title: An MRI study of the myelination pattern in West syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Tenri Hospital, Nara, Japan.
authors.forename: J
authors.initials: J
authors.lastname: Muroi
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: C
authors.initials: C
authors.lastname: Kuno
authors.forename: T
authors.initials: T
authors.lastname: Yorifuji
authors.forename: K
authors.initials: K
authors.lastname: Shimizu
authors.forename: M
authors.initials: M
authors.lastname: Matsumura
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: M
authors.initials: M
authors.lastname: Matsuo
mesh_terms: D001284:Atrophy; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009186:Myelin Sheath / Q000473:pathology*; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D011379:Prognosis; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000473:pathology