CEREBRAL HEMORRHAGE
Cerebral amyloid angiopathy (CAA)
Created 13/04/2021, last revision 23/01/2023
- a heterogeneous group of sporadic or familial diseases characterized by amyloid deposits in the walls of small and medium-sized cerebral vessels
- mainly affects older people, rare familial forms (Icelandic and Dutch type) occur in younger age
- CAA is responsible for ~2-10% of primary intracranial hemorrhages (30-70% of lobar hematomas in elderly patients) [Chao, 2006]
Pathology
Pathology- amyloid deposits in small and medium-sized cerebral arteries without systemic amyloidosis
- there is some association with typical Alzheimer’s changes, such as neuritic plaques and neurofibrillary tangles
Clinical presentation
- Transient Focal Neurological Episodes (TFNE), also called “amyloid spells“
- recurrent lobar intracerebral hemorrhages (ICH)
- convexial SAH
- hemocephalus
- arteriolopathy with leukoaraiosis and encephalopathy
- development of vascular dementia at an advanced stage of the disease
- transient positive and negative symptoms (also “amyloid spells”)
- positive symptoms – “aura-like” spreading paresis, visual phenomena (monocular blurred vision, flashes, teichopsia), twitching of limbs
- negative symptoms – transient focal symptoms – paresis, speech and visual disturbances
- positive symptoms predominate
- usually, there are multiple stereotyped episodes, typically lasting 10-30 minutes
- occurrs in about 14% of CAA patients [Charidimou, 2012]
- very often caused by convexial SAH
- FLAIR, DWI/ADC, and GRE/SWI are optimal for diagnostic workup
- frequently, convexial SAH is seen along with microbleeds or parenchymal hematoma, but also recent lesions on DWI – etiology is heterogeneous
- FLAIR, DWI/ADC, and GRE/SWI are optimal for diagnostic workup
- may be confused with TIA (antithrombotic drugs further increase the risk of ICH) or stroke (high risk of ICH during thrombolysis)
- TFNE is often followed by subsequent ICH (37.5% / 2 months)!! [Charidimou, 2012]
Diagnostic evaluation
| Content available only for logged-in subscribers (registration will be available soon) |
Therapy
- no causal treatment
- treat acute hematoma according to standard protocols → management of intracerebral hemorrhage
- antiplatelet and anticoagulant therapy increases the risk of bleeding ⇒ assess individual risk-benefit ratio in each patient (Biffi, 2010]
Anticoagulation in patients with suspected CAA
| Content available only for logged-in subscribers (registration will be available soon) |
Thrombolysis in patients with suspected CAA
- in a patient with CMBs < 10, IVT is possible (AHA/ASA 2019 IIa/B-NR)
- with CMBs >10, IVT is associated with a higher risk of ICH; the expected benefit of treatment must outweigh the risk (AHA/ASA 2019 IIb/B-NR)
- consider IVT in a severe deficit in a patient without a severe premorbid condition
