Functional limitation of the masticatory system in patients with bulbar involvement in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) with bulbar dysfunction affects the motor neurons responsible for controlling the muscles in the jaw, face, soft palate, pharynx, larynx and tongue. This cross-sectional study aimed to determine the functional limitation of the jaw in patients with ALS and bulbar dysfunction who had upper motor neuron (UMN), lower motor neuron (LMN) or balanced involvement. One hundred and fifty-three patients with ALS and 23 controls were included. All participants answered using the 8-item Jaw Functional Limitation Scale (JFLS-8). Patients with ALS were grouped by neurologic examination as follows: non-bulbar ALS, bulbar UMN-predominant ALS; bulbar LMN-predominant ALS; and bulbar balanced (UMN + LMN) ALS. Jaw limitation between the different groups was compared using the Kruskal-Wallis test. Patients with non-bulbar ALS had similar mandibular limitations to healthy participants. Only patients with balanced UMN and LMN bulbar manifestations reported greater difficulties in chewing soft food or in jaw mobility compared to the non-bulbar ALS group. Patients with bulbar involvement also had greater difficulties in chewing tough food or chicken and in swallowing and talking compared to the non-bulbar group, regardless of whether UMN or LMN predominant. No significant differences were found between the groups in smiling and yawning difficulties. Bulbar involvement in patients with ALS is associated with functional limitation of the masticatory system. However, balanced bulbar UMN and LMN involvement is associated with the worst impairments in chewing soft food and in opening the jaw widely.