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Surgical Resection Can Be Successful in a Large Fraction of Patients With Drug-Resistant Epilepsy Associated With Multiple Cerebral Cavernous Malformations

Brelie, Christian von der MD*,‖; von Lehe, Marec MD*; Raabe, Anna MD; Niehusmann, Pitt MD; Urbach, Horst MD§,**; Mayer, Christian MD§; Elger, Christian Erich MD; Malter, Michael P. MD

doi: 10.1227/NEU.0000000000000210
Research-Human-Clinical Studies

BACKGROUND: Multiple cerebral cavernous malformations (mCCMs) are known as potentially epileptogenic lesions. Treatment might be multimodal. Management of patients with mCCMs and epilepsy is challenging.

OBJECTIVE: To evaluate (1) algorhythmic therapeutic sequences in patients with epilepsy associated to mCCMs, (2) whether there are predictive parameters to anticipate the development of drug-resistant epilepsy, and (3) seizure after epilepsy surgery compared to conservatively-treated drug-resistant patients.

METHODS: All inpatients and outpatients with epilepsy associated to mCCMs from 1990 to 2010 and follow-up >12 months available were retrospectively analyzed.

RESULTS: Twenty-three patients matched inclusion criteria. Epilepsy became drug-resistant in 18/23 (78%) patients. No predictors were found for development of drug-resistant epilepsy. Median follow-up for both groups was 7.8 years. Nine patients did not qualify for surgical therapy and were treated conservatively. One patient of this cohort (11%) was seizure-free (International League Against Epilepsy [ILAE] class 1). Surgical treatment was performed in 9 patients; 7/9 (78%) of these patients were seizure-free (ILAE class 1) after epilepsy surgery for at least 12 months compared with 1/9 patients in the non-operated group. In 7/9 cases (78%) the largest CCM was resected. In 8/9 (89%) not all CCMs were resected.

CONCLUSION: After initial diagnosis of epilepsy associated to mCCMs, a primary conservative approach is reasonable. Surgical treatment can be successful in a large fraction of cases with drug-resistant epilepsy where an epileptogenic lesion is identified. Cases where surgery is not undertaken are likely to remain intractable.

ABBREVIATIONS: AED, antiepileptic drug

CCMs, cerebral cavernous malformations

DRE, drug-resistant epilepsy

ILAE, International League Against Epilepsy

mCCMs, multiple cerebral cavernous malformations

*Department of Neurosurgery,

Department of Epileptology,

Department of Pathology/Neuropathology,

§Department of Radiology, University of Bonn Medical Centre, Bonn, Germany;

Department of Neurosurgery, Unfallkrankenhaus Berlin, Berlin, Germany;

**Department of Neuroradiology, University of Freiburg Medical Center, Freiburg, Germany

Correspondence: Christian von der Brelie, MD, Department of Neurosurgery, Unfallkrankenhaus Berlin, Warener Strasse 7, 12683 Berlin, Germany. E-mail:

Received July 11, 2013

Accepted October 07, 2013

Copyright © by the Congress of Neurological Surgeons