Epileptic Seizures in Behc ¸et Disease Ebru Aykutlu, Betu ¨ l Baykan, Piraye Serdarog ˇlu, Aysen Go ¨kyig ˇit, and Gu ¨ lsen Akman-Demir Department of Neurology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey Summary: Purpose: To outline the clinical characteristics of seizures in our large series of Behc ¸et disease (BD) patients with neurologic involvement. Methods: All files of 223 patients with neuro-BD were evaluated retrospectively, and the group with clearly docu- mented seizures was included in the current study. Clinical characteristics, EEG, neuroimaging and cerebrospinal fluid findings were reevaluated, and the seizures were classified ac- cording to the new proposed criteria of the International League Against Epilepsy. On excluding the patients in whom the sei- zures were due to possible seizure-provoking factors, the sei- zures that appeared during a neurologic exacerbation were noted. Results: Seizures were seen in 10 (4.48%) of 223 patients. There were one female and nine male patients. In five of the patients, seizures occurred during neurologic exacerbation. Therefore, the actual prevalence of seizures due to BD in our group is 2.2%. In the remaining five patients, the seizures were not related to neurologic BD attacks, but probably were due to some seizure-provoking factors. The predominating seizure type was generalized tonic–clonic convulsions accompanied by focal motor seizures. It is notable that four patients died 1–5 years after the onset of the seizures. Conclusions: Our study showed that seizures are rare in BD. As the seizures due to some interventions and drugs are as frequent as neuro-BD–related seizures, seizure-provoking fac- tors must be considered before attributing them to the patho- genetic mechanism of BD. The occurrence of seizures seems to be associated with a high mortality rate. Key Words: Behc ¸et disease—Neuro-Behc ¸et disease—Epilepsy—Seizure. Behc ¸et disease (BD) is a multisystemic disease of un- known cause with variable clinical features (1). Accord- ing to the diagnostic criteria formed by the International Study Group, recurrent oral ulceration is a prerequisite, with any two of genital ulcerations, skin lesions, uveitis, or hyperreactivity of skin to nonspecific physical insult (pathergy test) (2). The central nervous system (CNS) also is involved in ∼5% of the patients with BD (3). In large series of neuro-BD patients, epileptic seizure fre- quency is rare (3). Recently, a single case of BD was reported to have partial seizures as the presenting feature of neurologic involvement (4). However, the prevalence and clinical course of the seizures in neuro-BD have not been studied. This study sought to outline the frequency and clinical characteristics of seizures in our large series of BD pa- tients with neurologic involvement. PATIENTS AND METHODS A total of 223 cases with BD and neurologic involve- ment was seen since 1984 in our clinic. All files were reevaluated retrospectively, and the group with clearly documented seizures was included in the current study. Clinical characteristics, EEG, neuroimaging, and ce- rebrospinal fluid (CSF) findings were evaluated, and the seizures were classified according to the new proposed criteria of the International League Against Epilepsy (5). Seizures that appeared during a neurologic exacerba- tion (attack) were noted. Special emphasis was given to the possible role of seizure-provoking factors such as high fever, pathologic blood biochemistry results, neu- rosurgical interventions, inappropriate discontinuation of antiepileptic drugs (AEDs), or use of other drugs with seizure threshold–lowering effects (6). RESULTS Seizure characteristics, neurologic pictures, clinical and laboratory findings, and outcomes are summarized in Table 1. Seizures were seen in 10 (4.48%) of 223 patients. There were one female and nine male patients. Age at onset of BD was 24.5 ± 8.8 years, and at onset of neuro- BD was 30.4 ± 10.1 years. One of the cases did not have sufficient follow-up (patient 2), whereas the others have been followed up for between 5 months and 9.5 years (mean, 44.3 months). None of the patients had any fam- ily history of epilepsy or febrile convulsions. Accepted March 17, 2002. Address correspondence and reprint requests to Dr. B. Baykan at University of Istanbul, Istanbul Faculty of Medicine, Department of Neurology, Millet Cad.; C ¸ apa 34390, Istanbul, Turkey. E-mail: baykankurtg@superonline.com.tr and/or baykanb@istanbul.edu.tr Epilepsia, 43(8):832–835, 2002 Blackwell Publishing, Inc. © International League Against Epilepsy 832