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

ABSTRACT

BACKGROUND: The symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on communicative need in ALS. AIMS: To investigate how long natural speech will remain functional and to identify the changes in the speech of persons with ALS. METHODS & PROCEDURES Altogether 30 consecutive participants were studied and divided into two groups based on the initial type of ALS, bulbar or spinal. Their speech disorder was evaluated on severity, articulation rate and intelligibility during the 2-year follow-up. OUTCOME & RESULTS: The ability to speak deteriorated to poor and necessitated augmentative and alternative communication (AAC) methods with 60% of the participants. Their speech remained adequate on average for 18 months from the first bulbar symptom. Severity, articulation rate and intelligibility declined with nearly all participants during the study. To begin with speech deteriorated more in the bulbar group than in the spinal group and the difference remained during the whole follow-up with some exceptions. CONCLUSIONS & IMPLICATIONS The onset of bulbar symptoms indicated the time to loss of speech better than when assessed from ALS diagnosis or the first speech therapy evaluation. In clinical work, it is important to take the initial type of ALS into consideration when determining the urgency of AAC measures as people with bulbar-onset ALS are more susceptible to delayed evaluation and AAC intervention.