Semin Neurol 2017; 37(03): 339-350
DOI: 10.1055/s-0037-1603914
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Cerebral Arteriopathies, Venous Thrombosis, and Migraine

Erica C. S. Camargo
1   Stroke Service, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
2   Department of Neurology, Harvard Medical School, Boston, Massachusetts
,
Aneesh B. Singhal
1   Stroke Service, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
2   Department of Neurology, Harvard Medical School, Boston, Massachusetts
› Author Affiliations
Further Information

Publication History

Publication Date:
31 July 2017 (online)

Abstract

In this article, the authors review stroke preventive strategies in a diverse group of conditions, namely the nonatherosclerotic cerebral arteriopathies, migraine-associated or migraine-induced stroke, and cerebral venous sinus thrombosis. Although these conditions are less common causes of stroke in the aggregate, they are frequent causes of stroke in teenagers and young adults. Aside from posing unique diagnostic challenges, their management is limited by the absence of randomized clinical trials or high-level evidence that is specific to these conditions. Therapeutic decision-making is largely based on expert opinion, clinical experience, and retrospective studies; it is often empiric. It remains uncertain whether otherwise routine secondary stroke-preventive strategies, such as long-term antiplatelet treatment, lipid-lowering medications, and antihypertensive agents, are applicable to patients with stroke from a cerebral arteriopathy or migraine-associated stroke.

 
  • References

  • 1 George MG, Tong X, Kuklina EV, Labarthe DR. Trends in stroke hospitalizations and associated risk factors among children and young adults, 1995-2008. Ann Neurol 2011; 70 (05) 713-721
  • 2 Kissela BM, Khoury JC, Alwell K. , et al. Age at stroke: temporal trends in stroke incidence in a large, biracial population. Neurology 2012; 79 (17) 1781-1787
  • 3 Rutten-Jacobs LC, Maaijwee NA, Arntz RM. , et al. Long-term risk of recurrent vascular events after young stroke: The FUTURE study. Ann Neurol 2013; 74 (04) 592-601
  • 4 Tibæk M, Dehlendorff C, Jørgensen HS, Forchhammer HB, Johnsen SP, Kammersgaard LP. Increasing incidence of hospitalization for stroke and transient ischemic attack in young adults: a registry-based study. J Am Heart Assoc 2016; 5 (05) 5
  • 5 Rutten-Jacobs LC, Arntz RM, Maaijwee NA. , et al. Long-term mortality after stroke among adults aged 18 to 50 years. JAMA 2013; 309 (11) 1136-1144
  • 6 Ji R, Schwamm LH, Pervez MA, Singhal AB. Ischemic stroke and transient ischemic attack in young adults: risk factors, diagnostic yield, neuroimaging, and thrombolysis. JAMA Neurol 2013; 70 (01) 51-57
  • 7 Putaala J, Haapaniemi E, Kaste M, Tatlisumak T. How does number of risk factors affect prognosis in young patients with ischemic stroke?. Stroke 2012; 43 (02) 356-361
  • 8 Rutten-Jacobs LC, Maaijwee NA, Arntz RM. , et al. Risk factors and prognosis of young stroke. The FUTURE study: a prospective cohort study. Study rationale and protocol. BMC Neurol 2011; 11: 109
  • 9 von Sarnowski B, Schminke U, Tatlisumak T. , et al; sifap1 investigators. Prevalence of stenoses and occlusions of brain-supplying arteries in young stroke patients. Neurology 2013; 80 (14) 1287-1294
  • 10 Putaala J, Metso AJ, Metso TM. , et al. Analysis of 1008 consecutive patients aged 15 to 49 with first-ever ischemic stroke: the Helsinki young stroke registry. Stroke 2009; 40 (04) 1195-1203
  • 11 Yesilot Barlas N, Putaala J, Waje-Andreassen U. , et al. Etiology of first-ever ischaemic stroke in European young adults: the 15 cities young stroke study. Eur J Neurol 2013; 20 (11) 1431-1439
  • 12 Mackay MT, Wiznitzer M, Benedict SL, Lee KJ, Deveber GA, Ganesan V. ; International Pediatric Stroke Study Group. Arterial ischemic stroke risk factors: the International Pediatric Stroke Study. Ann Neurol 2011; 69 (01) 130-140
  • 13 Debette S, Compter A, Labeyrie MA. , et al. Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection. Lancet Neurol 2015; 14 (06) 640-654
  • 14 Lyrer PA, Brandt T, Metso TM. , et al; Cervical Artery Dissection and Ischemic Stroke Patients (CADISP) Study Group. Clinical import of Horner syndrome in internal carotid and vertebral artery dissection. Neurology 2014; 82 (18) 1653-1659
  • 15 Schwedt TJ, Matharu MS, Dodick DW. Thunderclap headache. Lancet Neurol 2006; 5 (07) 621-631
  • 16 Georgiadis D, Lanczik O, Schwab S. , et al. IV thrombolysis in patients with acute stroke due to spontaneous carotid dissection. Neurology 2005; 64 (09) 1612-1614
  • 17 Engelter ST, Rutgers MP, Hatz F. , et al. Intravenous thrombolysis in stroke attributable to cervical artery dissection. Stroke 2009; 40 (12) 3772-3776
  • 18 Qureshi AI, Chaudhry SA, Hassan AE. , et al. Thrombolytic treatment of patients with acute ischemic stroke related to underlying arterial dissection in the United States. Arch Neurol 2011; 68 (12) 1536-1542
  • 19 Engelter ST, Dallongeville J, Kloss M. , et al; Cervical Artery Dissection and Ischaemic Stroke Patients-Study Group. Thrombolysis in cervical artery dissection--data from the Cervical Artery Dissection and Ischaemic Stroke Patients (CADISP) database. Eur J Neurol 2012; 19 (09) 1199-1206
  • 20 Ohta H, Natarajan SK, Hauck EF. , et al. Endovascular stent therapy for extracranial and intracranial carotid artery dissection: single-center experience. J Neurosurg 2011; 115 (01) 91-100
  • 21 Lyrer P, Engelter S. Antithrombotic drugs for carotid artery dissection. Stroke 2004; 35 (02) 613-614
  • 22 Georgiadis D, Arnold M, von Buedingen HC. , et al. Aspirin vs anticoagulation in carotid artery dissection: a study of 298 patients. Neurology 2009; 72 (21) 1810-1815
  • 23 Markus HS, Hayter E, Levi C, Feldman A, Venables G, Norris J. ; CADISS trial investigators. Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial. Lancet Neurol 2015; 14 (04) 361-367
  • 24 Debette S, Metso T, Pezzini A. , et al; Cervical Artery Dissection and Ischemic Stroke Patients (CADISP) Group. Association of vascular risk factors with cervical artery dissection and ischemic stroke in young adults. Circulation 2011; 123 (14) 1537-1544
  • 25 Biller J, Sacco RL, Albuquerque FC. , et al; American Heart Association Stroke Council. Cervical arterial dissections and association with cervical manipulative therapy: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2014; 45 (10) 3155-3174
  • 26 Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol 2006; 5 (06) 501-512
  • 27 Earley CJ, Kittner SJ, Feeser BR. , et al. Stroke in children and sickle-cell disease: Baltimore-Washington Cooperative Young Stroke Study. Neurology 1998; 51 (01) 169-176
  • 28 Ohene-Frempong K, Weiner SJ, Sleeper LA. , et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998; 91 (01) 288-294
  • 29 Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med 1978; 65 (03) 461-471
  • 30 Moser FG, Miller ST, Bello JA. , et al. The spectrum of brain MR abnormalities in sickle-cell disease: a report from the Cooperative Study of Sickle Cell Disease. AJNR Am J Neuroradiol 1996; 17 (05) 965-972
  • 31 Steen RG, Emudianughe T, Hankins GM. , et al. Brain imaging findings in pediatric patients with sickle cell disease. Radiology 2003; 228 (01) 216-225
  • 32 Pegelow CH, Macklin EA, Moser FG. , et al. Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood 2002; 99 (08) 3014-3018
  • 33 Adams R, McKie V, Nichols F. , et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 1992; 326 (09) 605-610
  • 34 Kossorotoff M, Brousse V, Grevent D. , et al. Cerebral haemorrhagic risk in children with sickle-cell disease. Dev Med Child Neurol 2015; 57 (02) 187-193
  • 35 Hariman LM, Griffith ER, Hurtig AL, Keehn MT. Functional outcomes of children with sickle-cell disease affected by stroke. Arch Phys Med Rehabil 1991; 72 (07) 498-502
  • 36 Roach ES, Golomb MR, Adams R. , et al; American Heart Association Stroke Council; Council on Cardiovascular Disease in the Young. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008; 39 (09) 2644-2691
  • 37 Adams RJ, McKie VC, Hsu L. , et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 1998; 339 (01) 5-11
  • 38 Armstrong-Wells J, Grimes B, Sidney S. , et al. Utilization of TCD screening for primary stroke prevention in children with sickle cell disease. Neurology 2009; 72 (15) 1316-1321
  • 39 Goldstein LB, Bushnell CD, Adams RJ. , et al; American Heart Association Stroke Council; Council on Cardiovascular Nursing; Council on Epidemiology and Prevention; Council for High Blood Pressure Research; Council on Peripheral Vascular Disease, and Interdisciplinary Council on Quality of Care and Outcomes Research. Guidelines for the primary prevention of stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011; 42 (02) 517-584
  • 40 Russell MO, Goldberg HI, Hodson A. , et al. Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood 1984; 63 (01) 162-169
  • 41 Kelly S, Quirolo K, Marsh A, Neumayr L, Garcia A, Custer B. Erythrocytapheresis for chronic transfusion therapy in sickle cell disease: survey of current practices and review of the literature. Transfusion 2016; 56 (11) 2877-2888
  • 42 Adams RJ, Brambilla D. ; Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med 2005; 353 (26) 2769-2778
  • 43 Abboud MR, Yim E, Musallam KM, Adams RJ. ; STOP II Study Investigators. Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell disease: data from STOP II. Blood 2011; 118 (04) 894-898
  • 44 Ware RE, Helms RW. ; SWiTCH Investigators. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH). Blood 2012; 119 (17) 3925-3932
  • 45 Ware RE, Davis BR, Schultz WH. , et al. Hydroxycarbamide versus Chronic Transfusion for Maintenance of Transcranial Doppler Flow Velocities in Children With Sickle Cell Anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. Lancet 2016; 387 (10019): 661-670
  • 46 Walters MC, Patience M, Leisenring W. , et al. Bone marrow transplantation for sickle cell disease. N Engl J Med 1996; 335 (06) 369-376
  • 47 Hall EM, Leonard J, Smith JL. , et al. Reduction in overt and silent stroke recurrence rate following cerebral revascularization surgery in children with sickle cell disease and severe cerebral vasculopathy. Pediatr Blood Cancer 2016; 63 (08) 1431-1437
  • 48 Singhal AB. Cerebral vasoconstriction syndromes. Top Stroke Rehabil 2004; 11 (02) 1-6
  • 49 Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007; 146 (01) 34-44
  • 50 Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007; 130 (Pt 12): 3091-3101
  • 51 Williams TL, Lukovits TG, Harris BT, Harker Rhodes C. A fatal case of postpartum cerebral angiopathy with literature review. Arch Gynecol Obstet 2007; 275 (01) 67-77
  • 52 Singhal AB, Topcuoglu MA, Fok JW. , et al. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol 2016; 79 (06) 882-894
  • 53 Singhal AB, Hajj-Ali RA, Topcuoglu MA, Fok J, Bena J, Yang D, Calabrese LH. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011; 68 (08) 1005-1012
  • 54 Chen SP, Fuh JL, Wang SJ. , et al. Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol 2010; 67 (05) 648-656
  • 55 Singhal AB. Postpartum angiopathy with reversible posterior leukoencephalopathy. Arch Neurol 2004; 61 (03) 411-416
  • 56 Topcuoglu MA, Kursun O, Singhal AB. Coexisting vascular lesions in reversible cerebral vasoconstriction syndrome. Cephalalgia 2017; 37 (01) 29-35
  • 57 Topcuoglu MA, McKee KE, Singhal AB. Gender and hormonal influences in reversible cerebral vasoconstriction syndrome. European Stroke Journal 2016; 1: 199-204
  • 58 Soo Y, Singhal AB, Leung T. , et al. Reversible cerebral vasoconstriction syndrome with posterior leucoencephalopathy after oral contraceptive pills. Cephalalgia 2010; 30 (01) 42-45
  • 59 Chatterjee N, Domoto-Reilly K, Fecci PE, Schwamm LH, Singhal AB. Licorice-associated reversible cerebral vasoconstriction with PRES. Neurology 2010; 75 (21) 1939-1941
  • 60 Topcuoglu MA, Singhal AB. Hemorrhagic reversible cerebral vasoconstriction syndrome: features and mechanisms. Stroke 2016; 47 (07) 1742-1747
  • 61 Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol 2011; 10 (06) 561-572
  • 62 Muehlschlegel S, Kursun O, Topcuoglu MA, Fok J, Singhal AB. Differentiating reversible cerebral vasoconstriction syndrome with subarachnoid hemorrhage from other causes of subarachnoid hemorrhage. JAMA Neurol 2013; 70 (10) 1254-1260
  • 63 Singhal AB, Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology 2017; 88 (03) 228-236
  • 64 Singhal AB, Caviness VS, Begleiter AF, Mark EJ, Rordorf G, Koroshetz WJ. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology 2002; 58 (01) 130-133
  • 65 Singhal AB. Reversible cerebral vasoconstriction syndromes: what the cardiologist should know. Curr Treat Options Cardiovasc Med 2014; 16 (03) 290
  • 66 Katz BS, Fugate JE, Ameriso SF. , et al. Clinical worsening in reversible cerebral vasoconstriction syndrome. JAMA Neurol 2014; 71 (01) 68-73
  • 67 Singhal AB, Kimberly WT, Schaefer PW, Hedley-Whyte ET. Case records of the Massachusetts General Hospital. Case 8-2009. A 36-year-old woman with headache, hypertension, and seizure 2 weeks post partum. N Engl J Med 2009; 360 (11) 1126-1137
  • 68 Chen SP, Fuh JL, Lirng JF, Wang YF, Wang SJ. Recurrence of reversible cerebral vasoconstriction syndrome: a long-term follow-up study. Neurology 2015; 84 (15) 1552-1558
  • 69 John S, Singhal AB, Calabrese L. , et al. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Cephalalgia 2016; 36 (04) 387-394
  • 70 Salvarani C, Brown Jr RD, Calamia KT. , et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62 (05) 442-451
  • 71 de Boysson H, Zuber M, Naggara O. , et al; French Vasculitis Study Group and the French NeuroVascular Society. Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol 2014; 66 (05) 1315-1326
  • 72 Miller DV, Salvarani C, Hunder GG. , et al. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 2009; 33 (01) 35-43
  • 73 Molloy ES, Singhal AB, Calabrese LH. Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system. Ann Rheum Dis 2008; 67 (12) 1732-1735
  • 74 Scolding NJ, Joseph F, Kirby PA. , et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005; 128 (Pt 3): 500-515
  • 75 Topcuoglu MA, Jha RM, George J, Frosch MP, Singhal AB. Hemorrhagic primary CNS angiitis and vasoconstrictive drug exposure. Neurol Clin Pract 2017; 7 (01) 26-34
  • 76 Hutchinson C, Elbers J, Halliday W. , et al. Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol 2010; 9 (11) 1078-1084
  • 77 Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet 2008; 372 (9634): 234-245
  • 78 Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology 1988; 38 (03) 352-359
  • 79 Villiger PM, Adler S, Kuchen S. , et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet 2016; 387 (10031): 1921-1927
  • 80 Mahr AD, Jover JA, Spiera RF. , et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007; 56 (08) 2789-2797
  • 81 Susac JO. Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44 (04) 591-593
  • 82 Mateen FJ, Zubkov AY, Muralidharan R. , et al. Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol 2012; 19 (06) 800-811
  • 83 Bousser M, Tournier-Lasserve E. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: from stroke to vessel wall physiology. J Neurol Neurosurg Psychiatry 2001; 70 (03) 285-287
  • 84 Chabriat H, Pappata S, Ostergaard L. , et al. Cerebral hemodynamics in CADASIL before and after acetazolamide challenge assessed with MRI bolus tracking. Stroke 2000; 31 (08) 1904-1912
  • 85 Dichgans M, Markus HS, Salloway S. , et al. Donepezil in patients with subcortical vascular cognitive impairment: a randomised double-blind trial in CADASIL. Lancet Neurol 2008; 7 (04) 310-318
  • 86 Kolodny E, Fellgiebel A, Hilz MJ. , et al. Cerebrovascular involvement in Fabry disease: current status of knowledge. Stroke 2015; 46 (01) 302-313
  • 87 Hallemeier CL, Rich KM, Grubb Jr RL. , et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 2006; 37 (06) 1490-1496
  • 88 Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007; 6 (02) 162-170
  • 89 Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F. ; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004; 35 (03) 664-670
  • 90 Einhäupl KM, Villringer A, Meister W. , et al. Heparin treatment in sinus venous thrombosis. Lancet 1991; 338 (8767): 597-600
  • 91 de Bruijn SF, Stam J. Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis. Stroke 1999; 30 (03) 484-488
  • 92 Ferro JM, Crassard I, Coutinho JM. , et al; Second International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT 2) Investigators. Decompressive surgery in cerebrovenous thrombosis: a multicenter registry and a systematic review of individual patient data. Stroke 2011; 42 (10) 2825-2831
  • 93 Kurth T, Winter AC, Eliassen AH. , et al. Migraine and risk of cardiovascular disease in women: prospective cohort study. BMJ 2016; 353: i2610
  • 94 Kurth T, Chabriat H, Bousser MG. Migraine and stroke: a complex association with clinical implications. Lancet Neurol 2012; 11 (01) 92-100
  • 95 Welch KM. Relationship of stroke and migraine. Neurology 1994; 44 (10) (Suppl. 07) S33-S36