An autopsy case of globular glial tauopathy presenting with clinical features of motor neuron disease with dementia and iron deposition in the motor cortex. Issue 4 (6th March 2018)

• Record Type: Journal Article
• Authors: Hasegawa, Itsuki; Takeda, Akitoshi; Hatsuta, Hiroyuki; Kubo, Yuki; Ohsawa, Masahiko; Nakano, Yuta; Ikeuchi, Takeshi; Hasegawa, Masato; Murayama, Shigeo; Itoh, Yoshiaki
• Is Part Of: Neuropathology. Volume 38:Issue 4(2018)
• Page Start: 372
• Page End: 379
• Publication Date: 2018-03-06
• Subjects: 4 repeat tau -- cortical atrophy -- globular glial tauopathy -- iron deposition -- precentral gyrus; Nervous system -- Diseases -- Periodicals; Nervous system -- Pathophysiology -- Periodicals; 616.8047
• DOI: 10.1111/neup.12457
• Languages: English
• ISSNs: 0919-6544

Abstract

Abstract : Globular glial tauopathy (GGT) is a 4‐repeat (4R) tauopathy in which 4R tau accumulates to form globular glial inclusions (GGIs), predominantly in oligodendroglia. To date, little has been reported on iron deposits in patients with GGT. 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.