Assessment of swallowing in patients with bulbar-onset ALS
Among all buALS patients, 49 (45%) underwent at least one swallowing function test, FEES in 15 patients and VFSS in 39 patients, with 5 (4.5%) patients undergoing both tests, and among them 32 (82%) with VFSS and 9 (60%) with FEES had either a strongly suspected or confirmed ALS at the time of referral. VFSS was indicated by a previous aspiration in 18 (46%) patients and 18 (46%) patients underwent VFSS in order to evaluate the safety of oral nutrition, whereas 10 (26%) procedures served to evaluate an esophageal obstruction. Among patients undergoing FEES, the corresponding figures are 4 (27%), 6 (40%), and 5 (33%). The findings of the swallowing function tests appear in Table 2. (Table 2).
Among the VFSS procedures, 8 (21%) resulted in a recommendation for PEG placement. None of the FEES procedures resulted in an immediate PEG recommendation. Outright swallowing interdictions were not provided based on either test. However, patients undergoing a swallowing function test more often received PEG at some point (r = 0.207; p< 0.05). The incidence of PEG placement among all buALS patients reached 78% (n = 86): 94% (n = 46) among those who underwent a swallowing function test and 66% (n = 40) among those who did not.
Among all study patients, 192 of 327 (59%) underwent PEG placement, which was more frequent in the buALS group (86/110 patients, 78%; r = 0.281; p < 0.001) compared to the extALS group (106/217, 49%). PEG was associated with a shorter survival time in the extALS group [53 months with PEG (95% CI 44.1–62.1; p < 0.001) vs. 57 months without PEG (95% CI 42.3–72.1;p < 0.05) and a longer survival time in the buALS group [35.6 months (95% CI 29.9–41.4; p < 0.001) and 26.8 months (95% CI 18.1–35.5; p < 0.05); (Figures 2 & 3).
The mean time from symptom onset to death in patients with buALS was not significantly longer among those primarily referred to a neurologist compared to those referred to an ORL or phoniatrician. Mean survival in the buALS patient group was 32 months (95% CI 27.3–36.9) compared to 33 months (95% CI 23.0–44.2) and 37 months (95% CI 25.9–47.4), respectively, among those initially referred to an ORL and a phoniatrician.