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Journal of Child Neurology
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Gliomatosis Cerebri Presenting as Intractable Epilepsy During Early Childhood

Mark T. Jennings, MD

Department of Neurology, Vanderbilt School of Medicine, Nashville

Meghana Frenchman

University of Tennessee Medical School Memphis, TN

Thomas Shehab

Wayne State Medical School Detroit, MI

Mahlon D. Johnson, MD, PhD

Department of Neuropathology, Vanderbilt School of Medicine, Nashville

Jeffrey Creasy, MD

Department of Neuroradiology Vanderbilt School of Medicine, Nashville

Kazel LaPorte, RN

Department of Neurology, Vanderbilt School of Medicine, Nashville

Wolf D. Dettbarn, MD

Department of Neurology, Vanderbilt School of Medicine, Nashville

We review 160 cases of gliomatosis cerebri from the literature and report 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 examination, electroencephalograms, magnetic resonance imaging, and positron emission tomographic scans. The natural history of gliomatosis cerebri was determined by a retrospective review of the literature of 160 cases in 85 reports. The most common neurologic symptoms and signs included corticospinal tract deficits (58%), dementia/mental retardation (44%), headache (39%), seizures (38%), cranioneuropathies (37%), increased intracranial pressure (34%), and spinocerebellar deficits (33%). The most commonly involved central nervous system structures were the centrum semiovale and cerebrum (76%), mesencephalon (52%), pons (52%), thalamus (43%), basal ganglia (34%), and the cerebellum (29%). Fifty-two percent of patients were dead within 12 months of onset. Different grades of glial neoplasm may also coexist within gliomatosis cerebri such as astrocytoma with anaplastic astrocytoma, atypical or anaplastic oligodendroglioma, and glioblastoma multiforme. Hypotheses regarding the pathogenesis of gliomatosis cerebri include blastomatous dysgenesis, diffuse infiltration, multicentric origin, in situ proliferation, 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 proliferative are not understood. (J Child Neurol 1995;10:37-45).

Journal of Child Neurology, Vol. 10, No. 1, 37-45 (1995)
DOI: 10.1177/088307389501000111
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