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.
Goals of Treatment
Arresting an Attack
Many neurological diseases cause episodic attacks. These include strokes, migraine, MS, epilepsy, paroxysmal dyskinesias, and periodic paralyses, and in some of these diseases, treatment may prevent or halt the attacks. Although it does not cure the underlying disease, aborting the attacks is of great help to the patient. Triptan-class drugs generally arrest a migraine, and valproate, a beta-blocker, or a calcium channel blocker will reduce the frequency of the attacks (see Chapter 69). Status epilepticus usually can be arrested by intravenous antiepileptic drugs, and the frequency of epileptic attacks can be reduced by the use of chronic oral anticonvulsant drugs (see Chapter 67). Intravenous and intraarterial thrombolytics may terminate and potentially reverse an otherwise disastrous “brain attack” (cerebral ischemia) (see Chapter 51A).
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