Gliomatosis cerebri in a 10-year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumour

Gliomatosis cerebri is the unifying term used when diffuse glial infiltrati on occurs throughout the cerebral hemispheres. The very few eases reported in children have presented with intractable epilepsy, corticospinal tract d eficits, unilateral tremor. headaches. and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiologic al and pathological findings. Adult cases may simulate an acute diffuse enc ephalomyelitis and show postmortem evidence of a marked swelling of the spi nal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10-year-old girl who presented with a hist ory and physical signs suggestive of benign intracranial hypertension. A di ffuse encephalomyelopathy occurred, which was complicated by spinal cord sw elling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination.