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Microsoft word - abn short guidelines, sept 2002
The medical management of motor neurone disease – a UK perspective of current practice
The UK MND Interest group#
Guidance on the management of motor neurone disease (MND), sometimes also known as
amyotrophic lateral sclerosis (ALS), has previously appeared in documents including the Practice
Parameter on the Care of the Patient with Amyotrophic Lateral Sclerosis
 and the Practice Advisory
on the use of Riluzole in the Management of Amyotrophic Lateral Sclerosis
, both developed under
the auspices of the American Academy of Neurology. While these documents give an excellent
overview of their respective topics, this paper is aimed at hospital doctors and general practitioners
looking after MND sufferers, their carers and families, in the UK. It is intended to emphasise the
opportunities for symptomatic treatments which can ease the burden of suffering in this most
This document is evidence-based where possible and, where evidence is not available, it is based on
expert opinion of best practice. It has been developed by the UK MND Interest Group, based on an
earlier document endorsed by Council of the Association of British Neurologists in June 1999
which was widely circulated among neurologists and others but not formally published. This
document has been updated to take account of developments in practice since that time as well as
recent guidance from the National Institute for Clinical Excellence (NICE)  on the use of riluzole
Current management of MND however does not depend solely on medical treatment. The
variability of symptoms and the rapid development of significant neurological disability also
necessitate timely intervention from a wide range of staff in other health care disciplines. The
quality of life of MND sufferers is increased when patients are managed by a multi-disciplinary
team with a specific commitment to MND. Although it is not possible to provide access to such
teams everywhere, it is hoped that this paper will increase awareness of the opportunities for
symptomatic, as well as disease modifying, therapies among clinical colleagues.
The World Federation of Neurology (WFN) Research Group on Neuromuscular Diseases has
[http://www.wfnals.org/Airlie criteria/]. Although widely accepted these criteria were designed as
an aid to clinical trials and research. They might thus be considered more restrictive than the
“burden of proof” used in routine neurological practice. They do however accommodate differing
levels of certainty of diagnosis and their use is encouraged.
• The diagnosis should be communicated by a senior doctor, in privacy, with the opportunity
• Patients should be accompanied by a relative and/or health professional who would be able
• Regular feedback is essential, to ensure patients have understood the information that they
have been given  and they should be encouraged to express their concerns.
• Written information should be available about the disease, the Motor Neurone Disease
Association and other follow-up contacts.
• The diagnosis should be communicated to the General Practitioner (and family if not present
when patient informed of diagnosis) without delay.
A co-ordinated multidisciplinary approach is required to meet the rapidly changing physical and
psychosocial needs of patients and carers throughout the course of the disease. This should be
underpinned by the following principles:
• Care encompassing the whole person and those that matter to them.
• Prompt provision of treatments to secure symptom control and quality of life.
• Planning for the future and timely liaison e.g. with the palliative care team.
The maintenance of optimal functional independence and well-being requires a multi-disciplinary
• Drooling can be alleviated by anti-cholinergic drugs such as hyoscine (sublingual or
transdermal), glycopyrrolate (subcutaneously), atropine (orally), tricyclic antidepressants
(such as amitryptiline) and beta-blockers . Non- pharmacological approaches such as
salivary gland irradiation and duct ligation have been described.
• Dysphagia is best managed in association with speech and language therapists as well as
dieticians and, as it progresses, more invasive approaches such as PEG may need to be
considered in consultation with patient and carer. Expert opinion favours early PEG .
• Speech and language therapists also help in the management of communication difficulties.
When a communication aid is needed it is essential that it is provided promptly .
• Muscle cramps may be treated by quinine, diazepam, phenytoin and naftidrofuryl. Quinine,
diazepam and phenytoin are often usefully given at bedtime. There is considerable
individual variation in levels of response to these drugs. If one does not help, others might.
• Spasticity can be helped by physiotherapy or drug treatment e.g. baclofen, dantrolene,
• The impact of increasing limb weakness may be alleviated by specific interventions ranging
from low-tech equipment such as simple splints and neck support collars designed for MND
sufferers to environmental control systems and computer access technology [9, 10].
• Respiratory symptoms are distressing for patients and carers. Nocturnal hypoventilation may
cause early morning headache and lethargy. There is a strong suggestion that non-invasive
respiratory support improves quality of life  and should be considered at an early stage
with appropriate specialist respiratory management in discussion with patients and carers
. Other symptomatic treatments such as opioids can relieve dyspnoea, coughing and
choking. The *Breathing Space Kit contains medication which can be used by the carer,
nurse or general practitioner for the emergency treatment of the acute episodes of respiratory
distress which may occur in the terminal stages [13, 14]. The use of invasive ventilation is a
complex issue which, if contemplated, requires early and careful discussion with patients
• Depression should be differentiated from the natural sadness of disability. When present it
may be treated with an appropriate antidepressant such as amitryptiline or a selective
serotonin reuptake inhibiting drug (SSRI). These drugs may also relieve emotional lability
and anxiety. Psychiatric advice may also be required.
• Pain is common and may be managed at all stages with anti-spasticity drugs, non-steroidal
agents and analgesics , using the principles of the WHO ladder including opioids such
as oral morphine or fentanyl transdermal patches. Significant benefits can be achieved with
Reviews of some of these therapies are currently registered with the Cochrane Collaboration
[http://www.cochrane.co.uk]. It is hoped that coverage of this area will become more
comprehensive in the future. These comments are based on clinical effectiveness only and do not
Riluzole is the only drug currently licensed in the UK for the treatment of MND. Riluzole is a
disease-modifying therapy. It does not cause symptomatic improvement and does not prevent death.
Patients and carers need to be informed of the implications of the trial data before starting
treatment. Evidence for its efficacy comes from two randomised placebo-controlled trials (RCT's)
[17,18] see footnote+. The NICE (National Institute for Clinical Excellence) guidance  states that
“1.1 Riluzole is recommended for the treatment of individuals with the amyotrophic lateral
sclerosis (ALS) form of motor neurone disease (MND)”
and “1.2 Riluzole therapy should be
initiated by a neurological specialist with expertise in the management of MND
supervision of therapy should be managed by locally agreed shared care protocols undertaken by
general practitioners”. The Cochrane Systematic Review  concluded that “Riluzole 100mg per
day is reasonably safe and probably prolongs survival by about two months in patients with ALS.
More studies are needed, especially to clarify its effect in older patients (over 75 years) and those
with more advanced disease.”
Riluzole treatment is appropriately discussed with MND patients as
soon as a working diagnosis has been made, although RCT evidence only exists currently for
patients with definite or probable MND within the El Escorial Criteria . Patient choice or adverse
effects such as fatigue and nausea, may lead to cessation of therapy. Efficacy evidence from
placebo-controlled randomised clinical trials only extends to 18 months of therapy. Nonetheless it is
reasonable to continue therapy for longer than 18 months when considered appropriate by the
clinician, patient and carers. However, it is not appropriate to start patients with advanced disease
on riluzole or to prolong treatment into the terminal phase. .
• Although there is published evidence from one placebo-controlled RCT  that insulin-
like nerve growth factor (rhIGF-I) slows disease progression, this was not confirmed in a
second RCT . This drug is not licensed.
• Many other putative disease-modifying therapies for MND have been investigated but have
Consultant Neurologist, Exeter Neurosciences, Royal Devon and Exeter Hospital
and Peninsular Medical School, Barrack Road, EXETER, EX2 5DW: D Jefferson;
Neurologist, Queen’s Medical Centre, Nottingham, NG7 2UH: PN Leigh;
Professor of Neurology,
Institute of Psychiatry, de Crespigny Park, London, SE5 8AF: JD Mitchell;
Consultant Neurologist &
Honorary Professor of Clinical Neurology, Royal Preston Hospital, Sharoe Green Lane, Fulwood,
PRESTON, PR2 9HT (Chairman): KE Morrison;
Professor of Neurology, Medical School,
University of Birmingham, Edgbaston, BIRMINGHAM, B15 2TT: DJ Oliver;
Palliative Medicine, Wisdom Hospice, St William Way, Rochester, Kent, ME1 2NU; Honorary Senior
Lecturer in Palliative Care, Kent Institute of Medicine and Health Science, University of Kent at
Canterbury: RW Orrell;
Senior Lecturer and Consultant Neurologist, Royal Free & University
College Medical School, University College, London, NW3 2PF: H Pall;
Senior Lecturer in
Neurology, University of Birmingham, B15 2TH: PJ Shaw;
Professor of Neurology, E Floor, Medical
School, University of Sheffield, Beech Hill Road, SHEFFIELD, S10 2RX: M Swash;
Neurologist, The Royal London Hospital, London, E1 1BB: RJ Swingler;
Ninewells Hospital, DUNDEE, DD9 1SY: E Williams;
Consultant in Rehabilitation Medicine,
Walton Centre for Neurology and Neurosurgery, LIVERPOOL, L9 7LJ: CA Young;
Neurologist, Walton Centre for Neurology and Neurosurgery, LIVERPOOL, L9 7LJ.
The UK MND Interest Group is funded by unconditional educational grants from Aventis, the
manufacturers of riluzole, and Amgen. Members of the group have accepted speakers honoraria
from several pharmaceutical firms, including Aventis, the manufacturer of riluzole. Some members
of the group were also investigators in the second large trial of riluzole in MND, but only PNL
participated in the data analysis and manuscript preparation. The Association of British
Neurologists has received financial support from the pharmaceutical industry including the
manufacturers of medications for motor neurone disease.
*Obtainable on medical request through the Motor Neurone Disease Association, David Niven
House, PO Box 246, NORTHAMPTON, NN I 2PR. (Telephone 01604-250505, fax 01604-24726).
Kit includes suggestions for the management of acute respiratory symptoms. It is then stocked with
drugs through prescription by the GP. The following contents are suggested:
enema (Stesolid) 10mg x 3 (for use by carers)
Chlorpromazine 25mg x 3 for injection is suggested as a possible alternative to Midazolam.
Glycopyrronium bromide 200µg x 3 is suggested as a possible alternative to Hyoscine
+These RCT's report a modest decrease in risk of tracheostomy or death with riluzole (relative risk
riluzole 100mg/day= 0.66 (95% confidence interval 0.42-1.02), p = 0.05 over 12 months treatment,
; relative risk, riluzole 100mg/day (Cox model) = 0.65 (95% confidence interval 0.50-0.85), p =
0.002 over 18 months treatment, . It has been calculated  that the number of patients needed
to treat with riluzole 100mg/day to prevent one death or tracheostomy after one year (based on )
is 9.2 (95% confidence interval 5.2-38). A post-hoc analysis of the data from the dose-ranging trial
 has suggested that riluzole delays the progression of the disease to severe disability. The
findings of this preliminary study require confirmation.
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amyotrophic lateral sclerosis (an evidence based review). Neurology 1999; 52: 1311-23
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treatment of amyotrophic lateral sclerosis with riluzole. Neurology 1997; 49: 657-9
3. National Institute for Clinical Excellence. Technology Appraisal Guidance No 20 – Guidance on
the use of riluzole (Rilutek) for the treatment of motor neurone disease. 2001
4. World Federation of Neurology Research Group on Neuromuscular Diseases. El Escorial World
Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci
5. Johnston M, Earll L, Mitchell E, Morrison I; Wright S. Communicating the diagnosis of motor
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