BACKGROUND: Anti-IgLON5 disease is a recently described neurological disorder with multisystemic features. The disease is characterized by the presence of IgLON5 antibodies in serum and cerebrospinal fluid. Our objective is to describe in detail the otorhinolaryngological manifestations of this disease, which are frequent and may include dysphagia, dysarthria, vocal cord paralysis and laryngospasm.
METHODS: In this study, we present a series of 9 patients with anti-IgLON5 disease and otolaryngological manifestations. Patients were evaluated between July 2012 and March 2022 by video-polysomnography, fiber-optic laryngoscopy, and functional endoscopic evaluation of swallowing.
RESULTS: The median age was 71 years, and 5 (56%) were female. Video-polysomnography showed a NREM/REM parasomnia in 6 patients (67%), obstructive sleep apnea in 8 (88%), stridor during sleep in 7 (78%) and central apneas in 1 (11%). Six out of the 9 patients (67%) presented episodes of acute respiratory failure that required mechanical ventilation, 6 had vocal fold palsy with 4 of them requiring tracheostomy (3 had to be performed on an emergency basis). Dysphagia occurred in 8 patients (89%). Prominent upper airway secretion and sialorrhea was also present in 3 cases.
CONCLUSIONS: The anti-IgLON5 disease exhibits extensive otolaryngological symptoms, mainly affecting the upper airway. These symptoms affect the quality of life and can be life-threatening. Prompt acute management is essential for stridor, dyspnea, and dysphagia. Given the potential severity of the symptoms and rarity of the disease, it is important for otolaryngologists to be familiar with anti-IgLON5 disease.
METHODS: Level 4.

BACKGROUND: The continuous intraoperative neuromonitoring (C-IONM) of the recurrent laryngeal nerve (RLN) could help reducing the incidence of nerve paralysis after thyroid surgery, in comparison with the mere anatomical visualization of the RLN. The objective is to assess the efficacy and utility of C-IONM as a predictive test for recurrent laryngeal nerve paralysis after thyroidectomy.
METHODS: A prospective observational study was performed in 248 patients who underwent thyroid surgery where C-IONM was applied, between September 2018 and December 2019, in a high-volume center. A previous and later laryngoscopy was performed, which allowed to evaluate the reliability of the C-IONM as a predictive test for recurrent paralysis. Sensitivity (SE), specificity (SP), positive predictive value (PPV), negative predictive value (NPV) were studied.
RESULTS: A total number of 171 thyroidectomies, 62 hemithyroidectomies, 15 totalization thyroidectomies and 27 thyroidectomy with cervical dissections were performed. Postoperative laryngoscopy was altered in 40 patients (16.12%). The SE, SP, PPV and NPV values ​​were 65%, 94.7%, 70.2% and 93.4% respectively.
CONCLUSIONS: C-IONM is a safe technique that provides real-time information about anatomical and functional integrity of the RLN and can improve the results of thyroid surgery.

OBJECTIVE: The risk of producing bilateral laryngeal paralysis (BLP) in total thyroidectomy (TT) is low, but it is a concern for the surgeon and a serious safety incident that may compromise the airway, require reintubation or tracheostomy and cause serious sequelae or death. Neuromonitoring (NM), as an early diagnostic tool for the existence of injury to the recurrent laryngeal nerve (RLN), has not been shown to have reduced the risk, even though published series show lower incidences. Our objective was to estimate the risk of bilateral RLN paralysis with and without NM TT by systematic review and meta-analysis.
METHODS: We performed a systematic review of clinical trials, cohort studies and case series with total thyroidectomy without NM published in the period 2000-2014. A database search was performed using PubMed, Scopus (EMBASE) and the Cochrane Library. Heterogeneity between studies was explored and weighted risks grouped according to random effects models were estimated.
RESULTS: We selected 40 articles and estimates of risk were identified in 54 case series (without NM, 25; with NM, 29) with 30,922 patients. The prevalence of BLP in the series with NM was lower compared to that without NM (2.43‰, [1.55 to 3.5‰] versus 5.18‰ [2.53 to 8.7‰]). This difference is equivalent to an absolute risk reduction of 2.75‰ with a number needed to treat of 364.13. The NM group was more homogeneous (I2=7.52%) than those without NM (I2=79.32%). The observed differences in the subgroup analysis were very imprecise because the number of observed paralysis was very low.
CONCLUSIONS: The risk of bilateral paralysis is lower in studies with neuromonitoring.

OBJECTIVE: Bilateral laryngeal paralysis cause serious respiratory complications. In thyroid surgery, neuromonitoring helps in identifying the recurrent laryngeal nerve, reports on its functioning at the end of surgery, supports decision making, and may reduce the risk of bilateral paralysis. Our objective was to estimate the influence of neuromonitoring in operative strategy and extubation safety in total thyroidectomy.
METHODS: A non-randomized prospective study was conducted on 210 patients undergoing total thyroidectomy (420 laryngeal nerves stimulated included). We collected qualitative neuromonitoring variables (presence or absence of final signal after stimulation of the vagus nerve), and postoperative indirect laryngoscopy (normal motility or paralysis), performed until 3rd day after the surgery.
RESULTS: The accuracy of the test was 99.5% (95% CI 98.3 to 99.9). The positive predictive value was 100% (95% CI 99.1 to 100), which showed the high ability of neuromonitoring to predict paralysis in case of loss of signal, and the negative predictive value was 99.5% (95% CI 98.3 to 99.9), which indicated its predictive capacity for normal motility when there is a normal signal.
CONCLUSIONS: In our group of patients, recurrent laryngeal nerve monitoring was useful in total thyroidectomy as it provided information on the prognosis of laryngeal motility, and helped in making decisions during surgery when there was signal loss. Due to the risk of serious respiratory complications due to bilateral recurrent laryngeal nerve paralysis, we opted for the performing of the 2-stage total thyroidectomy in case of signal loss in the first lobectomy. Thereby, neuromonitoring contributed to the safety of the airway in tracheal extubation, aiding in the prevention of a possible bilateral laryngeal paralysis.