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

Itsuki Hasegawa; Akitoshi Takeda; Hiroyuki Hatsuta; Yuki Kubo; Masahiko Ohsawa; Yuta Nakano; Takeshi Ikeuchi; Masato Hasegawa; Shigeo Murayama; Yoshiaki Itoh

doi:10.1111/neup.12457

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

Neuropathology. 2018 Mar 6. doi: 10.1111/neup.12457. Online ahead of print.

Authors

Affiliations

¹ Department of Neurology, Osaka City University Graduate School of Medicine, Osaka, Japan.
² Neurology and Bioresource Center (Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.
³ Department of Pathology, Osaka City University Graduate School of Medicine, Osaka, Japan.
⁴ Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
⁵ Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.

PMID: 29508434
DOI: 10.1111/neup.12457

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.

Keywords: 4 repeat tau; cortical atrophy; globular glial tauopathy; iron deposition; precentral gyrus.

Publication types

Case Reports