Delayed myelination in children with West syndrome: an MRI-study

Eighteen children with West syndrome underwent MRI-examination of the brain. Five children were examined twice. Myelination of ten regions of the brain was retrospectively assessed on the MRI-examinations. The children could be divided in three groups of main diagnosis (cryptogenic West syndrome, tuberous sclerosis, pre-/perinatally acquired brain lesions). Delays in myelination were found in 17 out of a total of 23 examinations. In children with cryptogenic West syndrome and with tuberous sclerosis myelination was normal or only mildly delayed in the first month after onset of the spasms, but delays seemed to increase in the course of the West syndrome in these groups. In children with pre- or perinatally acquired brain lesions severe delays of myelination could be found already in the first weeks after the onset of the West syndrome. The assessment of myelination can therefore contribute to the diagnosis and understanding of the underlying disease in children with West syndrome.