10/19/12. Uncommon Causes of Stroke. José Biller, MD, FACP, FAAN, FAHA Disclosures. Dr. Biller has no disclosures to report

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1 10/19/12 Uncommon Causes of Stroke José Biller, MD, FACP, FAAN, FAHA Loyola University Chicago Stritch School of Medicine Chicago, IL José Biller, MD, FACP, FAAN, FAHA Disclosures Dr. Biller has no disclosures to report 1

2 10/19/12 Learning Objective Define the uncommon causes of stroke and their consequences Ischemic Stoke Ischemic stroke 20% 20-25% Large-artery Cardiac disease atherosclerosis Atrial fibrillation Extracranial Intracranial LVT Valvular heart disease Other 20-25% 5% Small-artery disease Penetrating artery occlusion due to: Lipohyalinosis Microatheroma 795,000 strokes annually 2/3 first events 1/3 recurrent attacks Other causes CA dissections Non-atherosclerotic vasculopathies Migrainous stroke Drug abuse Hypercoagulability 25-30% Cryptogenic stroke Silent AF PFO in some cases? Fabry disease 80-87% ischemic strokes 8-12% mortality within 30 days 14% of all ischemic strokes in young adults and adolescents Non-Atherosclerotic Cerebral Vasculopathies Cervicocephalic arterial Traumatic cerebrovascular Radiation-induced dissections Fibromuscular dysplasia Moyamoya disease Sickle cell vasculopathy Vasculitis Post-varicella angiopathy Focal cerebral arteriopathy in children Migrainous infarction Ergotism disease arteriopathy Tumoral encasement of cervicocephalic vessels Hypoplasia and agenesis of cervicocephalic vessel Congenital arterial fenestration Coils, kinks and loops Biller J. J Stroke Cerebrovasc Dis. 1997;6(4): PMID:

3 10/19/12 53/y/o man with left jaw discomfort and right hand clumsiness DWI FLAIR DWI=diffusion-weighted imaging FLAIR=fluid-attenuated inversion recovery (FLAIR) 53 y/o man MRA MRA=Magnetic resonance angiography 53 y/o man Left ICA ICA=Internal Carotid Artery CTA CTA=Carotid Artery Stenosis 3

4 10/19/12 Cervicocephalic Arterial Dissection 2.5% of all strokes 5% - 22% of strokes in young patients Among leading causes of ischemic stroke in patients younger than 45 years Arnold M, et al. Int J Stroke. 2011;6(3): PMID: Cervicocephalic Arterial Dissections: Clinical Features Headache Neck pain Horner syndrome (no anhidrosis) Cerebral ischemia, retinal ischemia, brainstem/ cerebellar ischemia, spinal cord ischemia Cranial neuropathies (IX-XII, III, IV, VI, others) Pulsatile tinnitus Cervical root injury Biller J, et al. Arch Neurol. 1986;43(12): PMID: Cervicocephalic Arterial Dissections: Treatment Anticoagulants Antiplatelets UK survey: 50 % anticoagulants; 30 % antiplatelets; 20 % either Duration? Heightened risk of recurrence in patients with FMD, connective tissue disorder, family Hx of CAD Thrombolytics not to be withheld in patients with AIS due to extracranial ICA Surgery or endovascular treatment usually not recommended for extracranial dissections UK = United Kingdom; FMD = fibromuscular dysplasia; CAD = cervicocephalic arterial dissections; AIS = acute ischemic stroke; ICA = internal carotid artery Fusco MR, et al. Neurosurgery. 2011;68(1): PMID:

5 10/19/12 Moyamoya Disease Chronic progressive non-inflammatory occlusive intracranial vasculopathy of unknown etiology More frequent in Japan Reported in all ethnic groups Children and adults, especially women in the first or third decades of life Familial cases have been reported with autosomal dominant inheritance Janda PH, et al. J Am Osteopath Assoc. 2009;109(10): PMID: Other Conditions Neonatal anoxia Head trauma Basilar meningitis Post-infectious or postradiation vasculopathies TS complex, NF1, SW syndrome Phakomatosis pigmentovascularis type IIIB PXE Hypomelanosis of Ito Sickle cell anemia Β-thalassemia Fanconi anemia Hereditary spherocytosis Brain tumors PAN Marfan syndrome Turner syndrome NF1 = neurofibromatosis type 1; TS = Tuberous sclerosis; SW = Sturge Weber syndrome; PXE = pseudoxanthoma elasticum; PAN = polyarteritis nodosa; FMD = fibromuscular dysplasia; OCP = oral contraceptive pill Janda PH, et al. J Am Osteopath Assoc. 2009;109(10): PMID: Other Conditions Williams syndrome Arteriosclerosis Oral Contraceptives Fibromuscular dysplasia Cerebral dissecting Saccular aneurysms Sneddon syndrome Homocystinuria Type I glycogenosis Hirschsprung disease Alagille syndrome Hyperphosphatasia Graves disease Coarctation of the aorta Factor XII deficiency Renal artery stenosis Down syndrome Janda PH, et al. J Am Osteopath Assoc. 2009;109(10): PMID:

6 10/19/12 Sickle Cell Disease Stroke major complication (HbSS > HbSC) Ischemic strokes in children Hemorrhagic strokes in adults TCD screening every 6-12 months from age 2-18 years TCD may not be good screening for individuals older than 18 years HbSS = genotype of sickle cell disease; HbSC = genotype of sickle cell disease; TCD = transcranial Doppler Switzer JA, et al. Lancet Neurol. 2006;5(6): PMID: Sickle Cell Disease STOP trial (ages 2-16 years) Much lower incidence of brain infarctionm with long-term transfusion to maintain HbS < 30% When long-term transfusions were discontinued, TCD velocities returned to abnormal and a few children developed acute stroke HbS = abnormal form of hemoglobin; TCD = transcranial Doppler Switzer JA, et al. Lancet Neurol. 2006;5(6): PMID: Case Slides Image courtesy of Dr. Biller. 6

7 10/19/12 Case Slides Image courtesy of Dr. Biller. Case Slides Image courtesy of Dr. Biller. Clinical Connections Cervicocephalic arterial dissection is one of the leading causes of ischemic stroke in patients younger than 45 years old Stroke is a major complication in Sickle Cell Disease 7

8 10/19/12 Co-sponsored by Save the Date! 6th Annual Chair Summit September 26-28, 2013 Westin Tampa Harbour Island Tampa, Florida 8