Original article| Volume 23, ISSUE 7, P586-592, November 2001

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West syndrome: a university hospital based study from Oman


      Forty-four children (20 male: 24 female) with West syndrome (infantile spasms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Qaboos University Hospital (Pediatric Neurology Division of the Department of Child Health) are reported, with thirty-four (77.3%) children constituting the symptomatic group. All children were followed up for an initial 1 year at this hospital. Thirty-seven cases (84%) still continue their follow-up with us. The age of onset ranged from 1 to 14 months (mean, 6.0 months). Developmental delay before the onset of infantile spasms was noted in 29 (65.9%) children. Brain computed tomography was abnormal in 29 (65.9%). Sodium valproate and vigabatrin were the most often used drugs, though other antiepileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could be weaned off antiepileptic drugs completely. There was one death in the whole series, related to aspiration pneumonia.


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        • West W.J
        On a peculiar form of infantile convulsions.
        Lancet. 1841; 1: 724-725
        • Gibbs F.A
        • Gibbs E.L
        Atlas of electroencephalography. Addison–Wesley, Cambridge, MA1952
        • Dulac O
        • Chugani H.T
        • Dalla Bernardina B
        Introduction: Royaumont workshop on West syndrome.
        Epilepsia. 1993; 34: 670
        • Commission on Classification and Terminology of the International League Against Epilepsy
        Proposal for revised classification of epilepsies and epileptic syndromes.
        Epilepsia. 1989; 30: 389-399
        • Al-Rajeh S
        • Abomelha A
        • Awada A
        • Bademosi O
        • Ismail H
        Epilepsy and other convulsive disorders in Saudi Arabia: a population-based study of 1,000 consecutive cases.
        Acta Neurol Scand. 1990; 82: 341-345
        • Kalra V
        • Passi G.R
        Analysis of childhood epileptic encephalopathies with regard to etiological and prognostic factors.
        Brain Dev. 1998; 20: 14-17
        • Kramer U
        • Nevo Y
        • Neufeld M.Y
        • Fartal A
        • Leitner Y
        • Harel S
        Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients.
        Pediatr Neurol. 1998; 18: 46-50
        • Shields W.D
        Catastrophic epilepsy in childhood.
        Epilepsia. 2000; 41: 2-6
        • Dalla Bernardina B
        • Dulac O
        Introduction to etiology.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 166-171
        • Watanabe K
        West syndrome: etiological and prognostic aspects.
        Brain Dev. 1998; 20: 1-8
        • Riikonen R
        • Donner M
        Incidence and etiology of infantile spasms from 1960 to 1976: a population study in Finland.
        Dev Med Child Neurol. 1979; 21: 333-343
        • Dulac O
        • Feingold J
        • Plouin P
        • Chiron C
        • Pajot N
        • Ponsot G
        Genetic predisposition to West syndrome.
        Epilepsia. 1993; 34: 732-737
        • Claes S
        • Devriendt K
        • Lagae L
        • Ceulemans B
        • Dom L
        • Casaer P
        • et al.
        The X-linked infantile spasms syndrome (MIM 308350) maps to Xp11.4-Xpter in two pedigrees.
        Ann Neurol. 1997; 42: 360-364
        • Heiskala H
        CSF ACTH and beta endorphin in West syndrome and ACTH therapy.
        Brain Dev. 1997; 19: 339-342
        • Riikonen R.S
        • Soderstrom S
        • Vanhala R
        • Ebendal T
        • Lindhom D.B
        West syndrome: CSF nerve growth factor and effect of ACTH.
        Pediatr Neurol. 1997; 7: 224-229
        • Castano G
        • Lyons C.J
        • Jan J.E
        • Conolly M
        Cortical visual impairment in children with infantile spasms.
        J Pediatr Ophthalmol Strabismus. 2000; 4: 175-178
        • Curatolo P
        Tuberous sclerosis.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 192-202
        • Diebler C
        • Dulac O
        Structural imaging.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 118-141
        • Committee on Safety of Medicines and the Joint Committee on Vaccination and Immunization
        Whooping cough. Department of Health and Social Security. Her Majesty's Stationary Office, London1981
        • Bellman M.H
        • Ross E.M
        • Miller D.L
        Infantile spasms and pertussis immunisation.
        Lancet. 1983; 1: 1031-1034
        • Bogaert P.V
        • Chiron C
        • Adamsbaum C
        • Robain O
        • Diebler C
        • Dulac O
        Value of magnetic resonance imaging in West syndrome of unknown etiology.
        Epilepsia. 1993; 34: 701-706
        • Chiron C
        • Hwang P
        Functional imaging: SPECT.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 142-155
        • Chiron C
        • Hwang P
        Functional imaging.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 156-165
        • Aicardi J
        • Mumford J.P
        • Dumas C
        • Wood S
        • Sabril IS Investigator and Peer Review Groups
        Vigabatrin as initial therapy for infantile spasms: a European retrospective survey.
        Epilepsia. 1996; 37: 638-642
        • Granstrom M.L
        • Gaily E
        • Liukkonen E
        Treatment of Infantile spasms: results of a population-based study with vigabatrin as the first drug for spasms.
        Epilepsia. 1999; 40: 950-957
        • Appleton R.E
        • Peters A.C.B
        • Mumford J.P
        • Shaw D.E
        Randomized placebo-controlled study of vigabatrin as first-line treatment of infantile spasms.
        Epilepsia. 1999; 40: 1627-1633
        • Snead O.C
        • Chiron C
        Medical treatment.
        in: Dulac O Chugani H.T Dalla Bernardina B Infantile spasms and West syndrome. W.B. Saunders, London1994: 244-256
        • Koul R.L
        • Aithala G.R
        • Joshi R.M
        • Elbualy M.S
        • Al Riyami K
        Lamotrigine and vigabatrin in intractable seizures in children.
        Neurol India. 1998; 46: 130-133
        • Gross-Tsur V
        • Banin E
        • Shahar E
        • Shalev R.S
        • Lahat E
        Visual impairment in children with epilepsy treated with vigabatrin.
        Ann Neurol. 2000; 48: 60-64
      1. Koul RL, Alexander PC, Ganesh A. Safety of vigabatrin in childhood epilepsy. Paper submitted for 2nd Annual Epilepsy Conference, Jeddah, Saudi Arabia, 2000.

      2. The Royal College of Ophthalmologists. The ocular side-effects of vigabatrin (Sabril): information and guidelines for screening. London, 2000.

        • Glauser T.A
        • Clark P.O
        • McGee K
        Long term response to topiramate in patients with West syndrome.
        Epilepsia. 2000; 41: 591-594
        • Baram T.Z
        Myoclonus and myoclonic seizures.
        in: Swaiman K.F Ashwal S Pediatric neurology. Principles and practice. 3rd ed. Mosby, St. Louis, MO1999: 668-675
        • Ohtsuka Y
        • Matsuda M
        • Ogino T
        • Kobayashi K
        • Ohtahara S
        Treatment of the West syndrome with high dose pyridoxal phosphate.
        Brain Dev. 1987; 9: 418-421