Perinatal puzzlers
This is the age cut off for perinatal stroke.
What is 28 days?
Perinatal stroke is defined as a stroke occurring between day of life 0-28.
What are arteriopathies?
These processes can include focal cerebral arteriopathy (FCA) and moyamoya disease.
This congenital heart defect is most commonly encountered by adult neurologists.
What is a PFO?
About 25% of the population has a PFO, but 50% of young adults with stroke are found to have a PFO.
Although typically associated with younger women, this type of stroke is 5 times more common than in neonates than younger children.
What is a cerebral venous thrombosis?
This type of stroke occurs due to back pressure from clotting in the sinuses and veins of the CNS.
These are the two most common symptoms of perinatal stroke.
What are acute encephalopathy and acute symptomatic seizures?
These symptoms often occur in the absence of focal neurological deficits. If focal deficits are seen through development with imaging consistent with chronic stroke, a presumed diagnosis of perinatal stroke can be made.
These are the two subtypes of focal cerebral arteriopathy.
What are inflammatory and dissection?
Dissection-type is often related to a traumatic injury while inflammatory-type is often thought to be related to an active or post-infectious inflammatory process. Steroids have been proposed treatment for the latter, with ongoing studies investigating the validity.
These two clinical decision tools can be used when considering the likelihood of stroke related to PFO.
What are the RoPE and PASCAL scores?
Risk of Paradoxical Embolism: age, hx of diabetes/TIA/smoking, and cortical stroke
PASCAL: RoPE>7 and high-risk PFO feature (shunt size or presence of atrial aneurysm)
The TO-ACT trial evaluated this intervention for management of CVT with adverse outcomes but was stopped early due to futility.
What is endovascular intervention?
This treatment is not used routinely and is reserved for declining patients that are refractory to medical treatment. In life threatening circumstances, decompressive surgery is warranted.
This is the proposed mechanism for perinatal stroke.
What is emboli from placental vasculature?
With this thought to be the most common etiology, antiplatelet therapy is not warranted due to low concern for recurrence.
Progressive occlusion of the distal ICA with development of smaller collaterals found in isolation of other diagnoses can be called this.
What is moyamoya disease?
Moyamoya syndrome is the presence of aforementioned process in the presence of a known inciting disease (NF-1, trisomy 21, sickle cell disease).
This type of cardiac lesion is the highest risk factor for pediatric cardioembolic stroke.
What are cyanotic lesions?
These include transposition of the great arteries or hypoplastic left heart syndrome.
This type of anticoagulant can be used for long term management of CVT.
What are warfarin and DOACs?
Studies have shown that DOACs are non-inferior to warfarin after parenteral anticoagulation. Duration of 3-6 vs. 12 months did not show differences in bleeding or recurrence, so length should be chosen based on existing risk factors.
Pathogenic variants in these genes (1 of 2 for points) can be found in neonates with no risk factors for perinatal stroke.
What are COL4A1/COL4A2 genes?
These genes encode for collagen fibers, and pathogenic variants should be considered especially in the presence of antenatal stroke, intracranial hemorrhage, or porencephalic cysts.
This gene is the thought to increase susceptibility to developing moyamoya disease.
What is RNF213?
This gene is typically associated with early onset, rapid progression, and bilateral involvement.
There are six major risk factors for perinatal stroke. Name 4 for the points!
Intrauterine growth restriction, chorioamnionitis, perinatal asphyxia, infections, inherited thrombophilia, and complex congenital heart disease.
In almost half of cases, no risk factor is found. Interestingly, birth trauma has not been shown to increase risk of perinatal stroke.
About 1 in 5 spontaneous arterial dissections occur in patients with monogenic connective tissue diseases. Name three for the points!
What are EDS type IV, Marfan syndrome, osteogenesis imperfecta, and Loeys-Dietz syndrome?
These syndromes all impact collagen function and lead to weakening in the arterial wall integrity.