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<p>Neuronal<?tk 2?> intranuclear inclusion disease (NIID) is a neurodegenerative condition characterized by the presence of intranuclear inclusions in neuronal and visceral cells. Patients with NIID can present respiratory symptoms; however, data on pulmonary functions in NIID are lacking. This study investigated the respiratory conditions in NIID patients diagnosed with histopathological and genetic studies. We conducted two spirometries before and after administrating the bronchodilator in NIID patients with asthmatic histories or symptoms. We statistically compared pre- and post-measured values including forced vital capacity (FVC), forced expiratory volume in one second (FEV<sub>1</sub>), and peak expiratory flow (PEF). Before two spirometries, we also measured fractional concentration of exhaled nitric oxide (FeNO), if possible. Of the 51finally enrolled patients, 17 (33.3%, 95% CI 20.8% to 47.9%) patients had asthmatic histories or symptoms, and 14 patients received two spirometries. After administrating the bronchodilator, FEV<sub>1</sub> and PEF significantly increased by 150&#xa0;mL (6.01%, <i>p</i> = 0.002) and 260&#xa0;mL/s (6.72%, <i>p</i> = 0.017), respectively. The median (interquartile range) of FeNO measured in nine patients was 15 (10–21) ppb. Patients with NIID have airflow reversibility like asthma. This condition appears to be less associated with airway inflammation and may be related to autonomic dysfunction.</p>

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Asthma-like bronchodilator responsiveness in patients with neuronal intranuclear inclusion disease

  • 期刊论文

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  • 发表日期：

    2026年4月9日

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  • Daisuke Tahara,
  • Nao Tahara,
  • Chisato Tamai,
  • Akio Akagi,
  • Yuichi Riku,
  • Hiroaki Miyahara,
  • Rei Kobayashi,
  • Hisashi Okada,
  • Michi Kawamoto,
  • Junko Ishii,
  • Hiroki Yamazaki,
  • Takashi Kurashige,
  • Atsuhiko Sugiyama,
  • Akiko Nagaishi,
  • Katsuya Nishida,
  • Kazuma Sugie,
  • Takayasu Fukudome,
  • Kazuko Hasegawa,
  • Hiroyuki Ishiura,
  • Haruki Koike,
  • Takashi Kasai,
  • Toshiki Mizuno,
  • Masahiro Ando,
  • Yujiro Higuchi,
  • Fumiaki Tanaka,
  • Yuishin Izumi,
  • Gen Sobue,
  • Yasushi Iwasaki,
  • Satoru Ito,
  • Jun Sone,
  • Keiko Hayashi,
  • Arisa Yamamoto,
  • Takayuki Katayama,
  • Mai Fujikura,
  • Atsushi Sakurai,
  • Toshiyuki Hayashi,
  • Manabu Araki,
  • Keizo Sugaya,
  • Takamura Nagasaka,
  • Yuzo Tanaka,
  • Takanobu Kita,
  • Nobuyuki Iwade,
  • Takao Kiriyama,
  • Yosuke Osakada,
  • Megumi Toko,
  • Motoharu Kawai,
  • Yoshiaki Takahashi,
  • Tomoyasu Matsubara,
  • Noriyuki Miyaue,
  • Masahiro Shijo,
  • Toshihiro Ide,
  • Masaaki Yoshikawa,
  • Hidenori Matsuo

摘要

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative condition characterized by the presence of intranuclear inclusions in neuronal and visceral cells. Patients with NIID can present respiratory symptoms; however, data on pulmonary functions in NIID are lacking. This study investigated the respiratory conditions in NIID patients diagnosed with histopathological and genetic studies. We conducted two spirometries before and after administrating the bronchodilator in NIID patients with asthmatic histories or symptoms. We statistically compared pre- and post-measured values including forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and peak expiratory flow (PEF). Before two spirometries, we also measured fractional concentration of exhaled nitric oxide (FeNO), if possible. Of the 51finally enrolled patients, 17 (33.3%, 95% CI 20.8% to 47.9%) patients had asthmatic histories or symptoms, and 14 patients received two spirometries. After administrating the bronchodilator, FEV1 and PEF significantly increased by 150 mL (6.01%, p = 0.002) and 260 mL/s (6.72%, p = 0.017), respectively. The median (interquartile range) of FeNO measured in nine patients was 15 (10–21) ppb. Patients with NIID have airflow reversibility like asthma. This condition appears to be less associated with airway inflammation and may be related to autonomic dysfunction.