An autopsy case of globular glial tauopathy presenting with clinical features of motor neuron disease with dementia and iron deposition in the motor cortex

We report a case of GGT with iron deposits in a 78‐year‐old woman presenting with an 8‐year history of slowly progressing limb weakness and cognitive decline. Susceptibility‐weighted imaging revealed a low signal intensity in the right precentral gyrus, suggesting iron deposition. A clinical diagnosis of motor neuron disease with dementia was made 4 years after onset. At autopsy, gross pathological findings showed atrophy of the frontal and temporal lobes. A localized area of the precentral gyrus corresponding to the most severely affected limb showed the strongest atrophy, macroscopically, and displayed 4R tau‐immunoreactive GGIs and microscopically many ferritin‐immunoreactive neurons. We diagnosed this patient as having GGT. This is the first GGT case with iron deposition confirmed both radiologically and pathologically.

Source: Neuropathology - February 1, 2018 Category: Neurology Authors: Itsuki Hasegawa, Akitoshi Takeda, Hiroyuki Hatsuta, Yuki Kubo, Masahiko Ohsawa, Yuta Nakano, Takeshi Ikeuchi, Masato Hasegawa, Shigeo Murayama, Yoshiaki Itoh Tags: Case Report Source Type: research