APPENDIX 5: MOTOR NEURONE DISEASESYMPTOMS
AND TREATMENT NOTE BY DR RICHARD HILLIER |
Motor Neurone Disease (MND) is a rare, incurable
and invariably fatal condition. Many would consider it worse than
cancer. Because of its slow, relentless course, it has attracted
considerable attention within the assisted dying debate.
It is characterised by gradually increasing weakness,
initially of the limbs, and culminates in complete paralysis so
that the patient depends on others for every activity of daily
living. Ultimately, only the eye muscles may be spared, although
85% of patients normally retain control of bowel and bladder.
Sensation remains normal: pain is only a later consequence. Most
patients retain complete intellectual function although depression
occurs in 7% to 50% of them, dementia in 10% and significant cognitive
impairment in some.
Incidence and Prevalence
Across the United Kingdom, there are between 1.5
and 2 new patients diagnosed with MND per 100,000 population per
year. There are approximately 1000 deaths from the condition per
year. In some areas of the United Kingdom, e.g. West Sussex, there
is an unexplained prevalence. A general practitioner with a list
of 2000 patients might expect to have 1 or 2 patients in his/her
own professional lifetime, but a group practice of, say, 5 doctors
may obviously see more. Because of the rarity of MND, there are
few neurologists who specialise in its management. The same is
true of palliative medicine physicians.
The literature: strength of evidence
Most of the sparse literature on the symptom control,
management and patients' /carers' views about the disease and
assisted suicide consists of descriptive surveys or editorials.
There is very little data evaluating the effects of symptom control
on patients' quality of life in MND. There are two original articles
on assisted dying from Oregon  and The Netherlands .
How does MND affect patients?
In addition to increasing weakness and associated
muscle cramps, symptoms reported by carers towards the end of
life include gradual loss of speech (62%), choking episodes (52%),
breathlessness (50%), insomnia (42%), discomfort other than pain
(48%), depression (40%) and anxiety (30%) . Others mention
drooling, cough and fear. In Oregon, 30% of patients are supported
by a hospice.
In the United Kingdom, of 170 hospices surveyed,
all provided in-patient care, 60% provided care at home, 76% provided
hospice day-care and support for 28% in hospital. Of these hospices,
17% became involved at the time of diagnosis, rising to 48% in
the terminal stages .
Symptoms that are particularly difficult
Surprisingly, most of the symptoms mentioned above
can be relieved or reduced to some extent but the effect of treatment
overall has not been studied in randomised controlled trials.
Some services in the United Kingdom and Germany have produced
clear guidelines for management. The Association of British Neurologists
has identified standards of care for MND.
Publicity and media stories about MND, coupled with
the graphic horror of a relentlessly paralysing disease which
ends in death, ensures that at diagnosis most patients are frightened.
The fear is always real and often leads to anxiety and depression.
It is incumbent on the physician to provide explanations in order
to diminish this fear. For example, there is no pain at this stage,
discomfort can be relieved by good physiotherapy and appropriate
drugs, intellectual capacity remains unimpaired, and the feared
incontinence is rarely a problem. Even at this stage, involvement
of a full multi-professional expert team should be instigated
as the burden of this condition on both patients and families
will be considerable.
Patients will have read that this happens and will
have heard it reported in the media; and the Committee has heard
the evidence of Dr Taylor who said, "I shall die of pneumonia
caused by the inhalation of saliva". With this in the public
perception, the first coughing fit or sensation of choking often
causes inordinate terror and requires extremely careful management
and reassurance, particularly about the end stage of life.
What are the facts? Of 124 patients with MND cared
for in a London hospice, only one appeared to choke to death.
Autopsy revealed however that that patient had died, not of MND,
but of myocardial infarction . In this patient and 19 others
who had autopsies, none had any foreign matter in the mouth, throat
or breathing tubes. In another study by the caregivers of 121
patients in Germany, no patient choked to death . Ganzini's
study of the views of caregivers was significantly different .
How then do they die?
The majority of MND patients die of respiratory failure.
What occurs is that the muscles which affect breathing gradually
weaken. Over time this creates a build-up of carbon dioxide in
the blood which, instead of stimulating breathing, actually depresses
it with the result that the patient, in effect, dies in his/her
sleep. This often occurs in previously stable patients and can
be quite sudden. Of 113 patients dying in a London hospice, 48%
deteriorated suddenly and died within 24 hours. Only 2% survived
longer than one week .
Although it is possible to put patients on a ventilator,
this option is rarely discussed because the necessary resources
are often not available. Less than 2% of patients in the United
Kingdom accept ventilation: only 2.9% of patients in New York
Diagnosis and Management
It is crucial that an accurate diagnosis of MND is
made at the outset because there are a number of treatable neurological
conditions which can mimic it, e.g. myasthenia gravis. The diagnosis
must be made by a neurologist and, even then, a second opinion
may be required.
Management consists of three aspects:
- Relief of pain and symptoms
- Management of the physical effects of the disease,
- Psychological and emotional support of patients
There are a few drugs under trial to slow progression
of the disease, but so far these have not been remarkable and
their effects have not been evident to either the patient or his/her
family as the disease progresses.
Food and fluids
In the late stages of the disease, swallowing may
become difficult to some extent, although this is not invariable.
Expert speech and language therapists can successfully teach patients
new techniques to deal with this. Because these therapists are
not commonly involved, more patients than necessary have a feeding
tube inserted through the stomach walla painless and effective
Most of the symptoms can be helped or relieved but
there are no randomised controlled trials to measure this. Nevertheless
considerable interest and progress has been made in the general
management of MND over the past ten years. Because general practitioners
see patients with this condition only rarely, MND should be managed
in a centre with a specialist MND team consisting of a physician,
nurses, occupational therapist, physiotherapist, social worker,
speech and language therapist and counsellors, who can work with
the primary health care team in offering patient and family care
and support. Palliative care teams are increasingly involved and
combined clinics with neurologists and palliative care specialists
are becoming more available. Involvement with the MND Association,
which can provide invaluable advice and respite, is essential.
When the above exists and is coordinated well, the
care and support for patients and families can be superb. Unfortunately
such centres are rare (though increasing) and patients who are
not involved can suffer considerably.
Some questions concerning MND
How effective is palliative care?
In a properly constituted team, it can be good. Anecdotal
evidence suggests that many symptoms can be relieved, fear of
dependency and choking can be diminished, and pro-active management
can prevent the occurrence of many problems. Obviously, not all
suffering can be relieved.
Can palliative care relieve depression?
Yes, provided it is diagnosed and that treatment
(drug and non-drug) is given.
Can palliative care relieve "loss of self-worth"?
Of 56 patients, 84% of those who did not want PAS,
and 52% of those who would, looked forward to the future with
"hope and enthusiasm" .
Does morphine in these patients shorten life?
In cancer patients, the starting dose of morphine
for pain control is 30-60 mg per day and may rise up to, and even
beyond, 1000 mg per day with the patient remaining conscious,
alert and active. In one study of patients with MND, symptoms
were controlled with a morphine mean dose of 60 mg per day ;
in another study, the mean dose was 30 mg per day in MND patients
. There is no evidence that morphine shortens life when used
appropriately in these patients, nor that excessively high doses
are needed to control their pain.
Is the issue of PAS for MND and other non-cancer
conditions different from the issues concerning patients with
To some extent. Prognosis is often longer for MND,
and can be longer if artificial ventilation is used (up to 17
years) . Assessment of depression/competence may be more difficult
in a patient if left too late. As one patient said "don't
let go too soon but don't hang on too long". Being a rare
disease, the burden on the attending or consulting physician may
fall on very few specialists and distort clinical practice.
In countries where it is legal, what proportion
of patients seek or accept assisted suicide?
In the Netherlands, 20% of 203 patients with MND
received euthanasia or PAS . In a study in Oregon, 56% of 100
patients said they would consider assisted suicide. In 73% of
these, caregivers and patients agreed. Many would request a prescription
well before they would use it .
Is prognosis a problem?
In MND, deterioration is often rapid. As mentioned
previously, 50% may deteriorate suddenly and die in 24 hours 
and it is hard to anticipate this. There is no objective measure
and the assessment of prognosis is a combination of experience
and professional judgment in consultation with others who know
the patient well.
1. Ganzini et al., 2002, Neurology, Vol. 59, p.428-431.
2. Oliver et al, 2000, Palliative Medicine, Vol.
14, p. 427-8.
3. O'Brien, 1992, British Medical Journal, Vol. 304,
4. Neudert et al, 2001, Journal of Neurology, Vol.
248, p. 612-6.
6. Ganzini et al, 1998, New England Journal of Medicine,
Vol. 339, p. 967-73.
7. Oliver, 1998, Palliative Medicine, Vol. 12, p.
8. Veldink, 2002, New England Journal of Medicine,
Vol. 346, p. 1638-44.
Some relevant papers and websites consulted:
Borasio et al, 1997, Journal of Neurology, Vol. 244,
p. SII-SI7. Miller et al, 1999, Neurology, Vol. 52, p.1311-23.
Howard et al, 2002, Postgraduate Medical Journal,
Vol. 78, p. 736-41.
Association of British Neurologists: firstname.lastname@example.org