Dysautonomia in anti-Hu paraneoplastic neurological syndromes

We describe anti-Hu patients with dysautonomia, estimate its frequency, and compare them to patients without dysautonomia.MethodsPatients with anti-Hu antibodies diagnosed in the study centre (1990 –2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified.ResultsAmong 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 f...
Source: Journal of Neurology - Category: Neurology Source Type: research