抄録
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease classified into limb-onset and bulbar-onset types. Patients with bulbar-onset ALS frequently manifest oral dysfunction, including dysphagia, dysarthria, and tongue muscle atrophy, from an early stage. Evaluation of swallowing function and appropriate rehabilitation are crucial for maintaining quality of life. While the effectiveness of palatal augmentation prostheses (PAP) and palatal lift prostheses (PLP) for ALS patients has been documented, their application in bulbar-onset cases remains limited. This report describes two cases of bulbar-onset ALS patients treated with a palatal augmentation prosthesis with palatal lift function (PAP+PLP), evaluated using videofluoroscopic swallowing study (VFSS).
The two cases were male patients aged 40 and 60 years old who developed progressive bulbar symptoms post-ALS onset, subsequently requiring gastrostomy, glottic closure, and permanent tracheostomy due to severe aspiration. Despite these surgical interventions, both patients wished to maintain oral intake. Neither patient had prior experience of prosthetics. Both maintained natural dentition. The PAP+PLP was designed to enhance the anchoring function of the anterior tongue and velopharyngeal closure. VFSS demonstrated improvements in swallowing function, reduction in nasal regurgitation, and enhanced coordination of oral and pharyngeal structures. These outcomes suggest that PAP+PLP represents a promising prosthetic intervention for managing swallowing disorders in ALS patients.