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Clinical and Pathologic Aspects

Ann M. Kazee, MD; Lowell W. Lapham, MD; Carlos F. Torres, MD; David D. Wang, MD

Arch Neurol. 1991;48(8):850-853.

Abstract
• 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.

Author Affiliations
From the Departments of Pathology and Laboratory Medicine (Drs Kazee and Lapham), Neurology (Drs Torres and Wang), and Pediatrics (Dr Torres), University of Rochester (NY); and Rochester (NY) General Hospital (Dr Wang).

Footnotes
Accepted for publication January 25, 1991.

Reprint requests to the Department of Pathology, University of Rochester Medical Center, 601 Elmwood Ave, Box 626, Rochester, NY 14642 (Dr Kazee).

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