Prayson Vascular Flashcards
Carbon Monoxide
Bright pink edematous brain, necrosis of globus pallidus and substantia nigra, may show Grenker’s myelinopathy: demyelination and cerebral white matter destruction. Form of anemic/hypoxemic anoxia
Air embolism
spinal cord microinfarcts, “the bends” and cardiac bypass
Hypoglycemic damage
selective neuronal necrosis in superficial layers of the cortex, hippocampus (CA1 and dentate), and caudate. NO purkinje cell necrosis
Most common sites of venous thrombosis
Superior saggital sinus, lateral sinuses, and straight sinus
Most common site of Septic thrombosis
Cavernous sinus, ususally 2/2 contiguous spread from sinus/soft tissue infection
Berry aneurysm, how many bilateral / multiple?
Defective media and gap in internal elastic lamina85% occur in the circle of willis, 25% multiple, 20% bilateral. Most common sites: MCA trifurcation, anterior communicating and ACA jxn, Internal carotid-posterior communicating jxn
Berry aneurysm rupture risk
50% in aneurysms > 1cm
Berry aneurysm associations
Polycystic kidney, Ehlers-Danlos, NF1, coarctation of the aorta, fibromuscular dysplasia, Marfan’s, pseudoxanthoma elasticum
Wyburn-Mason synd
multiple AVMs, predominantly affecting brain (particularly midbrain), optic nerve/ retina, and face
AVM
Most supratentorial, superficial wedge-shaped w/ apex toward the ventricle, usually in MCA distribution, most common vascular association w/ hemorrhage. 4% of people have multiple. Most common vascular malformation and most common to casue hemorrhage. 2nd most common cause of SAH (after berry aneurysm)
Foix-Alajouanine synd (angiodysgenetic myelomalacia)
spinal cord A-V fistula/vascular malformation with thrombosis causing infarction and progressive myelopathy (areflexic parapersis, sensory disturbance, bladd, bowel, and sexual dysfxn) in middle aged men. Vessels with no internal elastic lamina which may be mineralized.
Cavernous malformation
Most supratentorial, CCM gene mutations. Seen on MRI, not angiography. Presents w/ siezures. Assoc w/ hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)
Polyarteritis nodosa (PAN)
Necrotizing vasculitis with fibrinoid necrosis of small to medium sized vessels. M>F, 40-50 yoa, systemic (except lungs and spleen). CNS invloved in 60-80%
Wegner’s granulomatosis
Necrotizing granulomatous inflammation, cANCA directed agains proteinase 3, 40-60 yoa, systemic w/ respiratory component. Respiratory tract +/- glomerulonephritis. CNS effect in 20-40% of cases.
Giant cell arteritis
Granulomatous lymphoplasmacytic, Assoc. w/ polymyalgia rhuematica, F>M, >50yoa
Takayasu’s arteritis
Transmural granulomatous and lymphoplasmacytic inflammation of the media w/ fibrosis. Aorotic arch and descending aorta, younger (15-40 yoa) asian females
Churg-Stauss
necrotizing vasculitis with esoinophilia w/ fibrinoid necrosis in medium arteries, capillaries, and venules. Lung involvement
Primary angiitis of the CNS (PACNS)
M>F, 30-50 yoa, Leptomeninges is highest yeild area (bx temporal tip of non-dominant lobe leptomenignes, cortex and white matter), affetcts small and medium sized arteries, may be granulomatous.
what vasculitides typically involve the brain parencyma
Lupus and lymphomatoid granulomatosis
What vasculitides involve the vessels of the subrachnoid space, PNS, and extracranial vessels
PAN, Wegner’s granulomatosis, and giant cell arteritis
Binswanger’s (aka subcortical arterioclerotic encephalopathy)
5-60 yoa, evolves over 3-5 years. Intermittent HTN and stepwise/ progressive profound dementia (memory, mood and cognition). Widespread, vascular alterations and white matter changes, ventricular dilation, focal and diffuse myelin loss and astrocytosis in deep white matter most severe in temporal and occipital lobes. sparing of subarcuate U-fibers
CADASIL (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencepholapathy)
NOTCH3 gene (EGFR), Slowly progressive (15-25 yr) strokes and dementia. Thickined vessels (media and adventicia) w/ basophilic PAS+ granules within smooth muscle. GRANULAR OSMIOPHILIC MATERIAL. Cortical and subcortical atrophy, astrocytosis, and lacunar infarcts/foci of encephalomalacia. Onset 45 yoa, systemic (can dectect osmophilic material by skin bx)
Charcot-Bouchard microaneurysms
aka miliary aneurysms, chronic HTN, dilations/ outpouchings 2/2 fibrinoid change and fragmentation of IEL. Usually basal ganglia and pons. Also seen in CAA and CADASIL
Moyamoya Synd
Occlusion of the internal carotid, PCA, or Pcom arteries with the development of abnormal collateralization (dilation of lenticulostriate arteries), thrombus and intimal fibroplasia w/out atherosclerosis or inflammation. Hemiparesis and hemorrhage. Most pt.s <20 yoa, F>M
Regions most vulnerable to ischemia in adults
Hippocampus (CA1), cerebral cortex (layers 3 and 5), purkinje cells, thalamus, basal ganglia, brainstem, hypothalamus, and spinal cord
Regions most vulnerabtle to ischemia in infants
Thalamus, hippocampus, colliculi
Cystic necrosis of globus pallidus
Carbon monoxide, cyanide, heroin overdose, global hypoxia/ischemia
Red neurons occur when
First seen after survival for 4-6 hours and last for 2 weeks
MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke)
Infarct like lesions that don’t conform to vascular teritories. Muscle bx: Ragged red fibers with mitochondrial cytopathy. Due to several mutations in mitochondrial DNA leading to defective oxidative metabolism.
Hereditary Hemorrhagic Telangiectasia (Osler-Webber-Rendu)
Vascular dysplasia and recurrent hemorrhages, AVMs, endoglin and ACVRL1 genes
Doliechoectasia
Vertebral and basilar arteries, Increased diameter w/ fibrosis and degeneration of the internal elastic lamina. Can progress to fusiform aneuyrism, may casue compression of adjacent structures (hydrocephalus, CN palsy). Assoc w/ Fabry’s dx
Large vessel vasculitidies
Giant cell arteritis and Takayasus
Hemorrhagic conditons
Arteriosclerosis/arteriolosclerosis, lipohyalinosis, cerebral amyloid angiopathy
Ischemic conditions
Vasculitis/angitis
Infectious agents that cause CNS angiitis
HIV, CMV, VZV, Hep C, syphillis, Borrelia burgdorferi, TB, fungi
CARASIL (Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy)
(aka MAEDA sydn) Mutations in HTRA1, very rare, Japan. 29-50 yoa, presenile dementia, alopecia, spondylosis, white matter infarcts, athero/arteriosclerotic changes
Retinal vasculopathy and Cerebral Leukodystrophy (HERNS, cerebroretinal vascuolpathy, and hereditary vascular retinopathy)
Obliterative vasculopathy, mutations in TREX1. HERNS (hereditary endotheliopathy with retionpathy, nephropathy, and stroke): Lareas of ischemic necrosis and small unusual angiomatoid formations surrounded by calcifications. Visual impairment and dementia
TREX1 mutations
Aicardi-Goutieres synd, HERNS/retinal vasculopathy with cerebral leukodystrophy, SLE, familial chilblain lupus
COL4A1 mutations
vascuolpathy (affects small cerebral arteries and causes berry aneurysms), familal porenecephaly, hereditary angiopathy with nephropathy, aneurysms, and muscle cramps.
MCA infarct
hemiparesis or complete hemiplegia, (face and arm, often leg), aphasia (if dominant hemisphere), hemisensory disturbance
ACA infarct
Hemiparesis (leg consistently possibly face and arms), transcortical motor aphasia (halting speech), abulia (absence of willpower)
PCA infarct
thalamic pain synd., hemianopia (cortical blindness if bilateral), and alexia (word blindness/can’t read, if dominant hemisphere)
Date the infarct: red neurons, vacuolization of neuropil
1-4 days
Date the infarct: endothelial proliferation and neovascularization, neutrophils around necrotic vessel, microglial activiation, and macs
5-12 days
Date the infarct: neuronla ghosts, foamy macs, gliosis, neutrophils around necrotic vessel, microglial activiation
8-14 days
Date the infarct: no red neurons, necrotic white matter w/ macs, axonal spheroids, early wallerian degen in non-infarcted white matter
15-30 days
Epidural hemorrhage
Almost always caused by trauma, middle meningeal artery, lucid interval, temporal in adults, posterior in peds, () shape. Microscopic: usually only difuse axonal injury. 75-100 ml is fatal, Less common than subdural
Subdural Hemorrhage
Concave (between dura and brain), bridging veins. Truama or spontaneous- can be acute or chronic (elderly)
Subarachnoid hemorrhage
between arachnoid and pia, spontaneous or traumatic. Berry aneurysm is most common cause, also mycotic aneurysm, fusiform aneurysm
Basis pontis hemorrhage
Locked in syndrome
Thalamic hemorrhage
Hemiparesis, hemisensory loss, gaze abn, vomitting
Cerebellar hemorrhage
Vomiting, HA, Tuncal/limb ataxia, cranial nerve abn, coma
Large intrapontine hemorrhage
Coma, quadraplegia, small reactive pupils
small intrapontine hemorrhage
gaze paresis, ataxia, contralateral sensorimotor defecit
Putaminal hemorrhage
Hemiparesis, hemisensory loss, visual field deficits
Cerebral Amyloid Angiopathy
APOE4, APOE2
Familial (ususally autosomal dominant):
HCHWA- Dutch type: mutation in APP, severe dementia, rare NFTs and plaques
Hereditary Cystatin C AA (HCHWA- Icelandic type): Mutation in cystatin C, young and middle aged adults, hemorrhage often lobar and fatal, no AD changes
Capillary telangiectasia
Usually pons, vessels are smaller and have thinner walls than venous angioma, usually asymptomatic
