Cavernoma‐related epilepsy: Review and recommendations for management—Report of the Surgical Task Force of the ILAE Commission on Therapeutic Strategies

Cerebral cavernous malformations (CCMs) are well‐defined, mostly singular lesions present in 0.4–0.9% of the population. Epileptic seizures are the most frequent symptom in patients with CCMs and have a great impact on social function and quality of life. However, patients with CCM‐related epilepsy (CRE) who undergo surgical resection achieve postoperative seizure freedom in only about 75% of cases. This is frequently because insufficient efforts are made to adequately define and resect the epileptogenic zone. The Surgical Task Force of the Commission on Therapeutics of the International League Against Epilepsy (ILAE) and invited experts reviewed the pertinent literature on CRE. Definitions of definitive and probable CRE are suggested, and recommendations regarding the diagnostic evaluation and etiology‐specific management of patients with CRE are made. Prospective trials are needed to determine when and how surgery should be done and to define the relations of the hemosiderin rim to the epileptogenic zone.

[1]  A. Marowsky,et al.  In-Vitro Characterization of mCerulean3_mRuby3 as a Novel FRET Pair with Favorable Bleed-Through Characteristics , 2019, Biosensors.

[2]  E. Chang,et al.  Predictors of seizure freedom in the surgical treatment of supratentorial cavernous malformations. , 2011, Journal of neurosurgery.

[3]  I. Sandalcioglu,et al.  In Vitro Characterization of the Angiogenic Phenotype and Genotype of the Endothelia Derived From Sporadic Cerebral Cavernous Malformations , 2011, Neurosurgery.

[4]  R. Salman,et al.  Seizure risk from cavernous or arteriovenous malformations , 2011, Neurology.

[5]  Oliver Kraff,et al.  7 tesla MRI of microbleeds and white matter lesions as seen in vascular dementia , 2011, Journal of magnetic resonance imaging : JMRI.

[6]  M. Lehecka,et al.  Microsurgical treatment of temporal lobe cavernomas , 2011, Acta Neurochirurgica.

[7]  J. Schramm,et al.  Cerebral cavernous malformations and intractable epilepsy: the limited usefulness of current literature , 2011, Acta Neurochirurgica.

[8]  Maria Thom,et al.  The clinicopathologic spectrum of focal cortical dysplasias: A consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission 1 , 2011, Epilepsia.

[9]  A. Reuss,et al.  Epileptogenicity of Cavernomas Depends on (Archi-) Cortical Localization , 2010, Neurosurgery.

[10]  Robert J. Maciunas,et al.  Triple pathology in epilepsy: Coexistence of cavernous angiomas and cortical dysplasias with other lesions , 2010, Epilepsy Research.

[11]  M. Ladd,et al.  Susceptibility weighted magnetic resonance imaging of cerebral cavernous malformations: prospects, drawbacks, and first experience at ultra-high field strength (7-Tesla) magnetic resonance imaging. , 2010, Neurosurgical focus.

[12]  D. Kondziolka,et al.  Stereotactic radiosurgery for the treatment of symptomatic brainstem cavernous malformations. , 2010, Neurosurgical focus.

[13]  Gary W. Mathern,et al.  Erratum: Definition of drug resistant epilepsy. Consensus proposal by the ad hoc task force of the ILAE commission on therapeutic strategies (Epilepsia (2010) 51 (1069-77)) , 2010 .

[14]  I. Awad,et al.  Emerging clinical imaging techniques for cerebral cavernous malformations: a systematic review. , 2010, Neurosurgical focus.

[15]  D. Kondziolka,et al.  Stereotactic radiosurgery for symptomatic solitary cerebral cavernous malformations considered high risk for resection. , 2010, Journal of neurosurgery.

[16]  T. Schwartz Epilepsy Surgeons, Rather than Vascular Neurosurgeons, Should Operate on Cavernous Malformations that Cause Seizures—A Modest Proposal , 2010, Epilepsy currents.

[17]  J. H. Cross,et al.  Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009 , 2010, Epilepsia.

[18]  G. Mathern,et al.  Definition of drug resistant epilepsy: Consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies , 2009, Epilepsia.

[19]  L. Chahine,et al.  Clinical Reasoning: Cerebral cavernous malformations , 2009, Neurology.

[20]  G. Lanzino,et al.  DEVELOPMENTAL VENOUS ANOMALIES: CURRENT CONCEPTS AND IMPLICATIONS FOR MANAGEMENT , 2009 .

[21]  Gregory A Worrell,et al.  Electrocorticography-guided resection of temporal cavernoma: is electrocorticography warranted and does it alter the surgical approach? , 2009, Journal of neurosurgery.

[22]  A. Schulze-Bonhage,et al.  Epilepsy surgery in patients with multiple cerebral cavernous malformations , 2009, Seizure.

[23]  U. Sure,et al.  Involvement of PTEN Promoter Methylation in Cerebral Cavernous Malformations , 2009, Stroke.

[24]  U. Sure,et al.  Phosphatase and tensin homolog in cerebral cavernous malformation: a potential role in pathological angiogenesis. , 2009, Journal of neurosurgery.

[25]  A. Schulze-Bonhage,et al.  Zur Notwendigkeit prächirurgischer Video-EEG-Registrierungen bei läsionellen Epilepsiepatienten , 2009, Der Nervenarzt.

[26]  Seung-Chyul Hong,et al.  Supratentorial cavernous angiomas presenting with seizures: Surgical outcomes in 60 consecutive patients , 2009, Seizure.

[27]  C. Baumgartner,et al.  LONG‐TERM SEIZURE CONTROL AFTER RESECTION OF SUPRATENTORIAL CAVERNOMAS: A RETROSPECTIVE SINGLE‐CENTER STUDY IN 53 PATIENTS , 2008, Neurosurgery.

[28]  W. Whetsell,et al.  Hypermotor seizures in patients with temporal pole lesions , 2008, Epilepsy Research.

[29]  Y. Itoyama,et al.  Neuromagnetic Localization of Spike Sources in Perilesional, Contralateral Mirror, and Ipsilateral Remote Areas in Patients with Cavernoma , 2007, Epilepsia.

[30]  P. V. van Rijen,et al.  Electrocorticography discharge patterns in patients with a cavernous hemangioma and pharmacoresistent epilepsy. , 2007, Journal of neurosurgery.

[31]  R. Delfini,et al.  Intramedullary cavernous angioma of the spinal cord in a pediatric patient, with multiple cavernomas, familial occurrence and partial spontaneous regression: case report and review of the literature , 2007, Child's Nervous System.

[32]  C. Tseng,et al.  Treatment of Epileptogenic Cavernomas: Surgery versus Radiosurgery , 2007, Cerebrovascular Diseases.

[33]  H. Stefan,et al.  Prediction of postoperative outcome with special respect to removal of hemosiderin fringe: A study in patients with cavernous haemangiomas associated with symptomatic epilepsy , 2007, Seizure.

[34]  C. Elger,et al.  Seizure Outcome after Resection of Supratentorial Cavernous Malformations: A Study of 168 Patients , 2007, Epilepsia.

[35]  G. Worrell,et al.  3D source localization of interictal spikes in epilepsy patients with MRI lesions , 2006, Physics in medicine and biology.

[36]  U. Felbor,et al.  Genetics of Cerebral Cavernous Angioma , 2006, Zentralblatt fur Neurochirurgie.

[37]  I. Awad,et al.  Cerebral cavernous malformations and epilepsy. , 2006, Neurosurgical focus.

[38]  F. Andermann,et al.  Seizure Outcome after Resection of Cavernous Malformations Is Better When Surrounding Hemosiderin‐stained Brain Also Is Removed , 2006, Epilepsia.

[39]  Christian Steinhäuser,et al.  Astrocyte dysfunction in neurological disorders: a molecular perspective , 2006, Nature Reviews Neuroscience.

[40]  P. Ferroli,et al.  Cerebral cavernomas and seizures: a retrospective study on 163 patients who underwent pure lesionectomy , 2006, Neurological Sciences.

[41]  Q. Wang Update on the molecular genetics of vascular anomalies. , 2005, Lymphatic research and biology.

[42]  Thomas Rülicke,et al.  Astrogliosis in epilepsy leads to overexpression of adenosine kinase, resulting in seizure aggravation. , 2005, Brain : a journal of neurology.

[43]  C. Elger,et al.  Response: Definitions Proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) , 2005 .

[44]  C. Elger,et al.  Epileptic Seizures and Epilepsy: Definitions Proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) , 2005, Epilepsia.

[45]  W. Hauser,et al.  Comment on Epileptic Seizures and Epilepsy: Definitions Proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) , 2005, Epilepsia.

[46]  A. Siegel,et al.  Biological activity of adult cavernous malformations: a study of 56 patients. , 2005, Journal of neurosurgery.

[47]  H. Stefan,et al.  Cavernous haemangiomas, epilepsy and treatment strategies , 2004, Acta neurologica Scandinavica.

[48]  Jens P Dreier,et al.  Lasting Blood-Brain Barrier Disruption Induces Epileptic Focus in the Rat Somatosensory Cortex , 2004, The Journal of Neuroscience.

[49]  I. Blümcke,et al.  [Supratentorial cavernoma and epileptic seizures. Are there predictors for postoperative seizure control?]. , 2004, Der Nervenarzt.

[50]  H. Stefan,et al.  Supratentorielle Kavernome und epileptische Anfälle , 2004, Der Nervenarzt.

[51]  M. Manfredi,et al.  Postoperative EEG and seizure outcome in temporal lobe epilepsy surgery , 2004, Clinical Neurophysiology.

[52]  C. Liquori,et al.  Mutations in a gene encoding a novel protein containing a phosphotyrosine-binding domain cause type 2 cerebral cavernous malformations. , 2003, American journal of human genetics.

[53]  F. Woermann,et al.  Cavernous angioma associated with ipsilateral hippocampal sclerosis , 2002, European Radiology.

[54]  H. Mehdorn,et al.  Therapeutical considerations in patients with intracranial venous angiomas , 2002, European journal of neurology.

[55]  H. Lüders,et al.  Presurgical evaluation of epilepsy. , 2001, Brain : a journal of neurology.

[56]  P. Blumbergs,et al.  Cavernous haemangiomas (angiomas) of the brain. , 2001, Australasian radiology.

[57]  A. Siegel,et al.  Treatment-induced neoangiogenesis in cerebral arteriovenous malformations , 2001, Clinical Neurology and Neurosurgery.

[58]  F. Vale,et al.  MRI evidence of mesial temporal sclerosis in patients with psychogenic nonepileptic seizures , 2000, Neurology.

[59]  A. Joutel,et al.  Truncating mutations in CCM1, encoding KRIT1, cause hereditary cavernous angiomas , 1999, Nature Genetics.

[60]  J. Rabinov,et al.  Diagnostic imaging of angiographically occult vascular malformations. , 1999, Neurosurgery clinics of North America.

[61]  Crone,et al.  The natural history of cavernous malformations: a prospective study of 68 patients , 1999, Neurosurgery.

[62]  S. Shorvon,et al.  Supratentorial cavernous haemangiomas and epilepsy: a review of the literature and case series , 1999, Journal of neurology, neurosurgery, and psychiatry.

[63]  B. Pohlmann-Eden,et al.  Epileptology of the first-seizure presentation , 1998, The Lancet.

[64]  Graeme D Jackson,et al.  Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients , 1998, The Lancet.

[65]  C. Lindquist,et al.  Radiosurgery for cavernous malformations. , 1998, Journal of neurosurgery.

[66]  D. Eberhard,et al.  Histopathology of arteriovenous malformations after gamma knife radiosurgery. , 1997, Journal of neurosurgery.

[67]  P. Cappabianca,et al.  Supratentorial cavernous malformations and epilepsy: seizure outcome after lesionectomy on a series of 35 patients , 1997, Clinical Neurology and Neurosurgery.

[68]  S. Chung,et al.  An analysis of the natural history of cavernous malformations. , 1997, Surgical neurology.

[69]  T. Hurley,et al.  Perimesencephalic nonaneurysmal subarachnoid hemorrhage: review of the literature. , 1997, Neurosurgery.

[70]  H. Schroeder,et al.  Supratentorial cavernous angiomas and epileptic seizures: preoperative course and postoperative outcome. , 1996, Neurosurgery.

[71]  G. Broggi,et al.  Supratentorial cavernous angiomas and epileptic seizures: preoperative course and postoperative outcome. , 1996, Neurosurgery.

[72]  I. Awad,et al.  Intracranial cavernous malformations: lesion behavior and management strategies. , 1995, Neurosurgery.

[73]  R. Goodman,et al.  Seizure outcome after lesionectomy for cavernous malformations. , 1995, Journal of neurosurgery.

[74]  M. Giulioni,et al.  Surgical management of cerebral cavernous angiomas causing epilepsy. , 1995, Journal of neurosurgical sciences.

[75]  John R. Robinson,et al.  Factors predisposing to clinical disability in patients with cavernous malformations of the brain. , 1993, Neurosurgery.

[76]  S. Phipps,et al.  Cavernous haemangiomas (angiomas) of the brain: clinically significant lesions. , 1993, Australasian radiology.

[77]  C. V. van Donselaar,et al.  Value of the electroencephalogram in adult patients with untreated idiopathic first seizures. , 1992, Archives of neurology.

[78]  A. Elster,et al.  An analysis of the natural history of cavernous angiomas. , 1991, Journal of neurosurgery.

[79]  John R. Robinson,et al.  Natural history of the cavernous angioma. , 1991, Journal of neurosurgery.

[80]  F. Morrell,et al.  Secondary epileptogenesis in man. , 1985, Archives of neurology.

[81]  G. Sypert,et al.  Chronic focal epileptiform discharges induced by injection of iron into rat and cat cortex. , 1978, Science.

[82]  A. Berkowitz,et al.  Physiology of Connective Tissue , 1969 .

[83]  W. Mccormick,et al.  Vascular malformations ("angiomas") of the dura mater. , 1966, Journal of neurosurgery.

[84]  John W. Scott,et al.  Selected Writings of John Hughlings Jackson , 1959 .

[85]  H. Kufs Über heredofamiliäre Angiomatose des Gehirns und der Retina, ihre Beziehungen zueinander und zur Angiomatose der Haut , 1928 .

[86]  R. Prayson,et al.  Cavernous angiomas in chronic epilepsy associated with focal cortical dysplasia. , 2013, Clinical neuropathology.

[87]  Oliver Kraff,et al.  Cerebral cavernous hemangiomas at 7 Tesla: initial experience. , 2010, Academic radiology.

[88]  D. Rigamonti,et al.  Cavernous malformations: natural history, diagnosis and treatment , 2009, Nature Reviews Neurology.

[89]  A. Schulze-Bonhage,et al.  [Presurgical video EEG monitoring of lesional epilepsy patients]. , 2009, Der Nervenarzt.

[90]  U. Ebeling,et al.  Seizure control following surgery in supratentorial cavernous malformations: a retrospective study in 77 patients , 2005, Acta Neurochirurgica.

[91]  M. Clanet,et al.  Mutations within the programmed cell death 10 gene cause cerebral cavernous malformations. , 2005, American journal of human genetics.

[92]  P. Schmiedek Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients , 2001, Neurosurgical Review.

[93]  H. Steiger,et al.  Cavernous Haemangiomas of the Spinal Cord. A Review of 117 Cases , 1999, Acta Neurochirurgica.

[94]  G. Broggi,et al.  Lesionectomy in epileptogenic temporal lobe lesions: preoperative seizure course and postoperative outcome. , 1997, Acta neurochirurgica. Supplement.

[95]  T. Sundt,et al.  Seizure outcome in patients with surgically treated cerebral arteriovenous malformations. , 1993, Journal of neurosurgery.

[96]  J. Tew,et al.  Seizure control after surgery on cerebral arteriovenous malformations. , 1993, Journal of neurosurgery.