angiopathy in familial British-, Danish-, and non-Alzheimer's dementias. J Neurochem 163: 233-246.
Yamada M, Naiki H. Cerebral amyloid angiopathy. Prog Mol Biol Transl Sci. 2012;107:41-78.Abeta Amyloidosis.

Last updated on: 12.08.2023