The Japanese journal of neuropsychology

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ArticleTitle	Neuropsychological and neuroradiological findings of early-stage semantic variant primary progressive aphasia -Comparison of progression between an early onset case and a late onset case-
Language	J
AuthorList	Ryota Kobayashi¹⁾, Shinobu Kawakatsu²⁾, Hiroshi Hayashi¹⁾, Nobuyuki Okamura³⁾⁴⁾, Koichi Otani¹⁾
Affiliation	¹⁾Department of Psychiatry, Yamagata University School of Medicine ²⁾Department of Neuropsychiatry, Aizu Medical Center, Fukushima Medical University ³⁾Department of Pharmacology, Faculty of Medicine, Tohoku Medical and Pharmaceutical University ⁴⁾Cyclotron and Radioisotope Center, Tohoku University
Publication	Japanese Journal of Neuropsychology: 35 (4), 238-248, 2019
Received	Jan 8, 2019
Accepted	Sep 6, 2019
Abstract	Semantic variant primary progressive aphasia (svPPA) is typically early-onset (EO, before 65 years of age) and associates with TDP-43 type C pathology. In the early stages of the disease and late-onset (LO) cases, differentiation of svPPA from Alzheimer's disease (AD) is often difficult. In this study, we revealed characteristics of the early-stage svPPA patients in whom AD was excluded by amyloid imaging, and compared clinical symptom progression and imaging findings between an EO and an LO svPPA case. Both cases showed cerebral atrophy progression and cerebral blood flow decrease over the course of 1 year; however, the progression of clinical symptoms and brain imaging findings were more prominent in the EO case. Our results suggest that LO cases show slower progression than EO cases. Further investigation is needed to clarify the underlying pathological differences between EO and LO svPPA cases.
Keywords	primary progressive aphasia, semantic dementia, amyloid PET, neuropathology