Richard Leigh, M.D. Johns Hopkins University School of Medicine

 Generally stroke in a less than 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  Cryptogenic

 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

 Hart & Miller, Stroke, 1983

 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  A bad prognosis is associated with an atherosclerotic risk profile Varona et al., J Neurol, 2004

 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.  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 angiography  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 asymptomatic  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  Complications  Pseudo aneurysms

 PFO (patent foramen ovale)  20-25% of adults have a PFO  Some times associated with an ASA (atrial septal aneurysm)  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.  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 course  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

 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