Motor Neurone Disease
(hereinafter MND) is a degenerative disease that directly affects both upper
and lower motor neurones in the brain and/or spinal cord, which in turn reduces
muscle movement. Those afflicted with the condition on average die within 3-5
years, and in the majority of cases this is through respiratory failure
(Schiffer et al. 1987). Since the muscle wastage caused by the disease occurs
at a global level, it has wide implications for the patient; the care provided
spreads across many medical disciplines.
One of these disciplines is
Speech and Language therapy (hereinafter SLT). The two key areas of focus for
SLT are communication and swallowing function; both are vital, but arguably for
different reasons. Being unable to swallow has consequences for the medical
health of the patient as there is greater risk of malnutrition and weight loss
et al. 2013), as well as the
additional risk of pneumonia caused by aspiration
(Langmore et al. 1998). Communication impairment interferes less with the
physical health of the patient, but can have negative effects on their mental
and emotional health. Therefore the role of SLT is unique in its responsibility
in maintaining communication between the patient and their loved ones, as well
as to the staff managing the health of the patient. The failure of a patient to
be able to communicate their wishes has been reported to be linked with depression,
a sense of powerlessness and emotional disengagement (Hecht et al, 2002).
Additionally, many relevant sources such as the Royal College of Speech &
Language Therapists (hereinafter RCSLT) do not provide specific documents that
focus on the role of SLT for this population.
This piece aims to catalogue
the relevant issues for this field in a holistic context, presenting the
evidence and rationale for therapies at the disposal of SLTs to maximise the
patient’s quality of life both physically and emotionally.
Following on from a diagnosis of MND the organisation of
care should be established using interprofessional teams, with the patient and
their family at the nucleus. This approach aligns with the fourth objective of
the NHS Constitution (2015), which aims to have care tailored and coordinated
around the patient and their family. These interprofessionals will usually be
led by a neurologist and may also involve the general practitioner, a nurse
specialist, occupational therapist (OT), physiotherapist (PT), SLT and/or
dietician. In the later stages of the disease the involvement of
gastroenterology and respiratory physicians may be required; also the
involvement of other organisations such as the MNDA (Motor Neurone Disease
Association), hospices, social workers or advocates should be linked to the
patient (Wood-Allum & Shaw, 2010). The interprofessional method is highly
recommended by the National Institute for Clinical Excellence (hereinafter
NICE) (NICE, 2016) guidelines and is further endorsed by qualitative studies
that have explored the opinions of healthcare professionals (Hogden et al.
2012) and of patients themselves (Oishi & Murtagh, 2014). Although there
does seem to be a lack of systematic research exploring the effectiveness of
interprofessional working in MND, the evidence currently available would imply
that this method is effective.
The progression of muscular
degeneration and reduced muscle control means that patients with MND are at
risk of dysphagia. Dysphagia is the term that denotes an impairment of the
swallow mechanism. SLTs are one of the primary professionals managing a
patient’s swallow mechanism, but other professionals that can also advise
include: dieticians, OTs and PTs (MNDA, 2017). The cooperation of these
professions means that the patient’s weight, risk of aspiration and positioning
are evaluated so that the patient’s comfort is ensured. The patient’s swallow
mechanism is an important factor for many other treatments to be carried out,
for example, the administration of drugs or the management of nutrition. These
depend on the SLT to assess and manage any dysphagic symptoms that may occur.
Whilst the NICE (2016) guidelines state the importance of the swallowing
assessment, there is seemingly a lack of SLT specific guidance in relation to
MND dysphagia assessment; this may be due to the rarity of the condition and
therefore a lack of participants to carry out research upon. Thus, SLTs may use
different assessment techniques depending on the setting in which the patient
resides at the time of assessment. If the patient is receiving care in their
own home then a bedside swallowing assessment, monitoring the sound of the
swallow using a stethoscope and measuring the blood-oxygen levels, may be
carried out to establish if the patient is experiencing difficulties in their
swallow such as aspiration, which will have a detrimental effect on their
respiration, which is the leading cause of death in patients with MND (Rowland
& Shneider, 2001). Despite evidence suggesting that these methods of
assessment are determined to be less accurate (Ramsey et al. 2003), they are
easily accessible and timely. If the patient is in a specialist facility then
the options of more in-depth and thorough assessments such as videofluroscopy
become available. Such assessments have found to be accurate but are also
uncomfortable for the patient (ibid, 2003). It can be argued that the comfort
of the patient in a degenerative condition is paramount unless the clinical
risk of aspiration outweighs the patient’s comfort. In such instances
discussions with the patient and their family would be ideal and would reflect
the NHS constitution (2015) in providing dignity, respect and compassion.
Overall it would appear that the assessment of swallowing for patients with MND
solely relies on the judgement of the SLT and their own experience as there are
no specific guidelines or research to suggest that certain methods are more
suitable for patients with MND.
Dysarthria, a disorder of motor
speech that causes a slurring of speech to occur, can become a symptom of the
disease in the first instance; Duffy (2013) claims that 25% of patients with
MND develop dysarthric symptoms. Whether or not the patient has dysarthria post
diagnosis is arguably irrelevant as dysarthria develops in over 80% of patients
with MND at some point in the disease (Tomik & Guiloff, 2000). Therefore
early assessment and subsequent management should be put in place to help the
patient and their family ease through the transition of the disease. To this
end, the SLT plays a vital role in maintaining the patient’s ability to
communicate, which in turn has wide implications that range from the patient
giving consent, to ultimately, their quality of life (Hecht et al. 2002), which
the NHS constitution states as vital (NHS, 2015). Therefore all parties
involved need the SLT to allow the patient to have a meaningful method of
communication to express their thoughts and desires.
The SLT should initially carry
out assessments to establish whether the patient at the early stage of the
disease has dysarthria and if so, its severity. In order to establish a
baseline the SLT could use a standardised dysarthria assessment such as the
Frenchay Dysarthria Assessment (Enderby, 1983), which would allow a
comprehensive assessment of the patient’s potential dysfluency; furthermore it
would allow the SLT to track the deterioration of the patient and therefore
begin to develop contingencies for their communicative deterioration. Duffy
(2013) has reported that in a sample of dysarthric MND patients in the Mayo
clinic, nearly 99% had mixed dysarthria. Mixed dysarthria impairs the larynx,
resulting in a quiet, breathy voice as well as weakening the oral musculature
leading to imprecise consonants (ibid, 2013). If the SLT decides that speech is
still a functional option for the patient then adjustments would need to be
made to accommodate the patient’s dysarthria. The SLT may employ strategies
including: reduced speech rate, over-articulation and the use of breath control
(Tomik & Guiloff, 2010). In order to help facilitate the patient’s speech
the patient’s family may need to be trained to develop helpful conversational
practices that will enable the patient to be heard, for example, a reduction in
background noise via home adaptations. Such decisions can be challenging for
the patient’s family to undertake and therefore discussions alongside
occupational therapy should be held to make suitable adaptations for carrying
out activities for daily living (Scott & McPhee, 2014).
There is little evidence in the
literature regarding therapy in MND patients with dysarthria, and the evidence
itself consists of limited case series designs (Murphy, 2004; Yorkston et al.
1996). Scott & McPhee (2014) state that dysarthria management should be
used with caution as the inevitable deterioration of a patient’s speech may
lead to the patient feeling powerless, despite efforts to manage the dysarthria
symptom. Studies have also claimed that overexertion of oral mechanisms through
therapies can be detrimental in the long term (Beukelman et al. 2011). Neither
the RCSLT nor the NICE guidelines provide any information for SLTs regarding
managing dysarthria in the context of patients with MND. In comparison with
other degenerative diseases such as Parkinson’s disease, the evidence for
dysarthria in MND is lacking. This is particularly worrying as the patient
literature also suggests that patients with MND wish to retain their own speech
for as long as possible (Murphy, 2004). Therefore, more research needs to find
dysarthria management strategies in MND otherwise patients’ own perception of
their quality of life may be harmed.
As the disease progresses the
risk of aspiration in a patient increases, therefore the SLT, as well as other
health professionals, have a duty of care to ensure that the risk of swallowing
difficulties is managed. One method of managing swallowing is texture
modifications of food. Texture modifications involve blending food so that it
becomes a thicker consistency to allow for a patient to swallow it more easily;
this has been shown to slightly improve swallowing safety in patients with risk
of dysphagia (Steele et al. 2015). Texture modifications allow the patient to
continue orally feeding and arguably retain some normality in their quality of
life (Leigh et al, 2003). However texture modification intervention will need
the input of the dietician as weight loss and malnutrition may become a risk
for those with MND (ibid, 2003).
Initially the SLT may opt to
teach the patient safer swallowing techniques such as the chin tuck or the
supraglottic swallow (Desport et al. 1999; Heffernan et al. 2004) to reduce the
risk of aspiration during the swallow. This method allows the patient to feel
that they are managing their own condition (NHS England, 2015). The difficulty
may be that further in the disease process the ability for the patient to
voluntarily carry out these techniques will be limited. Therefore, the SLT may
adopt this technique in the early stages of the disease but they may not be
appropriate in the later stages so the SLT should carefully monitor the
swallowing of the patient.
Other professionals are also
able to contribute to the management of a patient’s swallow mechanism in MND.
OTs and PTs are able to suggest optimal positioning and modified cutlery to
enable a more comfortable and safer entry for food/fluid into the mouth
(Wood-Allun & Shaw, 2010) and therefore collaboration would allow the
symptoms of the muscular degeneration to be less impactful on eating and
drinking. In the later stages of the disease, patients with MND can begin to
have difficulties with saliva control, which in turn can lead to aspiration. In
such cases the SLT may need to begin to consult with nurses to carry out
suctioning or specialist nurses to administer drugs to manage the saliva
production and its thickness. Finally, when the disease process reaches a
severity whereby the patient becomes unable to physically swallow, then the SLT
will need to raise the possibility of inserting a PEG
in the patient. Such a decision would first and foremost require the consent of
the patient and their families. Moreover, a meeting would need to be held to
discuss the implication of the PEG for other healthcare professionals; for
example the SLT would need to balance the risk of aspiration via oral feeding
as opposed to enteral as there is still debate as to whether PEG feeding
prevents aspiration (Heffernan et al. 2004; Blumenstein et al. 2014).
Nevertheless, a referral to a gastroenterologist to assess the viability of a
PEG insertion would be required. Considerations at this stage of the disease
must balance the desires and quality of life of the patient and family,
alongside the clinical risk of aspiration.
When the patient reaches a
stage in which the SLT decides it is no longer viable for them to use their own
speech to communicate, then the SLT must consider alternative methods in which
the patient is able to communicate. The SLT may incorporate Alternative
Augmentative Communication devices (hereinafter AAC), but prior to their
implementation the SLT should consult both the patient and their family to
establish the desires and motivations behind the use of an AAC device and
implement it based on the will of these parties (MNDA, 2016; Scott &
McPhee, 2014). A series of assessments will be carried out to examine the
eligibility of the patient and whether the stage of their disease allows them
to physically access either low-tech or hi-tech devices. Low-tech AAC devices
include: voice amplifiers, pen and paper, alphabet boards which need little
training and can be easily adapted for the specific needs of the individual. In
MND the control of the eyes is usually spared from the disease (Duffy, 2013)
therefore a patient can use hi-tech AAC eye-gaze systems to communicate with
others if they are unable to use their limbs for low-tech devices. However, the
downfall of using such a device is that the effective use of them requires
prolonged training as well as the installation and continued management of the
If the patient is deemed to
have sufficient need to use hi-tech AAC, then the equipment is loaned from a
specialist AAC hub complying with the NICE (2016) guidelines that state that
patients should be given a suitable device without delay. Once the AAC device
is able to be used at a functional level by the patient, the SLT should
specifically train family members, since they will be the prime facilitators of
the patient and their communication (Ball et al. 2005). The SLT’s role in
training the family members is vital as studies have found that successful
implementation and use of AAC comes down to the training that the SLT provides
to the patient, their family and other professionals (Ball et al. 2010; Murphy,
The SLT must ensure that the
interprofessional team is aware of the AAC system and how it is used so that
they can effectively communicate and carry out their interventions with the
consent of the patient. Furthermore, the SLT may consult with PTs and OTs to
ensure that the AAC equipment is in a position that is accessible and
comfortable for the user (Scott & McPhee, 2014). The patient literature
overwhelmingly suggests that the successful implementation of AAC has positive
impacts to patients with MND; studies report feelings of purpose, happiness and
mental and emotional wellbeing (Beukelman et al. 2011; Murphy, 2004; MNDA,
2016). Although, Murphy (2004) also found that some patients felt that AAC
correlated to admitting defeat and wished to use their own voice for as long as
possible. Clearly, the wishes of the patient are crucial as their own perceived
quality of life could be changed if an AAC device is applied without the
patient’s full acknowledgement and consent; these actions are supported by the
NHS constitution (2015). Ultimately it is the right of the patient to decide
the care pathway they use from the options they have available to them, and as
healthcare professionals we must respect the patient’s dignities and choices.
End of Life
As MND is terminal, the outcome
of the disease will be death; ergo the care aim of these professionals is
palliative (Maclomness, 2005). At this stage the SLT should regularly review
the communication of the patient (NICE, 2016) to ensure that it is functional.
Functionality of communication is vital at this stage as discussions with
health professionals regarding end of life preparations (advanced care planning
or lasting power of attorney) will likely take place (ibid, 2016). The SLT may be
required to help facilitate the communication of the patient to enable the
messages of the patient to be unambiguous. These should be documented in a
health plan for all professionals to be aware of so that they can act in
accordance with the patient’s requests. If the patient wishes to die in a new
location then accommodations must be made to ensure this is carried out; the
SLT may have to retrain those around the patient in their preferred method of
communication. The interprofessional team may refer the patient to specialist
palliative services, if there are additional concerns regarding family members
surrounding the patient, then referrals may be made to mental health teams and
social services to aid in the affairs of the patient once they have died (NHS
England, 2015; MNDA, 2013). Clearly, at the final stages of the patient’s life,
the ability to communicate with loved ones is of the upmost importance and all
staff involved with the patient must be receptive to the wishes of the
The role of the SLT in MND is
twofold, firstly the assessment and management of the swallow has implications
on the medical health of the patient, the second is to assess and provide a
suitable method of communication in which the patient can express themselves.
This piece aimed to document the unique responsibilities of SLT within this
population and has subsequently highlighted multiple areas in which further
research is needed to improve our practices in terms of both swallowing and the
recognition and facilitation of communication.
Ball, L. J., Beukelman, D. R., Ullman, C.,
Maassen, K., & Pattee, G. L. (2005) ‘Monitoring speaking rate by telephone
for persons with amyotrophic lateral sclerosis’,
Journal of Medical
Ball, L. J., Nordness, A. S., Fager, S. K.,
Kersch, K., Mohr, B., Pattee, G. L., & Beukelman, D. R. (2010) ‘Eye Gaze
Access of AAC Technology for People with Amyotrophic Lateral Sclerosis’,
of medical speech-language pathology
(3), p. 11.
Beukelman, D., Fager, S., & Nordness,
A. (2011) ‘Communication support for people with ALS’,
, pp. 1-6. doi:10.1155/2011/714693.
Blumenstein, I., Shastri, Y. M., &
Stein, J. (2014) ‘Gastroenteric tube feeding: techniques, problems and solutions’,
Chio, A., Logroscino, G., Traynor, B. J.,
Collins, J., Simeone, J. C., Goldstein, L. A., & White, L. A. (2013) ‘Global
epidemiology of amyotrophic lateral sclerosis: a systematic review of the
(2), pp. 118-130.
Dafinca, R., Scaber, J., Ababneh, N.,
Lalic, T., Weir, G., Christian, H., Vowles, J., Douglas, A.G., Fletcher
Jones, A., Browne, C. and Nakanishi, M., (2016) ‘C9orf72
Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum
Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem
Derived Neurons from Patients with Amyotrophic Lateral
Sclerosis and Frontotemporal Dementia’,
Desport, J. C., Preux,
P. M., Truong, T. C., Vallat, J. M., Sautereau, D., & Couratier, P. (1999) ‘
Nutritional status is a prognostic factor
for survival in ALS patients’,
(5), pp. 1059-1059.
Duffy, J. R. (2013)
disorders: Substrates, differential diagnosis, and management
Enderby, P., & Palmer, R. (1983)
Frenchay Dysarthria Assessment.
Hecht, M., Hillemacher, T., Gräsel, E.,
Tigges, S., Winterholler, M., Heuss, D., & Neundörfer, B. (2002) ‘Subjective
experience and coping in ALS’,
Amyotrophic Lateral Sclerosis and Other
Motor Neuron Disorders
Heffernan, C., Jenkinson, C., Holmes, T.,
Feder, G., Kupfer, R., Leigh, P. N. & Sidhu, P. (2004) ‘Nutritional
management in MND/ALS patients: an evidence based review’,
Lateral Sclerosis and Other Motor Neuron Disorders
(2), pp. 72-83.
Hogden, A., Greenfield, D., Nugus, P.,
& Kiernan, M. C. (2012) ‘What influences patient decision-making in
amyotrophic lateral sclerosis multidisciplinary care? A study of patient
Patient Prefer Adherence
, pp. 829-838.
Hoppitt, T., Pall, H., Calvert, M., Gill,
P., Yao, L., Ramsay, J. & Sackley, C. (2011) ‘A systematic review of the
incidence and prevalence of long-term neurological conditions in the UK’,
(1), pp. 19-28.
Körner, S., Hendricks, M., Kollewe, K.,
Zapf, A., Dengler, R., Silani, V., & Petri, S. (2013) ‘Weight loss,
dysphagia and supplement intake in patients with amyotrophic lateral sclerosis
(ALS): impact on quality of life and therapeutic options’,
(1), p. 84.
Langmore, S. E., Terpenning, M. S., Schork,
A., Chen, Y., Murray, J. T., Lopatin, D., & Loesche, W. J. (1998) ‘Predictors
of aspiration pneumonia: how important is dysphagia?’,
(2), pp. 69-81.
Leigh, P. N., Abrahams, S., Al-Chalabi, A.,
Ampong, M. A., Goldstein, L. H., Johnson, J. & Shaw, C. (2003) ‘The management
of motor neurone disease’,
Journal of Neurology, Neurosurgery &
Malcomess, K. (2005) ‘The care aims model’, in
Anderson, C. and Van Der Gagg, A. (eds.)
Speech and language therapy: Issues
in professional practice.
Mitchell, J. D., Callagher, P., Gardham,
J., Mitchell, C., Dixon, M., Addison-Jones, R. & O'Brien, M. R. (2010) ‘Timelines
in the diagnostic evaluation of people with suspected amyotrophic lateral
sclerosis (ALS)/motor neuron disease (MND)–a 20-year review: Can we do
Amyotrophic Lateral Sclerosis
(6), pp. 537-541.
Motor Neuone Disease Association (2013)
. Available at:
4 February 2017).
Motor Neuone Disease Association (2014)
Red Flag tool for MND: Painless, progressive weakness - Could this be
Motor Neuone Disease?
. Available at: www.mndassociation.org/wp-content/uploads/2015/02/red-flags-final-2.pdf
(Accessed on 4 February 2017).
Motor Neuone Disease Association (2016)
Speech and communication support
. Available at: www.mndassociation.org/wp-content/uploads/07c-speech-and-communication-support.pdf
(Accessed: 4 February 2017).
Motor Neuone Disease Association (2017)
. Available at:
(Accessed: 4 February 2017).
Murphy, J. (2004) ‘"I Prefer Contact
This Close": Perceptions of AAC by People with Motor Neurone Disease and
their Communication Partners’,
Augmentative and Alternative Communication
(4), pp. 259-271.
National Institute for Clinical Excellence
Motor Neurone disease: Assessment and Management.
NHS England (2015). The Handbook to the NHS
London: Department of Health
Oishi, A., & Murtagh, F. E. (2014) ‘The
challenges of uncertainty and interprofessional collaboration in palliative
care for non-cancer patients in the community: A systematic review of views
from patients, carers and health-care professionals’,
(9), pp. 1081-1098.
Ramsey, D. J., Smithard, D. G., &
Kalra, L. (2003) ‘Early assessments of dysphagia and aspiration risk in acute
Rowland, L. P., & Shneider, N. A.
(2001) ‘Amyotrophic lateral sclerosis’,
New England Journal of Medicine
(22), pp. 1688-1700.
Saccon, R.A., Bunton-Stasyshyn, R.K.,
Fisher, E.M. and Fratta, P., (2013) ‘Is SOD1 loss of function involved in
amyotrophic lateral sclerosis?’,
, 136(8), pp.
Brignolio, F., Chio, A., Leone, M., & Rosso, M. G. (1987)
‘A study of prognostic factors in motor
neuron disease’ in Cosi et al. (eds.)
Amyotrophic Lateral Sclerosis:
Therapeutic, Psychological, and Research aspects.
New York: Springer,
Scott, A. & McPhee, M. (2014)
'Multidisciplinary care: Speech & Language Therapy' in Oliver, D., Borasio,
G. D., & Johnston, W. (eds.)
Palliative care in amyotrophic lateral
sclerosis: from diagnosis to bereavement
. Oxford: Oxford University Press,
Shaw, P. J., & Wood-Allum, C. (2010) ‘Motor
neurone disease: a practical update on diagnosis and management’,
Steele, C. M., Alsanei, W. A., Ayanikalath,
S., Barbon, C. E., Chen, J., Cichero, J. A., & Hanson, B. (2015) ‘The
influence of food texture and liquid consistency modification on swallowing
physiology and function: a systematic review’,
(1), pp. 2-26.
Tomik, B., & Guiloff, R. J. (2010) ‘Dysarthria
in amyotrophic lateral sclerosis: a review’,
Yorkston, K. M., Strand, E. A., &
Kennedy, M. R. (1996) ‘Comprehensibility of Dysarthric Speech Implications for
Assessment and Treatment Planning’,
American Journal of Speech-Language
Bebbington. This article is licensed under a Creative Commons Attribution 4.0
International Licence (CC BY).
the term given for when food/fluid matter enters the airway. If substantial
enough this can lead to a chest infection (aspiration pneumonia).
of a patient’s swallowing function using an X-ray machine.
used to insert foods and fluids directly into a patient’s stomach through the