Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy.
Hippocampal sclerosis is defined as selective neuronal loss and gliosis of the hippocampus with heterogeneous etiologies, including neurodegenerative tauopathies. We report a 78-year-old woman who presented with depression, in whom postmortem examination revealed almost complete loss of neurons with gliosis in the subiculum and CA1-3 regions of the hippocampus and abundant neuronal cytoplasmic inclusions in the dentate gyrus. The inclusions were round, slightly basophilic and argyrophilic, resembling Pick bodies. However, they were Gallyas- and 4-repeat tau-positive, and 3-repeat tau- and ubiquitin-negative. To our knowledge, the histopathological features in this case were different from those in hippocampal sclerosis or 4-repeat tauopathies reported previously. It is likely that this case is a new variant of 4-repeat tauopathy presenting with hippocampal sclerosis.
Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan. yasuomiki@hotmail.com
Yasuo
Y
Miki
Fumiaki
F
Mori
Emiko
E
Hori
Mitsuomi
M
Kaimori
Koichi
K
Wakabayashi
D000368:Aged; D016923:Cell Death; D018891:Dentate Gyrus / Q000473:pathology* / Q000648:ultrastructure; D003937:Diagnosis, Differential; D005260:Female; D006624:Hippocampus / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology*; D008854:Microscopy, Electron; D016874:Neurofibrillary Tangles / Q000473:pathology; D009474:Neurons / Q000473:pathology* / Q000648:ultrastructure; D020033:Protein Isoforms; D017966:Pyramidal Cells / Q000473:pathology; D012598:Sclerosis / Q000473:pathology; D024801:Tauopathies / Q000473:pathology*; D025801:Ubiquitin / Q000378:metabolism; D016875:tau Proteins / Q000378:metabolism
C054369:MAPT protein, human; D020033:Protein Isoforms; D025801:Ubiquitin; D016875:tau Proteins