This information is for health professionals and service providers.
More than 80% of people with MND will experience communication difficulties at some point in the disease trajectory.
Advances in technology have improved opportunities to maintain and enhance communication. Timely referral to a speech pathologist is vital to ensure early discussion regarding options available and to support optimal decision making and planning ahead.
Weakness in the bulbar region affects the muscles of the mouth, throat and tongue leading to difficulties with speech. Weakness of the respiratory muscles may also have an impact on speech and the volume of a person's voice.
Speech is not the only form of communication that can be affected by MND.
Muscles used for non-verbal forms of communication can also be weakened by MND. These include muscles used for facial expressions, hand gestures and body language; and the muscles used for writing, typing and operating a mouse.
Up to 60% of people with MND will experience some cognitive changes which may also impact their ability to communicate effectively.
The results of these studies provide evidence that language impairment is as prevalent as executive dysfunction in MND; however, the profiling of linguistic dysfunction is challenged by the association between language and executive functions, impaired speech, and other motor deficits on language assessment and compounded by the heterogeneity of presentations within the population. A small number of cases of aphasia in MND have been described in the literature with patients presenting with comprehension deficits, non‐fluent aphasia, anomia, and dysgraphia. In fact, changes in language function should be considered an important characteristic of MND and are not necessarily explained by an over-lap of ALS and FTD. Other well‐described pragmatic features of communication dysfunction in MND include impaired social communication, impaired facial expression, emotional lability, and pseudo bulbar affect, which all impact on interpersonal communication. The inability to express, or to read, social communication cues has the potential to negatively impact interpersonal relationships. In the context of severe physical and speech disability, familial and social relationships have an increased significance and correlation to quality of life. In this review, motor speech impairment, linguistic, and social communication difficulties are collectively referred to as communication impairment.
Loss of or reduced effectiveness of communication prevents patients from participating in many activities, can lead to social isolation, and can reduce quality of life for both patients and carers. Difficulty with expressive communication results in significant vulnerability and increases reliance on caregivers for communication support, including making health care decisions. The loss of effective communication can result in intellectual and emotional isolation for carers. Communication impairment is linked to increased medical error rates. Preventable adverse events during acute hospital admissions are three times more likely to occur in patients with a communication impairment than in patients without communication difficulties. Additionally, the failure to assess for cognitive deficits and decision‐making capacity has significant health care implications for patients. Communication and/or cognitive difficulties may manifest as problems making decisions, managing finances, planning, and learning new tasks.
Motor impairments such as upper limb dysfunction make writing and the use of alternative communication devices problematic, resulting in additional difficulties engaging in health care decisions without support. Decision making for people with communication impairment associated with motor impairment can be more easily circumnavigated with alternative communication devices and communication strategies, whereas decision making for people with cognitive difficulties requires more structured facilitation (Paynter et al 2019).
The majority of clinically apparent communication difficulties in ALS result from dysarthria. However, subtle changes in language function may also occur, as evidenced by reduced verbal output, reduced spelling ability, increased word-finding difficulties and impaired auditory comprehension of specific classes of language (e.g. verbs more than nouns) and more complex language constructs. Deficits may be subtle and only identifiable with formal neuropsychological testing. Language impairment can reduce the quality of life of both patients and carers and can make clinical management difficult (Class IV). Formal neuropsychological evaluation and support may be required in patients with concomitant evolving language deficits (see previous section).
The overall goal should be to optimize the effectiveness of communication, concentrating on meaningful interpersonal communication with the primary carer and family. This should include strategies for effective conversation and the introduction of alternative communication devices where appropriate (Andersen et al 2012).
Impairment of communication in ALS can also be due to language changes. ALS patients with cognitive impairment, such as frontal lobe type dementia, may have reduced verbal output preceding or following a bulbar syndrome with dysarthria, and often leading to complete mutism within a few months, reduced spelling ability, word finding difficulty, non-fluent aphasia, impaired comprehension and changes in spoken and written language. However, aphasic symptoms can be found independently of dementia in ALS patients and overt dementia and aphasic syndrome may precede the onset of upper and lower motor neuron signs in bulbar regions and limbs (Hanson et al 2011).
Most communication difficulties are as a result of dysarthria, impairment of speech production, caused by:
Impairment of speech production may begin with slurring, hoarseness or weak voice which may progress to total loss of speech (anarthria).
The onset of dysarthria is insidious and may mimic other conditions such as adductor spasmodic dysphonia. In a study of consecutive cases (n = 111) seen in an outpatient speech clinic, the appearance of first speech symptoms varies widely from 33 months before diagnosis to 60 months after diagnosis. Although speech changes occur earlier in those with bulbar onset, functional changes in speech are noted in both bulbar-and spinal-onset groups. Furthermore, after symptoms appear, the rate of bulbar progression is similar for the bulbar-and the spinal-onset groups (Hanson et al 2011).
ALS patients usually have a mixed dysarthria (spastic-flaccid). It is characterized by defective articulation, slow laborious speech, imprecise consonant production, marked hypernasality with nasal emission of air during speech and harshness. A strained/strangled voice (spastic dysphonia) and disruption of prosody may also be present. Decreased respiratory function leads to a weak (low volume) voice, also referred to as inappropriate vocal loudness for conversational utterances. Abnormal vowel production, which may result in mono-pitch voice, short phrases, distorted vowels, mono-loudness and ‘breathy’ voice quality are also seen .
Dysarthria occurs in more than 80% of ALS patients and may cause major disability, earlier in those with bulbar onset, who may become anarthric after a few months. Careful neurological examination, including cranial nerves, and monitoring the rate of progression in affected muscles are important. Loss of communication prevents ALS patients from participating in a number activities and leads to social isolation. Dysarthria significantly reduces the quality of life of ALS patients.
Twenty-five to thirty percent of ALS patients have dysarthria as a first or predominant sign in the early stage of the disease. Dysarthria as an initial symptom is eight times more frequent than dysphagia in ALS. It affects up to 70% of patients with limb onset at a later stage.
Dysarthria in ALS can be rapidly progressive. The bulbar (LMN) ALS patients are generally more severely affected than the corticobulbar (UMN) or spinal ALS patients (Tomik and Guiloff 2010).
Speech and communication should be assessed by a speech pathologist soon after diagnosis and reviewed at each clinic visit. For some people speech will be impacted early on in the disease trajectory particularly if they present with the bulbar form of MND. For others speech may only be affected in the later stages.
Difficulties with communication can lead to decreased social interaction and feelings of isolation loss of control, lowered self-esteem and increased vulnerability.
Additionally, communication aids can cause frustration for both the communication partner and the person with MND.
Depression and/or cognitive changes may be present but can be masked by the progression of the disease and physical changes, and exacerbated by communication difficulties.
Regular review and assessment by a speech pathologist, and other members of the multidisciplinary team including a neuropsychologist and occupational therapist, supports early intervention and optimal decision making.
Guidance regarding Speech referrals:
The speech section recommended SLP speech evaluations at the initial clinic visit and recommended that all clinic visits should gather routine clinical information as listed in Figure 1. Follow-up speech assessments are suggested as an integral component of each ALS clinic visit, although frequency and visit duration may vary depending on multiple factors including patient needs, SLP availability, physician recommendations, and clinic resources. All patients who present with an oral motor exam atypical for bulbar ALS (i.e., asymmetrical tongue or pharyngeal weakness, oral/throat pain, raspy voice, or swallow difficulties preceding speech impairment) should undergo an otolaryngology evaluation to rule out alternative causes (Pattee et al 2019).
The presence of dysarthria in individuals with ALS is both common and distressing. In a survey of people with ALS, the potential loss of speech is rated as the one of the worst aspects of the condition (Hanson et al 2011).
Dysarthria is first assessed in clinical practice by listening to speech when taking a history, also called ‘perceptual’ assessment. The next step is the neurological examination of bulbar function and classifying the dysarthria as UMN, LMN, or mixed with or without predominance or UMN or LMN features. ENT examination of the vocal cords may or may not be required. The ALS Severity Scale – Speech and ALSFRS-R, Appel scale, Norris score and Charing Cross quantitative and qualitative scales are clinically simple and useful ways of grading the severity of the dysarthria and its evolution during follow-up. For timed tests it is important to consider learning effects in establishing baselines for follow-up assessments. There are no comparative studies between these scales to guide the clinician on which one to choose.
The assessment of dysarthria and the neurological examination, including the type of bulbar syndrome, are essential for the diagnosis of, and the condition causing, dysarthria, and will inform subsequent decisions about investigation and management in the ALS clinic. There is currently no hard evidence to support particular assessment methods or management strategies for dysarthria in ALS. Based on clinical opinion, dysarthria in ALS should be assessed early and monitored regularly. Perceptual assessment of the intelligibility of speech by the patient, carers and professionals, remains the main criterion for decisions on communication support. Assessment methods in common use include also various qualitative and quantitative clinical scales of bulbar function and standardized dysarthria scales. A few pharmacological approaches can be tried but are of no proven value (Tomik and Guiloff 2010).
Managing communication difficulties requires a coordinated, multidisciplinary approach and regular review.
This systematic review summarizes the characteristics of dysarthria associated with ALS and evidence related to speech and AAC intervention as well as additional factors that may influence management decisions. The speech characteristics of ALS dysarthria are well documented to be a mixed flaccid--spastic dysarthria with a progressive course leading eventually to loss of speech. Tongue and laryngeal impairments are common early symptoms. As the condition progresses, all speech components become involved. Change in speech and movement rates precede changes in speech intelligibility.
Much less research emphasis has been placed on documenting the effectiveness of speech intervention in people with ALS. There is strong consensus of expert opinion that compensatory strategies such as alphabet supplementation to maintain communication are useful in mild to moderate dysarthria. Limited evidence exists suggesting that prosthetic management of the velopharyngeal system may be helpful to some for a limited period. No evidence is available to support strengthening exercises to improve speech in individuals with ALS.
A variety of AAC devices are available when speech effectiveness declines. Consensus of expert opinion and observational data support the following practices: early assessment and monitoring of speaking rate to inform the timing of AAC intervention, thorough training of both the person with ALS and his or her communication partners in use of the device and communication strategies, and continued follow-up to monitor changing communication needs and abilities. All communication intervention should be carried out within the context of cognitive and respiratory status. Because most of the evidence reported in this review is based on expert opinion and class IV evidence, higher level studies using more rigorous research design are needed to address many issues related to the management of dysarthria in individuals with ALS (Hanson et al 2011).
The management of dysarthria in the ALS clinic starts with a neurological diagnosis of ALS and of the type of dysarthria. The otolaryngologist assessment may be helpful. Neurological assessments, clinical scales and SLT examinations should be performed periodically. Voice quality, speaking rate and communication effectiveness measured perceptually are one of the bases for making the appropriate decision about future speech support.
The goal of management of dysarthria in ALS is to optimize the intelligibility of speech for as long as possible and to concentrate not only on the disabled person, but also on partner-to-partner communication. The timing for assessments, interventions, and the methods of intervention, should be tailored to each patient. Effective evaluation and management of dysarthria in ALS patients may be limited by the availability and accessibility of neurological and SLT services, as well as by age, sex, psychological and psychiatric factors (cognitive status, motivation, personality, psychiatric illness), language function, physical function, hearing status and socioeconomic circumstances (Tomik and Guiloff 2010).
To maintain communication it is important to 'problem solve' and to use preserved muscle function. The speech pathologist, and other members of the multidisciplinary team, will need to work together with the person with MND and their carer to find the best way for them to communicate. Strategies include:
Care alerters are very important as they give the person with MND a greater sense of security. Switches are also often adapted onto modified door chimes and intercom system so they can be used as a care alert device. Other alert systems include commercial pendant-type back to base alerters and pressure buzzers. An occupational therapist can help to find the person’s best means of operating a call device or intercom.
Augmentative and assistive communication (AAC) devices include low-tech options such as:
and high- tech options such as:
Over the last few years there have been significant developments in MND-related AAC including:
AAC systems vary from non-technology aids such as communication books and boards to higher levels including speech synthesis systems. AAC has been described by family members of pwMND as critical for; maintaining social roles, emotional expression, and decision making. It has also been shown that AAC confers an independent positive effect on depression and psychological distress. The holistic benefit of AAC cannot be underestimated; suitable aids can be selected and used either individually or in combination to support communication throughout the disease course.
As a complex and personalized intervention, the provision of communication support equipment requires coordinated care by SLT and Occupational Therapists (OT) within the clinical multidisciplinary team. Ongoing OT support ensures that the accessibility of communication systems is matched to the individual physical capabilities of pwMND at successive disease stages and that these systems can be integrated with other AT needs such as computer access and environmental control.
Recommendations:
The results of this study indicate areas of improvement and the following recommendations have been detailed to address this:
- Earlier SLT referral; offered at the point of diagnosis of MND, irrespective of the subtype and ongoing 3-6 monthly SLT review thereafter.
- Co-ordinated SLT and OT assessment as part of an assistive technology team and development of provision funding pathways for communication devices in conjunction with access methods.
- Investigation of the reasons for declining or abandoning AAC, training provision to support those unfamiliar with higher technology AAC.
- Consideration of cognitive impairment as a possible barrier to AAC use, additional support system provision such as lower technology strategies or working with communication partners.
- Future detailed audit of service provision including qualitative AAC user and communication partner feedback (Elliott et al 2020).
For patients with dysarthria there are many augmentative assistive communication solutions available. These include simple writing boards, text-to-speech apps and eye-gaze systems. Voice banking is a relatively new option that can provide a more natural voice to patients who use text to speech apps such as “Predictable”. Patients can use software such as Model Talker to record their own voice although the quality of the synthesised voice relies on patients recording 1600 phrases which is time consuming. Patients can adopt a “donated” voice and voice banks are now able to combine multiple voices to provide an individual voice matched to age, sex and accent. There is some way to go before communication devices are fast enough to maintain a conversation and produce a voice that can match the prosody of natural speech. In the future brain-computer interfaces may allow patients with severe disability to communicate using electroencephalogram (EEG) control. Currently this technology has been evaluated in only a small number of patients, mostly in experimental environments and is limited by the usability and reliability of the technology (Hobson and McDermott 2016).
The survey findings indicated there were groups of respondents with different needs and preferences for communication technology. Some were early adopters of technology, with the skills, equipment and confidence to engage with technology. Others were willing but lacked either the confidence or skills to use technology, while some had access to equipment but were not willing to engage with the technology. For this sample, there appears to be a need for ongoing training and support in the use of technology to overcome a lack of confidence or skills in using the devices and software, and to maintain individuals as their disease progresses. To achieve this would require resourcing of technical support and expert advice. Ultimately, some people with MND will choose not to utilize communication technology, but there is an opportunity to target those who are willing to use technology but currently lack the necessary access to devices or skills to use them. All study participants would appear to benefit from the involvement of knowledgeable health professionals to create the right ongoing match between technology solutions, functional limitations and personal preferences.
The study results have identified recommendations for service providers to consider when addressing the needs of people with MND:
- Development of awareness-raising activities to allow opportunities for people with MND to adopt technology at an early stage.
- Training in technology devices for people with MND, particularly in the early stages of the disease, and targeted to those who are willing to use technology but currently lack skills or confidence.
- Provision of information on high-quality technology options to counter less helpful information derived from a free Internet search.
- A formal assessment by a qualified health professional is an important step in accessing effective solutions.
- Opportunities for health professionals to maintain and enhance their technology knowledge, so they can offer the best technology solutions matched to the needs of people with MND.
- Future developments in technology are inevitable and will continue to challenge health professionals in working with people with MND with communication needs (Mackenzie et al 2016).
In conclusion, our results suggest that assistive communication tools based on touchscreen-tablet devices (including text input, speech synthesizer, and internet access) should be introduced in early stages of bulbar-onset ALS, particularly when the dysarthria score on the ALSFRS-R is between 2 and 3. These findings demonstrate the positive impact of assistive communication on QoL in the early stages of bulbar-onset ALS, both for patients and caregivers. We believe that early intervention will improve patients’ skills in using communication devices in later stages, when more complex alternative access strategies are necessary. Further research is needed to understand the effect of early intervention on general performance of communication in later disease stages (Londrel et al 2015).
Message banking is a way for a person to digitally record and store words, phrases, sentences, personally meaningful sounds and/or stories using their own voice, inflection and intonation. These messages are catalogued as .wav files and may then be linked to messages in a variety of augmentative communication technologies or sound storage files. This allows people to ‘retrieve’ a message and speak it in their own voice but does not allow them to create novel messages through spelling. Individual words may be combined to create unique messages but the ‘voice’ will sound more staccato than their natural speaking voice.
Voice banking is the process that enables people to record a large inventory of their speech that can then be used to create a synthetic voice that approximates their own voice. Done successfully it allows people to create unique messages and then speak them through a synthesiser or speech generating device. This area of technology is advancing rapidly and there are several options (free and fee based) to create a voice through the use of apps using a smartphone, tablet, computer or other communication device.
More than 80% of people living with MND (plwMND) develop difficulties with their speech, affecting communication, self-identity and quality of life. Most plwMND eventually use an augmentative and alternative communication device (AAC) to communicate. Some AAC devices provide a synthesized voice for speech, however these voices are often viewed as impersonal and a factor in AAC acceptance. Voice banking creates an approximation of the person’s own voice that can be used in AAC and is argued to go some way to preserve a person’s identity when natural voice is lost, but there has been little supporting research.
This research is the first study of its kind to examine decision making surrounding voice banking for plwMND. It shows how preserving identity is critically important in how people deal with a diagnosis of MND. For those choosing to voice bank, this is seen as an effective way of preserving their identity, a way of ‘fighting back’ against MND, helping to maintain social networks and giving a positive psychological benefit. Those deciding against voice banking do not believe it could maintain their identity and cannot bring back the natural voice they once had. Timing was important and some plwMND may not be ready to consider voice banking while their natural voice is unaffected and believe other ways of maintaining social networks to be more effective. Significant others want to support the wishes of the plwMND, believe a voice bank will help maintain their relationship with the plwMND and support the wider social network (Cave and Bloch 2021).
Shoesmith et al 2021, Canadian best practice recommendations for the management of amyotrophic lateral sclerosis - See table 1: symptom management recommendations
National Institute for Health and Clinical Excellence (2016) NICE Guideline: Motor Neurone Disease Assessment and Management – see recommendation:
1.11 Communication
Andersen et al 2012, EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force - Communication in patients with ALS recommendations:
MND Aware e-training program MND Hub – online training course for health professionals and service providers developed by MND NSW – Session 14: Symptom Management – Communication
The Southern Metropolitan Region Palliative Care Consortium (SMRPCC), Robyn Reid, MND Shared Care Worker- MND podcasts - Communication
MND Association of England, Wales and Northern Ireland, Podcast series, MND Matters Episode 4: Voice banking
International ALS/MND Connect 2020:
International Alliance of ALS/MND Associations,18th Allied Professionals Forum, 2020:
International Alliance of ALS/MND Associations,17th Allied Professionals Forum, Perth 2019:
International Alliance of ALS/MND Associations, Annual Meeting, 2018:
International Alliance of ALS/MND Associations,14th Allied Professionals Forum, 2016:
Record me now - recording a video legacy
Smartbox brings the Amazon Echo to Grid 3 - control amazon echo from your device
Model Talker - speech synthesis sorftware package
my-own-voice by acapela - voice banking
speak unique - voice banking
VocaliD - voice banking
ALS guide for speech and language therapists, Eat, think and speak
AAC options for MND, MND Association of England, Wales and Northern Ireland
Suggestions and information about speech changes and AAC, - ALS Association
MND Australia, Motor Neurone Disease: More Facts - for people living with MND
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Cognitive and behaviour change in MND
Breathing and MND an introduction
Equipment, assistive technology and home modifications
MND NSW, Living well with MND 2019:
MND Australia, 4th MND Connect 2018:
Health Talk - healthtalk.org - stories collected by academic researchers who interview people in their own homes, using their own words:
Speech and communication with MND
Royal College of Speech and Language Therapists, podcast, Richard Cave, Voice banking for people living with motor neurone disease: Views and expectations