• Horst Urbach
Part of the Medical Radiology book series (MEDRAD)


A vascular etiology of epileptic seizures is common in the fetal and perinatal period and again in adults. In adults, more than 50 % of newly diagnosed epilepsy cases are related to cerebrovascular diseases, but the exact underlying mechanism is often difficult to prove. This chapter summarizes different vascular lesions with characteristic MRI patterns.


Epileptic Seizure White Matter Lesion Moyamoya Disease Periventricular Leukomalacia Cavitary Lesion 
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  1. Aguglia U, Gambardella A, Breedveld GJ et al (2004) Suggestive evidence for linkage to chromosome 13qter for autosomal dominant type 1 porencephaly. Neurology 62(9):1613–1615PubMedCrossRefGoogle Scholar
  2. Alamowitch S, Plaisier E, Favrole P et al (2009) Cerebrovascular disease related to COL4A1 mutations in HANAC syndrome. Neurology 73(22):1873–1882PubMedCrossRefGoogle Scholar
  3. Arboix A, Comes E, Garcia-Eroles L et al (2003) Prognostic value of very early seizures for in-hospital mortality in atherothrombotic infarction. Eur Neurol 50:78–84PubMedCrossRefGoogle Scholar
  4. Barkovich AJ, Ali FA, Rowley HA, Bass N (1998) Imaging patterns of neonatal hypoglycemia. AJNR Am J Neuroradiol 19:523–528PubMedGoogle Scholar
  5. Behunova J, Zavadilikova E, Bozoglu TM et al (2010) Familial microhydranencephaly, a family that does not map to 16p13.13-p12.2: relationship with hereditary fetal brain degeneration and fetal brain disruption sequence. Clin Dysmorphol 19(3):107–118PubMedCrossRefGoogle Scholar
  6. Bladin CF, Alexandrov AV, Bellavance A et al (2000) Seizures after stroke: a prospective multicenter study. Arch Neurol 57:1617–1622PubMedCrossRefGoogle Scholar
  7. Breedveld G, de Coo IF, Lequin MH et al (2006) Novel mutations in three families confirm a major role of COL4A1 in hereditary porencephaly. J Med Genet 43(6):490–495PubMedCrossRefGoogle Scholar
  8. Burn J, Dennis M, Bamford J et al (1997) Epileptic seizures after a first stroke: the Oxfordshire community stroke project. BMJ 315:1582–1587PubMedCrossRefGoogle Scholar
  9. Chabriat H, Levy C, Taillia H et al (1998) Patterns of MRI lesions in CADASIL. Neurology 51(2):452–457PubMedCrossRefGoogle Scholar
  10. Chabriat H, Pappata S, Poupon C et al (1999) Clinical severity in CADASIL related to ultrastructural damage in white matter: in vivo study with diffusion tensor MRI. Stroke 30(12):2637–2643PubMedCrossRefGoogle Scholar
  11. Chabriat H, Joutel A, Dichgans M et al (2009) Cadasil. Lancet Neurol 8(7):643–653 (Review)Google Scholar
  12. Counsell SJ, Allsop JM, Harrison MC et al (2003) Diffusion-weighted imaging of the brain in preterm infants with focal and diffuse white matter abnormality. Pediatrics 112:1–7PubMedCrossRefGoogle Scholar
  13. Dichgans M, Mayer M, Uttner I et al (1998) The phenotypic spectrum of CADASIL: clinical findings in 102 cases. Ann Neurol 44:731–739PubMedCrossRefGoogle Scholar
  14. Friede R (1989) Developmental neuropathology. Springer-Verlag, BerlinGoogle Scholar
  15. Gould DB, Phalan FC, Breedveld GJ et al (2005) Mutations in Col4a1 cause perinatal cerebral hemorrhage and porencephaly. Science 308(5725):1167–1171PubMedCrossRefGoogle Scholar
  16. Gurses C, Gross DW, Andermann F et al (1999) Periventricular leukomalacia and epilepsy: incidence and seizure pattern. Neurology 52:341–345PubMedCrossRefGoogle Scholar
  17. Hauser WA, Annegers JF, Kurland LT (1993) Incidence of epilepsy and un-provoked seizures in Rochester, Minnesota: 1935–1984. Epilepsia 34:453–468PubMedCrossRefGoogle Scholar
  18. Ho SH, Kuzniecky RI, Gilliam F et al (1998) Congenital porencephaly: MR features and relationship to hippocampal sclerosis. AJNR Am J Neuroradiol 19:135–141PubMedGoogle Scholar
  19. Humphreys P, Deonandan R, Whiting S et al (2007) Factors associated with epilepsy in children with periventricular leukomalacia. J Child Neurol 22:598–605PubMedCrossRefGoogle Scholar
  20. Jung S, Schindler K, Findling O et al (2012) Adverse effect of early epileptic seizures in patients receiving endovascular therapy for acute stroke. Stroke 43:1584–1590PubMedCrossRefGoogle Scholar
  21. Kavaslar GN, Onengüt S, Derman O et al (2000) The novel genetic disorder microhydranencephaly maps to chromosome 16p13.3-12.1. Am J Hum Genet 66(5):1705–1709PubMedCrossRefGoogle Scholar
  22. Kleinloog R, Regli L, Rinkel GJ, Klijn CJ (2012) Regional differences in incidence and patient characteristics of moyamoya disease: a systematic review. J Neurol Neurosurg Psychiatry 83(5):531–536PubMedCrossRefGoogle Scholar
  23. Kudo T (1968) Spontaneous occlusion of the circle of Willis. A disease apparently confined to Japanese. Neurology 18(5):485–496PubMedCrossRefGoogle Scholar
  24. Loiseau J, Loiseau P, Duche B et al (1990) A survey of epileptic disorders in southwest France: seizures in elderly patients. Ann Neurol 27:232–237PubMedCrossRefGoogle Scholar
  25. Lutterman J, Scott M, Nass R, Geva T (1998) Moyamoya syndrome associated with congenital heart disease. Pediatrics 101:57–60PubMedCrossRefGoogle Scholar
  26. Myers RE (1989) Cerebral ischemia in the developing primate fetus. Biomed Biochim Acta 48:S137–S142PubMedGoogle Scholar
  27. Myint PK, Staufenberg EF, Sabanathan K (2006) Post-stroke seizure and post-stroke epilepsy. Postgrad Med J 82(971):568–572PubMedCrossRefGoogle Scholar
  28. Okroglic S, Widmann CN, Urbach H, Scheltens P, Heneka M (2013) Clinical symptoms, risk factors and cardiovascular medication in patients diagnosed with cerebral microangiopathy. PLoS ONE 8(2):e53455PubMedCrossRefGoogle Scholar
  29. Raju TN, Nelson KB, Ferriero D (2007) NICHD-NINDS perinatal stroke workshop participants. Ischemic perinatal stroke: summary of a workshop sponsored by the national institute of child health and human development and the national institute of neurological disorders and stroke. Pediatrics 120(3):609–616PubMedCrossRefGoogle Scholar
  30. Shinton RA, Gill JS, Melnick SC et al (1988) The frequency, characteristics and prognosis of epileptic seizures at the onset of stroke. J Neurol Neurosurg Psychiatry 51:273–276PubMedCrossRefGoogle Scholar
  31. Subirana A, Subirana M (1962) Malformations vasculaires du type de l’angiome arterial racemeux [in French]. Rev Neurol 107:545–550Google Scholar
  32. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299PubMedCrossRefGoogle Scholar
  33. Tournier-Lasserve F, Loutel A, Melki J et al (1993) Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy maps to chromosome 19q12. Nat Genet 3:256–259PubMedCrossRefGoogle Scholar
  34. Ulmer S, Moeller F, Brockmann MA et al (2005) Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly. Pediatrics 116(1):242–245PubMedCrossRefGoogle Scholar
  35. van der Knaap MS, Smit LM, Barkhof F et al (2006) Neonatal porencephaly and adult stroke related to mutations in collagen IV A1. Ann Neurol 59(3):504–511PubMedCrossRefGoogle Scholar
  36. Velioza R, Mourand I, Serafini A et al (2011) Focal epilepsy as first symptom in CADASIL. Seizure 20:502–504CrossRefGoogle Scholar
  37. Williams D, Patel C, Fallet-Bianco C et al (2010) Fowler syndrome—a clinical, radiological, and pathological study of 14 cases. Am J Med Genet A 152A(1):153–160PubMedCrossRefGoogle Scholar
  38. Yousry TA, Seelos K, Mayer M et al (1999) Characteristic MR lesion pattern and correlation of T1 and T2 lesion volume with neurologic and neuropsychological findings in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). AJNR Am J Neuroradiol 20:91–100PubMedGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Department of NeuroradiologyUniversity Hospital FreiburgFreiburgGermany

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