Abstract：
OBJECTIVE: Dysautonomia has been associated with paraneoplastic neurological syndrome (PNS)-related mortality in anti-Hu PNS, but its frequency and spectrum remain ill-defined. We describe anti-Hu patients with dysautonomia, estimate its frequency, and compare them to patients without dysautonomia.
METHODS: Patients with anti-Hu antibodies diagnosed in the study centre (1990-2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified.
RESULTS: Among 477 anti-Hu patients, 126 (26%) had dysautonomia (the only PNS manifestation in 7/126, 6%); gastrointestinal (82/126, 65%), cardiovascular (64/126, 51%), urogenital (24/126, 19%), pupillomotor/secretomotor (each, 11/126, 9%), and central hypoventilation (10/126, 8%). Patients with isolated CNS involvement less frequently had gastrointestinal dysautonomia than those with peripheral (alone or combined with CNS) involvement (7/23, 30% vs. 31/44, 70% vs. 37/52, 71%; P = 0.002); while more frequently central hypoventilation (7/23, 30% vs. 1/44, 2.3% vs. 2/52, 4%; P < 0.001) and/or cardiovascular alterations (18/23, 78% vs. 20/44, 45% vs. 26/52, 50%; P = 0.055). Median [95% CI] overall survival was not significantly different between patients with (37 [17; 91] months) or without dysautonomia (28 [22; 39] months; P = 0.78). Cardiovascular dysautonomia (HR: 1.57, 95% CI [1.05; 2.36]; P = 0.030) and central hypoventilation (HR: 3.51, 95% CI [1.54; 8.01]; P = 0.003) were associated with a higher risk of death, and secretomotor dysautonomia a lower risk (HR: 0.28, 95% CI [0.09; 0.89]; P = 0.032). Patients with cardiovascular dysautonomia dying ≤ 1 year from clinical onset had severe CNS (21/27, 78%), frequently brainstem (13/27, 48%), involvement.
CONCLUSIONS: Anti-Hu PNS dysautonomia is rarely isolated, frequently gastrointestinal, cardiovascular and urogenital. CNS dysfunction, particularly brainstem, associates with lethal cardiovascular alterations and central hypoventilation, while peripheral involvement preferentially associates with gastrointestinal or secretomotor dysautonomia, being the latest more indolent.
METHODS: Patients with anti-Hu antibodies diagnosed in the study centre (1990-2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified.
RESULTS: Among 477 anti-Hu patients, 126 (26%) had dysautonomia (the only PNS manifestation in 7/126, 6%); gastrointestinal (82/126, 65%), cardiovascular (64/126, 51%), urogenital (24/126, 19%), pupillomotor/secretomotor (each, 11/126, 9%), and central hypoventilation (10/126, 8%). Patients with isolated CNS involvement less frequently had gastrointestinal dysautonomia than those with peripheral (alone or combined with CNS) involvement (7/23, 30% vs. 31/44, 70% vs. 37/52, 71%; P = 0.002); while more frequently central hypoventilation (7/23, 30% vs. 1/44, 2.3% vs. 2/52, 4%; P < 0.001) and/or cardiovascular alterations (18/23, 78% vs. 20/44, 45% vs. 26/52, 50%; P = 0.055). Median [95% CI] overall survival was not significantly different between patients with (37 [17; 91] months) or without dysautonomia (28 [22; 39] months; P = 0.78). Cardiovascular dysautonomia (HR: 1.57, 95% CI [1.05; 2.36]; P = 0.030) and central hypoventilation (HR: 3.51, 95% CI [1.54; 8.01]; P = 0.003) were associated with a higher risk of death, and secretomotor dysautonomia a lower risk (HR: 0.28, 95% CI [0.09; 0.89]; P = 0.032). Patients with cardiovascular dysautonomia dying ≤ 1 year from clinical onset had severe CNS (21/27, 78%), frequently brainstem (13/27, 48%), involvement.
CONCLUSIONS: Anti-Hu PNS dysautonomia is rarely isolated, frequently gastrointestinal, cardiovascular and urogenital. CNS dysfunction, particularly brainstem, associates with lethal cardiovascular alterations and central hypoventilation, while peripheral involvement preferentially associates with gastrointestinal or secretomotor dysautonomia, being the latest more indolent.
摘要：
目的：自主神经失调与抗HuPNS的副肿瘤神经综合征（PNS）相关死亡率有关，但是它的频率和频谱仍然不确定。我们描述了患有自主神经障碍的抗Hu患者，估计它的频率，并将它们与没有自主神经障碍的患者进行比较。
方法：对在研究中心（1990-2022年）诊断出的具有抗Hu抗体的患者进行回顾性分析；对具有自主神经体征和症状的患者进行鉴定。
结果：在477名抗Hu患者中，126（26％）有自主神经障碍（7/126，6％的唯一PNS表现）;胃肠道（82/126，65％），心血管（64/126，51%），泌尿生殖系统（24/126，19%），瞳孔运动/分泌运动(每个，11/126，9%），和中枢通气不足（10/126，8%）。与周围（单独或合并中枢神经系统）受累的患者相比，孤立中枢神经系统受累的患者发生胃肠道自主神经紊乱的频率较低（7/23，30％vs.31/44，70%与37/52，71%；P=0.002）；而更常见的是中枢通气不足(7/23，30%vs.1/44，2.3%与2/52，4%；P<0.001）和/或心血管改变（18/23，78%vs.20/44，45%与26/52,50%;P=0.055)。在有（37[17;91]个月）或无（28[22;39]个月；P=0.78）的患者之间，中位[95％CI]总生存期没有显着差异。心血管自主神经障碍(HR:1.57,95%CI[1.05;2.36];P=0.030)和中枢通气不足(HR:3.51,95%CI[1.54;8.01];P=0.003)与较高的死亡风险相关,分泌运动功能障碍的风险较低(HR:0.28,95%CI[0.09;0.89];P=0.032)。心血管自主神经障碍患者死亡≤1年，从临床发作（21/27，78％），有严重的中枢神经系统，经常脑干（13/27，48%），involvement.
结论：很少分离抗HuPNS自主神经障碍，经常是胃肠的，心血管和泌尿生殖系统。CNS功能障碍,尤其是脑干，与致命的心血管改变和中枢通气不足有关，虽然外周受累优先与胃肠道或分泌运动性自主神经障碍相关，成为最新的更懒惰的人。
方法：对在研究中心（1990-2022年）诊断出的具有抗Hu抗体的患者进行回顾性分析；对具有自主神经体征和症状的患者进行鉴定。
结果：在477名抗Hu患者中，126（26％）有自主神经障碍（7/126，6％的唯一PNS表现）;胃肠道（82/126，65％），心血管（64/126，51%），泌尿生殖系统（24/126，19%），瞳孔运动/分泌运动(每个，11/126，9%），和中枢通气不足（10/126，8%）。与周围（单独或合并中枢神经系统）受累的患者相比，孤立中枢神经系统受累的患者发生胃肠道自主神经紊乱的频率较低（7/23，30％vs.31/44，70%与37/52，71%；P=0.002）；而更常见的是中枢通气不足(7/23，30%vs.1/44，2.3%与2/52，4%；P<0.001）和/或心血管改变（18/23，78%vs.20/44，45%与26/52,50%;P=0.055)。在有（37[17;91]个月）或无（28[22;39]个月；P=0.78）的患者之间，中位[95％CI]总生存期没有显着差异。心血管自主神经障碍(HR:1.57,95%CI[1.05;2.36];P=0.030)和中枢通气不足(HR:3.51,95%CI[1.54;8.01];P=0.003)与较高的死亡风险相关,分泌运动功能障碍的风险较低(HR:0.28,95%CI[0.09;0.89];P=0.032)。心血管自主神经障碍患者死亡≤1年，从临床发作（21/27，78％），有严重的中枢神经系统，经常脑干（13/27，48%），involvement.
结论：很少分离抗HuPNS自主神经障碍，经常是胃肠的，心血管和泌尿生殖系统。CNS功能障碍,尤其是脑干，与致命的心血管改变和中枢通气不足有关，虽然外周受累优先与胃肠道或分泌运动性自主神经障碍相关，成为最新的更懒惰的人。
