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Richard Leigh, M.D.
Johns Hopkins University School of Medicine
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Generally stroke in a less than 40-45 years old
 Different from pediatric stroke
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Unique causes that are more common in the young
 Cervical Artery Dissection
 Hypercoagulable States
 Vasospasm
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Typical causes that are atypical in the young
 Hypertension
 Diabetes
 Hyperlipidemia
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Cryptogenic
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Appears to be trending toward younger
populations.
This trend appears to be larger in the
hemorrhagic stroke population.
Preferentially affecting lower socioeconomic
classes.
Attributable to modifiable risk factors:
hypertension, obesity and diabetes.
▪ Krishnamurthi et al., Lancet Global Health 2012
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Hart & Miller, Stroke, 1983
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Hart & Miller, Stroke, 1983
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Hart & Miller, Stroke, 1983
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Long-term prognosis
for stroke in the young
is better than the
elderly but higher
than the general
population
 Mostly in the first year
after stroke
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A bad prognosis is
associated with an
atherosclerotic risk
profile
Varona et al., J Neurol, 2004
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Dissection
Cryptogenic with PFO
Reversible vasoconstriction syndrome (RCVS)
 Not vasculitis!
Caused by separation of the arterial wall layers resulting in a false
lumen.
 A history of trauma is often but not always elicited.
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 Can be associated with major, minor or trivial trauma
 Can be spontaneous or cryptogenic
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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
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Often associated with headache/neck pain acutely and chronically
 Responds to migraine therapies
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Heparin or ASA are reasonable treatments
 With heparin only for 3-6 months then switch to ASA
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Can be detected with CT
angiography and MR
angiography
Conventional angiography
is the gold standard
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Angiography allows
for detection of FMD
in other vessels
Renal arteries can
also be affected
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Prognosis is good
 Many dissections are
asymptomatic
 Recurrent stroke after dissection
is rare with treatment
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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
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Complications
 Pseudo aneurysms
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PFO (patent foramen ovale)
 20-25% of adults have a PFO
 Some times associated with an ASA (atrial septal
aneurysm)
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PFO can serve as a source of paradoxical
embolism
 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.
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 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
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Primariy CNS Vasculitis?
 No! its almost never vasculitis
 Systemic rheumatologic diseases should be ruled out
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Vasculitis mimicks
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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
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Frequently misdiagnosed
as vasculitis
 Vasculitis = smoldering
course
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Presents with
thunderclap HA
 Initial w/u is often
negative
 Patients re-present with
ICH/SAH
 Can progress to ischemic
strokes
Most common trigger at
Hopkins:
SSRI
Ducros et al., Brain 2007
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Does not respond to steroids
 Data suggests patients treated with steroids do worse
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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?
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 RCVS <-> Migraine <-> PRES (posterior reversible
encephalopathy syndrome)
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Prognosis is good for young stroke survivors
 Better recovery
 Less recurrent stroke
▪ Identifying the cause is key
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Vascular risk factor associated stroke is on the
rise in the young
 Preventative medicine

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