TY - JOUR
T1 - Megalencephaly with formation of Rosenthal fibers in symmetric subependymal gliomatous proliferations
T2 - Clinicopathologic report
AU - Pietrini, V.
AU - Tagliavini, F.
AU - Tedeschi, F.
AU - Lechi, A.
PY - 1983
Y1 - 1983
N2 - 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.
AB - 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.
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M3 - Article
C2 - 6839573
AN - SCOPUS:0020624654
VL - 2
SP - 16
EP - 22
JO - Clinical Neuropathology
JF - Clinical Neuropathology
SN - 0722-5091
IS - 1
ER -