Cerebrovascular Diseases

Question 1

What is a common presentation for stroke in a child < 2 years of age?

Answer 1

In children < 2 years of age, a large majority of patients present with seizures and hemiparesis.

Question 2

What is one way that strokes in utero might present in the infant?

Answer 2

These strokes can present as early-onset hand dominance or hemiplegic CP.

Question 3

How do perinatal strokes present?

Answer 3

Focal neonatal seizures and mental status changes. The focal neurologic deficits don’t show up for weeks to months.

Question 4

How do strokes present in older children?

Answer 4

Acute focal neurological deficit, with or without seizures.

Question 5

What four etiologies need to be on the differential in children who present with acute hemiplegia?

Answer 5

Stroke, transient postictal hemiparesis (Todd paralysis), complex migraine, and alternating hemiplegia of childhood.

Question 6

What is transient postictal hemiparesis? How long does it last?

Answer 6

It is a neuronal exhaustion phenomenon following seizure activity. Its efffects usually last 24-48 hours, but can last up to one week.

Question 7

If acute hemiplegia occurs as a result of a complex migraine, how long would you expect the deficits to last?

Answer 7

The focal deficits can last hours or up to one week.

Question 8

How would imaging be helpful in differentiating between stroke, transient postictal hemiparesis, and complex migraine.

Answer 8

MRI brain should be negative for infarction in both complex migraine and transient postictal hemiparesis, but should be abnormal if the symptoms are due to an acute stroke.

Question 9

What is alternating hemiplegia of childhood?

Answer 9

It is a rare genetic disorder, generally beginning in children < 2 years of age, which presents as hemiplegia lasting minutes to hours, with weakness fluctuating between the two sides for each attack. Seizures are common but do not occur during the periods of weakness. Most children have progressive neurologic or developmental deterioration.

Question 10

What CNS event can occur in children with congenital heart disease?

Answer 10

Congenital heart disease complications cause ~25% of pediatric strokes. Most are due to embolic phenomena from the heart or shunted through the heart.

Question 11

What is the most common inheritable cause of venous thrombosis?

Answer 11

Activated Protein C resistance (Factor V Leiden). In older children, it has been known to cause arterial thrombosis as well.

Question 12

The presence of antiphospholipid antibody (including anticardiolipin antibody and lupus anticoagulant) is a risk factor for what event?

Answer 12

Pediatric stroke.

Question 13

What is the most common single risk factor cause of stroke in children?

Answer 13

Sickle cell disease

Question 14

Patients with sickle cell disease have the highest incidence of stroke within what age-range?

Answer 14

Children 2-5 years of age

Question 15

T/F: Children with sickle cell disease can have MRI evidence of prior stroke without symptoms.

Answer 15

True. ~20% of children with sickle cell disease have MRI/CT evidence of stroke without recognizable clinical symptoms (“silent strokes”).

Question 16

What protocol is used to prevent stroke recurrence in children with sickle cell disease.

Answer 16

Chronic transfusion protocols should be followed to prevent recurrences.

Question 17

Describe Moyamoya disease.

Answer 17

It is a chronic, occlusive, cerebrovascular disease which presents as an extensive collection of collateral vessels resembling a “puff of smoke” resulting from prior occlusion of arteries around the circle of Willis.

Question 18

List four known predisposing conditions for the development of Moyamoya disease in children.

Answer 18

Sickle cell disease, neurofibromatosis type 1, trisomy 21, and cranial irradiation.

Question 19

What is the treatment for Moyamoya disease?

Answer 19

Revascularization surgery

Question 20

Which arteries are most commonly affected by cerviocephalic arterial dissection?

Answer 20

The internal carotid arteries.

Question 21

What are the typical symptoms in children with cervicosephalic arterial dissection?

Answer 21

Focal cerebral symptoms with ipsilateral headache, neck, or eye pain.

Question 22

How does treatment differ for extracranial vs intracranial arterial dissection?

Answer 22

Treatment is anticoagulation for extracranial dissection. Anticoagulation is contraindicated for intracranial dissection due to the risk for developing a subarachnoid hemorrhage.

Question 23

What is the most common cause of CNS vasculitis?

Answer 23

Bacterial meningitis.

Question 24

Which virus has been shown to be a risk factor for causing infarcts in the basal ganglia or internal capsule in children?

Answer 24

Varicella has been shown to be a risk factor for childhood stroke, with cases occurring in children < 10 years of age. The infarcts affect the basal ganglia or internal capsule and usually occur within 9 months of developing the varicella rash.

Question 25

What two drugs increase the risk of vasculopathy and vasospasm?

Answer 25

Cocaine and diet pills

Question 26

Name two metabolic diseases which are known to cause strokes due to vascular occlusion in children.

Answer 26

Fabry disease and homocystinuria

Question 27

In which cerebral artery location do most neonatal infarcts occur? Are they usually embolic or thrombotic in character?

Answer 27

Most neonatal cerebral infarctions are embolic and occur in the distribution of the middle cerebral artery.

Question 28

What is the most common presentation for an infant with neonatal cerebral infarct?

Answer 28

Focal seizures within the first 3-4 days of life.

Question 29

How does subarachnoid hemorrhage usually present?

Answer 29

It presents with “the worst headache of my life”, followed by findings of meningeal irritation and increased intracranial pressure.

Question 30

What is the most common cause of intracranial bleed?

Answer 30

Head trauma, including nonaccidental trauma.

Question 31

What are arteriovenous malformations?

Answer 31

Abnormal collections of arteries and veins without the normal intervening capillary bed.

Question 32

What is the recommended management of cerebral AVMs?

Answer 32

AVMs require embolization or surgical removal.

Question 33

What symptoms are associated with intracranial AVMs?

Answer 33

Smaller AVMs appear with focal seizures. Large AVMs present with headache, focal neurologic findings, intracranial bruits, and seizures. Large AVMs can be fatal with rupture.

Question 34

What are intracranial cavernous malformations?

Answer 34

Collections of thin-walled vessels with only a single layer of epithelium and nothing else. Recurrent bleeds are typical, but they are rarely life-threatening.

Question 35

What is the most common vascular malformation?

Answer 35

Venous angiomas

Question 36

What symptoms are typically associated with saccular aneurysms?

Answer 36

They are typically asymptomatic, but they can cause acute subarachnoid hemorrhage.

Question 37

Saccular aneurysms are associated with which (4) diseases?

Answer 37

They are seen in conjunction with coarctation of the aorta, polycystic kidneys, Ehlers-Danlos syndrome, and Marfan syndrome.

Question 38

In neonates, how do most cerebral vascular thrombotic events present?

Answer 38

Seizures

Question 39

In older children, how do most cerebral vascular thrombotic events present?

Answer 39

Headache and vomiting

Question 40

What symptoms are associated with thrombosis of the superior sagittal sinus?

Answer 40

Thrombus in this location leads to obstruction of CSF absorption and causes increased intracranial pressure. Patients present with headache, papilledema, nausea, vomiting, and CN 6 palsy.

Question 41

What symptoms are associated with right lateral sinus thrombosis?

Answer 41

Pseudotumor cerebri, with papilledema and CN 6 palsy due to increased ICP.

Question 42

What symptoms are associated with thrombosis of the cavernous sinuses?

Answer 42

Proptosis, chemosis, and uni- or bilateral ophthalmoplegia.

Question 43

List three risk factors for the development of cerebral venous thrombosis?

Answer 43

Prothrombotic conditions, infection, and dehydration.

Question 44

What imaging modality is best for identification of cerebral vein thrombosis?

Answer 44

MRV is the best method of diagnosis. CTV is also a sensitive diagnostic tool if MRV is unavailable.