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 infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. Adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10-year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination.
Jayawant
S
S
Department of Paediatric Neurology, University Hospital of Wales, Heath Park, Cardiff, UK.
Neale
J
J
Stoodley
N
N
Wallace
S
S
D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D004679:Encephalomyelitis / Q000175:diagnosis*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D019586:Intracranial Hypertension / Q000209:etiology; D008279:Magnetic Resonance Imaging; D018302:Neoplasms, Neuroepithelial / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D013120:Spinal Cord Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology