Semantic deficits in ALS related to right lingual/fusiform gyrus network involvementResearch in context
Aya Ogura,
Hirohisa Watanabe,
Kazuya Kawabata,
Reiko Ohdake,
Yasuhiro Tanaka,
Michihito Masuda,
Toshiyasu Kato,
Kazunori Imai,
Takamasa Yokoi,
Kazuhiro Hara,
Epifanio Bagarinao,
Yuichi Riku,
Ryoichi Nakamura,
Yoshinari Kawai,
Masahiro Nakatochi,
Naoki Atsuta,
Masahisa Katsuno,
Gen Sobue
Affiliations
Aya Ogura
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Hirohisa Watanabe
Brain and Mind Research Centre, Nagoya University, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan; Department of Neurology, School of Medicine, Fujita Health University, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi, Japan
Kazuya Kawabata
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Reiko Ohdake
Brain and Mind Research Centre, Nagoya University, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Yasuhiro Tanaka
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan; Brain and Mind Research Centre, Nagoya University, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Michihito Masuda
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Toshiyasu Kato
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Kazunori Imai
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Takamasa Yokoi
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Kazuhiro Hara
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Epifanio Bagarinao
Brain and Mind Research Centre, Nagoya University, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Yuichi Riku
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Ryoichi Nakamura
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Yoshinari Kawai
Department of Neurology, Oyamada Memorial Spa Hospital, 5538-1 Yamadacho, Yokkaichi, Mie, Japan
Masahiro Nakatochi
Department of Nursing, Bioinformatics Section, Nagoya University Graduate School of Medicine, 1-1-20 Daiko-Minami, Higashi-ku, Nagoya, Japan
Naoki Atsuta
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Masahisa Katsuno
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan
Gen Sobue
Brain and Mind Research Centre, Nagoya University, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan; Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan; Aichi Medical University, Nagakute, Aichi, Japan; Corresponding author at: Brain and Mind Research Centre, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Japan.
Background: The clinicopathological continuity between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) is well known. Although ALS demonstrates language symptoms similar to FTLD, including semantic dementia, word reading impairments in ALS have not been well studied. “Jukujikun” are Kanji-written words with irregular pronunciation comparable to “exception words” and useful for detecting semantic deficits in Japan. We conducted a cross-sectional study to investigate Jukujikun reading impairments and related network changes in ALS. Methods: We enrolled 71 ALS patients and 69 healthy controls (HCs). Age-, sex-, and education matched HCs were recruited from another cohort study concurrently with patient registration. We examined neuropsychological factors including low frequency Jukujikun reading. We performed resting-state functional magnetic resonance imaging with voxel-based graph analysis on a subset of participants who agreed. Findings: Low frequency Jukujikun score was decreased in ALS (15·0[11·0–19·0](median[25–75 percentile])) compared with HCs (19·0[17·3–20·0]) (p < 0·001, effect size = 0·43). Fifty-two percent of ALS (N = 37) with low frequency Jukujikun score ≤ 5th percentile of HCs was classified as ALS with positive Jukujikun deficit (ALS-JD+). Compared with HCs, ALS-JD+ showed decreased degree centrality in the right lingual/fusiform gyrus, where connectivities with regions associated with word perception, semantic processing, or speech production were decreased. They also showed increased degree centrality in the left inferior/middle temporal gyrus, associated with increased connectivities involving semantic processing. Interpretation: Dysfunction of the “hub” in the right lingual/fusiform gyrus can affect semantic deficit in ALS. Considering neuropsychological symptoms as network impairments is vital for understanding various diseases. Fund: MHLW and MEXT, Japan. Keywords: Amyotrophic lateral sclerosis, Semantic deficits, Language impairments, Resting-state functional MRI, Voxel-based graph theoretical analysis
