Generalized cortical dysplasia. Clinical and pathologic aspects
A. M. Kazee, L. W. Lapham, C. F. Torres and D. D. Wang
Department of Pathology and Laboratory Medicine, University of Rochester, NY.
Three children with profound mental retardation and intractable seizures
died at ages 10 months, 3 years, and 7 years, respectively. Complete
examination of their brains showed generalized cortical dysplasia, without
any major malformation of the external gyral pattern. The neuropathologic
features of cortical dysplasia include abnormally thickened cortex with
indistinct demarcation of the gray-white matter junction. In many areas,
the cortex contained increased numbers of large neurons with disordered
cortical lamination. Heterotopic neurons were scattered throughout the
white matter with decreased myelination of the underlying white matter. To
our knowledge, these cases represent the first fully detailed
neuropathologic study of diffuse cortical dysplasia--a newly recognized
entity of abnormal neuronal migration.
