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A total of 25 patients with gliomatosis cerebri (19 males and 6 females; median age 51 years, range 10-73 years) were diagnosed and treated at the Sheba Medical Center between 1995 and 2009. Of these, 3 patients were 10 years old at the time of diagnosis. Seizures were the initial clinical presentation in 19 patients, focal signs in 16 patients, headaches in 7 patients, cognitive disorder in 4 patients and rapidly progressive hemiparesis in 1 patient. Magnetic resonance imaging (MRI) was performed in the patients and demonstrated a diffuse infiltrative process with a hyperintensity signal on T2-weighted images and a minimal mass effect. Some level of enhancement on MRI was observed in 6 patients. The infiltrative process involved at least two lobes in each patient. Biopsy was performed for diagnosis in the majority of patients. In 1 patient with a markedly rapid deterioration, the diagnosis was established at autopsy. The pathology was compatible with gliomatosis with a diffuse infiltrative low-grade astrocytoma in 21 patients and anaplastic astrocytoma in 5 patients. The patients were treated with whole-brain radiation therapy and 7 patients were treated with combined whole-brain radiation therapy and chemotherapy. Treatment appeared to stabilize 6 patients or improve the clinical condition in 7 patients. Due to the small number of patients in the present study, however, further studies are required to determine the effect of treatment on the natural history of the disease.

Presentation patterns and outcome of gliomatosis cerebri