Paediatric gliomatosis cerebri presenting with deep gray matter lesions

Abstract

Gliomatosis cerebri (GC) is a rare extensively infiltrating growth pattern of diffuse glioma with wide clinical heterogeneity, often mimicking other disorders. GC usually affects the white matter and involvement predominantly of the deep gray matter at onset is uncommon. We describe a teenager who presented with bi-thalamic and left hippocampal-parahippocampal lesions at first presentation with a 1-month history of right-sided motor hemiparesis and hemiataxia. Over a 1-year period the lesion spread to the bi-temporal, right insular cortex, and bi-frontal cerebral hemisphere associated with multiple cranial palsies and progressive dementia. Histological examination revealed a high- grade anaplastic astrocytoma consistent with GC. Our case widens the phenotypic spectrum of GC. Clinicians need to consider GC in children with lesions in the deep gray matter in combination with pyramidal, cranial nerve palsies and extrapyramidal signs.