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THE MANAGEMENT OF MND
The role of the GP. It used to be stated that a GP might expect to see a
maximum of one or two patients with MND during their professional lifetime. Now with large shared lists, exposure to and experience of this condition in primary care is probably more frequent, although it remains very uncommon. As such, it is entirely understandable that the index of suspicion is low when a patient presents with what appear to be non-specific symptoms. If there are lengthy waits for neurology appointments and subsequent tests, this can lead to anxiety, dissatisfaction and may delay symptomatic intervention. However, most neurology departments in England and Wales work to a maximum 11 week outpatient wait and some even less. Urgent referral to a neurologist is helpful where the following features of MND are present: • Patients >50 with progressive painless weakness • Wasted muscles with retained reflexes • Lack of prominent sensory symptoms (tingling as well as loss)
How does ALS/MND present? Presentation is commonly in 2 patterns: either
‘spinal’ (75%) with weakness in one or more limbs, or ‘bulbar’ with speech
and swallowing problems (25%). The earliest symptoms may be non-specific
such as muscle cramps or persistent muscle twitching (fasciculation).
However, both symptoms are probably more frequently a normal
phenomenon in healthy people. There may be insidious 'clumsiness' and/or
falls, which go unreported. The recognition of progressive weakness without
sensory involvement is the key to prompting a consideration of MND as a
diagnosis.
The diagnosis of ALS/MND is clinical and depends on the finding of 'lower
motor neurone' signs (fasciculation, wasting, weakness, flat reflexes), in combination with 'upper motor neurone' signs of varying degree (increased tone, weakness and brisk reflexes). The finding of these so-called ‘mixed signs’ is typical of the ‘classical ALS (amytrophic lateral sclerosis) form of MND’, which comprises 75% of all cases, (but the terms ALS and MND are often used interchangeably, particularly in the US and may parts of Western Europe). Routine blood tests are typically entirely normal, although the creatinine kinase (CK) level may be mildly elevated (300-1000iu/L), reflecting muscle breakdown secondary to denervation. However this test is neither sensitive nor specific. The implications of making a diagnosis of MND are profound, hence the need to exclude all other potentially treatable causes for the patient’s symptoms. Whilst true ‘mimic’ conditions are very unusual in reality, in most situations patients benefit from prompt referral to a neurologist, although not necessarily detailed investigation. Unexplained sub-acute painless progressive weakness should be regarded as an indication for urgent referral to minimise diagnostic delay and anxiety and to implement appropriate support. Most patients will undergo detailed neurophysiological examination (EMG/nerve conduction studies, (EMG/NCS)), but this is not always necessary if the history and examination findings are clear. Differential diagnoses. Other diseases which lead to muscular wasting,
especially of the upper limbs, must be considered. They are (in
approximate order of frequency):
1. Sensory loss is usually present but upper limb weakness and lower limb spasticity may be remarkably similar to that seen in MND. 2. MND has a more rapid course and cervical disc protrusion may 3. Not infrequently, MND will co-exist with cervical spondylosis 1. Forearm and finger flexors or quadriceps weakness 2. Can produce bulbar weakness 3. Differentiated by electromyography and muscle biopsy 4. CK modestly elevated 1. Bulbar signs but rarely muscular wasting 2. Responds rapidly to cholinesterase inhibitors • Multi-focal Motor Neuropathy with Conduction block (MMN/CB) 1. Pure lower motor neurone syndrome 2. May be associated with anti-GM1 auto antibodies 3. Conduction block demonstrated on EMG/NCS 1. Fasciculation absent 2. Pain prominent 3. Sensory loss usually present 4. Characteristic radiology and focal EMG/NCS findings 2. Usually accompanied by sensory loss. 3. Distribution clearly limited to distribution of a single (or 1. A rare condition with prominent sensory involvement 2. Fasciculations are rare 3. Signs can be localised to focal lesion (usually cervical spinal 1. Sensory loss usually prominent 2. Signs can be localised to focal spinal lesion 1. Differentiate by electromyography and muscle biopsy 2. Raised CK 3. Proximal weakness and myalgia prominent
Telling the diagnosis. Many neurologists regard giving a diagnosis of
ALS/MND to a patient as one of their hardest tasks, because of the grave
prognosis typically associated with this condition. The parallels with
oncology are very close (although most cancer patients will be offered some
form of disease modifying treatment). There is an inevitable balance to be
struck between avoiding delay and waiting until the diagnosis is certain. Occasionally time is the greatest and only diagnostic aid. Once the term MND is mentioned as a possibility it is very hard to take back. Patients need to be informed as soon as possible so that they can make arrangements and maximise their remaining good quality of life before significant progression of their symptoms and disability takes place. Commonsense rules apply, such as having a private, interruption-free area to give the diagnosis, and suggesting that a relative and a senior nurse are present. Generally one should try to give honest answers to questions about prognosis, recognising that people differ in the amount of information they wish to receive, and bearing in mind the enormous and largely unpredictable variation in progression and prognosis from patient to patient. Familial MND (FALS) accounts for only 5-10% of the total MND caseload. It is estimated that there are approximately 200-250 cases of familial MND in the UK. When someone is newly diagnosed with familial MND it is possible that the whole extended family will need support. In these circumstances, the family has often seen MND at close hand in another family member or members and frequently recognise the symptoms before a confirmed diagnosis has been given. FALS is typically an autosomal dominant condition. Offspring have a 1 in 2 chance of inheriting the disease from a parent carrying the faulty gene. Viewed in isolation familial cases are indistinguishable from more common sporadic disease. It is therefore important to ask about a family history. There is also ‘incomplete penetrance’, (80% by 85 years of age), meaning that up to twenty percent of people carrying the faulty gene may reach the age of 85 without developing the disease. This gives rise to the appearance of ‘obligate carriers’ in the family tree (family members who have passed on the disease gene but who have not developed the disease themselves). There are other exceptions to this; perhaps due to the late onset of the disease family members may die of something else before they develop MND (heart disease for example). This can be misleading and give the appearance that the disease has skipped a generation. Other families appear to have had no MND cases previously, either through insufficient information about previous generations or because previous generations did not live long enough, but subsequently two or more siblings develop the disease. What are the specific problems in MND? A 'head to toe' approach to
screening is essential so that no symptoms are missed.
Patients and carers may suffer profound depression following the diagnosis
of ALS/MND. Even in the absence of this, emotional lability ('crying one
minute, laughing the next') is a frequent symptom seen in MND. It is important to reassure patients that it is not a sign of early dementia, (which affects less than five per cent of patients in an obvious way), although a spectrum of mild cognitive changes, that may not intrude on daily life, may affect over 30% of patients. It is clear that MND is not simply a motor system disorder and that other areas of the brain can be affected. Patients with bulbar involvement may complain of excess saliva or drooling (sialorrhoea) because of impaired swallowing (rather than excess production). Impairment of swallowing can lead to distressing choking episodes, although patients and their carers should be reassured that choking to death is virtually unheard of. Nasal regurgitation of liquids may occur, due to poor palatal movement. Fear of eating leads to dehydration and malnutrition, and can cause problems with oral hygiene. Speech may range from mildly dysarthric to absent. Some patients lose speech early in the disease yet remain ambulant for a prolonged period, again illustrating the patchy, unpredictable nature of MND progression, and the dangers of being drawn into giving too accurate a prognosis. Neck weakness and the need for head support can be troublesome as the disease weakness progresses. This impairs communication and sometimes airway patency, compounding any swallowing difficulties. In the upper limbs, progressive loss of power leads to increasing dependence on others for feeding, dressing and other activities of daily living such as writing. If mobility is impaired due to leg involvement, progression to the arms may preclude the use of frame supports or a non-motorised wheelchair. In the chest, diaphragmatic weakness is important as a cause of respiratory insufficiency and may lead to hypoventilation. Intercostal muscle weakness may also occur. This can manifest as attacks of breathlessness, often worse when supine (orthopnoea), or with symptoms of chronic hypoventilation such as morning headaches, fatigue, poor appetite, broken sleep or excessive daytime somnolence. Reduced coughing ability can lead to recurrent and persistent chest infections. Seasonal influenza vaccination is advisable. Bulbar involvement may put the patient at risk of aspiration but this is much less common than expected. Truncal muscle involvement can cause difficulty in maintaining posture and support. There is generally disease-specific sparing of involvement of the spinal neurones responsible for sphincter control. However, reduced mobility and dehydration in the later stages of the disease can result in secondary urinary urgency and constipation respectively. This may be exacerbated by weakness of abdominal musculature. In the lower limbs, wasting and weakness impairs mobility and can lead to repeated falls with associated risk of injury. If the arms are involved then the head may bear the brunt of a fall. Bed transfers may become impossible without hoisting, and this is made more difficult if there is also upper limb involvement. Profound and persistent dependent oedema is a very common accompaniment of lower limb weakness often with acrocyanosis due to reduced lower limb circulatory demand. Poor mobility coupled with loss of muscle tone can lead to intractable joint pains. A long-acting NSAID can be helpful. In the longer term patients are at risk of pressure sores and deep venous thrombosis.
The role of the multi-disciplinary team. The optimal care of the MND
patient is best delivered by a well co-ordinated multi-disciplinary team
approach.
A full occupational therapy assessment often reveals far more potential obstacles that need to be overcome than both the patient and carer had at first considered. Adaptations may need to be made to housing - plans must be realistic, allowing for progressive disability. Aids for facilitating household tasks - for example, mobile arm supports, advice on driving (hand-control, wheelchair-accessible vehicles) and other aspects of community access - are also available. Physiotherapists have expertise in aspects relating to mobility and posture. Patients with foot drop may benefit from orthoses to prevent tripping. Hand exercises are useful to help prevent contractures and maximise function. A variety of neck supports are available and patients often need to try several. Patients can be taught strategies for turning in bed to prevent joint pains from prolonged immobility. Early referral to speech and language therapy services at the first suggestion of bulbar problems is important. Patients can be taught strategies to facilitate the passage of fluid/food without choking by maximising residual function, and the risk of aspiration can be assessed relatively easily using techniques such as video fluoroscopy. Specific advice on communication equipment, such as a Lightwriter or a more sophisticated PC-based system, can also be given. It is essential to maximise communication by whatever means to avoid the distress of reduced communication of needs and wishes, particularly as the disease progresses, when there may be significant speech and writing impairment. Prompt referral to a dietician is advised if there is concern regarding inadequate caloric intake. Weight loss has a significant adverse effect on survival. An assessment of needs can be made and 'build-up' drinks arranged. Advice regarding the timing of gastrostomy (see below) can be given in partnership. Patients should be encouraged to consider the procedure ‘prophilactically’ before respiratory involvement becomes a bar to even the mild sedation which may be needed for a PEG. Many patients benefit greatly from referral to local palliative care services, usually through a hospice. There is now a greater recognition of the role of hospice care in MND. The subject of referral needs to be approached sensitively but inclusion of the palliative team early in the disease supports both those affected by MND and the professionals involved. Local nursing support, in the form of home care teams, can be of great comfort to patients and carers even if they do not wish to attend the hospice as a source of respite. In the later stages, expertise in the management of respiratory failure is invaluable and can prevent inappropriate and distressing emergency admissions. In the face of rapidly progressive disease with no cure available, it is easy for the patient's GP and hospital physicians to develop a sense of helplessness. However, the GP's role is pivotal, not just in co-ordinating
services but also in the prompt management of symptoms, which can

greatly improve quality of life.
Putting patients on the Supportive Care Register (Gold Standards
Framework) will help those in the primary care team to ensure regular
reviews are undertaken and planning made for the future needs.
Specific interventions. Riluzole, a glutamate antagonist, has been licensed
for the treatment of MND. It is not a cure, but has been demonstrated to
have a modest positive effect on survival in large placebo-controlled trials.
Riluzole will not make the patient feel better, and there is no way to know
the extent of individual benefit from taking this drug. However, it remains
the only disease-modifying drug treatment for MND, and many patients feel
strongly that they should have the chance to take Riluzole despite its small
effect. It has been endorsed by the National Institute of Clinical Excellence.
Treatment requires monitoring of liver enzymes at baseline and monthly for
the first three months. Three-monthly tests are sufficient thereafter. Any significant rise in transaminases requires initial dose reduction and monitoring, and if persistent, then drug withdrawal, though such cases are very rare. Common side-effects of fatigue and nausea are more likely reasons for patients to stop riluzole therapy. Sialorrhoea may respond to amitriptyline, which is also a useful drug for the treatment of emotional lability. A low initial dose of 10mg, increasing as tolerated, is recommended. A hyosine (Scopoderm) patch, low dose atropine eye drops on the tongue or glycopyrrolate either orally or via a gastrostomy tube are alternative treatments. Judicious use of botulinum toxin injection into the salivary glands can be helpful but must be repeated every few months. Irradiation of the salivary glands is recommended by some Spasticity causing contractures or affecting mobility may respond to spasmolytics such as baclofen, dantrolene or tizanidine, but can cause unacceptable weakness due to the mode of action of these drugs. Joint pains related to immobility can be difficult to treat. A review of posture including wheelchair cushion replacement may help, but often it becomes necessary to use analgesia. Simple analgesics are preferred, or a long-acting non-steroidal preparation if tolerated. The use of opiate-based preparations may be necessary, and patch preparations should be considered. Many patients elect to take a variety of over-the-counter anti-oxidant preparations such as vitamin E, although firm evidence of benefit is lacking. Many patients see the insertion of a gastrostomy feeding tube as an admission of failure and progression towards death. Although some refuse the procedure outright, it is useful to initiate an early discussion of this intervention so that an informed decision can be made before a crisis is reached. If the discussion is left until oral feeding has become impossible, then malnutrition and respiratory weakness may render the procedure too risky. The radiologically inserted gastrostomy (RIG) requires less sedation and may be performed at a later stage of the disease. In practiced hands, standard endoscopically inserted gastrostomy (PEG) can be performed as safely in late disease. Inevitably, weakness spreads to the muscles of respiration. Severe breathlessness is extremely distressing, but may respond to low-dose lorazepam sub-lingually. A few patients elect to have permanent assisted ventilation via tracheostomy. However, use of non-invasive positive pressure ventilation (NIPPV) is much more widespread, and results in marked symptomatic relief and improved quality of life.
Support and information. The Motor Neurone Disease Association in
England, Wales and Northern Ireland, and the Scottish MND Association provide support to patients and their carers on all aspects of MND, along with information packs for GPs. Regional Care Development Advisers (MND Association) and MND Clinical Specialists (Scottish MND Association) are available to advise and educate professionals and patients. The Associations operate an equipment loan scheme and can provide limited financial assistance. The care of the MND patient undoubtedly represents a major challenge for all, but with adequate planning it is possible to maximise quality of life for patients with MND. Acknowledgement

Some of the information contained in this article was adapted from an article in The
Practitioner by Martin Turner in 2001. It was substantially edited and amended by Dr Tim Williams (Consultant Neurologist and Care Centre Director, Newcastle MND Care Centre, Newcastle General Hospital, Newcastle-upon-Tyne) and Dr Martin Turner (Consultant Neurologist and MRC/MND Association Lady Edith Wolfson Clinician Scientist, Oxford MND Care Centre, John Radcliffe Hospital, Oxford.
Additional reading
MND Resource File: A patient and carer-centred approach for Health and
Social Care Professionals. MND Association literature
GP Booklet: A problem-solving approach for General Practitioners and the Primary Health Care team MND Association literature Factsheet: Managing MND Scottish MND Association Talbot & Marsden. MND: The Facts. Oxford University Press Talbot, Turner, Marsden and Botell. Oxford Care Manual of MND. Oxford University Press. Estimated publication late 2009

Source: http://www.mndassociation.org/Resources/MNDA/Migrated%20Resources/Documents/G/GP%20MANAGEMENT%20OF%20MND_1778.pdf

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