Aß amyloidosis ICD-10: E85.4+ I68.0*; ICD-11: 8B22.3

Abeta amyloidosis, also called cerebral amyloid angiopathy or hereditary cerebral hemorrhage with amyloidosis, Dutch type, is a cerebrovascular disease characterized by the accumulation of amyloid beta peptide in the leptomeninges and small/medium cerebral blood vessels. The amyloid deposits lead to fragile vessels that may manifest as lobar intracerebral hemorrhages. The disease may also present with cognitive impairment, incidental microbleeds, hemosiderosis, inflammatory leukoencephalopathy, Alzheimer's disease, or transient neurologic symptoms.

The E22Q mutation of the amyloid beta protein (Abeta) in the rare disease hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) causes severe cerebral amyloid angiopathy with hemorrhagic stroke in middle age and dementia. Abeta E22Q aggregates more rapidly into more stable amyloid-like fibrils than wild-type Abeta. The severity of Abeta amyloidosis tends to increase with age but can vary widely among patients of comparable age.

1. Gandy S (2004) Cerebral Abeta amyloidosis and postmenopausal hormone deficiency: roles in the genesis of Alzheimer's disease. Hum Pathol 35:271-274.
2. Maat-Schieman M et al (2005) Hereditary cerebral hemorrhage with amyloidosis-Dutch type. Neuropathology. 25:288-297.
3. Pignataro A et al. (2017) Trans-Synaptic Spread of Amyloid-β in Alzheimer's Disease: Paths to β-Amyloidosis. Neural Plast:5281829.