Background: The epileptic encephalopathies collectivelyexact an
immense personal, medical, and financial toll onthe
affected children, their families, andthe
healthcare system. Objective: This study was aimed to delineate the
clinical spectrum of patients with Epileptic encephalopathies (EEs) and
classify them under various epileptic syndromes. Methods: This was a
cross-sectional study that was carried out in the department of Neurophysiology
of the National Institute of Neurosciences and Hospital, Bangladesh from July
2016 to June 2019.Children with recurrent seizures which were difficult to control and associated with developmental arrest or
regression in absence of a progressive brain pathology were considered to be
suffering from EE. Children under 12 years of age fulfilling the inclusion
criteria were enrolled in the study. These patients were evaluated clinically
and Electroencephalography (EEG) was done in all children at presentation.
Based on the clinical profile and EEG findings the patients were categorized
under various epileptic syndromes according to International League Against
Epilepsy (ILAE) classification 2010. Results: A total
References
[1]
Berg, A.T., Berkovic, S.F., Brodie, M.J., Buchhalter, J., Cross, J.H., van Emde Boas, W., Engel, J., French, J., Glauser, T.A., Mathern, G.W. and Moshé, S.L. (2010) Revised Terminology and Concepts for Organization of Seizures and Epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia, 51, 676-685. https://doi.org/10.1111/j.1528-1167.2010.02522.x
[2]
Jain, P., Sharma, S. and Tripathi, M. (2013) Diagnosis and Management of Epileptic Encephalopathies in Children. Epilepsy Research and Treatment, 2013, Article ID: 501981. https://doi.org/10.1155/2013/501981
[3]
Dulac, O. (2001) Epileptic Encephalopathy. Epilepsia, 42, 23-26.
https://doi.org/10.1046/j.1528-1157.2001.042suppl.3023.x
[4]
Nabbout, R. and Dulac, O. (2003) Epileptic Encephalopathies: A Brief Overview. Journal of Clinical Neurophysiology, 20, 393-397.
https://doi.org/10.1097/00004691-200311000-00002
[5]
Oka, E., Ishida, S., Ohtsuka, Y. and Ohtahara, S. (1995) Neuroepidemiological Study of Childhood Epilepsy by Application of International Classification of Epilepsies and Epileptic Syndromes (ILAE, 1989). Epilepsia, 36, 658-661.
https://doi.org/10.1111/j.1528-1157.1995.tb01042.x
[6]
MacAllister, W.S. and Schaffer, S.G. (2007) Neuropsychological Deficits in Childhood Epilepsy Syndromes. Neuropsychology Review, 17, 427-444.
https://doi.org/10.1007/s11065-007-9048-4
[7]
Holmes, G.L. and Lenck-Santini, P.P. (2006) Role of Interictal Epileptiform Abnormalities in Cognitive Impairment. Epilepsy & Behavior, 8, 504-515.
https://doi.org/10.1016/j.yebeh.2005.11.014
[8]
Chugani, H.T. (1995) Infantile Spasms. Current Opinion in Neurology, 8, 139-144.
https://doi.org/10.1097/00019052-199504000-00010
[9]
Khan, N.Z., Muslima, H., Begum, D., Shilpi, A.S. and Akhter, S. (2010) Rapid Neurodevelopmental Assessment: Validation of a New Tool for Functional Evaluation of 0-24 Month Old Children in Bangladesh. Pediatrics, 125, 755-762.
https://doi.org/10.1542/peds.2008-3471
[10]
Khan, N.Z., Muslima, H., Shilpi, A.B., Begum, D., Parveen, M., Akter, N., Ferdous, S., Nahar, K., McConachie, H. and Darmstadt, G.L. (2013) Validation of Rapid Neurodevelopmental Assessment for 2- to 5-Year-Old Children in Bangladesh. Pediatrics, 131, 486-494. https://doi.org/10.1542/peds.2011-2421
[11]
Kalra, V., Gulati, S., Pandey, R.M. and Menon, S. (2002) West Syndrome and Other Infantile Epileptic Encephalopathies—Indian Hospital Experience. Brain and Development, 24, 130-139. https://doi.org/10.1016/S0387-7604(02)00029-3
[12]
Ohtahara, S., Ohtsuka, Y. and Oka, E. (1997) Epileptic Encephalopathies in Early Infancy. The Indian Journal of Pediatrics, 64, 603-612.
https://doi.org/10.1007/BF02726112
[13]
Baram, T.Z. (1999) Myoclonus and Myoclonic Seizures. In: Swaiman, K.F. and Ashwal, S., Eds., Pediatric Neurology: Principles and Practice, 3rd Edition, Mosby Inc., St Louis, 668-675.
[14]
Ohtsuka, Y., Ogino, T., Murakami, N., Mimaki, N., Kobayashi, K. and Ohtahara, S. (1986) Developmental Aspects of Epilepsy with Special Reference to Age-Dependent Epileptic Encephalopathy. Psychiatry and Clinical Neurosciences, 40, 307-313.
https://doi.org/10.1111/j.1440-1819.1986.tb03152.x
[15]
Udani, V.P., Dhamidharka, V., Nair, A. and Oka, M. (1993) Difficult to Control Epilepsy in Childhood—A Long Term Study of 123 Cases. Indian Pediatrics, 30, 1199-1206.
[16]
Banu, S.H., Khan, N.Z., Hossain, M., Ferdousi, S., Boyd, S., Scott, R.C. and Neville, B. (2012) Prediction of Seizure Outcome in Childhood Epilepsies in Countries with Limited Resources: A Prospective Study. Developmental Medicine & Child Neurology, 54, 918-924. https://doi.org/10.1111/j.1469-8749.2012.04325.x
[17]
Seth, A., Aneja, S. and Taluja, V. (2001) Epileptic Encephalopathies of Early Childhood. Indian Pediatrics, 38, 390-396.
[18]
Chandra, S. and Kumar, R. (2016) Clinico-Aetiological Profile and Outcome of West Syndrome from North India. Hong Kong Journal of Paediatrics (New Series), 21, 262-265.
[19]
Kaushik, J.S., Patra, B., Sharma, S., Yadav, D. and Aneja, S. (2013) Clinical Spectrum and Treatment Outcome of West Syndrome in Children from Northern India. Seizure, 22, 617-621. https://doi.org/10.1016/j.seizure.2013.04.014
[20]
Matsuo, A., Matsuzaka, T., Tsuru, A., Moriuchi, H., Nakashita, Y., Tanaka, S., Baba, C. and Tomimasu, K. (2001) Epidemiological and Clinical Studies of West Syndrome in Nagasaki Prefecture, Japan. Brain and Development, 23, 575-579.
https://doi.org/10.1016/S0387-7604(01)00267-4
[21]
Kalra, V.N. and Passi, G.R. (1998) Analysis of Childhood Epileptic Encephalopathies: Neuroradiologic Aspect of West Syndrome. Pediatric Neurology, 19, 1-8.
[22]
Mackay, M.T., Weiss, S.K., Adams-Webber, T., Ashwal, S., Stephens, D., Ballaban-Gill, K., Baram, T.Z., Duchowny, M., Hirtz, D., Pellock, J.M. and Shields, W.D. (2004) Practice Parameter: Medical Treatment of Infantile Spasms: Report of the American Academy of Neurology and the Child Neurology Society. Neurology, 62, 1668-1681. https://doi.org/10.1212/01.WNL.0000127773.72699.C8
[23]
Tsuji, T., Okumura, A., Ozawa, H., Ito, M. and Watanabe, K. (2007) Current Treatment of West Syndrome in Japan. Journal of Child Neurology, 22, 560-564.
https://doi.org/10.1177/0883073807302606
[24]
Matsumoto, A., Watanabe, K., Negoro, T., Sugiura, M., Iwase, K., Hara, K. and Miyazaki, S. (1981) Infantile Spasms: Etiological Factors, Clinical Aspects, and Long Term Prognosis in 200 Cases. European Journal of Pediatrics, 135, 239-244.
https://doi.org/10.1007/BF00442097
[25]
Cusmai, R., Ricci, S., Pinard, J.M., Plouin, P., Fariello, G. and Dulac, O. (1993) West Syndrome Due to Perinatal Insults. Epilepsia, 34, 738-742.
https://doi.org/10.1111/j.1528-1157.1993.tb00455.x
[26]
Kalra, V. and Passi, G.R. (1998) Analysis of Childhood Epileptic Encephalopathies with Regard to Etiological and Prognostic Factors. Brain and Development, 20, 14-17. https://doi.org/10.1016/S0387-7604(97)00080-6
[27]
Wallace, S.J. (1993) Seizures in Children. In: Laidlaw, J., Richens, A. and Chadwick, D., Eds., A Textbook of Epilepsy, 4th Edition, Churchill Livingstone, Edinburgh, 77-164.
[28]
Kotagal, P. (1995) Multifocal Independent Spike Syndrome: Relationship to Hypsarrhythmia and the Slow Spike-Wave (Lennox-Gastaut) Syndrome. Clinical Electroencephalography, 26, 23-29. https://doi.org/10.1177/155005949502600105
[29]
Yamatogi, Y. and Ohtahara, S. (2006) Multiple Independent Spike Foci and Epilepsy, with Special Reference to a New Epileptic Syndrome of “Severe Epilepsy with Multiple Independent Spike Foci”. Epilepsy Research, 70, S96-S104.
https://doi.org/10.1016/j.eplepsyres.2006.01.013
[30]
Mohammad, Q.D., Saha, N.C., Alam, M.B., Hoque, S.A., Islam, A., Chowdhury, R.N., Hussain, M.E., Chowdhury, Y.S., Hossain, S., Chowdhury, M.A. and Rahman, M. (2020) Prevalence of Epilepsy in Bangladesh: Results from a National Household Survey. Epilepsia Open, 5, 526-536. https://doi.org/10.1002/epi4.12430
[31]
Hwang, S.K. and Kwon, S. (2015) Early-Onset Epileptic Encephalopathies and the Diagnostic Approach to Underlying Causes. Korean Journal of Pediatrics, 58, 407.
https://doi.org/10.3345/kjp.2015.58.11.407
[32]
Alam, S. and Lux, A.L. (2012) Epilepsies in Infancy. Archives of Disease in Childhood, 97, 985-992. https://doi.org/10.1136/archdischild-2011-301119
