[Amyloidoses in neuropathology]

Abstract

Amyloidoses play an important role in neuropathology, both in autopsies and biopsy specimens. Cerebral amyloidoses are typically characterized by the deposition of beta-amyloid and mostly affect patients >60 years. The cardinal symptom of cerebral amyloid angiopathy (CAA) is spontaneous intracerebral hemorrhage, whereas the clinical presentation of Alzheimer's disease is dementia. Rare familial forms of amyloidoses may affect young patients and need thorough neuropathological assessment, similar to the relatively infrequent prion diseases. Amyloidoses within neuromuscular tissues mainly occur in the setting of systemic amyloid diseases. Detailed evaluation including thorough characterisation of amyloid is essential for ensuring the neuropathological diagnosis.