Clinical profile and treatment outcome of epilepsy syndromes in children: A hospital-based study in Eastern Nepal

Abstract

Objective: It is often difficult to diagnose epilepsy syndromes in resource-limited settings. This study was aimed to investigate the prospect of ascertaining the diagnosis, clinical profile, and treatment outcomes of epilepsy syndromes (ESs) among children in a resource-limited setting.

Methods: This was a descriptive study done from 01/07/2009 to 15/06/2017 among children (1-17 years of age) with unprovoked seizures presenting to the pediatric neurology clinic of a university hospital in eastern Nepal. Diagnosis, classification, and treatment of seizures were based upon International League Against Epilepsy guidelines.

Results: Of 768 children with unprovoked seizures, 120 (15.6%) were diagnosed as ES. The age of onset of seizure was unique for each ES. Developmental delay and cerebral palsy were present in 47.5% and 28.3% children, respectively. Common ESs were West syndrome (WS)-26.7%, generalized tonic-clonic seizures alone (GTCSA)-21.7%, self-limited childhood epilepsy with centrotemporal spikes (SLCECTS)-12.5%, childhood absence epilepsy (CAE)-10.0%, Lennox-Gastaut syndrome (LGS)-10.0%, other developmental and epileptic encephalopathies (DEE)-5.8%, self-limited familial infantile epilepsy (SLFIE)-4.2%, and juvenile myoclonic epilepsy (JME)-3.3%. Among children with known outcomes (87/120), overall response to pharmacotherapy and to monotherapy was observed in 72.4% (63/87) and 57.5% (50/87) children, respectively. All children with GTCSA, SLFIE, genetic epilepsy with febrile seizure plus (GEFS+), CAE, SLCECTS, and JME responded to pharmacotherapy and they had normal computerized tomography scans of the brain. Seizures were largely pharmaco-resistant in progressive myoclonus epilepsy (PME)-100.0%, LGS-73.0%, WS-52.0%, and other DEEs-40%.

Significance: A reasonable proportion (15.6%) of unprovoked seizures could be classified into specific ES despite limited diagnostic resources. WS was the most common ES. GTCSA, SLCECTS, CAE, and LGS were other common ESs. GTCSA, SLFIE, CAE, SLCECTS, GEFS+, and JME were largely pharmaco-responsive. PME, WS, and LGS were relatively pharmaco-resistant. Electro-clinical diagnosis of certain ES avoids the necessity of neuroimaging.

Keywords: child; epilepsy; epileptic syndromes; seizure; treatment outcome.

FIGURE 1

Median age of onset (months and IQR^#) of seizure in various epilepsy syndromes. ^#‐IQR, interquartile range; *‐IQR not shown because there was a single child in each category. Abbreviations: JME, juvenile myoclonic epilepsy; CSWS, epileptic encephalopathy with continuous spike‐and‐wave during sleep; SLCECTS, self‐limited childhood epilepsy with centrotemporal spikes; GTCSA, generalized tonic‐clonic seizures alone; CAE, childhood absence epilepsy; MTLE, mesial temporal lobe epilepsy; PME, progressive myoclonus epilepsy; EMA, epilepsy with myoclonic absences; DEE, other developmental and epileptic encephalopathies; LGS, Lennox‐Gastaut syndrome; GEFS+, genetic epilepsy with febrile seizure plus; WS, West syndrome; and SLFIE, self‐limited familial infantile epilepsy

FIGURE 2

Final seizure control status in children with epilepsy syndromes