Gliomatosis Ceribri in childhood – case report

Introduction: Gliomatosis Cerebri (GC) is a rare neoplastic situation in childhood, characterized by diffuse infiltration of glial cells in the Central Nervous System (CNS), with a wide variety of presentations and an unfavorable outcome. Case report: An 11-years-old boy began with behavioral alterations, amnesic aphasia as well as right hemiplegia. Brain MRI showed diffuse infiltrative lesions involving both hemispheres. These lesions, identified largely on the white matter of the left hemisphere, encompassed frontal, temporal and parietal lobes, extending into basal ganglia, and mesencephalon, as well as bilateral thalami and white matter of the contralateral hemisphere, were hyperintense on T2 and flair sequences. The spectroscopy analysis showed increased peak of mioinositol, reduction of peak of N-acetyl-Aspartate, and some areas with a slight increase in the peak of choline. Brain biopsy evidenced grade 3 astrocytoma. He received combined treatment with temodal and radiotherapy. Discussion: In GC, different portions of the CNS are diffusely intermixed by neoplastic cells with preservation of anatomic architecture. It is not an unique cytogenetic entity, and the evolution depends on the histologic grade.The disease has a bimodal distribution, with peaks in the second and fifth decades of life. Usually, seizures are the first symptom, followed by hemiparesis, lethargy, other mental status alterations and visual loss.The MRI images are unspecific, and differential diagnosis includes ischemia, multiple sclerosis, encephalitis and leukodistrophy. Brain biopsy is extremely helpful to diagnosis confirmation. Treatment is based on chemotherapy.