Principles and Practice of Restorative Neurology: Butterworths International Medical Reviews

Switzerland

Series Preface

For almost a quarter of a century (1951–1975), subjects of topical interest were written about in the periodic volumes of our predecessor, Modern Trends in Neurology. Although both that series and its highly regarded editor, Dr Denis Williams, are no longer with us, the legacy continues in the present Butterworths series in Neurology. As was the case with Modern Trends, the current volumes are intended for use by physicians who grapple with the problems of neurological disorders on a daily basis, be they neurologists, neurologists in training, or those in related fields such as neurosurgery, internal medicine, psychiatry, and rehabilitation medicine.

Our purpose is to produce monographs on topics in clinical neurology in which progress through research has brought about new concepts of patient management. The subject of each monograph is selected by the Series Editors using two criteria: first, that there has been significant advance in knowledge in that area and, second, that such advances have been incorporated into new ways of managing patients with the disorders in question.

This has been the guiding spirit behind each volume, and we expect it to continue. In effect we emphasize research, both in the clinic and in the experimental laboratory, but principally to the extent that it changes our collective attitudes and practices in caring for those who are neurologically afflicted.

C.D. Marsden and A.K. Asbury

Series Editors

1

Introduction—why restorative neurology?

P.J. Delwaide and R.R. Young

Publisher Summary

This chapter discusses the importance of restorative neurology. Restorative neurology is a subspecialty of neurology, and it deals with techniques and strategies used to restore a disordered nervous system to a state of optimal function. Restorative neurology is characterized by a pathophysiological approach to nervous system disease and, as such, differs from neurosurgery, which is primarily an anatomical discipline, and from pharmacology, which relies essentially on neurochemistry. In addition to being multidisciplinary, restorative neurology is uniquely dependent upon the two endeavors. The first endeavor is quantitative evaluation of neurologic deficits, which is the cornerstone of restorative neurology. Proposed therapeutic efforts must prove their efficacy on an objective basis, and quantitative assessment is mandatory if one is to be able to compare results and outcomes. This goal is difficult to achieve because tools of assessment are in development and rarely incorporated into the routine practice of most neurology departments. The second essential endeavor is clinical neurophysiology. In addition to providing objective data to contribute to assessment, clinical neurophysiology also permits pathophysiological analyses of the consequences of nervous system lesions.

Neurologic impairments and disabilities constitute important medical and socio-economic problems. Paradoxically, treatment of chronic neurologic diseases has been considered frustrating and of little value. Neurology has been viewed as a medical specialty more concerned with diagnosis than with therapy.

This opinion should now be changed. In the last 20 years, indisputable progress has been made in the discovery of techniques which reduce patients’ disabilities and ameliorate their discomfort. However, to an extent unusual in other medical disciplines, these therapeutic modalities are not represented only by drugs but involve various procedures; surgical, psychological or rehabilitative in nature. Parkinson’s disease provides a good example of the way in which different useful techniques have evolved. In the 1960s, before the advent of dopamine precursors and agonists, patients benefited from stereotactic surgery. In the 1980s, the major problem was to define optimal strategies of pharmacologic treatment. The 1990s promise neural grafting although it will certainly not replace drug therapy and has yet to establish its proper place in overall management. New approaches have been developed for the management of patients with spinal cord injuries which aim to reduce the extent of their lesions and make their lives longer and more productive. In neurology, disability is not only the consequence of dysfunctions in cell metabolism but often results from disruptions of neuronal circuits; These disconnections trigger compensatory and adaptive mechanisms which, when understood, may be improved by drugs or by alternative strategies such as training or correction of imbalance between excitatory and inhibitory processes.

There are many new therapeutic techniques to complement well established ones but they may appear disparate and clinicians are faced with confusing choices, sometimes in competition with one another, to improve their patients’ function. Classically trained doctors may be reluctant to turn to new techniques but many clinicians are interested in the rationale, principles and achievements of these unusual or new procedures. They are anxious to identify those which are well-founded and derive directly from advances in the field of neuroscience and to discriminate them from other empirical ones which are advocated on the basis of anecdotal evidence. In addition, some clinicians are eager to learn about research techniques, such as neural grafting, which hold promise for the future.

The above mentioned considerations have promoted the development of this book, Principles and Practice of Restorative Neurology. Each term in the title deserves comment.

The principles describe the rationale behind available therapeutic techniques as well as future ones which will derive from actual achievements in experimental neurology. They indicate, from a theoretical point of view, the promises and limitations of transposition to human pathology of advances being made in animal laboratories. This intellectual approach, however, is not sufficient for clinicians who have to make decisions for individual patients, choose the best treatment and learn to apply it. Practice is thus a complementary aspect which derives from principles or sends investigators back to the laboratory to discover new principles. Practice refers to the well established procedures that have proved useful including how and when to apply them.

Although all of us would welcome critical and quantitative analyses of techniques currently employed in the practice of restorative neurology, such data, for the most part, do not exist. Collecting such data is an important aspect of the future of restorative neurology.

Restorative neurology is a subspecialty of neurology; it deals with techniques and strategies used to restore a disordered nervous system to a state of optimal function. Restorative neurology is characterized by a pathophysiological approach to nervous system disease and, as such, differs from neurosurgery which is primarily an anatomical discipline and from pharmacology which relies essentially on neurochemistry. However, restorative neurology tries to integrate these modalities of treatment into a comprehensive approach to the patient’s disability; it combines disparate disciplines, including new techniques derived from neurobiology, in a pragmatic attempt to improve neurologic functions. It is thus not surprising that restorative neurology encompasses a range of interests; some of these include pharmacology, plasticity, retraining, motivation, substitution, rehabilitation, functional surgery, neural grafting, and genetic engineering.

To be applied effectively, these various modalities of treatment require specialists so restorative neurology relies on a team of physicians, scientists and paramedical personnel assisting them. A coordinator experienced in many of the various aspects of care must define strategies of treatment and assess overall results. At this time, a neurologist seems best suited for that role.

In addition to being multidisciplinary, restorative neurology is uniquely dependent upon the following two endeavors. First is quantitative evaluation of neurologic deficits which is the cornerstone of restorative neurology. Proposed therapeutic efforts must prove their efficacy on an objective basis; quantitative assessment is mandatory if one is to be able to compare results and outcomes. This goal is difficult to achieve because tools of assessment are in development and rarely incorporated into the routine practice of most neurology departments. A continuous effort must be maintained to obtain reliable methods of assessment and, considering the interest raised in recent years by quantification of neurologic deficits, codification and generalization of these procedures which can be accepted worldwide may be expected. The second essential endeavor is clinical neurophysiology. In addition to providing objective data to contribute to assessment, clinical neurophysiology also permits pathophysiological analyses of the consequences of nervous system lesions. These results are useful to explain not only the general mechanisms involved in common syndromes such as spasticity but also to specify the unique functional particularities of individual patients. Clinical neurophysiology provides means to help understand defective function of the nervous system, develop strategies to correct abnormalities and assess the results. Finally, clinical neurophysiology has practical advantages because its equipment and techniques are widely available and not expensive; however, they are time-consuming.

One may, however, ask whether restorative neurology is a new field in neurology or is only a new name for an old practice, namely rehabilitation. Where does restorative neurology find its place and where are its borders with established medical specialities? We believe restorative neurology has a specific place in the treatment of individuals with neurologic disorders which can best be understood by considering schematically the sequence of events occurring immediately after a nervous system lesion. Similar considerations apply to patients following initial diagnosis of a chronic progressive disorder.

The first step is to make a correct diagnosis including a lesion’s localization and extent. If possible, adequate measures are taken immediately to halt progression of the lesion and suppress its effects. This phase is that of diagnosis and acute treatment and may require an Intensive Care Unit. If acute management does not succeed completely, the patient is left with a permanent lesion and following that, biological processes such as plasticity and compensatory mechanisms enter into play to rearrange the nervous system and restore function. A pathophysiological approach to the patient combined with good knowledge of neurobiology may at that stage help one to enhance the recovery process. This is the role of restorative neurology. Many complementary techniques, and new ones appear regularly, are put in place to limit or compensate for disability.

This part of the treatment is the responsibility of a neurologist who knows nervous system semeiology and has the background to understand future developments in neurobiology. Neurologists are accustomed to interpreting results of sophisticated procedures such as brain mapping, MRI and CT scans. The role of the neurologist is not to apply all the therapeutic techniques but to act instead as a coordinator, defining the best strategy and the timing for specific specialists, such as speech therapists, to intervene.

When the lesion has reached a stable state, rehabilitation and retraining may commence. This aspect is more concerned with protheses, occupational therapy and readaptation to society. The burden of socio-economic factors is particularly obvious at this stage and social workers assist the rehabilitation team to reintegrate the patient into society.

This sequence of medical care delivery is only a scheme; in many instances, one or two steps may be missing and multiple interactions and overlapping efforts are needed between the various phases. However, such a sequence corresponds to the evolution of a neural lesion and identifies individual responsibilities throughout the course of a neurologic disease. If not yet clearly or officially formulated, this opinion appears to be shared by an increasing number of clinicians.

This book is not intended to be a treatise on therapy for chronic neurological diseases. It aims, instead, to underline critically the convergence of distinct techniques in some selective exemplary areas of neurology. The editors are aware that it does not cover all the interesting aspects and apologize for that. For example, adult rather than pediatric neurologic disorders are emphasized and retraining of cortical functions after a stroke or a head injury is not discussed. These related aspects are well covered in other textbooks to which we recommend the reader (Ilis et al., 1982; Kaplan and Ceullo, 1985; Malony et al., 1985; Leek et al., 1986). We hope neurologists will find useful information here for understanding the rationale of standard as well as new therapeutic approaches and their application. However, our ultimate goal would be to stimulate young investigators to work on these problems to provide a firmer basis for an integrated therapy of neurological deficits.

References

Illis, LS, Sedgwick, EM, Glanville, HJRehabilitation of the Neurological Patient. Oxford: Blackwell Scientific, 1982.

Kaplan, PE, Ceullo, LSStroke Rehabilitation. Boston: Butterworths, 1985.

Leek, JC, Gershwin, ME, Fowler, WWPrinciples of Physical Medicine and Rehabilitation in Musculo-skeletal Diseases. Orlando: Straton, 1986.

Malony, FP, Burk, JS, Ringel, SPInterdisciplinary Rehabilitation of Multiple Sclerosis and Neuromuscular Disorders. Philadelphia: Lippincott, 1985.

2

Epidemiology of disability

D.L. McLellan

Publisher Summary

This chapter describes the epidemiology of disability. The relationship between disease and handicap is extremely complex. Many diseases or injuries cause impairments—that is, loss of a part or function. Some impairment lead to disability—that is, any restriction or lack, resulting from impairment, of ability to perform an activity in the manner or within the range considered normal for a human being. Most people with stroke, Parkinsonism, and multiple sclerosis have disability of some kind, whereas only one in every five people with arthritis is disabled at any point in time. There will be 30 times more people with disabilities caused by arthritis than there are with disabilities caused by multiple sclerosis because arthritis is much more common. Some indication of the propensity of different diseases to cause severe disability can be judged by comparing figures from disabled people living in private households with those living in communal establishments.

Discussion of this topic has become less complicated since the World Health Organization (WHO) formulated technical definitions for the terms disability, impairment, and handicap, which are described in the International Classification of Impairments, Disabilities, and Handicaps (WHO, 1980). The purpose for which epidemiological information is collected determines what information is sought (Wood and Badley, 1988).

From a health service perspective, knowing about the impairments and disabilities caused by different diseases helps in calculating the cost-effectiveness of their prevention and treatment. From the perspective of social services, or from the viewpoint of those responsible for housing and transport, the physical and social effects of disability are more immediately relevant than the impairments or the diseases (or trauma) that have caused them. Yet another perspective is that of the politician concerned to maximize the contribution to society by people with disadvantages. The effect of such disadvantages can often be reduced not by changing the people themselves, but by improving their physical environment, the resources available to them, and the willingness of the rest of society to recognize and encourage their contribution. These points are summarized in Table 2.1.

Table 2.1

Summary of criteria that determine the collection of information for epidemiological studies

The relationship between disease and handicap is extremely complex, even when acute and transient conditions are excluded from discussion. Many diseases or injuries cause impairments, that is loss of a part or function. Some impairments lead to disability, that is any restriction or lack, resulting from an impairment, of ability to perform an activity in the manner or within the range considered normal for a human being (WHO, 1980). For example, someone with severe arthritis of both hips has impairment of hip movement and of the capacity to bear weight, causing the disabilities of being unable to walk upstairs or for distances of more than 300 metres outside the house. These disabilities would not cause dependency in someone who lived in a bungalow next to a supermarket, but would do so for someone living in a small two-storey house in a rural location.

The wider dimension, which most closely approaches the experience of the disabled person, is captured by the term handicap. Handicap is the restriction of the social role that would otherwise be normal for that person, taking into account their personal attributes, age, resources, environment, and appropriate cultural factors. If the person with arthritis was unable to continue at work, play tennis, or maintain social contacts, there would be three handicaps, which could reduce the quality of life very significantly without necessarily leading to dependency.

A further degree of complexity that causes problems in surveys is the extraordinarily varied and unpredictable relationship between the operation of these factors in different individuals. It is often impossible to predict individual dependency from a list of disabilities, let alone from an inventory of their impairments or diseases. Dependency is crucially affected by the environment and resources of the disabled person. Handicap is even harder to predict because social attributes and objectives may be independent of impairment, but greatly constrained by the particular physical environment, and the attitudes and behaviour of other people.

For an individual, the cause or causes of impairment may themselves influence dependency and handicap. For example, multiple sclerosis may cause difficulty in walking, urgency of micturition, some impairment of judgement and decision making, and a tendency to unpredictable fatigue. These may seem modest impairments but they could translate in practice to daily incontinence (if the only toilet is upstairs), loss of employment (if the employer cannot accept unpredictable fluctuations in performance), and social isolation (if there is a breakdown in planning and consistency of making social contacts). Furthermore, this disabled person, his family and friends have to come to terms with the experience and prospect of a fluctuating and unpredictable decline in function over many years. The identity of the disease itself, multiple sclerosis in this example, has implications that contribute to the dependency and handicap experienced, which cannot be predicted by the impairments (or even the disabilities) alone.

People with disabilities have frequently expressed the view that advice provided through community services by people who are not familiar with their disease tends to miss the real target (Wood and Badley, 1988). It is equally clear that medical treatment given in the absence of any consideration of handicap may not only be wasteful but positively counter-productive.

These issues need to be kept clearly in mind when reading the data from surveys and considering their implications.

INSTRUMENTS FOR RECORDING IMPAIRMENT, DISABILITY, DEPENDENCY AND HANDICAP

One of the intentions of the WHO definitions (WHO, 1980) was to establish an agreed set of standards for the collection of data in the hope that all surveys would use them and therefore provide data that could be compared. However, this has not really succeeded, especially in relation to handicap where the necessary complexity of the inventories makes them impracticable for most surveys. By contrast, the concepts of impairment, disability, and handicap are now generally accepted and form part of most recent surveys of disability.

The most comprehensive survey yet undertaken in a developed country has been by the Office of Population Censuses and Surveys (OPCS) commissioned by the Department of Health and Social Security of the United Kingdom in 1984 (Green, 1988; Martin, Meltzer and Eliot, 1988; Martin and White, 1988; Bone and Meltzer, 1989; Martin and Meltzer, 1989; Meltzer, Smyth, and Robus, 1989; Smyth and Robus, 1989). The OPCS surveyed disabled people living in private households, disabled adults permanently resident in communal establishments, and ‘carers’, defined as people who are looking after, or providing some regular service for, a sick, handicapped, or elderly person, living in their own or another household.

For the survey of adults living in private households, a large sample of the general population (100 000 addresses) was screened to identify people with some form of disability. Subsequently, interviewers approached all disabled people under 60 years of age and half those 60 or more years of age. People with mental, intellectual, and sensory disabilities were surveyed as well as those with physical disabilities. Recognizing the hazards in attempting to predict overall disability from an inventory of impairments or disabilities, the survey team defined a set of scales of severity of disability. They are quite complex and the reader is referred to the illustrated cases given in the report (Martin, Meltzer, and Eliot, 1988).

The survey collected information about limitations in performing particular activities, but provided no direct measure of the overall, combined effect of all limitations. A criterion of overall severity was required in order to enable disabilities to be compared and to combine information about different disabilities into an overall measure. This was done by a panel of judges, which included professionals with expertise in disability, people carrying out research on disability including staff from the OPCS working on the surveys, people with disabilities, carers, and people from voluntary organizations concerned with disability. The panel performed a set of exercises designed to obtain an inventory of judgements in a systematic way. Scales were developed in 10 main areas of disability: locomotion, reaching and stretching, dexterity, sight, hearing, personal care, continence, communication, behaviour, and intellectual functioning.

The results showed that the average placement given by the panel could be predicted by taking into account only the three most severe disabilities, and considering only their level of severity, rather than which area of function they derive from. The three separate severity scores were then combined according to the