Spinal cord lesions in tuberous sclerosis

Spinal cord involvement is not widely recognized as part of the tuberous sclerosis complex. Necropsy study of the spinal cord from 2 patients revealed abnormalities in both. These consisted of clusters of abnormal fiber-forming astrocytes in gray and in white matter, bizarre giant glial forms in white matter in one patient, and neuronal loss. Myelin sheaths in subpial zones were also swollen and slightly fragmented in one patient. Reports of similar studies are sparse. Some investigators have also noted glial proliferation and white matter changes, although the latter most likely represented Wallerian degeneration.