Oral motor functions, speech and communication before a definitive diagnosis of amyotrophic lateral sclerosis

Abstract

The aim of this study was to explore the cranial nerve symptoms, speech disorders and communicative effectiveness of Finnish patients with diagnosed or possible amyotrophic lateral sclerosis (ALS) at their first assessment by a speech-language pathologist. The group studied consisted of 30 participants who had clinical signs of bulbar deterioration at the beginning of the study. They underwent a thorough clinical speech and communication examination. The cranial nerve symptoms and ability to communicate were compared in 14 participants with probable or definitive ALS and in 16 participants with suspected or possible ALS. The initial type of ALS was also assessed. More deterioration in soft palate function was found in participants with possible ALS than with diagnosed ALS. Likewise, a slower speech rate combined with more severe dysarthria was observed in possible ALS. In both groups, there was some deterioration in communicative effectiveness. In the possible ALS group the diagnostic delay was longer and speech therapy intervention actualized later. The participants with ALS showed multidimensional decline in communication at their first visit to the speech-language pathologist, but impairments and activity limitations were more severe in suspected or possible ALS. The majority of persons with bulbar-onset ALS in this study were in the latter diagnostic group. This suggests that they are more susceptible to delayed diagnosis and delayed speech therapy assessment. It is important to start speech therapy intervention during the diagnostic processes particularly if the person already shows bulbar symptoms.

Speech deterioration in amyotrophic lateral sclerosis (ALS) after manifestation of bulbar symptoms

“…Particularly those with bulbar‐onset ALS have obvious speech deterioration before a definitive diagnosis and the beginning of speech‐therapy intervention (Makkonen et al . ). The speaking rate and intelligibility was observed to be lower in bulbar‐onset than spinal‐onset participants after the diagnosis (Ball et al .…”

“…People with ALS demonstrate various speech symptoms at the time of diagnosis, ranging from normal speech to the use of augmentative communication (Yorkston et al 1993). Particularly those with bulbar-onset ALS have obvious speech deterioration before a definitive diagnosis and the beginning of speech-therapy intervention (Makkonen et al 2016). The speaking rate and intelligibility was observed to be lower in bulbar-onset than spinal-onset participants after the diagnosis (Ball et al 2002).…”

Factors Associated With Communicative Participation in Amyotrophic Lateral Sclerosis

“…Bulbar impairment is common and has received considerable research attention (Makkonen et al, 2016;Yunusova et al, 2010). The relationship between these impairments and their impact on communication in daily activities, such as conversation, is unclear, but understanding these associations is important for tailoring interventions to meet the needs that are relevant to clients (Bloch & Tuomainen, 2016).…”

Inteligibilidade de fala em pessoas com Esclerose Lateral Amiotrófica (ELA)

“…Frequent speech motor changes in ALS, such as dysarthria, are characterized by impaired oral production due to impaired neuromuscular control, slower, weak, and/or inaccurate communication, impaired breathing, phonation, resonance, articulation, and prosody, with wide a variability of symptoms (7) . As dysarthria progress, they compromise the functionality of communication in day-to-day activities, social participation, and negatively interfere with the person's quality of life (6,8) .…”

“…In this situation, the use of Supplementary and/or Alternative Communication (SAC) is configured as a communication strategy with the family, the health team, and close friends, facilitating socialization, autonomy, and decision making (12) . The communicative vulnerability situation experienced by the person with ALS, especially after the loss of communication, can lead to emotional problems and/or anxiety, frustration, fear, and sadness (8,12) , and can be considered as one of the worst aspects of the disease (6) .…”