Megalencephaly with formation of Rosenthal fibers in symmetric subependymal gliomatous proliferations: clinicopathologic report.

ABSTRACT

Clinical and neuropathologic observations are reported concerning a girl with retarded psychomotor development accompanied with a steady increase in the volume of the cranium and brain, tetraparesis, and a serious epileptic syndrome resulting in death at the age of 13 years. The neuropathologic study revealed a marked megalencephaly and the presence of two bilateral and symmetric areas of atypical glial proliferation, corresponding with the optostriatal groove, with endoventricular protrusion in this region the histologic findings gave evidence of a tapetum of Rosenthal fibers. Discussion is focused on the observed neuropathologic aspects in relation to the various diagnostic hypotheses Alexander's disease, primary megalencephaly associated with astrocytomas or reactive gliosis, or megalencephaly associated with subependymal hamartomas.