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Brain & Development xxx (2017) xxx–xxx Case Report Successful corpus callosotomy for Doose syndrome Sotaro Kanai a, Tohru Okanishi a,⇑, Mitsuyo Nishimura b, Kentaro Iijima c, Takuya Yokota a, Tomohiro Yamazo
  Case Report Successful corpus callosotomy for Doose syndrome Sotaro Kanai a , Tohru Okanishi a, ⇑ , Mitsuyo Nishimura b , Kentaro Iijima c ,Takuya Yokota a , Tomohiro Yamazoe c , Ayataka Fujimoto c , Hideo Enoki a ,Takamichi Yamamoto c a Department of Child Neurology, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 430-8558, Japan b Laboratory of Neurophysiology, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 430-8558, Japan c Comprehensive Epilepsy Center, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 430-8558, Japan Received 16 March 2017; received in revised form 6 June 2017; accepted 7 June 2017 Abstract Doose syndrome (epilepsy with myoclonic-atonic seizures) is an epilepsy syndrome with an incidence of approximately 1–2% of childhood-onset epilepsies. Although this syndrome is associated with multiple types of generalized seizures, the diagnosis is basedon the presence of myoclonic-atonic seizures. Eighteen percent of patients have refractory seizures and intellectual disabilities. Therehave, however, been a few reports on the efficacy of surgical treatment for Doose syndrome.We describe a case of Doose syndrome in a 10-year-old boy. He developed generalized tonic–clonic seizures at 3 years 8 monthsof age and subsequently developed myoclonic-atonic, myoclonic, and tonic seizures. The frequent myoclonic seizures were refractoryto multiple antiepileptic medications. His cognitive development was moderately delayed. Anterior four fifths corpus callosotomywas performed at 8 years 5 months of age. His seizures, especially myoclonic seizures, were markedly reduced. He was given vagusnerve stimulation therapy at 9 years and 1 month of age, which led to complete resolution of the myoclonic seizures. Corpus cal-losotomy can be a good treatment strategy in patients with Doose syndrome with medically refractory generalized seizures.   2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved. Keywords:  Doose syndrome; Epilepsy with myoclonic-atonic seizures; Myoclonic seizures; Corpus callosotomy; Vagus nerve stimulation 1. Introduction Doose syndrome is an epilepsy syndrome that wasfirst reported in 1970 as ‘‘centrencephalic myoclonic-astatic petit mal ”  [1]. This syndrome was otherwiseknown as myoclonic-astatic epilepsy, and was redefinedin 2010 by the International League Against Epilepsy as‘‘epilepsy with myoclonic-atonic seizures ”  [2]. Doosesyndrome is relatively common, with an incidence of approximately 1–2% of childhood-onset epilepsies.Although these patients can develop multiple types of generalized seizures, the diagnosis is based on the pres-ence of myoclonic-atonic seizures. The seizures are gen-erally refractory to antiepileptic medication. Theseizure-free ratio in each antiepileptic medication rangesfrom 0% to 36%. Cognitive impairment affects 20–74%of the patients with Doose syndrome [3]. An electroen-cephalogram (EEG) demonstrates a 2–5 Hz syn-chronous spike and wave, and polyspike and wavecomplexes with abnormal parietal theta activities,although most of the posterior background rhythmsand sleep architecture could be considered generallynormal for age. This can help to distinguish children   2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved. ⇑ Corresponding author. Fax: +81 53 475 7596. E-mail addresses:, (T. Okanishi). Brain & Development xxx (2017) xxx–xxx Please cite this article in press as: Kanai S et al. Successful corpus callosotomy for Doose syndrome. Brain Dev (2017),  with Doose syndrome from those with LGS, in whomthe EEG is much more abnormal with little or no nor-mal background activity [3]. Brain magnetic resonanceimaging (MRI) shows no abnormality.Antiepileptic medications such as valproate, ethosux-imide, and lamotrigine are beneficial for controlling theseizures [4–6]. For the refractory patients, a ketogenicdiet has been reported to be efficacious [4,6,7]. Recently,a few reports described vagus nerve stimulation (VNS)therapy, which led to complete resolution of seizuresin two patients [8,9]. However, there has been nodetailed report of open cranial surgery for thesyndrome.Corpus callosotomy (CC) is a valuable palliative sur-gical option for cases of medically refractory epilepsy,especially in cases that include drop attacks [10,11].CC is typically used in patients with diffuse or multifocalepileptic discharges resulting in generalized seizures [11].There have been only a few reports of CC for childrenwith refractory myoclonic seizures.Here, we present a case of a child with Doose syn-drome, whose myoclonic seizures were refractory tomultiple antiepileptic medications. CC markedlydecreased seizure frequency. 2. Case report A 10-year-old boy developed febrile generalizedtonic–clonic (GTC) seizures at the age of 3 years8 months. One week after the febrile seizure, he devel-oped daily myoclonic-atonic seizures. GTC seizures,myoclonic seizures, and tonic seizures also appearedafter the onset. He was treated with valproate, lamotrig-ine, and clobazam, which controlled the myoclonic-atonic seizures. Other antiepileptic medications, includ-ing zonisamide, topiramate, carbamazepine, nitraze-pam, and levetiracetam, were administered for theother types of seizures, and were partially effective inreducing the frequencies.The patient was referred to our hospital at the age of 8 years 3 months. Brain MRI showed no suspiciousepileptogenic lesions. The patient’s Tanaka-Binet intel-lectual test score was 45. Scalp video EEG monitoringfor 24 h showed dominantly bilateral parietal 6–7 Hztheta activity that was not attenuated by eye opening,and diffuse 1.5–2 Hz high-amplitude spike and waveburst interictally (Fig. 1). Bilateral myoclonic seizureswith frontal dominant high-amplitude spike or poly-spike and waves were captured approximately one Fig. 1. The interictal EEG showed (a) bilateral parietal dominant theta activity that was not attenuated by eye opening, (b) diffuse 1.5–2 Hz high-amplitude spike and wave burst.2  S. Kanai et al./Brain & Development xxx (2017) xxx–xxx Please cite this article in press as: Kanai S et al. Successful corpus callosotomy for Doose syndrome. Brain Dev (2017),  hundred times during drowsiness to light sleep (Fig. 2).Tonic and GTC seizures were also seen. From the clin-ical course and the EEG findings, we diagnosed him ashaving Doose syndrome.The parents did not want diet therapy. The patientsubsequently had anterior four fifths CC at the age of 8 years 5 months (Fig. 3). His myoclonic seizures mark-edly decreased to a few times a month. We added VNStherapy for his residual seizures at the age of 9 years1 month. Interictal EEG did not show any epileptiformdischarges at the age of 10 years 6 months. At the lastfollow-up at the age of 10 years 10 months, only simplepartial seizures once a month remained with valproate,lamotrigine, and levetiracetam. Although the Tanaka-Binet intelligence score was 44 and had not improved,the parents were satisfied that his concentration forstudy was improved compared to the status before CC. 3. Discussion Our patient showed frequent myoclonic seizures dur-ing drowsiness to light sleep, as well as tonic and GTCseizures, which were refractory to multiple antiepilepticmedications. CC markedly reduced those seizures, andadditional VNS therapy led to complete resolution of his residual myoclonic seizures. Fig. 2. The ictal EEG of myoclonic seizure. Bilateral muscular contractions on electromyogram (arrow) occurred simultaneously for 0.2 s associatedwith polyspikes.Fig. 3. The head computed tomography soon after anterior four fifthcorpus callosotomy (arrow). S. Kanai et al./Brain & Development xxx (2017) xxx–xxx  3 Please cite this article in press as: Kanai S et al. Successful corpus callosotomy for Doose syndrome. Brain Dev (2017),  Seizure and intellectual outcomes are variable inDoose syndrome. Eighteen percent of patients haverefractory seizures and intellectual disabilities [4]. Theketogenic diet is a widely reported therapy and may,in fact, be the most efficacious [4,6,7]. Efficacy of surgi-cal treatment for Doose syndrome is unclear for therefractory cases. Caraballo et al. reported clinicalcourses in 69 patients with Doose syndrome [7]. In thestudy, five patients underwent VNS implantation, andthree showed seizure reduction greater than 50%. Twoother case reports described VNS therapy leading tocomplete resolution of myoclonic, atonic, and absenceseizures [8,9]; one patient later presented serious brady-cardia. To our knowledge, there has been only a singlecase of CC for Doose syndrome, which was brieflydescribed in Caraballo’s retrospective study and didnot show the effectiveness.CC is a palliative surgical option for cases of medi-cally refractory epilepsy without resectable epileptic foci[10]. This procedure has a role in alleviating varioustypes of seizures, with the best efficacy in drop attacksand generalized seizures, including absence, atonic,myoclonic, and GTC seizures. Kasasbeh et al. reportedthat CC for children with refractory myoclonic seizuresresulted in complete resolution in more than half of thecases [11]. We therefore expected that CC could be effec-tive in the present case, because the main seizure type inthis patient was myoclonic seizures. From this casestudy, we cannot evaluate the efficacy of CC formyoclonic-atonic seizure, which is the most characteris-tic type in Doose syndrome.In conclusion, we report the a case of a child withDoose syndrome with refractory myoclonic, tonic, andGTC seizures, which were markedly reduced after CC.CC can be a valuable surgical option for children withmedically refractory generalized seizures in Doosesyndrome. References [1] Doose H, Gerken H, Leonhardt R, Volzke E, Volz C. Centren-cephalic myoclonic-astatic petit mal. Clinical and genetic inves-tigations (in German). Neuropediatrics 1970;2:59–78.[2] Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, vanEmde Boas W, et al. Revised terminology and concepts fororganization of seizures and epilepsies: report of the ILAEcommission on classification and terminology, 2005–2009. Epilep-sia 2010;51:676–85.[3] Kelley SA, Kossoff EH. Doose syndrome (myoclonic-astaticepilepsy): 40 years of progress. Dev Med Child Neurol2010;52:988–93.[4] Oguni H, Tanaka T, Hayashi K, Funatsuka M, Sakauchi M,Shirakawa S, et al. Treatment and long-term prognosis of myoclonic-astatic epilepsy of early childhood. Neuropediatrics2002;33:122–32.[5] Wallace SJ. Myoclonus and epilepsy in childhood: a review of treatment with valproate, ethosuximide, lamotrigine and zon-isamide. Epilepsy Res 1998;29:147–54.[6] Kilaru S, Bergqvist AG. Current treatment of myoclonic astaticepilepsy: clinical experience at the Children’s Hospital of Philadelphia. Epilepsia 2007;48:1703–7.[7] Caraballo RH, Chamorro N, Darra F, Fortini S, Arroyo H.Epilepsy with myoclonic atonic seizures: an electroclinical studyof 69 patients. Pediatr Neurol 2013;48:355–62.[8] Fan PC, Peng SS, Yen RF, Shieh JY, Kuo MF. Neuroimagingand electroencephalographic changes after vagus nerve stimula-tion in a boy with medically intractable myoclonic astaticepilepsy. J Formos Med Assoc 2014;113:258–63.[9] Cantarı´n-Extremera V, Ruı´z-Falco´-Rojas ML, Tamarı´z-Martel-Moreno A, Garcı´a-Ferna´ndez M, Duat-Rodriguez A, Rivero-Martı´n B. Late-onset periodic bradycardia during vagus nervestimulation in a pediatric patient. A new case and review of theliterature. Eur J Paediatr Neurol 2016;20:678–83.[10] Maehara T, Shimizu H. Surgical outcome of corpus callosotomyin patients with drop attacks. Epilepsia 2001;42:67–71.[11] Kasasbeh AS, Smyth MD, Steger-May K, Jalilian L, Bertrand M,Limbrick DD. Outcomes after anterior or complete corpuscallosotomy in children. Neurosurgery 2014;74:17–28.4  S. Kanai et al./Brain & Development xxx (2017) xxx–xxx Please cite this article in press as: Kanai S et al. Successful corpus callosotomy for Doose syndrome. Brain Dev (2017),
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