AN MRI STUDY OF THE MYELINATION PATTERN IN WEST SYNDROME

Using magnetic resonance imaging (MRI), we studied the myelination of the brains of 8 patients with West syndrome, All cases were symptomati c, 2 having severe asphyxia, 1 lissencephaly, 1 Leigh encephalopathy, 2 tuberous sclerosis, 1 multiple anomalies and 1 microcephaly. Myelina tion of the pens, cerebellum, thalamus, internal capsules, optic radia tion, centrum semiovale and cerebral white matter was separately asses sed, The 2 cases with tuberous sclerosis exhibited normal myelination patterns, although asymmetry of the cerebral white matter was noted, T hese cases had a moderate degree of mental retardation and persistent seizures. The other 6 cases exhibited a marked delay of myelination th roughout the central nervous system except for the midbrain, These cas es had severe psychomotor retardation and persistent seizures, Althoug h the difference in the outcome may simply reflect the different etiol ogical disorders, these results suggest that the myelination pattern i s related to the psychomotor retardation but not to the severity of th e seizures in West syndrome, Atrophy of the corpus callosum developed during ACTH therapy and disappeared after the therapy, It was thus sug gested that the callosal atrophy caused by the ACTH therapy was revers ible.