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Gliomatosis cerebri (GC) is an infrequent infiltrative glioma with an undefined natural history and poor response to treatment. Two articles in this issue of Neurology1,2⇓ contribute to our understanding of GC and the response of GC to primary chemotherapy.
Nevin first proposed the term GC in 1938 although he cites prior reports from Hildebrandt in 1907 and Landau in 1911. The majority of subsequent reports have been small retrospective case series from a single institution with few exceptions.3–6⇓⇓⇓ Many aspects of the biology of this peculiar glioma remain enigmatic. Several features of GC merit comment, including neurologic presentation, neuroradiographic appearance, neuropathology/etiopathogenesis, and treatment.
There is a wide range in age at presentation of GC in adults (17 to 85 years) but the majority of patients are male (as is true in all gliomas) and comparatively young (median age 44 years). Patients most often present with an insidiously evolving syndrome (median duration 3 to 5 months) comprised of progressive headache, hemiparesis, and seizures (partial with or without secondary generalization). Findings on neurologic examination include evidence of raised intracranial pressure (papilledema, ophthalmoplegia, gait disturbance), neurobehavioral changes, and hemiparesis. In general, the presentation in the majority of patients with GC recapitulates that of patients with low-grade astrocytoma (LGA).
MRI has changed the conceptualization of GC. CT imaging only poorly visualized GC because of the subtle widespread infiltration of white matter, lack of focal tumor mass, and usual lack …
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