Vascular Disease Flashcards

Question

What Borden grade for dural AVFs generally warrant treatment?

Answer 1

Borden II and III due to 15% annual event rate

Answer 2

Elimination of cortical venous reflux (CVR)

Answer 3

Dural venous drainage with no cortical venous reflux

Answer 4

Dural venous drainage WITH cortical venous reflux

Answer 5

Cortical Venous reflux only

Answer 6

Cognard scale separates dural venous drainage into anterograde and retrograde drainage. For Borden grade III, Cognard scale distinguishes between cortical venous reflux +/- venous ectasis

Answer 7

Transvenous coiling

Answer 8

Age 3 and 20-30s

Answer 9

In pediatric cases more often presents with ischemia (80%) In adult cases more often presents with hemorrhage (60%)

Answer 10

Cavernous malformations

Answer 11

Seizures, focal deficits, headaches Hemorrhage may occur and is more common in deeper lesions and in the posterior fossa

Answer 12

Medical mgmt/observance for seizure control Surgical removal is often very effective Radiosurgery usually doesn't alter their natural history

Answer 13

Age > 60, EtOH use, HTN, amyloid angiopathy, drugs (e.g. cocaine), coagulopathy

Answer 14

Strict BP control (varies on center, at UPMC SBP goal <140 and can later be liberalized to <180) Correct coagulation Manage ICPs and frequent neuro exams

Answer 15

Need to be prudently watched because if there are signs of neurologic deterioration, brainstem compression, or obstructive HCP then urgent evacuation may be needed

Answer 16

Basal Ganglia Thalamus Pons Cerebellum

Answer 17

Post-infarct edema which may cause obstructive HCP or malignant edema

Answer 18

Cerebellar: craniectomy and possibly strokectomy MCA; Hemicraniectomy can be done but has severe morbidity; outcomes are better in younger patients and in nondominant hemisphere strokes

Answer 19

70-99% stenosis

Answer 20

NASCET shows very mild absolute risk reduction at 5 years with CEA (6% ARR)

Answer 21

Carotid dissection

Answer 22

Antiplatelet/anticoagulate agents for 6-12 weeks

Answer 23

Antiplatelet/anticoagulate for 6-12 weeks (unless SAH part of presentation) Surgical options: proximal clipping, vessel reconstruction, endovascular stenting

Answer 24

High-flow (e.g. cavernous carotid aneurysm rupture) | Low-flow (e.g. dural carotid branch, ECA, etc.)

Answer 25

Pulsatile proptosis, retro-orbital/orbital pain, chemosis, ocular bruit, visual deterioration, diplopia

Answer 26

~50% thrombose on their own | (Medically managing is ok as long IOP < 25mmHg

Answer 27

Endovascular transarterial embolization | Endovascular transvenous coiling

Answer 28

## Footnote When compared to DWI (Diffusion-Weighted Imaging), ADC (Apparent Diffusion Coefficient) is a complementary MRI technique that provides additional information about tissue microstructure. While DWI measures the random motion of water molecules, ADC calculates the rate of diffusion in multiple directions. ADC is particularly useful for evaluating white matter integrity, detecting Alzheimer's disease, and differentiating between tumor types. In MR imaging, ADC and DWI are often used together to provide a more comprehensive understanding of tissue characteristics and abnormalities.

Answer 29

The minimal lumen diameter is divided by the diameter of the distal ICA where the walls become parallel so as to avoid any post-stenosis dilatation

Answer 30

Carotid endarterectomy (1) **Asymptomatic** stenosis of 60–99% has a 6% absolute risk reduc- tion for stroke over 5 years if operative mortality < 3% and if the patient has a 5-year life expectancy (a) stroke risk of untreated asymptomatic stenosis is 2% per year (2) **Symptomatic stenosis** of 70–99% has a 17% absolute risk reduction for stroke over 2 years if operative mortality < 6% and if the patient has a 5-year life expectancy (a) Stroke risk of untreated symptomatic stenosis is 13% per year (b) Beneﬁt of surgery persists for less than 1 year after stroke or TIA; therefore, surgery is best done as soon as possible after a stroke (usually within 4 to 6 weeks when the risk of stroke is highest) (c) 50%–70% symptomatic stenosis may also beneﬁt from surgery in (i) Male patients (ii) Conjunction with plaque ulceration (iii) Cases involving nonlacunar hemispheric stroke or TIA (d) Endovascular stenting is a treatment option for carotid ste- nosis in patients who are poor surgical candidates

Answer 31

Amyloid angiopathy:develops when ﬁbrillary proteins form a β-pleated sheet conformation that deposits in the walls of arterioles as amyloid, causing wall thickening and lumen narrowing; amyloid deposits can extend into the surrounding perivascular tissue, resem- bling senile plaques of Alzheimer disease (1) Histology: similar to lipohyalinosis except that amyloid angiopa- thy is congophilic (i.e., exhibits apple-green birefringence after Congo red staining) whereas lipohyalinosis is eosinophilic (i.e., stains with hematoxylin) (2) ICH from amyloid angiopathy often extends into an SAH because of the cortical location of the ICH (3) Genetics: most cases are sporadic and involve the amyloid pre- cursor protein (APP) (i.e., the amyloid β-protein 1-42 [Aβ1-42]) that is found in Alzheimer plaques (a) Rare hereditary forms of amyloid angiopathy (i) Icelandic-type amyloid angiopathy: mutations of cys- tatin C, a protease inhibitor that accumulates as amyloid when mutated; causes fatal ICH by 20–30 years of age (ii) Dutch-type amyloid angiopathy: mutations in APP that cause accumulation as amyloid; causes fatal ICH by 40 years of age (iii) Mutations of transthyretin, the thyroxine (T4) carrier protein

Answer 32

Pathophysiology: a systemic vascular disease of smooth muscle; caused by **autosomal dominant mutations of the Notch 3gene on chromosome 19**, the protein of which acts as a cell-cell adhesion molecule with nuclear transcription regulatory properties I. Transcription regulatory properties require cleavage of the Notch 3 protein by γ-secretase and transport to the nucleus ii. Notch 3 protein in the cell membrane binds the delta ligand on neigh-boring cells iii. Notch 3 gene is located very close to the CACNL1A4gene that is mutated in familial hemiplegic migraine (1) Sporadic mutation in 15% of patients with characteristic MRI ab- normalities and no family history b. Histology: **ﬁbrosis of the tunica adventitia and granular deposits in the tunica media that develop in arteries of 100–400 mm in diameter (penetrating arteries)** i. Deposits are of an unknown composition that is not amyloid ii. Deposits surround the smooth muscle cells, and also occur in muscle and skin arteries in some patients c. Symptoms: onset between 30–50 years of age i. Recurrent lacunar stroke and TIA, often occurring in the thalamus and sparing the cortex ii. Dementia (30%): usually develops > 10 years after the ﬁrst stroke from a combination of multiple strokes (i.e., a vascular dementia) and progressive leukoencephalopathy iii. Migraine with aura (25%): does not develop in childhood like other migraines, and typically involves fever and/or stupor {confusional migraine (see pg. 154)} iv. Mood disorders: develop early in the disease course

Answer 33

Pathophysiology: caused by point mutations in a transfer ribonucleic acid (tRNA) encoded by the mitochondrial genome; exhibits **maternal inheritance** i. Highly variable penetrance dependent on the number of aﬀected mitochondria b. Symptoms: usually presents in childhood i. Stroke, particularly in the occipital region and frequently in a distribution that is not deﬁned by a single artery ii. Migraine, often occurring at the time of a stroke iii. Vision loss from retinopathy; sensorineural hearing loss iv. Diﬀuse weakness from myopathy v. Seizures and dementia, which develop during adolescence c. Diagnostic testing: lactic acidosis; ragged-red ﬁbers on muscle biopsy d. Treatment: B-complex vitamins + niacin + coenzyme Q10; dichloroacetate

Answer 34

Infectious endocarditis: causative organisms are Streptococcus **viridans, Staphylococcus aureus, Enterococcus >> HACEK organisms** (Haemophilus, Acinetobacillus, Cardiobacterium, Eikenella, Kingella), fungi i. Risk factors include IV drug abuse (usually causes right-sided endo- carditis) and cardiac abnormalities (e.g., artiﬁcial heart valves) ii. Infectious endocarditis leads to an infectious arteritis, which pro- gresses to (1) Mycotic aneurysm formation on the distal superﬁcial cerebral arteries (2) Microabscess formation (3) Meningoencephalitis

Answer 35

a. Pathophysiology: **smooth muscle hyperplasia and ﬁbrosis of small- and medium-sized arteries** (renal > cervical > visceral > extremities); variants of the disease can involve hypertrophy of the tunica intima or adventitia i. A non-inﬂammatory condition that does not involve atherosclerosis or vascular calciﬁcation but that is associated with the **development of cerebral aneurysms** ii. Involvement of the extracranial carotid and vertebral arteries is much more common than involvement of the intracranial vasculature b. Epidemiology: usually occurs in middle-aged women c. Symptoms: ischemic stroke (30%); hypertension from renal artery stenosis; limb claudication; bowel ischemia d. Diagnostic testing: angiography demonstrates alternating segments of constriction and dilation (string of beads sign, tubular stenosis)); rarely, arterial dissection develops at sites of disease e. Treatment: antiplatelet agents or anticoagulation; surgical relief of obstruction in high-grade lesions

Answer 36

carotid injury occurs with compression against the transverse process of vertebrae during neck extension and rotation; vertebral injury occurs with stretching between C1 and C2 vertebrae during neck rotation Dissection locationi. Carotid artery: usually within 2–3 cm of the bifurcation; rarely en- ters the petrous bone or extends intracranially ii. Vertebral artery: generally starts around C1 or C2 vertebral level and often extends intracranially

Answer 37

(1) **Aspirin**: absolute risk reduction for recurrent stroke = 1% (a) Irreversibly *inhibits cyclooxygenase production* of thromboxane that causes platelet aggregation, but also inhibits vasodilatory prostacyclin production (b) Low-dose (e.g., 81 mg qd) or enteric-coated aspirin will inhibit platelet aggregation in only 40% of patients, in comparison with 70% of patients taking higher doses or uncoated aspirin, but higher doses are not known to be better for stroke prevention (2) **Dipyridamole** + aspirin (Aggrenox): modestly better than aspirin alone; higher side-eﬀect rate, particularly headaches (a) Dipyridamole reduces platelet aggregation by *inhibiting cyclic adenosine monophosphate (cAMP)-phosphodiesterase *and by blocking adenosine reuptake (3) Ticlopidine: seldom used; used for patients who cannot tolerate aspirin; has a higher side-eﬀect rate (diarrhea, neutropenia, thrombotic thrombocytopenic purpura) (a) Reduces platelet aggregation through a poorly understood mechanism (4) **Clopidogrel:**equivalent eﬃcacy against stroke in comparison to aspirin; beneﬁt of clopidogrel over aspirin is its superior protection against myocardial infarction in selected patients (a) *Reduces platelet aggregation by blocking adenosine diphosphate (ADP) binding to adenosine receptors* (b) Same side-eﬀect proﬁle as ticlopidine; fewer hemorrhagic side-eﬀects in comparison with aspirin (5) **Warfarin**: 67% relative risk reduction for stroke from atrial ﬁbrillation when International Normalized Ratio (INR) is 2–3 (a) For mechanical heart valves, target INR of 3–4; for bioprosthetic heart valve, target INR of 2–3 (6) **Dabigatran**: direct *thrombin inhibitor*; indicated for stroke pre-vention in non-valvular atrial ﬁbrillation (a) Requires dosing according to renal function (b) Has a lower rate of hemorrhagic complications than warfarin (7) **Heparin**: no proven beneﬁt in stroke management; used commonly as a bridge to warfarin therapy and for temporary management of unstable major artery stenosis prior to surgical or endovascular intervention (a) Side eﬀects: heparin-induced thrombocytopathy (HIT) syndromes, cauased by a decreased number of platelets and platelet malfunction (i) Type I HIT: develops < 5 days after starting heparin and is clinically benign; heparin causes platelet aggregation, thereby increasing blood viscosity (ii) Type II HIT: develops 1–2 weeks after starting heparin and is associated with arterial and venous thromboses; heparin-induced autoantibodies cause thrombocytope- nia but also a *paradoxical hypercoaguable* state

Answer 38

(1) For patients on **warfarin**, use (a) 2–3 units fresh-frozen plasma; titrate to normalization of coagulation measures, which may require up to 6 units (b) Vitamin K 10 mg IM: takes 12 hours to have any eﬀect (2) For patients on **heparin**, use protamine 1 mg per 100 units of heparin administered (3) For patients on **dabigatran**, no reversal agents are available; plasma clotting factor administration or hemodialysis may reduce drug levels

Answer 39

Anton-Babinski syndrome, also known as ABS or Anton syndrome, is visual anosognosia, or denial of vision loss, associated with confabulation, or making up experiences to compensate for memory loss, in the setting of cortical blindness.

Answer 40

type I: dural AVF. type II: intramedullary glomus AVM. type III: intramedullary juvenile AVM. type IV: perimedullary AVF. type V: extradural AVF. | dAVF, IM g, IM j, PM, ED

Answer 41

Patients with nidus-type AVMs were younger at presentation and more often presented with hemorrhage, with a higher proportion of hematomyelia than fistulous-type AVMs (P<0.05). Progression of clinical presentation from hemorrhage to congestive myelopathy during follow-up was noted in 5 patients, all of which had AVMs of the nidus type. Complete obliteration could be achieved more often in the fistulous type (72%) than in the nidus type (27%). Improved or stable clinical status at last follow-up was noted in 100% of fistulous-type and 77% of nidus-type patients. Long-term clinical deterioration was noted in 6 of 26 patients with nidus-type (23%) AVMs and was related to recurrent bleeding (n=3) or progressive venous congestion (n=3). Overall rebleed rate after presentation with hemorrhage was 7 in 145.5 patient-years (4.8%/y) if the lesion was not treated, 3 in 102 patient-years (2.9%/y) after partial treatment, and 0 in 47.5 patient-years (0%) after complete treatment. ## Footnote Clinical Features and Outcomes of Spinal Cord Arteriovenous Malformations: Comparison Between Nidus and Fistulous Types