Enzyme in Fabry
alpha-galactosidase
accumulation of globotriaosylceramide in vascular SmM and EndoT –> stroke
hemorrhage rate unruptured aneurysms
<10 mm = 0.05%/yr
10-25 mm = 1%/yr
>25 mm = 6%
TCD cutoff for Exchange Transfusion in SCD>
200 cm/s (in MCA)
target HbS 30%
preferred Rx of VOG malformation
embolization
goal MAP in ICH w known hypertensive pt
130 mmHg
PAN size arteries affected
small and medium sized arteries
Recurrent bleeding rates of vascular malformations
DVA: 0.15%/yr HTN: 2%/yr Cav mal: 4.5% AVM: 3-34% (dep. Characteristics) CAA: 10%
Seizure rates w acute cerebrovascular Dz
AIS : 6%
SAH: 5%
ICH: 30% (cortical), 5% subcortical
peak SAH periods
re-bleeding = 12-48 hr vasospasm = 3-?14 (Borsody = 4-7)
Basic pathophys of MTHFR mutation
Cannot convert Hcy back to Methionine, both increase in homocystinemia
Esoteric condition asoc w marantic endocarditis
AIDS, DIC, SLE
Synonym for Factor V Leiden
activated Protein C resistance (Factor V Leiden resists cleavage by Protein C)
AVM vs. CavMal vs. DVA
AVM = arterial-venous anastomosis, no parenchyma between vessels
CavMal (aka cavernoma/cavernous hemangioma) = abnormal venous structures, no parenchyma between vessels, +/- adj. cortical dysplasia
DVA = (aka venous angioma) = cluster of normal venous str
Arterial layer MC involved in FMD?
Tunica media
Usually results in tunica medica fibroplasia
CHADS2 components
CHF at onset of AF, Hypertension, Age >75, Diabetes, Stroke/TIA(+2)
Annualized stroke risks on ASA: 0 (1%), 1 (2%), 2 (4%), 3 (8%), 4 (10%), 5 (12%), 6 (14%)
ABCD2 components
Age > 60 +1
BP > 140/90+1
Duration (<10 min+0, 10-59min+1, >60 min+2)
Diabetes+1
Clinical (other symptoms+0, speech disturbance wo weakness+1, unilateral weakness+2)
Stroke recurrence rates by TOAST
Extracranial LAA > cardioembolic > intracranial LAA > lacunar
size of cerebellar ICH for surgery?
3 cm
re-bleeding rate SAH (first week; unsecured)
20%
Sturge Weber
Neurocutaneous capillary malformation
leptomeningal angioma + capillary malformation (port wne stain over V1) + eye capillary malformations
MC presentation = seizure in infancy, or stroke-like episodes
Sneddon syndrome
positive Lupus AC, livedo reticularis, stroke
Blood age on MRI
oxyHg (hyperacute) –> deoxyHg (acute) –> intrac metHg (early subacute) –> extrac metHg (late subacute) –> hemosiderin (chronic, intra)
Percent of CT-neg SAH?
5%; 70% CSF is positive for xantho within 6 hours, 90% within 12 of SAH
CADASIL vs. MELAS
CADASIL = Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. NOTCH3 mutation; deposition in media of end-arteries that surround SmM cells; subcortical stroke + dementia + migraine +/-mood d; MELAS = Mitochondrial Encephalopathy, Lactic acidosis, Stroke-like episodes. mRNA mutations; occipital, multi-territory stroke + dementia + migraine + seizures + SN hearing loss + ret
Gene in Pseudoxanthoma Elasticum
ABCC6 gene (encodes MRP6 protein, function unknown)
MOA of aspirin?
irreversible inhibitition of COX production of TXA;
also inhibits vasodilatory prostacyclin production
MOA of dipyridamole?
inhibition of cAMP-phosphodiesterase –> incr cAMP
blocking adenosine reuptake. Also vasodilation (HA)
end result = inhibition of platelet aggregation
MOA of clopidogrel?
Blocking of adenosine diphosphate (ADP) binding to Adenosine (R)
MOA of ticlopidine?
poorly understood, but inhibits platelet aggregation
MOA of warfarin
inhibits Factors II, VII, IX, X, C+S
basically acts on both intrinsic + extrinsic systems
MOA of heparins?
ALL: activates Antithrombin III, which then:
Unfractionated: inhibits Factor X = Factor II
Lovenox: X > II
MOA of dabigatran?
direct thrombin inhibitor (Factor II)
MOA of t-PA?
activates plasminogen, which turns Factor I (fibrin clot) into fibrin degradation products
MOA of edoxaban, apixaban, rivaroxaban
Factor Xa inhibitors (Factor Xa activates prothrombin into thrombin)
MOA of statins?
HMG-CoA reductase inhibition
inhibits enzyme that catalyzes production of mevalonic acid)
–> inhibits cell production of cholesterol
MOA of thiazide diuretics?
Block reabsorption of NaCl from distal tubule
increase Ca2+
decrease K+
decreases Na+ –> hyponatremia
MOA of furosemide?
Loop diuretic. Block Na+, Cl-, K+ reabsorption in Loop of Henle.
Can also cause hypoK/Na/Mg/Ca2+
Can cause hyperuricemia
MOA of nitrates?
venodilation through donation of NO.
MOA of different CCBs?
Dihydropyridine (-dipine): vasodilation (vessels>heart)
verapamil: cardioselective (AE = neg inotropy)
diltiazem: intermediate
MOA of cilostazol?
inhibition of phosphodiesterase (platelet inhibition) + vasodilation
MOA of abciximab/eptifibatide?
blocking binding of GP2a/3b with fibrinogen (humanized monoclonal ab against glycoprotein IIb/IIIa R).
MOA of eptifibatide?
GP2b/3a receptor antagonist
most common AE of clopidogrel?
rash
cardioselective beta blockers
Eskimos (esmolol) Ate (atenolol) Ace (acebutolol) Butt (??) Meat (metoprolol)
MOA of doxazosin
alpha blocker