Gliomatosis cerebri: A rare cause of progressive headache

A 49-year-old right-handed woman was admitted for an 8-month history of unu sual headache and transient diplopia. Clinical examination and brain CT sca n were normal. Two months later, symptoms of raised intracranial pressure d eveloped and a brain CT scan showed small lateral ventricles and sulci with out any abnormal contrast enhancement or tumor mass. Brain MRI with T2-weig hted spin echo sequences revealed a hyperintense signal in the right tempor oparietal region, whereas only a slight enlargement of this region was note d on T1 spin echo. The patient deteriorated rapidly and died with uncontrol lable raised cerebrospinal fluid pressure. The diagnosis of gliomatosis cer ebri was made at necropsy. Gliomatosis cerebri is a rare intracranial neopl asm of neuroepithelial origin. Spread of this tumor is particularly fast in the white matter compared with the gray matter and nuclei. Clinical sympto ms are not specific. The diagnosis can be suspected by MRI showing an isoin tense or hypointense signal in the deep white matter on T1-weighted images, and largely a hyperintense signal on T2-weighted sequences. The diagnosis is confirmed by stereotactic biopsy or necropsy. No curative treatment is c urrently available. Radiotherapy can delay the rapidly fatal outcome. Our c ase illustrates the possible onset of this rare disease by isolated cephalg ia with normal early CT scan.