Abstract
To study the profile of West syndrome (WS) in North Indian Children, 165 cases of
WS were analyzed. Details of seizure semiology, prenatal and perinatal events, developmental
milestones, treatment received, physical and neurological examination and investigations
were recorded. The response of seizures to various therapeutic modalities and the
final developmental status were taken as primary outcome variables. Analysis was done
to find the factors influencing these outcome variables. The age of onset of infantile
spasms ranged from 1 to 19 (mean 6.1±3.4) months. Age at presentation ranged from
1.5 months to 4.5 years (mean 14.7±11.4 months); 74% had flexor spasms. Other types
of seizures were associated in 31 children. Antenatal problems and adverse perinatal
events were reported in 26.7 and 59.4%, respectively. Developmental delay was recognized
in 69.7% prior to and in 27.9% after onset of spasms. Microcephaly was seen in 72.7%.
Interictal EEG showed hypsarrhythmia in 44; generalized spike and slow waves in 31%
and burst suppression in 7%. Computed tomography scan done in 94 cases showed cerebral
atrophy in 15%, infarcts in 8%, tubers in 7%, developmental malformations in 5%. Magnetic
resonance imaging done in 77 cases showed periventricular T2WI white matter hyper
intensities in 33.8% and cerebral atrophy in 21%. Prednisolone and ACTH were used
in 57 and 35 cases, respectively. Complete control of seizures was seen in 49 and
46% cases. No significant difference in seizure control or developmental outcome was
found in the two groups. Overall, 42.4, 30.9 and 16% children showed complete, partial
and no control of seizures. After therapy, developmental improvement was seen in 55.8%
and no change in 23.6% cases. The type of spasms had no correlation with the other
parameters including etiology, seizure or developmental outcome. An early age of onset
correlated with presence of antenatal problems (P<0.05). Seizure control and developmental improvement correlated significantly (P<0.005). Developmental outcome was better in cryptogenic as compared to symptomatic
cases (P<0.05). No other significant correlations were found. In India WS is often diagnosed
late because of lack of awareness. Adverse perinatal events are important etiological
factors. Nonaffordability of ACTH and Vigabatrin prompts the use of prednisolone in
most cases.
Keywords
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References
- Proposal for the revised classification of epilepsies and epileptic syndromes.Epilepsia. 1989; 30: 389-399
- Infantile spasms: West syndrome.Arch Neurol. 2002; 59: 317-318
- History of clinical identification of West syndrome—in quest after the classic.Brain Dev. 2001; 23: 779-787
- West's syndrome (infantile spasms). Clinical description and diagnosis.Adv Exp Med Biol. 2002; 497: 33-50
- Long-term prognosis of patients with West syndrome in Japan: social aspects.Brain Dev. 2001; 23: 695-697
- West syndrome: long-term prognosis and social aspects.Brain Dev. 2001; 23: 688-691
- Long-term outcome of patients with West syndrome.Brain Dev. 2001; 23: 683-687
- Incidence and aetiology of infantile spasms from 1960 to 1976: a population study in Finland.Dev Med Child Neurol. 1979; 21: 333-343
- A risk–benefit assessment of treatments for infantile spasms.Drug Saf. 2001; 24: 813-828
- What is West syndrome?.Brain Dev. 2001; 23: 447-452
- West syndrome and other infantile epileptic encephalopathies—Indian hospital experience.Brain Dev. 2002; 24: 130-139
- West's syndrome.J Child Neurol. 2002; 17: S76-S79
- Infantile spasms.in: Pendley T.A. Meldrum B.S. Recent advances in epilepsy-I. Churchill Livingstone, Edinburgh1983: 113-138
- Infantile spasms: etiologic factors, clinical aspects and long term prognosis in 200 cases.Eur J Pediatr. 1981; 135: 239-244
- A long term follow up study of 214 children with the syndrome of infantile spasms.Neuropediatrics. 1982; 13: 14-23
- Infantile spasms: unique syndrome or general age-dependent manifestation of a diffuse encephalopathy?.Int Rev Neurobiol. 2002; 49: 57-62
- Neurophysiology of spasms.Brain Dev. 2001; 23: 467-472
- Epidemiological and clinical studies of West syndrome in Nagasaki Prefecture Japan.Brain Dev. 2001; 23: 575-579
- Pathophysiology of infantile spasms.Adv Exp Med Biol. 2002; 497: 111-121
- West syndrome—the University of Hong Kong experience (1970–2000).Brain Dev. 2001; 23: 609-615
- The efficacy of corticotropin in primary IS.J Pediatr. 1982; 101: 294-296
- Prospective study of outcome of infants with infantile spasms therapy during controlled studies of adrenocorticotropic hormone and prednisolone.J Pediatr. 1988; 112: 389-396
- Ictal, clinical electroencephalographic findings of spasms in West Syndrome.Epilepsia. 1993; 34: 671-678
- Symptomatology of infantile spasms.Brain Dev. 2001; 23: 453-466
- Predicting favourable outcome in idiopathic West syndrome.Epilepsia. 1993; 34: 743-746
- Ictal pattern of EEG and muscular activation in symptomatic infantile spasms.Epilepsia. 2002; 43: 1559-1563
- Assymetric and asynchronous infantile spasms.Epilepsia. 1995; 36: 873-882
- Reappraisal of interictal EEGs in infantile spasm.Epilepsia. 1993; 34: 679-685
- West syndrome and Lennox Gestaut syndrome: a survey of natural history.Pediatrics. 1980; 65: 81-88
- West syndrome in Thailand: a hospital-based survey.Brain Dev. 2001; 23: 605-608
- West syndrome: a university hospital based study from Oman.Brain Dev. 2001; 23: 586-592
- Epidemiology of West syndrome in Singapore.Brain Dev. 2001; 23: 584-585
- Epidemiological and clinical study of West syndrome in Nagasaki Prefecture, Japan.No To Hattatsu (Tokyo). 2001; 33: 15-20
- Periventricular leukomalacia and West syndrome.Dev Med Child Neurol. 1996; 38: 13-18
- Cerebral magnetic resonance study in the study of West syndrome.Rev Neurol. 1999; 28: 685-687
- Histopathology of brain tissue from patients with infantile spasms.Int Rev Neurobiol. 2002; 49: 63-76
- Clinical and electroencephalographic study of infantile spasms.Indian J Pediatr. 1983; 50: 515-518
- Prenatal etiologies of West syndrome.Epilepsia. 1993; 34: 716-722
- Neuroradiological aspects of West syndrome.Pediatr Neurol. 1998; 19: 211-216
- Neuroradiological assessment of brain structure and function and its implication in the pathogenesis of West syndrome.Brain Dev. 2001; 23: 488-495
- Informative value of magnetic resonance imaging and electroencephalography in the prognosis of infantile spasms.Epilepsia. 2002; 43: 246-252
- Electroencephalographic characteristics of West syndrome.Zh Nevrol Psikhiatr Im SS Korsakova. 2002; 102: 40-44
- Infantile spasms in tuberous sclerosis complex.Brain Dev. 2001; 173: 502-507
- Double blind study of ACTH versus prednisolone in infantile spasms.J Pediatr. 1983; 103: 641-645
- High dose corticotrophin (ACTH) versus prednisone for infantile spasms: a prospective randomized blinded study.Pediatrics. 1996; 97: 375-379
- High dose long duration versus low dose short duration corticotropin therapy for infantile spasms.J Pediatr. 1994; 124: 803-806
- A comparative study of high dose and low dose ACTH therapy for West syndrome.Brain Dev. 1999; 21: 461-467
- Medical treatment of patients with infantile spasm.Clin Neuropharmacol. 2002; 25: 61-70
- The treatment of West syndrome: a Cochrane review of the literature to December 2000.Brain Dev. 2001; 23: 624-634
- Treatment of infantile spasms.Cochrane Database Syst Rev. 2002; : CD001770
Article info
Publication history
Accepted:
October 27,
2003
Received in revised form:
October 27,
2003
Received:
July 14,
2003
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
