Epidemiological aspects

Neurological disorders have certain notable characteristics:

• They are large in number

• Few neurological disorders are totally curable, although new, and expensive, treatments are currently being introduced for many of the major neurological disorders including multiple sclerosis, motor neuron disease, stroke, epilepsy, and Alzheimer’s disease

• Neurological disorders are associated with many symptoms

• Neurological disorders are a major cause of disability and account for a high proportion of severely disabled people under the age of 65.

• The adverse effects of most neurological diseases can be reduced.

Three arbitrary groups of neurological disorders can be identified according to their frequency in the general population. Neurological disease, looked at as a group, may be likened to an animal having a small head, a rather large body, and a very long tail. There are a few common diseases, a greater number of less common disorders, and a huge number of uncommon disorders comprising hundreds of separately identifiable conditions. The total cost of the many uncommon diseases taken together is considerable and may well approach that of the common diseases.

Neurological disorders characterised by full recovery

There are a few disorders that are followed by full recovery (for example, carpal tunnel syndrome and some forms of meningitis). Some are cured by neurosurgery (for example, benign tumours). Overall, these disorders probably do not contribute in any major way to the total burden of neurological disease although they have the potential to do so if they are not dealt with properly. Although the disease process may be arrested by neurological, neurosurgical, or other interventions, some patients are left with permanent disability.

Disorders that cannot be cured

Many patients in this group can be helped by various medical or surgical procedures. Rehabilitation is often of much importance. The group contains many disabled people. A large proportion of the total personal and national burden is produced by this group.

There are several ways of considering the problems of those with chronic neurological disorders. Each of the following classifications underscores the importance of prolonged disability on personal and economic capacity.

The first classification is by dominant clinical problem.

Those for whom the main problem concerns physical disability—Examples of disability are inability to walk, incontinence, and difficulty with performing activities of self care). The group includes those with multiple sclerosis, motor neuron disease, and Parkinson’s disease.

Cognitive impairment—This group includes the survivors of closed head injuries, Alzheimer’s disease, and Huntington’s disease. The burden of this group may include the difficult problems of wandering, aggressiveness, incontinence, and concerns about safety (for example, some people with dementia may risk their own and other lives through unsafe use of matches and cigarettes).

Intermittent disturbance—Examples include epilepsy, narcolepsy, movement disorders, and migraine. People in this group experience much difficulty with planning their lives because of the uncertainty caused by the condition.

Pain—This group contains those patients whose principal problem is pain. Example disorders are trigeminal neuralgia, sciatica and chronic back pain, migraine, and diabetic neuropathy.

Those in whom a mixture of two or more of the previously listed problems are present—For instance, Parkinson’s disease may be associated with dementia and indeed cognitive disturbance may eventually come to dominate the clinical picture. The above classification allows identification of the principal clinical problem(s).

Another classification involves a consideration of how the burden of disease is determined by the age of onset. This grouping was originally suggested by Warren.1

Disorders with onset in childhood —Disorders with onset in childhood include cerebral palsy, epilepsy, muscular dystrophy, and spina bifida. A huge personal burden is likely to fall on the family—often preventing one of the parents from working, with obvious financial implications. The main National Health Service (NHS) burden will fall initially on the paediatric services and the primary care team. The organisation of appropriate schooling is essential.

Disorders with onset in adolescence and early adult life—The objectives of normal adolescents include becoming independent of parents, moving away from home, completing education, obtaining employment, developing sexual competence, learning to drive, and becoming financially independent. Additionally, there are the usual emotional upheavals to be tolerated! All these objectives are more difficult to achieve if the person has a major neurological problem—especially if there is associated appreciable disability. Closed head injuries resulting from road traffic accidents are a particular problem at this age.

A major investment of time, skill, and resources is required if the young person is to achieve his or her full potential. Arrangements for transition from school to work and from paediatric to adult health services need to be considered at this time.

Age 26–55—This is the stage of life at which children are being conceived and reared. Employment and career are important, and often a house is being bought. Additionally, sexual activity is at a high level. Rates of separation and divorce are high. Parents are becoming increasingly frail and are often less able to help. All those social elements are likely to be important in considering the effects of a major neurological illness such as multiple sclerosis. Other relevant conditions include epilepsy, migraine, and other headache syndromes.

Diseases occurring in this age group have important effects on employability, with a resultant strain on personal finances, as well as loss of tax revenue on earned income and a drain on the social security budget.

Age 55–75—The average person retires in this time and is able to anticipate a number of years of disability free life. Many people remain physically and mentally fit. Parents have usually died by now. Neurological disease may affect this otherwise enjoyable phase of life. Some of the disorders of later life start to appear including stroke, Parkinson’s disease, Alzheimer’s disease, and motor neuron disease.

Age 75 onwards—The proportion of people who are disabled increases markedly from age 75 onwards. The period is responsible for a large proportion of health costs. There is increasing general frailty with multiple health problems. Geriatric services are heavily involved. An increasing number of people live alone. The important neurological diseases include stroke (responsible for about 25% of severe disability in people living in their own homes), Alzheimer’s disease, and Parkinson’s disease. Another way of examining the burden of neurological disorders involves the time course of disease. Six arbitrary groups of conditions emerge. Disorders which cause sudden death, or which are followed by rapid recovery, are not included in the discussion as they add little to the burden on health care services.

Sudden onset with severe damage and little, or no, subsequent improvement—This group includes catastrophic head injury, Herpes simplex encephalitis, severe stroke, and spinal cord transection. The results may be a persistent vegetative state, the locked in syndrome, or permanent paraplegia/tetraplegia. Some patients remain totally dependent and are a major burden on the hospital and other services, as well as to their families.

Sudden or gradual onset with at least some subsequent improvement—Many disorders fall into this group including stroke, head injury, incomplete spinal cord injury, and Guillain-Barré syndrome. In some instances there is complete recovery and in others there is permanent disability. High quality intensive care is often initially needed followed by rehabilitation input, representing a significant burden on the health services.

Lifelong disability from childhood—Many disorders in this group are non-progressive or only slowly so. Examples are spina bifida, cerebral palsy, treated hydrocephalus, and early onset epilepsy. These disorders represent a major burden for families—leading to, for instance, restricted employment for at least one of the parents.

Disorders characterised by fluctuation and uncertainty—Multiple sclerosis, epilepsy, narcolepsy, and migraine are examples. The personal burden involves difficulty in planning ahead and a major effect on employment opportunities and relationships. Secondary psychological and psychiatric problems are common.

Gradual onset with predictable death within six months to five years—Creutzfeldt-Jakob disease, malignant glioma, and motor neuron disease are in this group. Death is sometimes preceded by distressing symptoms. Expert terminal care is often needed.

Gradual onset with death due to the disease many years after onset—This group contains disorders that can occur at any age—Duchenne muscular dystrophy, Friedreich’s ataxia, some leukodystrophies, Huntington’s disease, severe multiple sclerosis, Alzheimer’s disease, and Parkinson’s disease are examples. Prolonged and severe disability may ensue. The group has major personal and national implications. For instance, the vast majority of the people in units for younger disabled people are there because of the effects of neurological disease.2

The perspective of the patient and his or her family

Many accounts of personal experiences of neurological diseases exist3-5 and illustrate that the burden on the patient and family may be enormous.

Many patients are reluctant to admit secret fears related to the diagnosis. Thus a patient with severe headache may harbour fears that he may have a brain tumour. A thorough clinical assessment may reassure him that his fears are groundless. The devotion of half an hour of consultant time (and possibly a brain scan) may totally relieve him of his secret burden.6

The effective making, and telling, of a serious diagnosis is much appreciated. If appropriate, a second opinion may be arranged. Unnecessary uncertainty regarding the diagnosis is a burden that should be avoided if possible. There may be anxieties about other matters including the availability of treatment, the outlook, and any genetic implications.

There are “medical” outcomes that may be particularly feared. It may be possible to provide reassurance in many instances or to adopt some other approach, again reducing burden.

They include the following:

• Dementia and change in personality—“will I lose my mind?”

• Incontinence

• Uncontrollable pain

• Severe physical disability and dependancy

• Change in physical appearance (for example, facial palsy in a teenage girl, loss of hair with neurosurgery or radiotherapy, loss of facial expression in Parkinson’s disease)

• Adverse effects on sexuality or reproductive life

• Inability to communicate with others (for example, pseudobulbar palsy due to motor neuron disease).

There are also socioeconomic concerns including the following;

• Dependency—“will I become a burden?”

• Children—“who will look after the children?”

• Employment—“what will happen if I lose my job?”

• Finances—“how will we cope?”

• Driving—loss of driving mobility can represent a major burden and may even result in loss of a job

• Leisure activities—“will I still be able to watch my favourite football club on Saturdays?”

• Effect on my family and career—“will I continue to be able to look after my spouse and children?”

 Patients require a considerable amount of help at the time the diagnosis is made and also at times of crisis, such as during a major relapse of multiple sclerosis. This help should come from various sources including the neurologist, the family doctor, and the multidisciplinary team (which will include a social worker, community nurse, and therapist). Many patients appreciate the availability of professional, and lay, support (including that given by voluntary organisations). Expert advice relating to state and other financial benefits and allowances is a further important matter and professional guidance on this topic is provided by social workers.

 Effective interventions along the above lines are likely to lessen personal burden. Patients need to know that they will not be abandoned by the specialist services, and that expert or informed help and advice is always available.

The national perspective

The nation has an interest in reducing state expenditure as much as possible, and in ensuring that money is “well spent”. It also has an interest in the existence of high quality services. Issues of effectiveness, efficacy, efficiency, and equity arise.

WHAT ARE THE CURRENT IDENTIFIABLE COSTS TO THE NATION OF NEUROLOGICAL DISEASE?

Direct costs fall on the NHS, which is responsible for the healthcare needs of the population; on the Department of Social Security, which deals with state benefits; and on Local Directorates of Social Services which have responsibility for community care.

Indirect costs include loss of economic activity of people with neurological disease and of the tax revenues generated through it.

There is little published information about the indirect costs of neurological disease. Some information, however, is available about NHS costs. Analysis of expenditure in England for the years 1992–3 showed that diseases of the nervous system (International Classification of Diseases-revision 9) accounted for 8.4% of the total health and the social services net public expenditure for the years 1992 and 1993.7

STROKE

The largest part of the direct healthcare costs associated with cerebrovascular disease results from treatment that is provided on an inpatient basis.8 The Hospital Inpatient Enquiry estimated that there were 118 500 admissions to hospital in England for cerebrovascular disease in 1985. The mean duration of each patient’s stay for each “case” was 48.8 days. The disease was estimated to have generated a total of 5.78 million bed days per annum. This gave, according to cost estimates existing at the time, an annual NHS expenditure of £550 million for England and Wales. This represented 3.9% of the total NHS expenditure.

More recent data related to costs in England for 1992–37indicate that stroke was the second most expensive disorder treated in hospital. The most expensive disorder was learning disability, responsible for 6.9% of inpatient expenditure. Stroke was responsible for 5.5%, about £607 million. Similar figures were obtained when community health care and personal social services costs were analysed. Stroke was responsible for 6.6% of costs. Again, learning disability was the only condition that was more expensive than stroke.

A study from Bristol9 showed that 41% of the inpatient costs of stroke in Frenchay Hospital was generated by patients who eventually went to long term care. A further recent Bristol experience indicates that on a rehabilitation ward, between 9% and 24% of patients, at any one time, are in hospital for “social” rather than medical reasons.

Despite vast expenditure of national funds on cerebrovascular disease, there has been severe criticism of the quality of inpatient care.10 Experience indicates that national funds are still not being spent to optimal effect.

DEMENTIA, INCLUDING ALZHEIMER’S DISEASE

There is less evidence on the cost of services for those with dementia than for other neurological disorders.11 Hospital costs are, however, substantial, accounting for 2.65% of expenditure in 1992–3.7 There is also a major burden on community resources and carers. There are also major nursing home costs.

EPILEPSY

The hospital costs for 1988 were reckoned to be £84.2 million—representing 0.6% of all hospital costs.12Between 0.2% and 0.5% of general practice consultations were for epilepsy. Antiepileptic drugs, in 1988, cost £21.4 million or 0.9% of the NHS drug bill. The total annual cost of epilepsy in 1988 was thought to be in the region of £109 million.

PARKINSON’S DISEASE

The total hospital, primary care, and medication costs of Parkinson’s disease were thought to be about £126 million in 1991.13 This figure included £99 million for inpatient care with 2175 beds being used daily on average by patients with Parkinson’s disease . They stayed in hospital for an average of 50.9 days. The general practice consultation costs were estimated at £2 million and medication at £25 million.

Another analysis found that the total burden of Parkinson’s disease was about £478 million with most costs incurred outside the NHS. The cost of home care management (including residential care) alone was estimated at £287 million annually.14

MULTIPLE SCLEROSIS

The Office of Health Economics (OHE) estimated that the cost of multiple sclerosis to the NHS is in the region of £27.3 million—the greater proportion of this being accounted for by inpatient costs (£20.1 million).15 An analysis of costs in 1992–3 showed that multiple sclerosis accounted for 0.29% of total hospital expenditure, about £47 million.7

There are other important costs including those incurred by caring for patients with multiple sclerosis in residential care. The impact of new developments, such as interferon therapy, is uncertain. The prescribing costs are potentially enormous. It has yet to be shown that these will result in significant savings in other areas.

HUNTINGTON’S DISEASE

Huntington’s disease is a relatively rare, genetically transmitted, form of dementia occurring in about 4000 people in the United Kingdom with perhaps a further 20 000 at risk of developing the disease.16 Although only a relatively few have the condition, the disease is so debilitating in its later stages that the costs to the NHS and social services could be as high as £12 million at 1980 prices.16

Discussion

It is difficult to be specific about the economic impact of neurological diseases—partly because of their large number and partly because many of them are so rare. The following general conclusions may be drawn.

Hospital costs are relatively easy to identify. The costs to patients and their families, although highly significant are often impossible to quantify. The available evidence seems to be that hospital costs in many areas, particularly stroke, represent the major identifiable burden on the NHS, but overall cost of support in the community is likely to be much higher.

Little is known about the complex and important needs of patients with neurological disorders. Little information is available to the health service as to precisely how hospital beds are used and why patients are in hospital and what resources they require when they are there. Unless this information is available it will be impossible to have a logical debate as to the precise use of hospital resources—for example, the need for intensive care or high dependency beds, the need for rehabilitation units, and whether the patient could be equally well looked after in a community or neighbourhood hospital or at home.

Studies from the 1960s onwards have shown that between 14.6% and 62% of acute hospital bed use may be considered inappropriate with both hospital admissions and hospital bed days being studied.17These studies have suggested that potential for improvement lies both in managerial domains—for example, discharge planning, and in the provision of alternative services. The appropriateness of acute hospital care has also been studied by the audit commission. The reportLying in wait; the use of medical beds in acute hospitals 18 examines the potential for improving efficiency in the use of hospital beds through good practice. Variations in bed provision and bed use throughout England were analysed and it was concluded that if all districts could achieve lengths of stay and turnover intervals at least as low as the current best 25% of districts, there would be the potential to reduce the number of beds in England from 85 000 to 58 000 while maintaining the present level of activity. Part of this reduction would result from the removal of organisational deficiencies identified in the report and part would require investment in community services.17 18It must be noted that the studies just mentioned deal with all hospital admissions and not simply those concerned with neurological disorders.

The costs of care in hospital are only one aspect of the cost to the nation. Innovations that have the potential for improving the use of resources include better discharge planning from hospital, improvement in the quality of nursing home care, more use of outpatient facilities, and the greater use of technological developments. The last includes the increased use of the telephone for consultations, the use of video technology for consultative purposes, and the telephonic transmission of images for expert interpretation elsewhere.

Prevention of disease and disability remains a most important matter. There have been recent important advances in genetics, counselling, and perinatal care. Some clinical problems, encountered in disabled people, may be susceptible to prevention—for example, falls and fractures, pressure sores, and contractures and deformities. All these topics are important in considering the economic aspects of neurological disorders.

Conclusion

The economic burden of neurological diseases falls on both individuals and the nation. The amount of high quality information available is small and this substantially hinders the planning of neurological services. The personal burden of neurological diseases for patients and their families can be identified but cannot easily be expressed in financial terms. There are major research opportunities in all of these fields.