Research Article| Volume 18, ISSUE 3, P179-184, May 1996

An MRI study of the myelination pattern in West syndrome

      This paper is only available as a PDF. To read, Please Download here.


      Using magnetic resonance imaging (MRI), we studied the myelination of the brains of 8 patients with West syndrome. All cases were symptomatic, 2 having severe asphyxia, 1 lissencephaly, 1 Leigh encephalopathy, 2 tuberous sclerosis, 1 multiple anomalies and 1 microcephaly. Myelination of the pons, cerebellum, thalamus, internal capsules, optic radiation, centrum semiovale and cerebral white matter was separately assessed. The 2 cases with tuberous sclerosis exhibited normal myelination patterns, although asymmetry of the cerebral white matter was noted. These cases had a moderate degree of mental retardation and persistent seizures. The other 6 cases exhibited a marked delay of myelination throughout the central nervous system except for the midbrain. These cases had severe psychomotor retardation and persistent seizures. Although the difference in the outcome may simply reflect the different etiological disorders, these results suggest that the myelination pattern is related to the psychomotor retardation but not to the severity of the seizures in West syndrome. Atrophy of the corpus callosum developed during ACTH therapy and disappeared after the therapy. It was thus suggested that the callosal atrophy caused by the ACTH therapy was reversible.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Bogaert P
        • Chiron C
        • Adamsbaum C
        • et al.
        Value of magnetic resonance imaging in West syndrome of unknown etiology.
        Epilepsia. 1993; 34: 701-706
        • Konishi Y
        • Yasujima M
        • Kuriyama M
        • et al.
        Magnetic resonance imaging in infantile spasms: effects of hormonal therapy.
        Epilepsia. 1992; 33: 304-309
        • Staudt M
        • Schropp C
        • Staudt F
        • et al.
        MRI assessment of myelination: an age standardization.
        Pediatr Radiol. 1994; 24: 122-127
        • Watanabe K
        • Haga T
        • Negoro T
        • et al.
        Focal spasms in clusters, focal delayed myelination, and hypsarrhythmia: unusual variant of West syndrome.
        Pediatr Neurol. 1994; 11: 47-49
        • Tamaki K
        • Okuno T
        • Ito M
        • et al.
        Magnetic resonance imaging in relation to EEG epileptic foci in tuberous sclerosis.
        Brain Dev (Tokyo). 1990; 12: 316-320
        • Schropp C
        • Staudt M
        • Staudt F
        • et al.
        Delayed myelination in children with West syndrome: an MRI-study.
        Neuropediatrics. 1994; 25: 116-120
        • Inoue Y
        Development of the brain and MRI (in Japanese).
        in: Committee of Postgraduate Education. 3rd edn. Shoni-Shinkeigaku No Shinpo. Shindan to Chiryosha, Tokyo1989: 28-39 (No 23)
        • Barkovich AJ
        • Lyon G
        • Evrard P
        Formation, maturation and disorders of white matter.
        AJNR. 1992; 13: 447-461
        • Yasujima M
        • Konishi Y
        • Kuriyama M
        • et al.
        MR imaging in infantile spasms (in Japanese).
        No to Hattatsu (Tokyo). 1989; 21: 537-542
        • Hrachovy RA
        • Frost JD
        Infantile spasms.
        Pediatr Clin North Am. 1989; 36: 311-329
        • Kjos BO
        • Umansky R
        • Barkovich AJ
        Brain MR imaging in children with developmental retardation of unknown cause.
        AJNR. 1990; 11: 1035-1040
        • Maeda N
        • Watanabe K
        • Negoro T
        • et al.
        Evolutional changes of cortical hypometabolism in West's syndrome.
        Lancet. 1994; 343: 1620-1623
        • Hiraiwa M
        • Funamoto N
        • Nonaka C
        • et al.
        A new concept for the symptomatic type of infantile spasms and early infantile epileptic encephalopathy using positron emission tomography.
        Ann Neurol. 1989; 26: 477
        • Chugani HT
        The use of positron emission tomography in the clinical assessment of epilepsy.
        Semin Nucl Med. 1992; 22: 247-253
        • Satoh J
        • Mizutani T
        • Morimatsu Y
        Neuropathology of the brainstem in age-dependent epileptic encephalopathy — especially of cases with infantile spasms.
        Brain Dev (Tokyo). 1986; 8: 443-449
        • Morimoto Y
        • Kubo M
        • Ouchi T
        • et al.
        Infantile spasms associated with brainstem and cerebellar hypogenesis (in Japanese).
        Nihon Shonika Gakkai Zasshi (Tokyo). 1990; 94: 2250-2254
        • Okuno T
        • Ito M
        • Konishi Y
        • et al.
        Cerebral atrophy following ACTH therapy.
        J Comput Assist Tomogr. 1980; 4: 20-23
        • Lagenstein I
        • Willig RP
        • Kühne D
        Reversible cerebral atrophy caused by corticotropin.
        Lancet. 1979; 1: 1246-1247