EPILEPSY IN INFANCY. AK Gururaj, R Pratap Chand, K E Choo SING MED J. 1988; 29: ABSTRACT

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1 EPILEPSY IN INFANCY AK Gururaj, R Pratap Chand, K E Ch ABSTRACT The clinical and electrencephalgraphic (EEG) filatures f epilepsy in the first year f life were studied in 5 infants admitted with a histry suggestive f seizures. The age incidence shwed bimdal peaks at and mnths. Parial seizures with r withut secndary generalisatin were seen in 67.% and primary generalised seizures in.7% f cases. Cmplex partial seizure with secndary generalisatin was the single cmmnest seizure type seen in infancy. A significant develpmental delay was seen in 6.5% f cases. The clinical and electrencephalgraphic features, underlying aetilgical factrs, and respnse t anticnvulsant therapy are described. KEY WORDS Epilepsy, Electrencephalgraphy. SING MED J. 988; 9: - 7 INTRODUCTION The reprts n epilepsy in infancy deal mstly with nenatal seizures and the ccurence f infantile spasms in the later half f infancy. The first half f infancy has been refered t as a silent perid fr epilepsy (). Sme f these cases, partial seizures in particular, may escape diagnsis if nt investigated by electrencephalgraphy (EEG) since the clinical presentatin may be cmplex and atypical (). There is a high "cnvulsive threshld" in early infancy and epilepsy at this age is ften symptmatic f a serius underlying brain disrder r abnrmality. There is a paucity f literature n the patterns f seizures in infancy. The present study describes the clinical and EEG features in infantile seizures seen in Kelantan, Malaysia. MATERIALS AND METHODS The material cnsisted f 5 infants frm newbrn perid up t the age f year, admitted fr epilepsy t Hspital Universiti Sains Malaysia frm August 98 t March 987. Patients with febrile fits were excluded frm the study. All cases were assessed by a detailed histry, neurlgical examinatin and EEG. Develpmental assessment was dne in all the cases using Denver develpmental scale. Investigatins such as lumbar puncutre, skull x-ray, CT Scans and a limited metblic screening including urine fr amin acidurias and PKU were dne if indicated. Serum electrlytes, bld sugar and calcium were dne in all the cases. The seizures were classified based n the Internatinal Classificatin f Epilepsies (98)() by analysis f clinical and EEG features. Anticnvulsants were administered in all cases and the respnse analysed. Department f Paediatrics Hspital Universiti Sains Malaysia Kubang Kerian 65 Kelantan Malaysia. A K Gururaj MBBS DCH MD M.MED (PAED) MRCPI. AM. K E Ch AM MBBS FRCP (PAED) Department f Medicine Hspital Universiti Sain Malaysia R PRATAP CHAND. MBBS DM (NEURO) RESULTS Out f the 688 cases admitted during the perid t Paediatric Wards, there were 5 cases (%) f infantile epilepsies. The age incidence is shwn in fig.. A bimdal distributin was seen with maximum incidence at and mnths f age. The breakdwn f the different seizure types in shwn in table. A striking feature was the high incidence f cmplex partial seizures especially at the age f t 5 mnths. A significant develpmental delay was seen in 6% f partial seizures and 7.5% f generalised seizures. All three cases f infantile spasms had significant mental retardatin. The clinical manifestatins f cmplex partial seizures were atypical in mst f the cases and are shwn in table. Identifiable aetilgical factrs were seen 5.% f partial seizures and 7.5% f generalised seizures (table ). The EEG shwed primary generalised seizure discharges in 7 cases (fig. ) secndary generalisatin was bserved in 7 infants. Fcal tempral seizure discharges were seen in 7% f the cases with partial (fig. ) seizures with r withut secndary generalisatin. Hypsarrhythmia was present in ut f cases diagnsed as infantile spasms. CT Scans dne in 7 ut f 5 cases f partial seizures shwed significant brain atrphy in 7, hydrcephalus in, hydranencephaly in and frnttempral infarctin in ; in cases, the scans were nrmal. In 5 cases with primary generalised epilepsy, CT Scan was nrmal in and revealed subdural fluid cllectin in. A detailed metablic wrkut was nt dne fr any f these cases since the facilities were nt available. Hwever, urine fr phenyl ketnuria and chrmatgraphy dne in selected cases were essentially nrmal. Bld urea, electrlytes, serum clacium and bld glucse were rutinely perfrmed, and were within nrmal range. The infants were treated with a single r multiple drugs including phenbarbitne, carbamazepine, dilantin, clnazepam and sdium valprate. Cmbinatin therapy was given in % f partial seizures and an equal number f generalised seizures..7% f cases were lst t fllw up. Of thse wh were fllwed up, seizures were well cntrlled in 75 / f the infants in bth grups. The rest did nt shw significant cntrl f seizures. DISCUSSION The prevalence f seizures ther than infantile spasm in the first year f life has nt been widely dcumented.

2 JO FIG Shws the age incidence f ed seizures which reveals a partial and generalisbimdal pattern. Age f Presentatin N y p 6 PARTIAL A 9 6 Ji GENERALISED G Y S 5 b LL 5 AGES IN MONTHS / 8 9 Table CLASSIFICATION OF SEIZURES IN 5 INFANTS PARTIAL SEIZURES [(NO = 5 (67.%)]. Simple partial seizures 5(9.6%).. Cmplex partial seizures (5.7%).. Cmplex partial seizures with secndary generalisatin 7(5.9%) PRIMARY GENERALISED SEIZURES [(NO = 7 (.7%)]. Generalised tnic/clnic/tnic clnic seizures (6.9%).. Infantile spasms (5.7%). in the early infancy are difficult t recgnise and are ften partial, fragmented and disrganized (). Recently there has been an increasing awareness that significant numbers f cases f tempral lbe epilepsy have their nset in early childhd (). The incidence f partial seizures in the infancy fund in ur study is high, with the highest incidence f the partial seizures in the first 6 mnths f life. The presence f significant develpmental retardatin is partial seizures during infancy has been dcumented by O'Dnhe(5). This is cnfirmed by the findings f ur study where 6% f infants with partial seizures and 7.5/ f infants with primary generalised seizures and significant develpmental retardatin. It is interesting t nte that all the cases With infantile spasms had develpmental delay. The aetilgy f infantile seizures is debatable. Althugh the initial suggestins by Penfield that in mst f the cases f tempral lbe epilepsy, birth asphyxia played a majr rle in prducing scarring f the tempral lbe have been strengthened by many studies(6), recently there have been a number f large scale studies, (),(7) which questin the relatinship between lw apgar scres and seizures in childhd. In ur study, the mst strik- ing feature has been a dcumented birth asphyxia, r ther perinatal factrs such as nenatal meningitis, nenata hypglycemia, kernicterus and intra uterine infectin; they were respnsible fr the infantile seizures in 59.6% f the cases. This is in keeping with the bservatin by O'Dnhe that seizures in early infancy are ften symptmatic f underlying structural brain disease(). In the state f Kelantan where this study was cnducted, the infant mrtality rate is believed t be 8/ live births and is knwn t be the highest amngst the states f Malaysia; yearly mre than 8 ut f 5, deliveres are cnducted at hmes by unskilled village midwives, the remaining 7 deliveries take place in hspitals. The ccurrence f birth asphyxia amng these babies delivered at hme is pssibly significantly high. This is cnfirmed by the fact that 7/ f the ttal admissins t the sick nursery was frmed by new-brn with birth asphyxia r respiratry distress. Bth partial and generalised seizures were fund t be the sequalae f significant perinatal brain damage in 56% f cases f ur study. The high incidence f brain atrphy in the CT Scans is supprtive f the fact. Frank infarctin was fund in nly tw cases, as against brain atrphy, which was fund in 7 ut f 9 cases f partial seizures by CT Scans. When the clinical descriptin f fits is studied, it is bvius that mst f the cases f cmplex partial seizures can be missed if nt subjected t Electrencephalgraphy Althugh generlised tnic/clnic r bth were fund in ut f 5 cases f partial seizures, blue spells, apnic spells and sudden pallr secndary t vasmtr phenmena were fund in % f cmplex partial seizures; these findings, and the thers such as staring spells, atnic spells, cnfusinal states and autmatism have been described as a cmmn feature in tempral lbe seizures, (),(9). Hwever, all three infants with infantile spasm had the characteristic flexr spasms. In primary generalised seizures, autnmic r autmatic features were uncmmn. Electrencephalgraphy plays a very imprtant rle in the diagnsis f seizures in infancy. It is an essential investiga -

3 Table CLINICAL DESCRIPTION OF FITS Partial Primary Generalised Seizrues Generalised Tnic/Clnic r bth 5 Flexr Spasm Blue Spells/Apnic Spells/ Sudden Pallr Staring Spells Atnic Spells Jitteriness Lip Smacking Cnfusinal States Vacant Smiling Up Rlling f Eye Balls (Withut Gen Fits) Vmiting with Fits Blank Lk Clenching f Fists Extensr Spasm FIG : EEG shwing generalised spike discharges. Table IDENTIFIABLE AETIOLOGICAL FACTORS - Birth Asphyxia - Nnatal Meningitis - - Nenatal Jaundice Nenatal Hypglycemia 5 - Cngenital Hydrcephalus 6 - Hydranencephaly 7 - Intra Uterine Infectins (CMV) PartialL 8 _ Generalised Ttal = 9 Ttal = 5 tin prviding supprtive evidence fr clinical diagnsis f epilepsy by demnstratin f epilepsy discharges(), althugh nrmal EEGs d nt exclude the presence f a seizure disrder. Frtunately, epileptic discharges are mre readily recrdable in infantile r childhd epilepsy(). In ur study the EEG revealed evidence r epilepsy in all the cases studied and was f immense value in detecting seizure type especially when seizure phenmena were atypical. Amng the drugs used fr management f epilepsy in infants, we fund carbamazepine and phenbarbitne extremely useful fr bth partial as well as primary generalised seizures. Carbamazepine was free f significant side effects unlike pehenbarbitne which tended t prduce drwsiness r hyperkinesis. The ther drugs used included Dilantin, Clnazepam and Sdium valprate. Generally, infants with primary generalised seizures respnded better; 5% f infants with partial seizures and.7% f generalised seizures prved t be difficult t cntrl with varis drug cmbinatins. Nearly ne third f all the cases were lst fr fllwup. Hwever, an verall pr prgnsis is a dcumented feature in infantile seizures (), (). 5

4 -art T I. I Y rr-r j- YYrrrrr -L7-7\FPI - Fr F7- ' T_T5 T5 - ' W V OI_ -T5 TB -T Tt Fd F8 _ FP _ FPI F _C C _P F - C,.. /.. /l,j '... C-P FIG : EEG shwing sharp waves in phase reversal acrss T indicating a tempiral lbe fcus. REFERENCES. O'Dnhe NV. Cnvulsins in early infancy and infantile spasms. In: Epilepsies f Childhd. Lndn; Butterwrth and C, Publishers, 985: -7.. Feindel W. Tempral lbe seizures. In: Vinken PJ, Bruyn GW. eds. Handbk f clinical Neurlgy: Vl: 5: Epilepsy, Amsterdam; Nrth Hlland Publishers, 977: Nelsn KB, Ellenberg JH: Antecedents f seizures disrders in early childhl: JAMA SEA Supplement. N.. 987: 6-.. Lee I.S. Electrencephalgraphy in infantile and Childhd Epilepsy. In: Dreifus FE. (Ed). Paediatric Epileptlgy. Bstn; Jhn Wright/PSG Inc. 98: -6. 6

5 5. O'Dnhe NV. Cmmunicatins t the British Paediatric Neurlgy study grup, Brightn. 97. Unpublished. 6. Brwn IA, Frunch LA, Ogle WS, Jhnsn S.: Tempral lbe epilepsy: Its Clinical Manifestatins and surgical treatment. Medicine: 966; 5: Rcca WA, Sharcrugh FW, Hausen WA, Annegers JF, Schenberg BS.: Risk Factrs fr Cmplex partial seizures. A ppulatin based case cntrl study. Ann.Neurl 987; : Gld AR: Psychmtr Epilepsy in Childhd. Pediatrics 97; 5 (): Oka E, Ogin T, Kbayashi K.: Cmplex partial seizures in Childhd. Fli Psychiatr Neurl Jpn 985; 9 (): Cmmissin n classificatin and Terminlgy, Internatinal League against Epilepsy: Prpsed revisins f clinical and electr encephalgraphic classificatin f epileptic seizures. Epilepsia 98; : Dinner, Luders H, Rthner A D, Erenberg G.: Cmplex partial seizures f Childhd nset: A clinical and Encephalgraphic study. Cleveland Clinic Quarterly 98; 5: Duchwny M S. Cmplex partial seizures in Infancy. Arch Neurl 987; : 9-. 7