Stroke in children and young adult

1. By
Ahmed Abd El Hady
Assistant lecturer of neurology
Assiut university
Stroke in children and young adult

2. Agenda
 Epidemiology
 Etiology of stroke in young patients
 Clinical presentation
 Evaluation of stroke in young patients
 Management
 Prognosis

3. Epidemiology
 Incidence of stroke in children older than 28 days
about 13/100.000 for all strokes
 Incidence of stroke in young adult (18-49) about
17/100.000 for all strokes
 In the geriatric the ischemic– hemorrhagic stroke
ratio is approximately 4 to 1 (80% of strokes are
ischemic), whereas in the young, the ratio is close
to 1.0–1.5 (60% are hemorrhagic)

4. Etiology of stroke in children (1 month to 18
years).
1. Hemorrhagic strokes in children
 Intracererebral hemorrhage represent the majority of
the cases (50–75%)
 Vascular malformations like AVM, cavernomas and Vein
of Galen malformation (VGAM) are most common
causes .
 SAH represent less than 25% and mostly due to
aneurysm (including mycotic aneurysm)
 Other causes of hemorrhagic strokes include
hematological disease, head truma and acute
hypertension as in pheochromocytoma and GN

5. In VGAM the vein of Galen is greatly enlarged, forming a
large varix, and the straight sinus and
confluence of sinuses is also large and tortuous.

6. Continue :- Etiology of stroke in children (1 month
to 18 years).
2- Arterial ischemic stroke in children (1 month-18
years)
Main categories of etiology are
 Arteriopathy
 Cardiac
 Hematologic
 Other etiology like metabolic/genetic

7. Continue :- Etiology of stroke in children (1 month
to 18 years).
 Cardiac etiology
Represent about 25% of ischemic strokes in
children
 Rheumatic heart disease, endocarditis,
congenital heart dieses, cardiomyopathies, and
myocarditis are important heart diseases
associated with ischemic strokes

8. Continue :- Etiology of stroke in children (1 month
to 18 years).
2. Arteriopathy represent about 50% of ischemic strokes in
children
Common causes of arteriopathy include
 Transient cerebral arteriopathy (TCA)
 Postvaricella and other viruses angiopathy (PVA)
 Systemic/secondary vasculitis (e.g., Takayasu arteritis)
 Moyamoya disease/syndrome
 Arterial infection (e.g., bacterial meningitis, tuberculosis)
 Fibromuscular dysplasia
 Traumatic or spontaneous carotid or vertebral artery
dissection
 Migraine (migrainous infarction?)
 Congenital arterial hypoplasia (e.g., PHACES syndrome)
 CADASIL syndrome

9. Continue :- Etiology of stroke in children (1 month
to 18 years).
Transient cerebral arteriopathy (TCA)
Is the most common arteriopathy in childhood
stroke (7% of ischemic stroke in children).
lenticulostriate infarction due to unilateral
intracranial arteriopathy affecting the supraclinoid
internal carotid artery and its proximal branches.
The pathogenesis of TCA is unknown to date
The arteriopathy mostly stabilizes, even
completely resolves.

11. Continue :- Etiology of stroke in children (1 month
to 18 years).
Trauma
 Is important cause of arteriopathy in childhood
 Direct trauma can lead to arterial occlusion and
intense vasoconstriction
 Dissection to extracranial carotid and vertebral
artery represent about 7% of ischemic stroke in
children and can develop after head or neck
injuries, spontaneous dissection also can occurs.
 Connective tissue disorders such as Ehlers-
Danlos syndrome and Marfan syndrome can
predispose to dissection.
 Oral trauma by penetrating objects can cause ICA
injuries.

13. Continue :- Etiology of stroke in children (1 month
to 18 years).
Infection
 Most serious complications of Varicella zoster virus
(VZV) is VZV vasculopathy
 VZV vasculopathies develop within 6 weeks after
VZ infection
 VZV gain access to the cerebral arteries through
trigeminal ganglia (trigeminovascular bundle) in
endothelium virus activate platelet and triggering
the coagulation cascade
 Brain infarction mostly occure deep in the basal
ganglia, internal capsule, and thalamus.
 vasculopathy is confirmed by the detection of VZV
DNA or anti-VZV antibody in CSF

14. Continue :- Etiology of stroke in children (1 month
to 18 years).
Migraine
 Hemiplegic migraine is a form of migraine with
aura that includes motor weakness.
 Familial hemiplegic migraine (FHM) is an
autosomal dominant form of migraine
 Neurologic deficits with FHM can be prolonged
for hours to days
 FHM frequently beginning in the first or second
decade
 There three genes are associated with
FHM: CACNA1A (FHM1), ATP1A2 (FHM2),
and SCN1A (FHM3).

15. Continue :- Etiology of stroke in children (1 month
to 18 years).
Moyamoya syndrome/disease is characterized by
progressive stenosis of the internal carotid arteries
and formation of collateral vessels that give a "puff of
smoke" appearance on angiography.
 8 percent of children with arterial ischemic stroke had
moyamoya syndrome/disesae
 Secondary moyamoya syndrome is seen in
association with neurofibromatosis, trisomy 21,
Williams syndrome, sickle cell disease, congenital
dwarfism, and as a sequela of cranial irradiation.
 In children, moyamoya typically presents with
recurrent TIAs or ischemic strokes, while intracranial
hemorrhage is more common in young adults

17. Continue :- Etiology of stroke in children (1 month
to 18 years).
Other cause of arteriopathy
Vasculitis
 Vasculitis (inflammatory changes in the cerebral
vessels) can be primary (like Takayasu arteritis)
or secondary to collagen vascular diseases
CADASIL (cerebral autosomal-dominant
arteriopathy with subcortical infarcts and
leukoencephalopathy) is caused by a mutation in
the Notch3 gene, which leads to progressive
degeneration of smooth muscle cells in the vessel
wall. Patients with CADASIL may present with
migraine, TIA, or ischemic stroke in late childhood
or early adulthood

18. Continue :- Etiology of stroke in children (1 month
to 18 years).
MELAS and disorders causing metabolic
stroke
 Some metabolic conditions are associated with
metabolic stroke rather than arterial stroke. This
category includes the syndrome of mitochondrial
encephalomyopathy with lactic acidosis and
stroke-like episodes (MELAS), a maternally
inherited multisystem disorder caused by
mutations of mitochondrial DNA.
 Fabry disease, an X-linked lysosomal storage
disorder may cause vessel narrowing and
infarction and is due to deficiency of alpha-
galactosidase A,

19. Continue :- Etiology of stroke in children (1 month
to 18 years)
Some hematological and coagulation disorders may cause strokes
in children and must be evaluated
Disorder Test
Anemia (particularly iron
deficiency), polycythemia and skill
cell disease
CBC and Hemoglobin
electrophoresis
Antiphospholipid syndrome Lupus anticoagulant ,
Anticardiolipin antibodies (IgG and
IgM) and Beta2-glycoprotein I
antibodies (IgG and IgM)
Factor V Leiden mutation Factor V Leiden gene mutation
Protein C and S deficiency Protein C and S functional
Antithrombin III deficiency Antithrombin III activity
Prothrombin gene mutation Prothrombin gene mutation
Elevated lipoprotein(a) Lipoprotein (a) level

20. Strokes in young adults (18–45 years
of age)
Etiology of hemorrhagic stroke
 The etiology of ICH in patients younger than 45 years is
similar to those older than 45 years, except for an
overrepresentation of AVMs, aneurysm, cavernomas,
reversible cerebral vasoconstriction syndromes associated
with drug abuse and bleeding disorders such as hemophilia
 Amyloid angiopathy and warfarin-related hemorrhages are
less frequent in young patients.
Etiology ischemic stroke
 Etiology ischemic stroke in young adult include vasculopathy
(such as arterial dissection), cardiac defects,
hypercoagulable states (include pregnancy), smoking, illicit
drug use, premature atherosclerosis, hypertension, low

21. Clinical presentation
 Presentation of ischemic stroke in young adult and
hemorrhagic stroke not different from elderly
 There delayed in diagnosis due to stroke in young is
relatively rare
 A major cause of delayed in diagnosis in young
patient is wide range of stroke mimics
 In children, seizures at stroke onset are frequent
 In infants and young children focal deficits are less
common because the brain is insufficiently mature to
demonstrate these
 Abnormalities of posture and movement, such as
dystonias, chorea, and athetosis, are more frequent

22. stroke mimics common in young
patients
Migraine central nervous system (CNS)
demyelination
Seizures Peripheral nerve disorder
Bell’s palsy CNS infection
Conversion disorder Drug intoxication
Syncope CNS tumors
Cerebellitis Cord demyelination

23. Evaluation of stroke in young
patients
Neuroimaging
 Some guidelines recommend cranial CT,
including CT angiography (CTA), within one hour
of arrival at hospital for every child with suspected
stroke; Brain MRI, including MRI and angiography
(MRA), should be obtained within 24 hours if the
initial CT is negative and stroke is still suspected.
 CTA or MRA should cover the aortic arch to the
vertex (ie, the extracranial and intracranial
cerebral vessels).
 For vascular imaging catheter angiography is
gold standard for diagnosis

24. Continue:- Evaluation of stroke in young patients
 Laboratory studies — The initial evaluation for
stroke should include the following studies
CBC INR ,PTT
Kidney function Electrolyte
Oxygen saturation Serum glucose
ECG Transthoracic
echocardiography

25. Continue:- Evaluation of stroke in young patients
 In selected patients additional studies may be
useful.
 Lumbar puncture, if there is clinical suspicion for
subarachnoid hemorrhage and head CT scan is
negative, or if there is suspicion for an infectious
Cardiac enzymes and troponin Transesophageal
echocardiography
Holter monitor Toxicology screen
Hypercoagulable evaluation Vasculitis evaluation
Lumbar puncture Pregnancy test in women of
child-bearing

26. Continue:- Evaluation of stroke in young patients
Hypercoagulable evaluation
Vasculitis evaluation — The following studies are suggested
whenever there is clinical suspicion of vasculitis:
 Cerebral digital subtraction angiography
 Erythrocyte sedimentation rate and C-reactive protein level
 Antinuclear antibody assay
 Anti-neutrophil cytoplasmic antibody (ANCAs)
 Varicella titers
MELAS evaluation
If there multisystem impairment (eye, hearing, cardiac,
hepatic, renal) and mitochondrial disease is suspected
Other genetic and chromosomal study if there suspicious
for CADASIL syndrome, marfan S, neurofibromatosis type
1, trisomy 21 syndrome and Ehler Danlos S

27. Management
 Management of ischemic stroke in young adult
and hemorrhagic stroke not different from elderly
 Thrombolysis and thrombectomy —
Alteplase (tPA) is not approved for use in children
less than 18 years old.
 Safety and efficacy of mechanical thrombectomy
in children has not been tested
 There are no randomized controlled trials
examining the effectiveness of antiplatelet or
anticoagulation therapy for the treatment of acute
arterial ischemic stroke in children

28. Continue :- management
 For children with arterial stroke due to a confirmed
cardioembolic source or hypercoagulable state initial
anticoagulation treatment is preferable
 Aspirin (3 to 5 mg/kg per day) should be given if there
is a contraindication to anticoagulation.
 For extracranial arterial dissection, initial treatment with
either aspirin or anticoagulation is reasonable.
 For children with arterial ischemic stroke related to
vasculopathy, lacunar disease, or cryptogenic etiology,
treatment with aspirin (3 to 5 mg/kg per day) rather
than anticoagulation is preferable. Immunosuppression
may be indicated for confirmed inflammatory vasculitis

29. Continue :- management
 For children with acute ischemic stroke resulting
from sickle cell disease, treatment generally includes
urgent intravenous hydration with normal saline and
transfusion to reduce the hemoglobin S fraction to
≤30 percent of total hemoglobin.
 For children with large "malignant" middle cerebral
artery associated with mass effect, midline shift, and
deterioration of consciousness, decompressive
hemicraniectomy is suggested
 Patients who have moyamoya syndrome may be
candidate for surgical revascularization,

30. Prognosis
 In young, the recovery is better than in adults.
 Good collateral circulation often minimizes the
eventual brain damage, making the infarction
smaller than in adults.
 The developing brain shows more plasticity and
undamaged areas can frequently assume the
functions of damaged regions