S. Jayawant et al., Gliomatosis cerebri in a 10-year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumour, DEVELOP MED, 43(2), 2001, pp. 124-126
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.