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

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).

Onset around the fifth decade of life

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.

1. 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.
2. Yamada M, Naiki H. Cerebral amyloid angiopathy. Prog Mol Biol Transl Sci. 2012;107:41-78.Abeta Amyloidosis.