Richard Leigh, M.D.
Johns Hopkins University School of Medicine
Generally stroke in a less than 40-45 years old
 Different from pediatric stroke
Unique causes that are more common in the young
 Cervical Artery Dissection
 Hypercoagulable States
 Vasospasm
Typical causes that are atypical in the young
 Hypertension
 Diabetes
 Hyperlipidemia
Appears to be trending toward younger
This trend appears to be larger in the
hemorrhagic stroke population.
Preferentially affecting lower socioeconomic
Attributable to modifiable risk factors:
hypertension, obesity and diabetes.
▪ Krishnamurthi et al., Lancet Global Health 2012
Hart & Miller, Stroke, 1983
Hart & Miller, Stroke, 1983
Hart & Miller, Stroke, 1983
Long-term prognosis
for stroke in the young
is better than the
elderly but higher
than the general
 Mostly in the first year
after stroke
A bad prognosis is
associated with an
atherosclerotic risk
Varona et al., J Neurol, 2004
Cryptogenic with PFO
Reversible vasoconstriction syndrome (RCVS)
 Not vasculitis!
Caused by separation of the arterial wall layers resulting in a false
 A history of trauma is often but not always elicited.
 Can be associated with major, minor or trivial trauma
 Can be spontaneous or cryptogenic
Typically the dissection occurs at the skull base
Can be diagnosed with CTA, angiogram typically not necessary
 Often associated with fibromuscular dysplasia
 Rarer conditions also have an increased incidence
▪ Ehlers-Danlos Syndrome Type IV
▪ Marfans Syndrome
Often associated with headache/neck pain acutely and chronically
 Responds to migraine therapies
Heparin or ASA are reasonable treatments
 With heparin only for 3-6 months then switch to ASA
Can be detected with CT
angiography and MR
Conventional angiography
is the gold standard
Angiography allows
for detection of FMD
in other vessels
Renal arteries can
also be affected
Prognosis is good
 Many dissections are
 Recurrent stroke after dissection
is rare with treatment
Treat with Aspirin or Coumadin
 Avoid anticoagulation of
intracranial dissections
▪ LP r/o SAH prior to a/c
 Transition to ASA after 3-6 months
 Pseudo aneurysms
PFO (patent foramen ovale)
 20-25% of adults have a PFO
 Some times associated with an ASA (atrial septal
PFO can serve as a source of paradoxical
 Venous clot (DVT) can traverse a right to left shunt and
enter the arterial circulation.
 Young people are felt to be at higher risk of
paradoxical emboli due to heart chamber pressures
that favor a right to left shunt.
There is an increase incidence of PFO and ASA in patients
who have had a cryptogenic stroke.
 There is no clear evidence that the PFO itself is the cause
of the stroke.
 This has lead to many centers advocating not to close PFOs since
they are not the cause.
 Instead, underlying causes of venous embolism are evaluated
and treated.
▪ Hypercoagulable states treated with anticoagulation
▪ Removal of triggers: Birth control, smoking
 If no cause if found other than PFO, treat with Aspirin
▪ Recurrent stroke very rare
▪ Data on PFO with ASA conflicting
▪ In the setting of recurrent stroke, PFO is closed
Primariy CNS Vasculitis?
 No! its almost never vasculitis
 Systemic rheumatologic diseases should be ruled out
Vasculitis mimicks
Intracranial Athero
RCVS reversible vasoconstriction syndrome
PRES posterior reversible leukoencephelopathy
Cerebral Amyloid Angiopathy
Intravascular lymphoma and other malignancies
Never treat a primary CNS vasculitis without a
positive brain biopsy
 Image guided biopsy is key
Frequently misdiagnosed
as vasculitis
 Vasculitis = smoldering
Presents with
thunderclap HA
 Initial w/u is often
 Patients re-present with
 Can progress to ischemic
Most common trigger at
Ducros et al., Brain 2007
Does not respond to steroids
 Data suggests patients treated with steroids do worse
Treated by removing the trigger
 Calcium Channel Blockers
 Magnesium
MRA should normalize by 3 months
Re-introduction of the offending agent can cause
recurrent RCVS
 Continuum?
 RCVS <-> Migraine <-> PRES (posterior reversible
encephalopathy syndrome)
Prognosis is good for young stroke survivors
 Better recovery
 Less recurrent stroke
▪ Identifying the cause is key
Vascular risk factor associated stroke is on the
rise in the young
 Preventative medicine

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