Standards of Care for Patients with Neurological Disease: A Consensus. Report of a Working Group



## Systems of care
The management of all the neurological diseases under discussion involves both family doctor and consultant neurologist. Some patients with Parkinson's disease may be under the care of a consultant geriatrician, and some cases of epilepsy under a consultant paediatrician. The consultant is usually involved in confirming or making the diagnosis and deciding upon appropriate investigation.
Each health district should formulate a policy for caring for those with chronic physical disabilities, with active rehabilitation services for those who can be improved, and with organised systems of care for these, and also for those whose diseases, like motor neurone disease, are advancing. Disabled people may be less handicapped if relatively modest adjustments to their homes are made, and if physical aids are promptly provided after skilled assessment. Every district should have a hospital based assessment team, including a physiotherapist and an occupational therapist, who can determine, together with the patient, the relatives and the family doctor, the patient's present and likely future needs.
Professional care must be shared. The neurologist and family doctor must liaise about advice on employment, eligibility to hold a driving licence, recreation, diet, and treatment of associated symptoms and diseases. Continuity of care is essential so that, even though care of the neurologically disabled patient is shared, the patient knows whom to contact, and how to make contact, for further advice. The extent to which the long-term care of each patient is hospital or community based will vary depending on the particular manifestations of the disease and the expectations held by individual patients and their relatives.
We believe that some districts should appoint and evaluate the effectiveness of 'keyworkers' assigned to individual patients. Keyworkers might have professional training in one of a number of disciplines, such as nursing, social work or physiotherapy, but would need to acquire some expertise across all relevant disciplines so that they could give 'first level' advice to their patients. Keyworkers should be appointed by and be professionally and managerially accountable to consultant members of a department of neurology. Keyworkers could be appointed within existing resources by replacing, for example, a physiotherapist. Since the Government has adopted the Griffiths report, budgetary arrangements for keyworkers' domiciliary care will need to be made with local authorities. The keyworkers should be part of the District Disability Assessment Team, and would seek advice from team members in other professions. The keyworker would devise and help maintain a record card held by the patient, on which all relevant professions made entries.
Arrangements for outpatient care should be flexible but, for the diseases considered here, consultant neurological supervision should be available at intervals that are determined by the rate of progression of the illness or by the difficulty in controlling symptoms. Patients and their relatives, their family doctors and neurologist must all define and agree on the objectives of outpatient visits. Outpatient appointments for 'old' patients may more readily be found if patients in remission with, for example, multiple sclerosis or epilepsy are not seen needlessly on a 'routine' basis. Particular problems arise in providing continuity of care for patients with epilepsy. Patients with continuing seizures are too often relegated to management by registrars, and therefore tend to suffer because of frequent, and often ill-advised, changes and additions to drug therapy. It is important that the consultant sees and reviews such patients at least once per year so as to provide other less experienced staff with a policy for ongoing management of that patient. Neurologists and others should be more ready to advise by telephone patients known to them, and their family doctors, so that the rigours of an outpatient appointment can be avoided. Other novel systems of care that should be tried and evaluated include the use of 'walk in' clinics, similar to those now popular with diabetic patients, at which advice can be obtained without prior appointment. Such clinics could be based upon a local neurological rehabilitation centre, or a unit with beds for the younger patient with chronic disabilities. All concerned with the management of chronic neurological illness can help the patient by counselling, focussing in particular on helping the patient's expectations adjust to the new realities brought about by his illness. All team members should also have sufficient knowledge to advise about how chronic neurological illnesses affect personal and sexual relationships.
Local branches of voluntary patient associations (for addresses, see below) should be encouraged to form partnerships with the local neurological services. At an appropriate stage in their illness, patients should be offered the opportunity to meet representatives of these associations. Members of patient associations offer valuable support to the patient, and often also to carers, by allowing both to know that there are others to share the problems created by the disease. The associations may also be a useful source of advice about obtaining appropriate welfare benefits, about employment, and about changing personal relationships. Members and volunteers may offer practical help, for example, with transport, with gardening and with collecting pension payments, and the association may also be able to help with respite care or a holiday for a carer. However, we do not believe that the voluntary sector, as exemplified by these associations, should have to make up for deficiencies in the provision of appropriate National Health Service care.
Many patients with neurological illness are concerned to know about recent advances in the management of their condition, and the results of current Corticosteroids, given as short pulses of high dose intravenous methylprednisolone, intramuscular ACTH or oral steroids in diminishing doses over a few weeks, improve the rate of recovery in the context of recent symptomatic change [bib_ref] A double blind controlled trial of high dose methylprednisolone in patients with..., Milligan [/bib_ref] [bib_ref] Relative efficacy of intravenous methylprednisolone and ACTH in the treatment of acute..., Thompson [/bib_ref]. There does seem to be a selective effect on pyramidal function, especially spasticity. On the present evidence, their use is not limited by adverse effects. These drugs have little role in patients with slowly progressive or persistent disability. In general, there is no place for the long-term use of steroids. It must be remembered that not every change in symptoms is necessarily due to relapse. A worsening of spasticity, for example, may be due to an intercurrent infection. The rare short-lived paroxysmal events (eg trigeminal neuralgia or dysarthria lasting a few minutes) are well prevented by carbamazepine.
Experimental treatments designed to influence the long-term course of multiple sclerosis are not usually considered until disability is well established [bib_ref] Diagnostic and therapeutic trials in multiple sclerosis: a new look, Weiner [/bib_ref] [bib_ref] A referendum on clinical trial research in multiple sclerosis: the opinion of..., Noseworthy [/bib_ref] -The agents currently in use are of uncertain benefit and carry an appreciable risk of adverse effects. It is noteworthy that, when those researching in multiple sclerosis were asked to consider themselves as patients, many said that they would not be prepared to be randomised into trials of immunosuppression or treatment with interferon [bib_ref] Diagnostic and therapeutic trials in multiple sclerosis: a new look, Weiner [/bib_ref]. Patients and clinicians are therefore understandably cautious early in the illness, even though that may be the best time for influencing the prognosis. Very occasionally, when the disease is pursuing a rapidly advancing malignant course, it is reasonable to try and stem progression with cyclophosphamide [bib_ref] Controlled pilot trial of monthly intravenous cyclophosphamide in multiple sclerosis, Killian [/bib_ref].
Symptomatic treatment. Treatment can be given at any stage to relieve persistent symptoms which, although they adversely affect daily living, do not cause overt disability. The complications of multiple sclerosis which are most amenable to symptomatic treatment include involvement of the bladder and relief of spasticity and paroxysmal symptoms.
Approximately 80% of patients describe urinary urgency or frequency which modifies or completely dominates social, domestic and economic life. A combination of mild urgency with moderate immobility usually results in incontinence and disturbed sleep, as a result of which all symptoms are tolerated less well and fatigue may develop. Bladder symptoms usually arise from loss of spinal inhibition or reflex emptying, resulting in the urge frequently to empty a partially filled bladder. If the normal mechanism of detrusor contraction and sphincter opening is uncoupled, hesitancy, retention and incontinence also occur. Drugs which inhibit detrusor contractions and maintain urethral tone (anticholinergics, such as propantheline) successfully relieve these symptoms but the therapeutic window is narrow, especially if sphincter dyssynergia co-exists. Full bladder emptying can occasionally be achieved by manual abdominal pressure or other manoeuvres, but a useful and under-used technique is intermittent self-catheterisation which achieves several hours freedom from urinary symptoms and thus enhances social activities and improves sleep. This technique requires some motivation, adequate vision and the use of one good arm. It can be performed by a partner, and may obviate the need for indwelling catheterisation or urinary diversion. Urinary infections should be promptly treated. In general, urinary problems are so prominent in patients with chronic multiple sclerosis that help from a urologist with a particular interest in neurological disease is often useful. Spasticity may be relieved by the use of muscle relaxants, acting peripherally (dantrolene) and centrally (baclofen or diazepam), by physiotherapy, and by the use of high dose intravenous methylprednisolone.
Occasionally, motor end point injections with procaine or tenotomy may be needed to release an abnormal flexed posture of the lower limbs.
Motor neurone disease Pharmacological treatment. No pharmacological treatment influences the natural history of the disease. Symptomatic treatment [bib_ref] Motor neurone disease: can we do better? A study of 42 patients, Newrick [/bib_ref]. Pain may be helped by appropriate gentle exercises, support, and by carefully adjusted analgesic treatment. Dysarthria may be helped, temporarily, by speech therapy, by intra-oral devices and, at a later stage of the disease, by the provision of appropriate aids to communication such as a CANON light-writer or POSSUM equipment linked to a microcomputer, which can also be used to control some aspects of the environment, such as lighting. Drooling of saliva may be helped by anticholinergic drugs, by radiotherapy to the salivary glands, or by surgical diversion of salivary ducts. Nutrition can be maintained via a nasogastric tube or a gastrostomy. Some patients may be helped by a cricopharyngeal myotomy, but experience shows that this is only effective if some tongue movement remains. Many patients suffer from insomnia, partly due to muscle pain and inability to get comfortable in bed. An electrically operated turning bed may be very useful. Some patients suffer from night terrors associated with hypoxia, and these may be helped by nasal catheters through which air is delivered at positive pressure. Very occasionally respiratory muscles fail before limb and bulbar muscles, and for these few patients support by a cuirasse ventilator is reasonable. Emotional lability may be helped by clomipramine.
It is helpful to explain to both patient and carer that incontinence seldom occurs, that mental clarity usually persists to the end, and that although choking attacks cause concern they are usually not fatal. Most patients die peaceably of respiratory failure. The severity of the symptoms can be conveniently separated into mild, moderate, severe, and terminal. 'Mild' is defined as with symptoms but no disability; 'moderate' is disability (that is restriction of activity) but preservation of personal independence; 'severe' is when the disease has deprived the patient of personal Patients with established disease often have more problems with associated symptoms than they do with the symptoms of Parkinson's disease. Constipation is common, especially in the elderly, and especially in patients taking anticholinergic drugs. Depression is common and frequently overlooked, even though it may well be relieved by tricyclic antidepressants. The retardation of depression may be mistaken for the bradyphrenia and bradykinesia of Parkinson's disease. Equally the limited range of facial expression and slow movements of a patient with Parkinson's disease may falsely suggest that the patient is depressed. Akathisia (pathological restlessness) due to the disease may disturb sleep for patient and household. It may be mistaken for insomnia but seldom responds to sedative drugs. Reversal of sleep rhythm may occur because the akathisia is relieved in the day-time by levodopa, so allowing the patient to sleep. Levodopa may exacerbate depressive symptoms, especially when treatment with this drug is first initiated. Levodopa induced dyskinesias, 'on-off fluctuations in response, or wearing off effects are difficult to treat. Small frequent doses of levodopa, or the addition of doses of bromocriptine or selegiline timed to the patient's daily activities, may help. Apomorphine by subcutaneous injection or infusion may become a more generally available and useful method of controlling these symptoms. The side effects of treatment usually require hospital outpatient supervision, with occasional admission to analyse the patient's response to treatment.
The mono-amine oxidase inhibitor selegiline has been shown to prevent the establishment of damage to the substantia nigra of experimental animals produced by l-methyl-4-phenyl-l ,2,3,6-tetrahydropyridine (MPTP), and there is now evidence that the course of Parkinson's disease in man may also be slowed by this drug. However, larger scale prospective studies are required before this type of medication should be recommended as standard. Symptomatic management. Patients will often need advice about a new symptom arising in the course of their disease. Depression of mood responds reasonably well to appropriate drugs, or may be due to levodopa. Confusion may be an adverse effect of medication, particularly the anticholinergic drugs. Constipation is often a problem, but may be helped by bulking or by stimulant laxatives. Advice may be needed about sexual dysfunction, incontinence, driving and interpersonal relationships.
Day centre care may provide valuable relief for the home carer of a severely affected patient. Holiday relief in a nursing home or geriatric or neurological ward may also be highly desirable. Early planning of these arrangements increases the family's trust in the neurologist's sympathetic understanding of their needs.
As the disease progresses, a visit by a district nurse or occupational therapist is often helpful. Home nursing assistance, especially for bathing, is often essential in the severe or terminal case, and hospice care is appropriate for some for the terminal stages of the illness. Home help, volunteer assistance and health visitors all have an important place in aiding and assisting the main carer. The patient and family should feel that there are others with whom they can share the problems the disease creates. At present the family doctor is best placed to organise the provision of these services, but a 'keyworker', as described above, could play a useful role.

## Epilepsy
Pharmacological treatment. There is little knowledge of the prognosis of untreated epilepsy. This makes it difficult to know when to begin drug treatment. In practical terms this is decided on an individual basis after discussion with the patient. For patients with occasional seizures or with seizures causing relatively minor symptoms, social factors such as the requirement for a driving licence and types of employment are important when reaching a decision. Similar factors apply to the decision to withdraw drugs after a period of remission of seizures. Treatment should be limited to a single appropriate drug wherever possible, although, in patients whose seizures are refractory to single drugs, brief trials of drugs in combination may be undertaken. In view of the known adverse effects, treatment should not now be initiated with phenobarbitone or primidone. These drugs also seem particularly difficult to withdraw without recurrent seizures. Sodium valproate may be preferable to these drugs for the primary generalised epilepsies (simple absences, epilepsy, juvenile myoclonic epilepsy).
There is little evidence that for other adult epilepsies there is any difference in efficacy between anticonvulsant drugs. The choice is determined by considering what potential adverse effects are particularly relevant to the patient. Estimation of serum levels of anticonvulsant drugs, although performed more often than is necessary, is useful if seizures are continuing, if there is doubt about the patient's compliance, or concern about whether symptoms are due to toxicity, or whether the patient will be able to tolerate an increase in dosage.
Symptomatic management. Most patients with intractable epilepsy will be in the community. Patients with added intellectual, neurological or psychiatric disability require liaison with professionals in ancillary disciplines social workers, day training centre staff, mental handicap teams, etc. Here again a keyworker would be valuable.
The medical role of the neurologist should be: ? to optimise pharmacological treatment of epilepsy in any given individual as quickly as possible ? to consider at an early stage (within two or three years) whether a patient's epilepsy is medically refractory, and to explore the advisability of surgical treatment. Such patients almost always have complex partial seizures. About 70% of carefully selected patients are cured by surgery. Current information suggests that, of the 50 per 100,000 population developing seizures each year, 1-2 per year would benefit from surgery to the temporal lobe. Adequate assessment of so many patients has resource implications for neurology, neurophysiology, neurosurgery, neuropsychology and neuroradiology. Regional and supra-regional teams will be required to fulfil a demand for surgery. For patients with medically refractory epilepsy unsuitable for a surgical approach, the neurologist's role is to ensure minimisation of therapy compatible with optimal control, so as to avoid an excessive incidence of chronic adverse effects from the drugs used in treatment. There may come a time when, because of adverse effects, it is just not worthwhile pursuing new combinations of drugs, and patients may feel better tolerating a few seizures rather than such effects. Patients who are unlikely ever to achieve a long-term remission should be told of this.
It is especially important that family doctors and neurologists are aware of the particular problems of women with epilepsy, notably the interaction between anticonvulsant drugs and oral contraceptives, the effect of pregnancy on epilepsy and on anticonvulsant drug metabolism, and the teratogenic effect of anticonvulsant drugs.
Status epilepticus continues to have a high mortality. District general hospitals should have protocols for prompt and adequate therapy. Points of particular concern to patients The Working Group felt that a number of aspects of care were not well handled by neurologists at present.

## Points raised included:
? a lack of a co-ordinated plan for management shared between family doctor and hospital ? a lack of continuity of care in hospital clinics poor transport facilities to outpatient clinics at which attendance is often inappropriate, and felt to be inappropriate ? the slow provision of physical aids, structural alterations to the home, or supporting services such as community physiotherapy ? the lack of adequate local facilities for welfare counselling, home nursing and physiotherapy, day care and holiday relief. Some patients feel that the most frustrating aspect of contact with some consultant neurologists is their nihilistic approach. Clinicians need to strike a balance between therapeutic optimism, based on an increasingly active research initiative, and the exposure of vulnerable individuals to improbable or overly hazardous approaches to treatment.
Priority areas for research Some areas are common to all the diseases we considered. We need better measures of functional status and of the quality of life, so that the quality of supportive management can be measured and become more appropriate. We need to evaluate more carefully the effectiveness of our present systems of care, and of newer proposals the effectiveness of 'keyworkers' and of special clinics such as epilepsy clinics.

## Multiple sclerosis
The priority for research into multiple sclerosis is to find an effective treatment. Because there has been no serendipitous breakthrough, systematic work on the mechanisms of demyelination is still required. The disorder has a reputation for poorly conducted and unimaginative research. The literature is contaminated by elaborate hypotheses often constructed merely to justify a particular therapeutic hunch. Understanding the mechanisms of myelin injury through an integrated approach using tissue culture, animal models and analysis of human samples may eventually lead to effective treatments. These may not immediately benefit the large number of patients in whom the disease is well established but, in the long term, learning about the aetiological factors genetic or environmental ? which render some individuals susceptible to multiple sclerosis may lead to prevention of the disease.

## Motor neurone disease
The issues for motor neurone disease are similar to those for multiple sclerosis. In addition, at a simpler level, we need more information about the relative merits of techniques used to deal with dysphagia, including nasogastric tubes and the relative merits of pharyngostomy versus gastrostomy. The symptomatic management of drooling is also unsatisfactory and requires further research.
Parkinson's disease Research priorities here are the structure and formation of the Lewy body ubiquitinated proteins and their relevance to exogenous toxins and the cause of Parkinson's disease. We also need further research into the biology of striatal implants, and the more effective delivery of dopamine agonists to the central nervous system.

## Epilepsy
Further research is needed into when it is appropriate both to start and to stop anticonvulsant treatment.
Auditing the care of patients We have here laid down standards for the treatment of four neurological illnesses. Much of what we have written about systems of care is likely to be relevant to other chronic diseases, the care of which is shared by family and hospital doctors. With defined standards, the quality of care may be audited.
The following are examples of general measures that could be used in audit: ? Accessibility to specialist care (eg waiting times and distance to travel for a neurological consultation). ? Accessibility to other aspects of care (eg availability of counselling, community physiotherapy, day hospitals, waiting times for provision of aids etc). ? Continuity of care (eg proportion of outpatient visits at which a patient is seen by the consultant).
? Information (eg information given about patients' associations; patients' understanding of their diagnosis; the family doctor's satisfaction with information received from the neurological clinic). Adverse outcomes (eg drug-induced confusional states; pressure sores). Inappropriate provision of medication (eg long continued steroids in multiple sclerosis, patients with continuing juvenile myoclonic epilepsy in whom sodium valproate has not been tried; inappropriate polytherapy for epilepsy). ? Adequate record of plan of investigation and management.

## Resource implications
Data on some of these audit measures (for example, on waiting times) can be obtained by simple secretarial work, and are suitable for routine recording for which a computer would be helpful. Other measures, such as a review of the medical records to ascertain whether these contain a written plan for future management, require first the retrieval of sets of notes and then the time of a skilled person, who may or may not be medically qualified, to review their contents. Yet other measures, such as an audit of patients' understanding of their diagnosis, require specific focused research projects. Such research audit may be small scale, but the methodology must be sound. All such initiatives will require time, and hence either additional resources or the displacement of more traditional clinical work. And yet there is evidence of an underprovision of neurological care at present. There are currently 190 neurologists in the United Kingdom, including six fulltime academic posts, for a population of 56.8 million ? roughly one per 373,000. The Association of British Neurologists has recently recommended that there be one neurologist per 200,000. An attempt has been made by some neurologists in the United Kingdom to judge the amount of time that they 'ought' to spend with patients [bib_ref] Norms of care in British and American neurological practice, Menken [/bib_ref] , but the following example will suffice. With three clinics a week, a neurologist will have available about 480 hours of outpatient time each year. And yet, with the prevalence of 240 patients with multiple sclerosis in a population of 200,000, half of this time would be used up if just one hour were spent each year with each patient with this one disease. If the community wishes to improve neurological care to the standards proposed in this paper, then there should be an increase in the number of consultant neurologists, and their training in the management of epilepsy and other causes of chronic disability should improve. Resources will also be necessary to pay keyworkers and therapists.
[table] Table 1: Drug treatment in Parkinson's disease [/table]