KEY FACTS Terminology Microangiopathy of brain, retina, and cochlea Imaging T2 hyperintense corpus callosum lesions in patient with clinical triad Encephalopathy, bilateral hearing loss, and branch retinal artery occlusions Multifocal T2 hyperintensities similar to MS More often round, midcallosal rather…
KEY FACTS Terminology Chronic, idiopathic relapsing-remitting multisystem vascular-inflammatory disease characterized by recurrent orogenital ulcerations and uveitis CNS involved in up to 20-25% of patients Imaging Best diagnostic clue: T2 hyperintense brainstem lesion in patient with oral and genital ulcers Midbrain…
KEY FACTS Terminology C erebral A utosomal D ominant A rteriopathy with S ubcortical I nfarcts and L eukoencephalopathy ( CADASIL ) Hereditary small-vessel disease due to mutations in NOTCH3 gene on chromosome 19, which causes stroke in young to…
KEY FACTS Terminology Cerebral amyloid deposition occurs in 3 morphologic varieties Common: Cerebral amyloid angiopathy (CAA) Uncommon: Mass-like lesion (amyloidoma) Rare: Inflammatory; diffuse (encephalopathic) white matter involvement Imaging General findings Normotensive demented patient Lobar hemorrhage(s) of different ages Multifocal “black…
KEY FACTS Terminology Systemic lupus erythematosus (SLE), neuropsychiatric (NPSLE), CNS lupus Multisystem autoimmune disorder that affects respiratory, cardiovascular, GI, GU, musculoskeletal systems, and CNS CNS involved in up to 75% Imaging 4 general patterns New infarcts (associated with ↑ anticardiolipin,…
KEY FACTS Terminology Reversible stenosis of intracranial arteries Common with aneurysmal subarachnoid hemorrhage (SAH) Caused by exposure to blood breakdown products Contraction of vascular smooth muscle Histological changes in vessel wall Imaging General features (CTA/MRA/DSA) Typically occurs 4-14 days after…
KEY FACTS Terminology Reversible cerebral vasoconstriction syndrome, a.k.a. Call-Fleming syndrome Group of disorders characterized by Reversible, multifocal cerebral artery vasoconstrictions Severe headaches ± focal neurological deficits Imaging Acute/recurrent headaches with vasculitic pattern (DSA) DSA = crucial for diagnosis (100% sensitive)…
KEY FACTS Terminology Heterogeneous group of CNS disorders Characterized by nonatheromatous inflammation and necrosis of blood vessel walls Both arteries, veins may be affected Imaging CTA/MRA useful screening; spatial resolution usually insufficient for subtle disease MR Basal ganglia, cortex, subcortical…
KEY FACTS Terminology Primary arteritis of CNS (PACNS) No evidence of secondary (systemic) vasculitis May involve intracranial arteries of any size 2 features essential for histopathologic diagnosis Inflammation and necrosis of blood vessel wall Imaging CT Look for secondary signs…
KEY FACTS Terminology Progressive narrowing of distal internal carotid artery/proximal circle of Willis (COW) vessels with secondary collateralization Collateral vessels → cloud-like “puff of smoke” (moyamoya) at angiography Moyamoya disease (MMD) = primary (idiopathic) moyamoya Marked East-West gradient (more common…