A 36 year-old patient presented with a dementia of frontal type, gait disturbances, incontinence and a pseudo-bulbar palsy, which caused death at age 40. Brain biopsy of the frontal lobe showed an extensive deep subcortical gliosis. A high level of GFAP was detected by immunoblotting in the biopsy. Clinical and neuropathological observations are similar to cases described as Neumann Progressive Subcortical Gliosis. Single Photon Emission Computer Tomography showed a bilateral frontotemporal hypoperfusion, and Magnetic Resonance Imaging large periventricular and subcortical hyperintensities in both hemispheres, the brainstem and the cerebellum. The hyperintensities on T2-weighted MR images might be related to the intense gliosis. The contribution of such imaging data to diagnosis must be confirmed by other clinico-pathological cases.