Nursing Management for Motor Neurone Disease: A Detailed Analysis

This report provides a comprehensive overview of nursing management for Motor Neurone Disease (MND), also known as amyotrophic lateral sclerosis (ALS). It begins with an introduction to MND, detailing its neurodegenerative nature, progressive characteristics, and the associated motor and bulbar dysfunction, often leading to premature death. The report delves into the pathogenesis of the disease, exploring genetic factors, oxidative stress, excitotoxicity, and the neurodegeneration of motor neurons. It discusses genetic mutations, particularly those involving SOD1, and their impact on cellular function. The report also examines the role of oxidative stress and excitotoxicity in neuronal damage. Furthermore, it details nursing care strategies, including the importance of multidisciplinary teams, the nurse's role in coordinating care, providing patient and carer education, and addressing specific complications such as aspiration and emotional demands. The report emphasizes the importance of person-centered nursing and palliative care, focusing on symptom control and enhancing the quality of life for patients with MND.

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Nursing Management for Motor Neurone Disease
Student’s Name
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Nursing Management for Motor Neurone Disease
1.0 Introduction:
Motor neuron disorder (MND) is characteristically a neurodegenerative disorder and is
alternatively referred to as amyotrophic lateral sclerosis (ALS) [1, 2, 3]. It is a degenerative
disorder and typically has undefined and unknown aetiological characteristics [1, 2, 3]. It is a
progressive disorder and is typically associated a weakness of the motor functionality and is
additionally associated with a dysfunction of the bulbar tissue [1, 2, 3]. It is likely to result in
premature death as well [1, 2, 3]. Researchers and physicians have forever and especially in the
19th century have associated the weakness of the muscles and nervous tissue dysfunction with
primary disorders of the muscle [1, 2, 3]. Additionally, it may be associated with secondary loss
of the integrity of neuromuscular tissues and cells [1, 2, 3]. Such a dysfunction is similar to the
dysfunction that results from a cut in the peripheral nerves [1, 2, 17]. This is even likely in the
cases of degeneration of motor neurones [1, 2, 3]. Researchers have additionally observed that
the motor neuron degeneration may be of different categories or types [1, 2, 15]. They may
selectively affect either the upper or the lower motor neurones causing selective dysfunction in
either of these neuronal junctions [1, 2, 3]. A condition in which there is a combination of the
upper and lower neurone systems is known as amyotrophic lateral sclerosis or ALS [1, 2, 15].
The terminology of ALS is more commonly used in the USA for diseases of the motor neurone
dysfunction [1, 2, 3]. The term ALS is used for most motor neurone dysfunction diseases
irrespective of whether the upper or the lower motor neurones are involved [1, 2, 14]. The term
MND is used more commonly in the UK [1, 2, 3]. MND occurs commonly in the middle or late
life period [1, 2, 17]. It is one of the most common diseases which onsets in the later adult life [3,
4]. There is no specific treatment protocol for the management of MND in adults [3, 4]. The

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substantial progression of the disease onset is not currently possible by any medication or known
treatment procedure [3, 4]. The incidence rates of the disease is considerably high as it affects at
least one or two individuals per 1, 00,000 individuals [3, 4, 18]. The disease is predominantly
prevalent in individuals who are middle aged or old [3, 4, 18]. The mean age of disease onset is
an average of 55 years old [3, 4]. However, occasional occurrences in younger individuals are
possible [3, 4].
The disease is typically associated with progressive injury to the motor neurones [3, 4, 12].
Additionally, the death of cells and groups of cells of the lower motor neurone are also
associated with MND [3, 4, 13]. The groups of cells of the lower motor neurone are present in
the spinal cord and the brain stem regions in the individuals [3, 4, 13]. The upper motor neurones
of the motor cortex are mostly affected in the condition of MND [3, 4, 20]. The affected
individuals typically present with a combination of upper and lower signs of the motor neurone
[3, 4, 21]. They also present with muscle weakness of a progressive nature [3, 4]. There is an
occurrence of muscle wasting that primarily occurs due to the presence of brisk reflexes in a
pathological manner [3, 4, 19, 21]. There is an eventual involvement of the muscles of the limb
and the bulbar region [3, 4, 20, 22]. There may be occasional occurrences of several clinical
variants of the disease which particularly affect the lower motor neurones predominantly [3, 4].
Progressive muscular atrophy and primary lateral sclerosis is predominantly associated with the
upper and lower motor neurones [3, 4, 23]. The occurrence of death is most often resulted from
the failure of the respiratory organs which may be due to the presence of weakness in the
ventilator muscles [3, 4, 24, 25]. The causes of the neurodegenerative process are not precisely
predicted and they are unknown to research [3, 4]. The disease process particularly includes
certain selectivity for the system of motor functionality in the human body [3, 4]. This type of

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sensitivity is particularly of a relative nature and is not observably absolute [3, 4]. Research and
extensive analysis of clinical and pathological data have unravelled that the extra-motor sections
of the central nervous system are additionally affected in these individuals [3, 4]. Therefore,
research considers the condition as that involving multiple systems and the failure of motor
neurones essentially occurs at the earliest stage [3, 4]. The motor neurones are affected severely
in the early onset stages of the disease [3, 4].
1.1. Pathogenesis:
1.1.1. Genetics: The degeneration of motor neurones is resulted from the presence of multiple
gene products of different classes and of abnormal classifications [3, 4]. In most familial
incidences of motor neurone disease, several gene mutations, particularly point mutations have
been found to exist in most of these cases [3, 4, 26]. The most common observation has been the
mutation in the chromosome 21 that encodes copper and zinc superoxide dismutase (SOD1)
(Cu/Zn) in the familial motor neurone disease [3, 4, 24]. The primary function of SOD1 is the
catalysis of conversion of radicals of the intracellular superoxide which are produced during the
event of metabolism of normal cellular hydrogen peroxide [3, 4]. These hydrogen peroxide
radicals are typically eliminated by scavenging enzymes that characteristically act on free
radicals [3, 4]. SOD1 is a ubiquitous enzyme and undergoes expression in cells present
throughout the body [3, 4, 27]. However, the reason for the specific vulnerability of motor
neurones to injury in the presence of several mutations lacks complete clarity [3, 4]. Motor
neurones display a great degree of expression of the Cu/Zn superoxide dismutase enzyme in the
compartments of the cells and the axons relative to the other cells of the nervous system [3, 4].
The mutant enzyme is able to generate a gain of function rather than a loss which results in a
toxic event [3, 4]. One of the primary hypotheses for this gain of function is the likely abnormal

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handling of biochemical compounds such as hydrogen peroxide resulting in elevated formations
of damaging hydroxyl radicals which are excessively damaging to the system [3, 4].
Additionally, the formation of nitrotyrosine residues on the intracellular proteins is indicated [3,
4]. Neurotoxicity is a common occurrence resulting due to the formation of intracellular
aggregates of Cu/Zn superoxide dismutase which are abnormal and lead to adverse cellular
biochemical effects along with a probable abnormal release of copper or zinc [3, 4].
1.1.2. Oxidative stress: Oxidative stress is considered one of the most important causes of
deterioration of neuronal function and age-related neurodegenerative disorders [3, 4]. The
mutations of the Cu/Zn superoxide dismutase are known to cause damage to free radicals and
several damaging biochemical changes leading to oxidative stress [3, 4]. An altered expression
of the individual components of the defence systems of the intracellular antioxidants is a major
reason for the presence of oxidative stress as indicated by post-mortem studies of the patients
with as an attempt of the system to compensate for oxidative stress [3, 4]. The fibroblasts present
in the fibroblast cells of the specimens of skin tissues of patients of familial and sporadic MND
indicate an increased sensitivity to oxidative insults [3, 4].
1.1.3. Excitotoxicity: The primary excitatory transmitter of the human nervous system,
glutamate, plays a vital role in the transmission of excitatory signals [3, 4]. The termination of
the excitatory signal occurs when glutamate is taken out of the cleft of the synapsis by the means
of several transporter proteins [3, 4]. They are primarily located in the perisynaptic glial cells [3,
4]. The stimulation of the receptors of the neuronal glutamate, if carried out in an excessive and
abnormal manner typically results in the damage and the death of these cells [3, 4]. The
mechanisms of the primary derangement of the homeostasis of intracellular moieties of calcium
along with the excessive production of free radicals are also some of the effects of the

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derangement [3, 4]. Glutamate mediated toxicity is one of the primary factors that contribute to
the injury of the cells in the condition of MND [3, 4]. One of the important findings of research
indicates that one of the primary reuptake transporter proteins of glutamate is typically impaired
in MND [3, 4]. The concentrations of glutamate are found to be abnormal in the extracellular
fluid and the cerebrospinal fluid in a few cases of MND [3, 4]. Research has additionally found
the presence of various RNA segments with abnormal splicing sequences [3, 4]. These encode
the excitatory molecule of amino acid transporter 2 [3, 4]. These have been found in the motor
system in most cases of MND [3, 4]. Research has suggested that one of the major aetiological
factors for the disease progression is the presence of a specific defect in the processing
mechanism of the RNA molecule in the disease pathology [3, 4]. However, certain theories of
disease progression suggest that the RNA segments which are alternatively spliced are typically
found within the central nervous system [3, 4].
1.1.4. Neurodegeneration of motor neurones: The neurons of the motor system are
characteristically cells of a post-mitotic and fixed type [3, 4]. They do not have the retained
capacity of replication [3, 4]. These cells tend to gradually reduce in number along with the
process of aging [3, 4]. One of the important features of the MND condition is that only certain
specific populations of the neurons of the motor system have additional vulnerability for
degeneration [3, 4]. These cells are susceptible to acquire the disease process [3, 4]. Certain cell
populations including the cell groups that are responsible for controlling the muscles of the
extraocular region and the floor of the pelvic region are typically immune to the disease
progression [3, 4]. Research has attempted to understand the particular features of the motor
neuron that render it either resistant to disease development or vulnerable to acquiring the motor
neuron disease condition [3, 4]. The features that mostly render the cells of the neuron

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susceptible to the disease include the following: The largest cells of the human system are those
of the nervous system and thus the motor neurons that supply nerves to the distal lower limbs
need to support the axonal processes that are up to one meter in length and thus the burden on
these neurons is considerably high [3, 4]. This leads to an excessively high demand of energy in
these cells [3, 4]. This requires a high level of mitochondrial activity along with the requirement
for a robust and energetic system containing the cytoskeleton [3, 4]. This in turn poses the
demand for a high content of intracellular material in the proteins of the neurofilament [3, 4].
Research has found evidence from human sources of tissue and several experimental animal
models that the proteins of the mitochondria and the neurofilament are specific target sites for
injury resulting in within the motor neurons [3, 4]. Another important feature is that most
neurons of the motor system have specific features of the cellular composition that renders them
susceptible to processes of a toxic nature that are mostly mediated by the calcium moiety [3, 4].
These processes are seen to result following the activation of the surface of their cells and the
receptors present on the cell surface [3, 4]. Most of the neurones of the motor system are
suggested to possess a particular profile for the receptors of d=glutamate which are permeable to
calcium molecules [3, 4]. These are in fact different from the receptors of several groups present
in other cell groups of the nervous system [3, 4]. Additionally, most motor neurons lack proteins
that are responsible for intracellular buffering of calcium [3, 4]. These are responsible for
protecting the various neurones from excessive increases in the calcium present in the cytosolic
region [3, 4]. Motor neurones specifically contain a high extent of expression of perisomatic
molecules of the excitatory amino acid transporter 2 moiety along with having a high expression
rate of Cu/Zn superoxide dismutase enzyme [3, 4].
Nursing care for patient with MND:

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The patients of MND have special needs and medical emergencies [5, 6, 7]. The carers of these
patients additionally have complex requirements in the caring of these patients [5, 6, 7]. The
common presentations include compromised respiratory mechanism and other emergency
presentation [5, 6, 7]. The needs of the patients are primarily addressed by the nurses [5, 6, 7].
However, most often, their needs can be addressed adequately by the involvement of several
teams and multidisciplinary units [5, 6, 7]. The management of progressive neurological
disorders is carried out by these multidisciplinary teams and their experience and skill set is
crucial for the effective management of the challenges in the treatment procedures [5, 6, 7]. The
multidisciplinary team may involve a wide range of professionals including a physician,
physiotherapist, counsellor or psychologist, dietician, community nurse, primary care general
practitioner team, neurologist, gastroenterologist, occupational therapist, palliative care team,
respiratory specialists, social service, speech and language therapist, and sociologist [28, 30].
The care protocol is carried out for 24 hours every day at the multidisciplinary centre or on an
outpatient basis as the care provided needs to incessant [28, 29, 30]. In the case of Mr. Chiswick,
the progression of MND is at an advanced stage. Due to this, the care provided is mostly
palliative care [28, 29, 30]. The palliative care setup is also multidisciplinary in nature and the
setup includes the afore-mentioned patients [28, 29, 30].
The role of the nurse is of specific importance in the multidisciplinary team [28, 29, 30]. The
nurse essentially needs to coordinate with the paramedics, physicians, and other related
multidisciplinary teams to provide complete care to the patients [28, 29, 30]. The role of the
nurse is increasingly critical in the subsequent stages of the progression of MND and the
progression of the disease process [28, 29, 30]. The requirement of the support for the patient
increases with time [28, 29, 30]. The patients require more attention and time during the

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progression of the disease process [28, 29, 30]. The nurse’s role extrapolates to the provision of
support and assistance for the performance of many activities of daily life [28, 29, 30]. Mr.
Chiswick complains of inability to speak effectively or speech deficit [28, 29, 30]. The nurse
would then be required to coordinate with the speech and language therapists to provide the
necessary care [28, 29, 30]. Additionally, Mr. Chiswick experiences inability to walk and
weakness due to which he has become wheelchair bound. The multidisciplinary team essentially
includes wheelchair services as well and thus the nurse needs to assist in the provision of the
physiotherapy and wheelchair services [28, 29, 30]. Mr. Chiswick has been advised to undergo
palliative care [28, 29, 30]. The anxiety in the patient is a essential challenge to the care provided
to him [28, 29, 30]. Thus, the role of the psychologist is increasingly important [28, 29, 30]. Mr,
Chiswick experiences anxiety with a belief of being ‘given up on’ and believes that he is being
driven to the process of death by lack of attempt of curative treatment [28, 29, 30]. Thus, the
appropriate counselling for the patient needs to be provided by the psychologist or the counsellor
[28, 29, 30].
The nurse’s role is critical for educating the patient and his carer or the consequences of MND
[28, 29, 30]. The awareness of the disease progression and pathology of the disease is essential in
the carer of the patient in order to appreciate the ceasing of curative medication [28, 29, 30]. The
role of the nurse extends from educating the patient and the carer to the coordinated provision of
the multidisciplinary care options [28, 29, 30]. The various multidisciplinary care services have
to be provided with much coordination by the involvement of the nurse in the palliation [28, 29,
30]. Palliative care mostly focuses on relief from pain and the requirement of the care protocol is
to provide the essential aides for activities of daily life [28, 29, 30]. Providing support to the
patient for the performance of regular activities is an essential aspect of palliative care [28, 29,

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30]. The nurse is involved in both community caring as well as provision of care to hospitalised
patients [28, 29, 30].
The most important complications during the palliative care for a patient with MND include
aspiration which can be potentially avoided by urging the patient to sit upright whilst consuming
food [28, 29, 30]. The carers of the patients also experience several emotional and physical
demands during this stage; therefore, the nurse is expected to deal with the situation with
empathy and provide adequate support to the carers [28, 29, 30]. The up keeping of records of
daily consumption of food and drinks, health and the moods of the patient, the pressure areas on
the skin and treatment provided, if any, care at night, bathing, washing up, getting dressed and
the aid provided, bowel habits of the patient and any reported constipation occurrences, and other
recordable activities [28, 29, 30]. Since Mr. Chiswick experiences difficulty in swallowing, the
speech therapist needs to provide support in the swallowing activity [28, 29, 30]. The dietician
may additionally suggest any changes or dietary supplements with better ease of swallowing, and
a diet to suit the patient can be prepared [28, 29, 30]. In extreme cases, tube feeding can be
undertaken [28, 29, 30]. The possibility of choking has to be avoided by managing the position at
the time of ingestion [28, 29, 30].
Conclusion: Person-centred nursing and multidisciplinary team care may be the best practice for
patients with MND. Non-invasive respiratory assistance and reduction of pain at all stages of
nursing and palliation are the most crucial for the improvement of the quality of life. The
palliative care protocol includes the entire course of the disease development without involving
only the final stage. Symptom control is crucial during the entire disease process. MND is
associated with the damage of motor neurones and thus injury or trauma to the nerve has to be

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avoided. Since MND is a crippling and devastating condition, the role of nursing and care
remains to enhance the independence and quality of life of the patients.
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