Management of Neurological Disease

Principles of Neurological Management

Once a neurological diagnosis has been established, it is necessary to develop a management plan. In addition to disease-specific treatments, steps must be taken to provide support for the patient, family, and caregivers. Communicating bad news should be done with compassion, with a goal to offer as much hope as realistically possible (discussed further in Chapter 114). If a genetic diagnosis is made, genetic counseling should be offered to support the patient and also to address the potential for disease occurrence in other family members. A detailed discussion of genetic diagnosis and management is provided in Chapter 48 .

At present, many neurological diseases are “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 51.1 ).

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. 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 is not only 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 51.1 . In fact, it is usually 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 the 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

No treatment should be given to a patient without a good rationale. Although there is growing emphasis on evidence-based medicine, it should be acknowledged that this approach has some limitations.

Subjects selected for double-blind placebo-controlled studies must meet criteria strictly defined by inclusion/exclusion, 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 demographic 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 may often 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 has taken place that efficacy and adverse effects are 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 relevant 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.

Goals of Treatment

In defining the goals of treatment, it is important to separate neurological impairment from disability. Neurological impairment (the presence of abnormal neurological signs) allows a diagnosis to be made. Impairment may cause disability. For instance, a stroke may cause a hemiplegia, which is the impairment. The hemiplegia may cause difficulty in walking, which is the disability. The patient may be concerned about the abnormal neurological signs but to a greater degree wants correction of the disability. It may not be possible to correct the underlying stroke lesion or reverse the hemiparesis or other neurological deficits, but symptomatic treatment such as providing physical therapy, a walker, and a wheelchair can mitigate these disabilities. The functional state of a stroke patient who has benefitted from neurological rehabilitation may be gratifying as 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 and are likely to die within 3 years; therefore, 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 is not only uncaring but also leaves the patient without hope and without the symptomatic treatment that could help the patient to circumvent the disabilities attending 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 will often have found these by searching the internet, but the physician should keep available the addresses and contact information of key organizations to give those who need them.

Symptomatic treatment depends on the nature of the disease. It can consist of arresting an attack of a disease such as multiple sclerosis (MS); circumventing the effects of the disease, as with antispasticity medications; or end-of-life care for a patient approaching death. This last is sometimes 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 individual patient.