The Japanese journal of neuropsychology

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ArticleTitle	Clinicopathological study of a semantic behavioral variant of frontotemporal dementia with TDP-type C pathology and corticospinal tract degeneration
Language	J
AuthorList	Shinobu Kawakatsu¹⁾, Ryouta Kobayashi²⁾, Daichi Morioka²⁾, Kazutaka Sakamoto²⁾, Akihito Suzuki²⁾
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: 40 (3), 195-204, 2024
Received
Accepted
Abstract	We reported a case of semantic behavioural variant frontotemporal dementia (sbv FTD) with TDP-type C pathology and corticospinal tract degeneration (CSTD) and reviewed the related literatures on right-predominant semantic dementia (rt-SD). A 62-year-old right-handed housewife developed difficulty identifying familiar faces and presented with loss of empathy and complex compulsions in routine shopping and dietary behaviours. At 63 years of age, she scored 16/30 on Mini-Mental state examination and could not identify any Japanese celebrities. Mild word meaning loss was also noted with surface dyslexia. Magnetic resonance imaging revealed severe right-predominant atrophy of the anterior and inferior temporal regions in the area in which hypoperfusion was observed on single-photon emission computed tomography. At 66 years of age, her comprehension was poor. However, no motor neuron diseases were observed. She died of pneumonia at 73 years of age, 11 years after onset of the initial illness. The brain weighed 740 g. Atrophy was predominantly evident in the right anterior temporal and bilateral frontal lobes, including the precentral cortices. Phosphorylated (p)-TDP-43-positive long dystrophic neurites were observed throughout the cerebral cortex; they were more abundant on the relatively spared side of the cortices and less abundant within the severely degenerated cortices. CSTD was observed at the level of the medulla oblongata and spinal cord with spongiform degeneration in the superficial layers of the motor cortices. The neurons in the anterior horn were spared; however some had p-TDP-43 positive inclusions. A literature review of the pathological backgrounds of rt-SD and sbv FTD revealed that, although the TDP type C pathology is the most common in rt-SD and left-predominant SD cases, rt-SD seems to have a more heterogeneous pathology. The combination of TDP-type C and CSTD being more common in rt-SD may have important implications for future research.
Keywords	right-predominant semantic dementia, semantic behavioural variant frontotemporal dementia, right temporal lobe atrophy, TDP-type C, prosopagnosia