Management of Neurological Disease

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Chapter 43 Management of Neurological Disease

How an experienced neurologist uses the history of the patient’s illness, the neurological examination, and investigations to diagnose neurological disease is discussed in Chapters 1 and 31. This chapter presents some general principles guiding the management of neurological disease. Chapters 44 to 48 cover individual areas of neurological management such as pain management, neuropharmacology, intensive care, neurosurgery, and neurological rehabilitation. Details about the management of specific neurological diseases are presented in Chapter 49, Chapter 50, Chapter 51, Chapter 52, Chapter 53, Chapter 54, Chapter 55, Chapter 56, Chapter 57, Chapter 58, Chapter 59, Chapter 60, Chapter 61, Chapter 62, Chapter 63, Chapter 64, Chapter 65, Chapter 66, Chapter 67, Chapter 68, Chapter 69, Chapter 70, Chapter 71, Chapter 72, Chapter 73, Chapter 74, Chapter 75, Chapter 76, Chapter 77, Chapter 78, Chapter 79, Chapter 80, Chapter 81, Chapter 82 . Many aspects of management are common to all neurological disorders; these management considerations are the subject of this chapter.

Principles of Neurological Management

As in all medical disciplines, many neurological diseases are, at present, “incurable.” This does not mean, however, that such diseases are not treatable and that nothing can be done to help the patient. Help that can be provided short of curing the disease ranges from treating the symptoms, to providing support for the patient and family, to end-of-life care (Box 43.1). Healthcare professionals are so committed to the scientific understanding of diseases and their treatment that the natural tendency of the clinician is to feel guilty when confronted with a patient with an incurable disease. The number of neurological diseases that are curable or arrestable is constantly expanding thanks to research.

Unfortunately, a physician who is fixated on the need to cure disease may simply strive to make the diagnosis of an as-yet incurable disease and then give no thought to patient management. Such a physician will tell the patient that he or she has an incurable disease, so coming back for further appointments is pointless (“diagnose and adios”). The aphorism “To cure sometimes, to relieve often, to comfort always” originated in the 1800s with Dr. Edward Trudeau, founder of a tuberculosis sanatorium. Any other attitude not only is an abrogation of the physician’s responsibility to care for the patient, but also leaves the patient without the many modalities of assistance that can be provided even to those with incurable diseases. The neurologist who accepts the responsibility for treating the patient will review with the patient and family all the issues listed in Box 43.1. In fact, it usually is necessary to spend more time with the patient with an incurable disease than with one for whom effective treatment is available. In addition to providing all practical help available, the compassionate neurologist should share the grief and provide consolation for the patient and family; both are essential aspects of patient management.

Evidence-Based Medicine in Neurology

Recent emphasis on evidence-based medicine is appropriate. No treatment should be given to a patient without a good rationale. The scientific management of disease has always involved using all of the information available in the literature, so evidence-based medicine is not new. Although considered to be the standard method of analysis of benefit, double-blind placebo-controlled studies have some serious limitations. Subjects selected for these studies often are strictly defined by inclusion/exclusion criteria, and they may not represent the population for whom the treatment will eventually be prescribed. Such patients, for example, may not necessarily have exactly the same demographics or clinical characteristics as those of the well-defined study population, and they may be taking other medications that could affect the response. For these and other reasons, the findings from controlled trials often may not be generalizable. Furthermore, most double-blind placebo-controlled drug trials are relatively short-term studies, and it is not until a long-term open-label trial that efficacy and adverse effects become better understood. Moreover, the cumulative experience of a seasoned physician whose clinical judgment relies not only on the published evidence-based literature but also on personal and often empirical experience can be of great importance in the management of a specific patient. It would be wrong if this resource were to be disregarded in areas where the literature is not definitive or available. Absence of evidence (usually because the appropriate studies have not yet been done or published) does not mean that support for a specific intervention or application is lacking. This applies at least as much to neurology as it does to other disciplines.

Goals of Treatment

In defining the goals of treatment, it is important to separate neurological impairment, disability, and handicap. Neurological impairment (presence of abnormal neurological signs) allows a diagnosis to be made. Impairment may cause disability, which in turn produces a handicap. For instance, a stroke may cause a hemiplegia, which is the impairment. The hemiplegia may cause difficulty in walking, which is the disability. The difficulty in walking may make it impossible for the patient to leave the house, which is the handicap. The patient does not care about the abnormal neurological signs but wants correction of the disability and relief from the handicap. It may not be possible to correct the underlying stroke lesion or reverse the hemiparesis, but symptomatic treatment such as providing physical therapy, a walker, and a wheelchair can help alleviate the handicap. Improvement in the functional state of a stroke patient resulting from neurological rehabilitation is gratifying when compared with the state of untreated patients.

Amyotrophic lateral sclerosis (ALS) is perhaps the disease that epitomizes the role of symptomatic care. Patients with ALS often report being told by their doctor that they have ALS, they are likely to die within 3 years, and because nothing can be done for them, they should go home, put their affairs in order, and prepare to die. A doctor who dispenses such advice not only is uncaring but also leaves the patient without hope and the symptomatic treatment that can help circumvent the disabilities and handicaps that attend the disease. The psychological support of a caring neurologist who is familiar with the disease can be of great help to the patient and family. An increasing number of lay organizations and support groups are available to provide information and services. Patients often will have found these by searching the Internet, but the physician should keep available the addresses and contact information of key organizations to give to patients.

Symptomatic treatment depends on the nature of the disease. It can consist of arresting an attack in a disease such as multiple sclerosis (MS); circumventing the effects of the disease, such as with antispasticity medications; or end-of-life care for a patient approaching death. The latter sometimes is called palliative care, but in fact every treatment short of cure, even in the early stages of a disease, is palliative. There is no “cookbook” approach to the management of any neurological disorder; therapy must be individualized, and the selection of the therapeutic strategy must be guided by the specific impairment and tailored to the needs of the patient.

Slowing Disease Progression

Examples of treatments that slow the progress of neurological disease are numerous. A malignant cerebral glioma is almost universally fatal, but high-dose corticosteroids, neurosurgical debulking, radiotherapy, and chemotherapy may slow tumor growth and prolong survival (see Chapters 52E and 52F). The β-interferons, glatiramer, natalizumab, or mitoxantrone may reduce relapses and slow the progress of MS (see Chapter 54). Liver transplantation in familial amyloid polyneuropathy may slow or arrest disease progression (see Chapter 76). Riluzole may slow the progress of ALS (see Chapter 74). Despite many efforts to slow the progression of Parkinson disease (PD), no neuroprotective therapy has proved to be effective, although certain monoamine oxidase B inhibitors and dopamine agonists delay the onset of levodopa-related motor complications.

Relieving Symptoms

Symptomatic treatment is available for many neurological diseases. Relief of pain, although not curative, is the most important duty of the physician and can be accomplished in many ways (see Chapter 44). Baclofen and tizanidine can reduce spasticity, particularly in spinal cord disease, without affecting the disorder causing it. Injections of botulinum toxin provide marked relief in patients with dystonia, spasticity, and other disorders manifested by abnormal muscle contractions. High-dose corticosteroid therapy reduces the edema surrounding a brain tumor, temporarily relieving headache and neurological deficits without necessarily affecting tumor growth. In PD, dopaminergic drugs partly or completely relieve symptoms for a period, without affecting the progressive degeneration of substantia nigra neurons (see Chapter 71

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