The Japanese journal of neuropsychology

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ArticleTitle	Neuropsychology and neuropathology of semantic dementia
Language	J
AuthorList	Shinobu Kawakatsu¹⁾, Ryouta Kobayashi²⁾, Daichi Morioka²⁾, Koichi Otani²⁾
Affiliation	¹⁾Department of Neuropsychiatry, Aizu Medical Center, Fukushima Medical University ²⁾Department of Psychiatry, Yamagata University School of Medicine
Publication	Japanese Journal of Neuropsychology: 38 (2), 86-95, 2022
Received
Accepted
Abstract	Semantic dementia is a unique clinical syndrome characterized by the loss of word meaning or Gogi aphasia. This condition has a heterogenous neuropathological basis, but the vast majority of cases involve proteinopathy of trans-activation responsive RNA-binding protein of 43kDa (TDP-43). The most common presentation is TDP type C pathology, accounting for up to 70 to 80% of cases. The remaining cases are reported to be Pick's disease, Alzheimer's disease or TDP type B with motor neuron disease. Here, we present the clinicopathological findings of a typical patient with semantic dementia with TDP type C pathology. The main degenerative changes were severe neuronal loss and astrogliosis in the anterior and inferior temporal cortices, which was consistent with antemortem neuroimaging findings. On the other hand, phosphorylated TDP-43 positive long dystrophic neurites were observed in the frontal and parietal cortices as well as the anterior and inferior temporal cortices, indicating a widespread distribution of TDP type C pathology.
Keywords	semantic dementia, gogi-aphasia, TDP-43, dystrophic neurites, astrogliosis