Symptom-based management of amyotrophic lateral sclerosis
- Nestor Galvez-Jimenez, MD, MSc, MHSA, FACP
Nestor Galvez-Jimenez, MD, MSc, MHSA, FACP
- The Pauline M. Braathen Endowed Chair
- Chairman, Department of Neurology
- Cleveland Clinic Florida
- Clinical Professor of Neurology
- Herbert Wertheim College of Medicine
- Section Editors
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- R Sean Morrison, MD
R Sean Morrison, MD
- Section Editor — Selected End Stage Conditions
- Hermann Merkin Professor of Palliative Care
- Mount Sinai School of Medicine
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually death, with a median survival of three to five years. Symptomatic management is the mainstay of treatment for ALS, and is reviewed in this topic. Other aspects of ALS are discussed separately. (See "Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease" and "Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease" and "Disease modifying treatment of amyotrophic lateral sclerosis".)
ALS is a progressive neurodegenerative disorder. Most ALS patients die within three to five years of diagnosis. However, longer survival is not rare. About 30 percent of ALS patients are alive five years after diagnosis, and 10 to 20 percent survive for greater than 10 years. Long-term survival is associated with a younger age at symptom onset, male gender, and limb rather than bulbar symptom onset [1-3].
BREAKING THE NEWS
Informing the patient and family of the diagnosis of ALS is a daunting task for the clinician. The diagnosis should always be given face-to-face, with enough time to ensure no rushing [4,5]. It may be helpful to have the patient's support network present; European guidelines note a perceived advantage if the patient’s network outnumbers the medical staff present at the meeting . It is important to give a warning that bad news is coming and to acknowledge and explore the patient's reaction. Allow for questions, give reassurance that the complications of ALS are treatable, and give reassurance that the patient will not be abandoned. Offer a second opinion if the patient wishes. It is often useful to schedule a return visit within a short time frame so that questions can be addressed and family members can attend if desired. (See "Communication of prognosis in palliative care".)
Many patients have a delay in diagnosis that is very distressing. For these patients, a diagnosis may be good news, in that uncertainty is reduced, and active therapy can be initiated. The discussion of diagnosis should always be accompanied by a discussion of the ways that symptoms can be treated, as well as by institution of disease modifying treatment, assuming cost is not prohibitive. (See "Disease modifying treatment of amyotrophic lateral sclerosis", section on 'Riluzole'.)
Multidisciplinary ALS clinics provide care from neurologists, physical therapists, occupational therapists, speech therapists, respiratory therapists, dietitians, social workers, and nursing care managers [5-9]. Thus, specialized clinics can guide the management of the complex issues related to ALS, which include respiratory symptoms, nutrition, dysarthria, dysphagia, functional decline, and psychosocial problems.
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- BREAKING THE NEWS
- MULTIDISCIPLINARY CARE
- RESPIRATORY MANAGEMENT
- Pulmonary tests
- Noninvasive positive pressure ventilation
- Invasive ventilation
- DYSPHAGIA AND NUTRITION
- SYMPTOM MANAGEMENT
- Dysarthria and communication
- Muscle spasms
- Muscle weakness and functional decline
- Thick mucus
- Pseudobulbar affect
- Psychosocial aspects
- Sleep problems
- HOSPICE CARE
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS