Benign Childhood Epilepsy with Centrotemporal Spikes (Rolandic) in Birjand City of Iranian Patients

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1 Benign Childhood Epilepsy with Centrotemporal Spikes (Rolandic) in Birjand City of Iranian Patients Hamid reza Riasi, MD 17, Mohammad Mehdi Hassanzadeh Taheri 18, Fariba Nakhaey, MD 19, oroud Salehi, MD 20 Iranian Journal of Neurology, Vol.7, No.21 & 22, Spring & Summer 2008, Abstract Introduction: Benign epilepsy of childhood with rolandic spikes (BECRS) is the most common childhood epilepsy syndrome and its epidemiological studies are of importance primarily to compare incidence and prevalence rates, age distribution, inheritance, seizure types, and treatment strategies in different populations. In this investigation we studied clinical and paraclinical aspects of Rolandic epilepsy. Methods: Between 2002 and 2007, 67 patients of Iranian nation in east of Iran, were studied retrospectively in Vali-e-Asr and Imam Reza hospitals in Birjand. All patients had first seizure between the age of 3 and 12 years and all of them were normal in neurologic exam without any sign of focal CNS lesion. Our study included only the patients who had definite past medical history with at least one EEG each six months. Results: In this study 37 cases (55.2%) were male and 30 cases (44.8%) were female. 30% of our patients (20 cases) had no further seizures after the age of 5 years. 42 cases (63%) had complete recovery by age of 12 years and all cases had complete recovery by age of cases (79.1%) of patients had only partial seizures while 20.9% (14 cases) had secondary generalization of focal seizure. In their paraclinic survey, epileptic discharges were confined to one hemisphere in patients (41.8%) but in 39 cases (58.2%) epileptic discharges were seen bilaterally in two hemispheres. Results: Our study showed that Rolandic seizure (BRE) is more prevalent in males than females, but sex difference was not significant. Responding to CBZ (Carbamazepine) was excellent (62 cases (92.5%)) but 5 cases (7.5%) not responded to CBZ or didn't tolerate it but had good response to sodium valproate. Conclusion: In regarding to BRE two aspects are of importance; first, this disorder is confined to active period of learning life, and its bad control can result in profound difficulty in later life and second, the disorder can easily be controlled with simple antiepileptic drugs without any side effect. Keywords: Benign Rolandic Epilepsy (BRE), Carbamazepine (CBZ), EEG, Clinical Features 17 Assistant professor of Neurology Department, Vali-e-Asr Hospital, Birjand University of Medical Sciences, Birjand, Iran 18 Ph.D, Assistant professor of Anatomical Sciences, Anatomy Department, Medical Faculty, Birjand University of Medical Sciences, Birjand, Iran 19 Internal Medicine Assistant, Baghiyatollah Hospital, Baghiyatollah University.Tehran, Iran 20 Assistant professor of Pediatric Department, Vali-e-Asr Hospital, Birjand University of Medical Sciences. Birjand, Iran

2 177/ Iran. J. Neurol. Vol.7; No. 21 & 22, Spring & Summer 2008 Introduction Epilepsy may compromise the ability of child and family to function effectively. One of the most important form of epilepsy is Rolandic epilepsy which constitutes about 15-24% of all childhood epilepsy. (1) The syndrome was not described and defined until 1950s. The EEG features were first reported by Gastaüt 1952 who called the discharges as prerolandique and Gibbs & Gibbs in the same year entitled them mid-temporal. (2) The first accurate description of electro-clinical syndrome was presented in 1958 by Nayrac and Beaussort and this was followed by several other reports in the 1960 and (2) The name proposed by commission and classification and terminology by International league against epilepsy 1969 is benign childhood epilepsy with centrotemporal spikes according to clinical and electroencephalographic features. (2) The term benign refers to the excellent prognosis of disorder regarding seizure control and the long-term seizure and developmental outcome. (3) Because of the benign nature of rolandic epilepsy it is generally presumed that quality of life is not significantly affected. Epidemiologic studies of childhood epilepsy are of importance primarily to compare incidence and prevalence rate, age distribution, inheritance, seizure types, epilepsy syndromes and treatment strategies in different populations. (4) Benign Rolandic Epilepsy (BRE) is the most common epileptic disorder in childhood period with an incidence of approximately 21 in of 5-20 years old population which is characterized with brief repetitive unprovoked attacks. (5) All Rolandic epileptic children are normal in physical exam who divided into two groups; A group present only with simple partial seizure without generalization which predominantly occur during sleep. The second group have secondary generalization of focal seizure which occur predominantly during day time. (5,6) Nocturnal seizures accounts for 20-25% of all childhood epilepsies (7,9) has characteristic EEG view: poly spike and sharp tall waves which is accentuated in temporal and centrotemporal areas in one hemisphere or bilaterally in two hemispheres (symmetric or asymmetric). Affected persons have no focal neurologic deficit and have characteristic (5, 6) remission after puberty. We studied general manifestations of BRE retrospectively including; age of onset, sex distribution, family history, febrile seizure in past medical history, age of remission, EEG pattern and drug strategies in patients who referred to Vali-e-Asr and Imam Reza neurologic ward of Birjand University of Medical Sciences (BUMS) including patients who were visited for other neurologic problem but had had BRE in their past medical history or visited for epilepsy

3 178/ Benign Childhood Epilepsy with Centrotemporal workup after their complete remission beyond two years. Patients & Method From Oct 2002 to March 2007, 67 patients (cases) were studied including only cases that had definite BRE in their history and visited for other neurologic problem (non organic problem) or epilepsy workup and all of them were seizure free beyond two years without drug taking. Diagnosis based on complete history of clinical presentation and paraclinic complementary data (EEG with epileptic discharge which accentuated in centrotemporal area) only cases were included that their neurologic exam and imaging during active epileptic disorder had no abnormality and their EEG background were normal and had at least one EEG during each 6 month in active period of disease. Other information which were taken included family history of epilepsy, positive history of febrile seizure, age of onset, age of recovery, features of seizures (only focal seizure or secondary generalization). For those patients who had attacks until March 2005 carbamazepine was the first choice and if carbamazepine (CBZ) can not be prescribed for each reason, sodium valproate was the second choice. Only EEGs were included which were done with 18 electrodes during wakefulness and HV (hyper ventilation) test, PS (photic stimulation) test had done. Results Population: In this study of 67 patients, 37 cases were male (55.2%) while 30 cases were female (44.8%). Mean age of onset was 6 years and all of our patients had the first seizure between 4-12 years. 42 cases (63%) had complete recovery by age of 12 years and all cases had complete recovery by age of 18 years. 25 cases had positive familial history which included 10 cases who had same epileptic attack in one of their parents (14.92%) and 15 cases had one affected sibling (22.39%) in summation 25 cases had positive family history (37.3%), [in 5 cases similar attack were seen both in one of parents and in one of siblings (7.46% )]. In assumption, positive family history in our study was 37.3%. 12 patients had FC (febrile convulsion) in their medical background (18%). 53 cases had only simple partial seizure (79.1%), 14 cases had secondary generalization (20.9%). Typically seizures occur on early phases of falling asleep, or on awakening. Events typical associate with BRE include speech arrest preservation of consciousness, unilateral colonic or tunic activities, excess saliva, seven cases (5%) had todd's palsy, three patients (4.5%) had status epilepticus during our study, 35 cases (52.2%) had only sleep attack, 11 cases had attacks exclusively on awakening 21 cases had both sleep and awaken attack (92.5%), 62 of patients had good response to carbamazepine (CBZ) but 5 patients

4 179 / Iran. J. Neurol. Vol.7; No. 21 & 22, Spring & Summer 2008 didn't tolerate CBZ or didn't have good response but had favorable response to valproate. Finally all patients had good response to one of these two drugs. all of our patients had at least one distinctive EEG pattern but all of them had basically normal background. Distinctive EEG in our patients was paroxysmal epileptic discharges which were accentuated in centrotemporal area. Epileptic discharges were confined to one hemisphere in 58.2% (39 cases), in other patients epileptic discharges were seen in two hemisphere symmetrically or asymmetrically ( 28 patients (41.8%)). In our study drug of choice which considered for BRE was CBZ which 62 cases had good response to it, but 5 cases (7.5%) had no favorable response or didn't tolerate CBZ but had good response to sodium valproate. Discussion Gastaüt was the first who described benign rolandic epilepsy (BRE) in (2) BRE is respected because of several unique characters. First; its benign course, nearly all seizure attacks will eventually ceased in affected persons by the end of second decade of life. Second; its excellent response to AEDs (anti epileptic drugs). Third; it is confined to a specific range of age. Fourth; it has partial relevance to genetic background. Fifth; it has a circadian diurnal rhythm which is seen less in other epileptic disorders (such as juvenile myoclonic epilepsy) ILAE considered it as a focal epilepsy. (5) BRE is primarily a focal seizure which has generalization potential especially in early sleep phase or on awakening from a deep sleep. Two studies (9,10) have reported that 20% of patients have seizure only on awakening and 15% of patients both on sleep and on awakening. In our study 54 of cases experienced seizure attack in sleep and in all of them the seizure was in early phase of falling asleep or in morning, near awakening. Other studies also confirmed our findings, beyond 50% of BREs cases had exclusively sleep attacks and all of them had seizure in early phase of falling asleep or near awakening. (7,11,12,13) 13 cases of our patients experienced seizure only in sleep period which 4 of them (near 6%) experienced seizure only soon after falling asleep and other experienced seizure both after falling asleep and before awakening. Other studies (8,9,14) showed 15-20% of patients had only sleep seizure. All studies confirm that sleep predisposes patient's condition for seizure, sleep lower threshold for neuronal hypersynchronous discharge and predisposes condition for generalization of focal discharges. BRE is basically a focal seizure with motor and sensory sign with generalization potential in most studies. (11) Some authors believe that a group of BREs are primary generalized but this is in contradiction with BRE character

5 180/ Benign Childhood Epilepsy with Centrotemporal then we rule out primary generalized seizure from our study. EEG of BRE patients have normal background with paroxysmal epileptic discharges which are accentuated in centrotemporal area. Discharges may seen individually or in cluster with frequency of 2 times per second. (5,7,11,14) Discharges can spread to frontal and temporal areas, confined to one hemisphere or spread to other hemisphere. (15,16) BRE is considered as a benign disease, thus some authors believe that it dose not need treatment with antiepileptic drugs (AEDs) or if there is indication for treatment we must use lowest doses of AEDs. (10,12) However Blom et al, (17) reported that few patients were refractory to AEDs but in our study all patients responded to AEDs. All of studies including Monjauze. (18) Bouma (9) indicate that the prognosis of BRE is excellent, in our study in all cases seizure attacks ceased by age of 18. Frequent and sever seizure attacks doesn't indicate poor prognosis. (7,11,19) In our study 30% of cases (20 cases) had complete remission by age of 5 years, 42 cases (63%) by age of 12 year and all cases by age of 18. In Z.H. Zhauang (15) and Massa (14) studies showed that near 90% of cases recovered completely by end of adolescence. In our study 25 cases had positive familial history which included 10 cases which had one affected parents and 15 cases had at least one affected sibling. Other studies also indicate significant positive family history [about 15%], then it can be concluded that a heritable factor should be found which need further genetic studies for identification of its precise genetic locus. (11,15) 18 percent of our cases had at least one febrile convulsion (FC) attack in their background and by considering of high frequency of FC in 2-6 years child, there is not significant relation between BRE and FC. Other studies, (13,16,19) also could not find significant relation between FC in childhood period and later BRE. In our study in 28 cases (41.8%), discharges were confined to one hemisphere and in 39 cases discharges were seen in both hemispheres synchronically or asynchronically. Some other studies (9,13,17) also show similar results, but Lerman (7) reported that 60% of seizure had unilateral focus whereas 40% showed bilateral discharges. Gregory et al (8) found that the spikes were likely generated in lower Rolandic region. In Messa study which included 60 cases he determined six distinctive interictal patterns of EEG. (14) Our study showed that about 20.9% (14 cases) had scattered epileptic discharges in spite of beyond two years of complete recovery. Other studies (16,18) indicate that complete recovery may achieve without complete EEG normalization. These

6 181 / Iran. J. Neurol. Vol.7; No. 21 & 22, Spring & Summer 2008 can be concluded that BRE patients can achieve recovery but their threshold for epileptic discharges or seizures in other seizure amenable situations is lower in comparison to other wise normal persons. EEG pattern of centrotemporal spike waves is characteristic for BRE but not pathogonomic because in Rett syndrome (fragile " X ") and sometimes in tumor lesions there is similar EEG pattern, but normal physical examination, normal imaging can differentiate BRE from other lesions. Normal imaging, normal physical examination is essential for diagnosis. Reference 1- Pazzaglia P, D Alessandro R, Lozito A, Lugaresi E. Classification of partial epilepsies according to the symptomatology of seizures:practical value and prognostic implications. Epilepsia 1982;23:pp Staffan Lundberg, Orvar Eeg-Olofsson. Rolandic epilepsy: a challenge in terminology and classification, European Journal of Paediatric Neurology 2003; 7: pp Connolly AM, Northcott E, Cairns DR, McIntyre J, Christie J, Berroya A, Lawson JA, Bleasel AF, Bye AME. Quality of life of children with benign rolandic epilepsy. Pediatr Neurol 2006;35:pp Katrin Larsson, Orvar Eeg-Olofsson. A population based study of epilepsy in children from a Swedish county. European Journal of Paediatric Neurology 2006;10: pp Bradley Walter G, Robert B Daroff, Jerald M Fenichel and Joseph Jahrovic. Neurology of clinical practice. 4 th Ed. 2004: pp David A Greenberg, Michael Aminoff and Roger P Simon. Clinical neurology. Appleton& Lange. 6 th Ed. 2007: pp P. Lerman, Benign partial epilepsy with centrotemporal spikes. In: J. Roger, M. Bureau. C. Dravet, F.E, Dreifuss, A. Perret and P. Wolf, editors, epileptic syndromes in infancy childhood and adolesecne, John Libby Eurotext, London 1992; pp D.L. Gregory and P.K. Wong, Clinical relevance of a dipole field in Rolandic spikes, Epilepsia 1992; 33: pp P.A. Bouma, A.C. Bovenkerk, R.G. Westendorp and O.F. Brouwer, The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis, Neurology 1997; 48: pp G.L. Holmes, benign focal epilepsies of childhood, Epilepsia 1993; 34: (Suppl. 3), pp. s49-s Xiuhe Zhao, Zhaofuchi, Department of neurology, Qthi hospital, Shanding University, China, 16 May E. Northcott, A.M. Connolly, A. Berroya, M. sabaz, J. McIntyre and J. Christie et al., The neuropsychological and language profile of children with benign Rolandic epilepsy, Epilepsia 2005; 46: pp I. Drury and A. Beyond, Benign partial epilepsy of childhood with monomorphic sharp waves in centrotemporal and other locations, Epilepsia 1991; 32: pp R. Massa, A. de Saint-Martin, R. Carcangiu, G. Rudolf, C. Seegnuller and C. Kleitz et al, EEG criteria predictive of complicated evolution in idiopathic Rolandic epilepsy, Neurology 2001; 57:pp

7 182 / Benign Childhood Epilepsy with Centrotemporal 15- Z.H. Zhuang, Z.M. Huang, Y.Q. Chen and Y.X. Xie, clinical and EEG characteristics of benign epilepsy of childhood with centro-temporal spikes [in Chinese], J Chin Physician 2004; 6: pp L.Y. Jin, L.W. Wu, W. Gao, X.Q. Shao and L.K. Ren, Features of clinical and EEG changes during follow-up in patients with benign epilepsy of childhood with central-temporal spikes [in Chinese], Chin J Neurol 2002; 35: pp S. Blom and J. Heijbel, Benign epilepsy of children with centrotemporal EEG foci: a follow-up in adulthood of patients initially studied as children, epilepsia 1982; 23: pp C. Monjauze, L. Tuller, C. Hommet, M.A. Barthez and A. Khomsi, Language in benign childhood epilepsy with centro-temporal spikes abbreviated form: Rolandic epilepsy and language, Brain Lang 2005;92: pp C.K. Ma and K.Y. Chan, Benign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children, Brain Dev 2003; 25: pp