Young Stroke Survivors

1. Richard Leigh, M.D. Johns Hopkins University School of Medicine Young Stroke Survivors

2. Stroke in the Young • 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 • Cryptogenic

3. Stroke Worldwide • 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

4. Differential Diagnosis • Hart & Miller, Stroke, 1983

5. Differential Diagnosis • Hart & Miller, Stroke, 1983

6. Differential Diagnosis • Hart & Miller, Stroke, 1983

7. Prognosis • 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

8. Overview – Common Causes • Dissection • Cryptogenic with PFO • Reversible vasoconstriction syndrome (RCVS) • Not vasculitis!

9. Dissection • 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

10. Dissection - Diagnosis • Can be detected with CT angiography and MR angiography • Conventional angiography is the gold standard

11. Dissection - Diagnosis • Angiography allows for detection of FMD in other vessels • Renal arteries can also be affected

12. Dissection - Prognosis • 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

13. Cryptogenic Stroke with PFO • 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.

14. Cryptogenic Stroke with PFO • 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

15. Vasculitis (Angiitis) • Primariy CNS Vasculitis? • No! its almost never vasculitis • Systemic rheumatologic diseases should be ruled out • Vasculitismimicks • 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

16. RCVS - Reversible Vasoconstriction Syndrome • 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

17. RCVS - Reversible Vasoconstriction Syndrome

18. RCVS triggers Most common trigger at Hopkins: SSRI Ducros et al., Brain 2007

19. RCVS - Reversible Vasoconstriction Syndrome • 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)

20. Conclusions • 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