Skip to main content

Cerebral cavernous malformations and intractable epilepsy: the limited usefulness of current literature

Abstract

Background

Cerebral cavernous malformations (CCM) are known to be highly epileptogenic lesions. A number of studies on CCM surgery deal with CCM-associated seizures and/or epilepsy. In order to counsel patients with CCM-associated epilepsy, clear results from such studies would be highly useful. This study reviews the current literature with the aim to assess its usefulness for presurgical decision-making with emphasis on differentiating outcomes in different epilepsy types.

Methods

A systematic Medline search identified 27 studies between 1991 and 2009 through the keywords “cavernomas, cavernous, hemangioma, AND epilepsy, AND surgery”. They were analysed with regard to clarity of definition of epilepsy subtypes, precision of definition of drug-resistant epilepsy, information on surgical procedure and presurgical workup, seizure outcome and length of follow-up.

Results

Twenty studies included only surgically treated patients. Three types of epilepsy were defined: drug-resistant epilepsy, epilepsy or single/sporadic seizures. In 12 of 27 studies, at least one of these categories remained unclear. The classic definition of drug-resistant epilepsy was not used in the vast majority of studies, with many groups using their own definition. In 30%, the surgical procedure was not described precisely, although 52% of studies used a differentiated preoperative evaluation. Seizure outcome was described using a widely accepted classification in only 48% of series, and in over half of the studies outcome results contained cases with insufficient length of follow-up.

Conclusions

A large proportion of recent studies on surgery for CCM-associated epilepsy are not using criteria and definitions for the classification of epilepsy and outcome that are commonly used by epileptologists or epilepsy surgeons. This results in the limited usefulness of a large part of the literature for the purpose of preoperative counselling a patient with CCM-associated epilepsy.

This is a preview of subscription content, access via your institution.

References

  1. Acciarri N, Giulioni M, Padovani R, Galassi E, Gaist G (1995) Surgical management of cerebral cavernous angiomas causing epilepsy. J Neurosurg Sci 39:13–20

    CAS  PubMed  Google Scholar 

  2. Awad I, Jabbour P (2006) Cerebral cavernous malformations and epilepsy. Neurosurg Focus 21:e7

    Article  PubMed  Google Scholar 

  3. Baumann CR, Acciarri N, Bertalanffy H, Devinsky O, Elger CE, Lo Russo G, Cossu M, Sure U, Singh A, Stefan H, Hammen T, Georgiadis D, Baumgartner RW, Andermann F, Siegel AM (2007) Seizure outcome after resection of supratentorial cavernous malformations: a study of 168 patients. Epilepsia 48:559–563

    Article  PubMed  Google Scholar 

  4. Baumann CR, Schuknecht B, Lo Russo G, Cossu M, Citterio A, Andermann F, Siegel AM (2006) Seizure outcome after resection of cavernous malformations is better when surrounding hemosiderin-stained brain also is removed. Epilepsia 47:563–566

    Article  PubMed  Google Scholar 

  5. Bernotas G, Rastenyte D, Deltuva V, Matukevicius A, Jaskeviciene V, Tamasauskas A (2009) Cavernous angiomas: an uncontrolled clinical study of 87 surgically treated patients. Medicina (Kaunas) 45:21–28

    Google Scholar 

  6. Cappabianca P, Alfieri A, Maiuri F, Mariniello G, Cirillo S, de Divitiis E (1997) Supratentorial cavernous malformations and epilepsy: seizure outcome after lesionectomy on a series of 35 patients. Clin Neurol Neurosurg 99:179–183

    CAS  Article  PubMed  Google Scholar 

  7. Casazza M, Broggi G, Franzini A, Avanzini G, Spreafico R, Bracchi M, Valentini MC (1996) Supratentorial cavernous angiomas and epileptic seizures: preoperative course and postoperative outcome. Neurosurgery 39:26–32, discussion 32–24

    CAS  Article  PubMed  Google Scholar 

  8. Chang EF, Gabriel RA, Potts MB, Garcia PA, Barbaro NM, Lawton MT (2009) Seizure characteristics and control after microsurgical resection of supratentorial cerebral cavernous malformations. Neurosurgery 65:31–37, discussion 37–38

    Article  PubMed  Google Scholar 

  9. Churchyard A, Khangure M, Grainger K (1992) Cerebral cavernous angioma: a potentially benign condition? Successful treatment in 16 cases. J Neurol Neurosurg Psychiatry 55:1040–1045

    CAS  Article  PubMed  Google Scholar 

  10. Clusmann H (2008) Predictors, procedures, and perspective for temporal lobe epilepsy surgery. Semin Ultrasound CT MR 29:60–70

    Article  PubMed  Google Scholar 

  11. Cohen DS, Zubay GP, Goodman RR (1995) Seizure outcome after lesionectomy for cavernous malformations. J Neurosurg 83:237–242

    CAS  Article  PubMed  Google Scholar 

  12. D'Angelo VA, De Bonis C, Amoroso R, Cali A, D'Agruma L, Guarnieri V, Muscarella LA, Zelante L, Bisceglia M, Scarabino T, Catapano D (2006) Supratentorial cerebral cavernous malformations: clinical, surgical, and genetic involvement. Neurosurg Focus 21:e9

    Article  PubMed  Google Scholar 

  13. Del Curling O, Jr KDL, Jr EAD, Craven TE (1991) An analysis of the natural history of cavernous angiomas. J Neurosurg 75:702–708

    Article  PubMed  Google Scholar 

  14. Dodick DW, Cascino GD, Meyer FB (1994) Vascular malformations and intractable epilepsy: outcome after surgical treatment. Mayo Clin Proc 69:741–745

    CAS  PubMed  Google Scholar 

  15. Engel JJ, Van Ness PC, Rasmussen TB, Ojemann LM (1987) Outcome with respect to epileptic seizures. In: Engel J (ed) Surgical treatment of the epilepsies. Raven, New York, pp 553–571

    Google Scholar 

  16. Ferroli P, Casazza M, Marras C, Mendola C, Franzini A, Broggi G (2006) Cerebral cavernomas and seizures: a retrospective study on 163 patients who underwent pure lesionectomy. Neurol Sci 26:390–394

    CAS  Article  PubMed  Google Scholar 

  17. Folkersma H, Mooij JJ (2001) Follow-up of 13 patients with surgical treatment of cerebral cavernous malformations: effect on epilepsy and patient disability. Clin Neurol Neurosurg 103:67–71

    CAS  Article  PubMed  Google Scholar 

  18. Giulioni M, Acciarri N, Padovani R, Galassi E (1995) Results of surgery in children with cerebral cavernous angiomas causing epilepsy. Br J Neurosurg 9:135–141

    CAS  Article  PubMed  Google Scholar 

  19. Hammen T, Romstock J, Dorfler A, Kerling F, Buchfelder M, Stefan H (2007) Prediction of postoperative outcome with special respect to removal of hemosiderin fringe: a study in patients with cavernous haemangiomas associated with symptomatic epilepsy. Seizure 16:248–253

    CAS  Article  PubMed  Google Scholar 

  20. Hsu PW, Chang CN, Tseng CK, Wei KC, Wang CC, Chuang CC, Huang YC (2007) Treatment of epileptogenic cavernomas: surgery versus radiosurgery. Cerebrovasc Dis 24:116–120, discussion 121

    Article  PubMed  Google Scholar 

  21. Jehi L (2008) Mesial temporal lobectomy: post-surgical seizure frequency. In: Lüders H (ed) Textbook of epilepsy surgery. Informa Healthcare, London, p 1228

    Google Scholar 

  22. Kondziolka D, Lunsford LD, Kestle JR (1995) The natural history of cerebral cavernous malformations. J Neurosurg 83:820–824

    CAS  Article  PubMed  Google Scholar 

  23. Kraemer DL, Awad IA (1994) Vascular malformations and epilepsy: clinical considerations and basic mechanisms. Epilepsia 35(Suppl 6):S30–S43

    Article  PubMed  Google Scholar 

  24. Kraemer DL, Griebel ML, Lee N, Friedman AH, Radtke RA (1998) Surgical outcome in patients with epilepsy with occult vascular malformations treated with lesionectomy. Epilepsia 39:600–607

    CAS  Article  PubMed  Google Scholar 

  25. Menzler K, Chen X, Thiel P, Iwinska-Zelder J, Miller D, Reuss A, Hamer HM, Reis J, Pagenstecher A, Knake S, Bertalanffy H, Rosenow F, Sure U (2010) Epileptogenicity of cavernomas depends on (archi-) cortical localization. Neurosurgery 67:918–924

    Article  PubMed  Google Scholar 

  26. Moran NF, Fish DR, Kitchen N, Shorvon S, Kendall BE, Stevens JM (1999) Supratentorial cavernous haemangiomas and epilepsy: a review of the literature and case series. J Neurol Neurosurg Psychiatry 66:561–568

    CAS  Article  PubMed  Google Scholar 

  27. Moriarity JL, Clatterbuck RE, Rigamonti D (1999) The natural history of cavernous malformations. Neurosurg Clin N Am 10:411–417

    CAS  PubMed  Google Scholar 

  28. Noto S, Fujii M, Akimura T, Imoto H, Nomura S, Kajiwara K, Kato S, Fujisawa H, Suzuki M (2005) Management of patients with cavernous angiomas presenting epileptic seizures. Surg Neurol 64:495–498, discussion 498–499

    Article  PubMed  Google Scholar 

  29. Ojemann RG, Ogilvy CS (1999) Microsurgical treatment of supratentorial cavernous malformations. Neurosurg Clin N Am 10:433–440

    CAS  PubMed  Google Scholar 

  30. Robinson JR, Awad IA, Little JR (1991) Natural history of the cavernous angioma. J Neurosurg 75:709–714

    CAS  Article  PubMed  Google Scholar 

  31. Robinson JR Jr, Awad IA, Masaryk TJ, Estes ML (1993) Pathological heterogeneity of angiographically occult vascular malformations of the brain. Neurosurgery 33:547–554, discussion 554–545

    Article  PubMed  Google Scholar 

  32. Rocamora R, Mader I, Zentner J, Schulze-Bonhage A (2009) Epilepsy surgery in patients with multiple cerebral cavernous malformations. Seizure 18:241–245

    CAS  Article  PubMed  Google Scholar 

  33. Salanova V, Markand ON, Worth R (1994) Clinical characteristics and predictive factors in 98 patients with complex partial seizures treated with temporal resection. Arch Neurol 51:1008–1013

    CAS  PubMed  Google Scholar 

  34. Schramm J, Clusmann H (2008) The surgery of epilepsy. Neurosurgery 62(Suppl 2):463–481, discussion 481

    PubMed  Google Scholar 

  35. Shih YH, Pan DH (2005) Management of supratentorial cavernous malformations: craniotomy versus gammaknife radiosurgery. Clin Neurol Neurosurg 107:108–112

    Article  PubMed  Google Scholar 

  36. Stavrou I, Baumgartner C, Frischer JM, Trattnig S, Knosp E (2008) Long-term seizure control after resection of supratentorial cavernomas: a retrospective single-center study in 53 patients. Neurosurgery 63:888–896, discussion 897

    Article  PubMed  Google Scholar 

  37. Tymianski M, Tator CH (1996) Normal and abnormal calcium homeostasis in neurons: a basis for the pathophysiology of traumatic and ischemic central nervous system injury. Neurosurgery 38:1176–1195

    CAS  Article  PubMed  Google Scholar 

  38. Van Gompel JJ, Rubio J, Cascino GD, Worrell GA, Meyer FB (2009) Electrocorticography-guided resection of temporal cavernoma: is electrocorticography warranted and does it alter the surgical approach? J Neurosurg 110:1179–1185

    Article  PubMed  Google Scholar 

  39. Volterra A, Trotti D, Tromba C, Floridi S, Racagni G (1994) Glutamate uptake inhibition by oxygen free radicals in rat cortical astrocytes. J Neurosci 14:2924–2932

    CAS  PubMed  Google Scholar 

  40. Wieser HG, Blume WT, Fish D, Goldensohn E, Hufnagel A, King D, Sperling MR, Luders H, Pedley TA (2001) ILAE Commission report. Proposal for a new classification of outcome with respect to epileptic seizures following epilepsy surgery. Epilepsia 42:282–286

    CAS  Article  PubMed  Google Scholar 

  41. Williamson A, Patrylo PR, Lee S, Spencer DD (2003) Physiology of human cortical neurons adjacent to cavernous malformations and tumors. Epilepsia 44:1413–1419

    Article  PubMed  Google Scholar 

  42. Yeon JY, Kim JS, Choi SJ, Seo DW, Hong SB, Hong SC (2009) Supratentorial cavernous angiomas presenting with seizures: surgical outcomes in 60 consecutive patients. Seizure 18:14–20

    Article  PubMed  Google Scholar 

  43. Zevgaridis D, van Velthoven V, Ebeling U, Reulen HJ (1996) Seizure control following surgery in supratentorial cavernous malformations: a retrospective study in 77 patients. Acta Neurochir (Wien) 138:672–677

    CAS  Article  Google Scholar 

Download references

Conflicts of interest

None.

Author information

Affiliations

Authors

Corresponding author

Correspondence to C. von der Brelie.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

von der Brelie, C., Schramm, J. Cerebral cavernous malformations and intractable epilepsy: the limited usefulness of current literature. Acta Neurochir 153, 249–259 (2011). https://doi.org/10.1007/s00701-010-0915-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-010-0915-z

Keywords

  • Cerebral cavernous malformation
  • Drug-resistant epilepsy
  • Epilepsy surgery