Cerebral amyloid angiopathy – an underdiagnosed entity in younger adults with lobar intracerebral hemorrhage?

Abstract

Cerebral amyloid angiopathy (CAA) is a progressive microvascular amyloidosis affecting the small- and medium-sized arterioles and the capillaries of brain parenchyma and leptomeninges, and is recognized as a cause of lobar intracerebral hemorrhage (ICH). We report two patients who experienced recurrent ICH due to CAA at an age of 37 (A) and 42 (B) years, respectively. The classic and modified Boston criteria for the diagnosis of CAA include an age limit of 55 years if no biopsy or postmortem examination is performed; CAA is typically not considered in the differential diagnosis of lobar ICH in younger patients. We assume that sporadic CAA is an underdiagnosed entity in younger adults with lobar ICH.

Abbreviations: Aβ, amyloid β protein; AβPP: amyloid precursor protein; CAA: cerebral amyloid angiopathy; CSF: cerebrospinal fluid; ICH: intracerebral hemorrhage; MRI: magnetic resonance image; SNP: single-nucleotide-polymorphism; SWI: susceptibility weighted MR imaging; TBI: traumatic brain injury; vwD: von Willebrand disease; vWF: von Willebrand factor.

• Boston criteria
• cerebral amyloid angiopathy
• sporadic intracerebral hemorrhage
• von Willebrand disease

Acknowledgements

Genetic diagnostics were performed in the Institute of Human Genetics, University Hospital Giessen and Marburg, Giessen, Germany (Director: Professor Dr. med. U. Müller).

Declaration of Interest: The authors report no conflicts of interest.