A 36 year-old patient presented with a dementia of frontal type, gait
disturbances, incontinence and a pseudo-bulbar palsy, which caused dea
th at age 40. Brain biopsy of the frontal lobe showed an extensive dee
p subcortical gliosis. A high level of GFAP was detected by immunoblot
ting in the biopsy. Clinical and neuropathological observations are si
milar to cases described as Neumann Progressive Subcortical Gliosis. S
ingle Photon Emission Computer Tomography showed a bilateral frontotem
poral hypoperfusion, and Magnetic Resonance Imaging large periventricu
lar and subcortical hyperintensities in both hemispheres, the brainste
m and the cerebellum. The hyperintensities on T2-weighted MR images mi
ght be related to the intense gliosis. The contribution of such imagin
g data to diagnosis must be confirmed by other clinico-pathological ca
ses.