在抗胡神经瘤神经综合征的dysautonomia
Macarena Villagrán-García1,2, Antonio Farina1,2,3, Joaquín Arzalluz-Luque1,4
1French Reference Centre on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, 59 Bd Pinel, 69677, Bron Cedex, France.
Journal of neurology
|March 18, 2024
概括
抗胡神经瘤神经综合征 (PNS) 的失自症很常见,影响了26%的患者,并且经常涉及胃肠道和心血管系统. 虽然整体存活率没有受到影响,但特定类型 (如心血管功能障碍症) 会增加死亡风险.
科学领域:
- 神经学 神经学
- 在瘤学瘤学.
- 自主医学是一种自主医学.
背景情况:
- 脱自主症与抗胡氏瘤神经综合征 (PNS) 的死亡率有关,但其患病率和临床范围尚不清楚.
- 这项研究调查了抗Hu抗体患者的dysautonomia的频率和特征.
研究的目的:
- 为了描述抗Hu PNS患者的dysautonomia的频谱.
- 为了估计这个人群中dysautonomia的频率.
- 为了在临床特征和生存率方面比较具有和没有dysautonomia的患者.
主要方法:
- 在1990年至2022年期间被诊断患有抗胡抗体的患者的回顾性审查.
- 识别表现出自主体征和症状的患者.
主要成果:
- 在26% (126/477) 的抗胡病患者中,存在失自症,很少作为唯一的PNS表现 (6%).
- 通常受到影响的系统包括胃肠道 (65%),心血管 (51%) 和泌尿器官 (19%).
- 心血管失调和中心低通风与死亡风险增加有关,而秘密运动失调显示风险较低. 中枢神经系统 (CNS) 的参与,特别是脑干,与致命的心血管变化和中央低通风相关.
结论:
- 抗Hu PNS中的失自性是常见的,通常涉及多个系统,主要是胃肠道,心血管和泌尿器官.
- 中枢神经系统的功能障碍,特别是脑干的干扰,与严重的,可能致命的心血管和呼吸道并发症有关.
- 周围部位的参与更常见地与胃肠道或机密运动症状有关,后者看起来更惰.
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