GIANT-CELLS IN CORTICAL TUBERS IN TUBEROUS SCLEROSIS SHOWING SYNAPTOPHYSIN-IMMUNOREACTIVE HALOS

We describe a characteristic pattern of immunoreactivity for synaptoph ysin in tuberous sclerosis. We analyzed cortical tubers from surgical specimens taken from six patients with tuberous sclerosis, which were obtained by surgical resections for the treatment of intractable seizu res. The cortical tubers were characterized by blurred lamination of t he cerebral cortex, hypercellularity, and gliotic changes. Neuropil in the cortex of cortical tubers showed reduced immunoreactivity for syn aptophysin in all patients. 'Giant cells' were investigated in the cor tex and white matter regions of cortical tubers. Some 'giant cells' ha d neuronal characteristics such as Nissl substance, a centrally placed chromatin-marginated nucleus, prominent nucleolus, positive immunorea ctivity for microtubule-associated protein 2, and negative immunoreact ivity for glial fibrillary acidic protein. Other 'giant cells' were in determinate in cell type because they lacked Nissl bodies, distinct nu cleolus, consistent immunoreactivity for microtubule-associated protei n 2 and glial fibrillary acidic protein. Almost all 'neuronal giant ce lls' and some of the 'indeterminate giant cells' in the white matter s howed intense immunoreactivity for synaptophysin: cell borders were su rrounded by an intense immunoreactive halo. In conclusion, these immun ohistochemical patterns for synaptophysin assist in characterizing the se abnormal cells in the cortical tubers of patients with tuberous scl erosis. (C) 1997 Elsevier Science B.V.