GLIOMATOSIS CEREBRI PRESENTING AS INTRACTABLE EPILEPSY DURING EARLY-CHILDHOOD

We review 160 cases of gliomatosis cerebri from the literature and rep ort an additional three infants and young children who presented with intractable epilepsy, corticospinal tract deficits, and developmental delay in whom a pathologic diagnosis was made. The progressive nature of the encephalopathy in our cases was documented by serial clinical e xamination, electroencephalograms, magnetic resonance imaging, and pos itron emission tomographic scans. The natural history of gliomatosis c erebri was determined by a retrospective review of the literature of 1 60 cases in 85 reports. The most common neurologic symptoms and signs included corticospinal tract deficits (58%), dementia/mental retardati on (44%), headache (39%), seizures (38%), cranioneuropathies (37%), in creased intracranial pressure (34%), and spinocerebellar deficits (33% ). The most commonly involved central nervous system structures were t he centrum semiovale and cerebrum (76%), mesencephalon (52%), pens (52 %), thalamus (43%), basal ganglia (34%), and the cerebellum (29%). Fif ty-two percent of patients were dead within 12 months of onset. Differ ent grades of glial neoplasm may also coexist within gliomatosis cereb ri such as astrocytoma with anaplastic astrocytoma, atypical or anapla stic oligodendroglioma, and glioblastoma multiforme. Hypotheses regard ing the pathogenesis of gliomatosis cerebri include blastomatous dysge nesis, diffuse infiltration, multicentric origin, in situ proliferatio n, and ''field transformation.'' The biologic determinants of whether a transformed glial cell behaves as a relatively localized tumor mass or truly loses anchorage dependence to become migratory as well as pro liferative are not understood.