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

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.

Is Old Age Just a Number in Language Skills? Language Performance and Its Relation to Age, Education, Gender, Cognitive Screening, and Dentition in Very Old Finnish Speakers.

PURPOSE: This study reports on how very old (VO) Finnish people without dementia perform in the Western Aphasia Battery (WAB) and two verbal fluency tasks and which demographic factors predict the performance. METHOD: The study included fifty 80- to 100-year-old community-dwelling Finnish speakers with no dementing illnesses or speech-language disabilities, who completed the WAB and two verbal fluency tasks. Multifactorial statistical analyses with recursive partitioning were carried out to determine the significant predictors out of five predictor variables (age, gender, education, dentition, and Mini-Mental State Examination [MMSE]) for four response variables (WAB Aphasia Quotient [AQ], Language Quotient [LQ], semantic, and phonemic word fluencies). RESULTS: Overall, individual variation was notable in VO speakers. All predictor variables were statistically significantly associated with one or more of the language skills. Age was the most significant predictor; the critical age of 85-86 years was associated with a decline in WAB-AQ and semantic fluency. Poor dentition and the MMSE score both predicted a decline in WAB-LQ and phonemic fluency. A high level of education was positively associated with the skills of the best-performing individuals in WAB-AQ, WAB-LQ, and semantic fluency. CONCLUSIONS: VO age is a significant factor contributing to language performance. However, a younger age, a good cognitive performance, intact teeth, and a higher educational level also seem to have a preservative power as regards language skills. Gender differences should be interpreted with caution. The results of this study provide culture- and language-specific normative data, which aids in differentiating typical aging from the signs of acute or degenerative neuropathology to ensure appropriate medical and therapeutic interventions.

Clinical management of voice and breathing problems in two patients with vagus nerve stimulation therapy.

We report two cases highlighting the diversity of vagal nerve stimulation (VNS)-related effects on voice and breathing in patients with refractory epilepsy. The patients had both implantation and stimulation-related side effects, which lasted for several months, impacting on their quality of life. The adverse effects appear to be due to recurrent laryngeal nerve paralysis-related vocal cord hypofunction and stimulation-related vocal fold spasms, however, their inter-relationship is complex. In one of the patients, we were able to utilize the novel programming capabilities of the VNS device to reduce the laryngeal side effects without compromising therapeutic efficacy. [Published with video sequences].

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

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.