Emergent Treatment of Amyotrophic Lateral Sclerosis (ALS) 

  • Author: Kathleen Clem, MD, FACEP; Chief Editor: Rick Kulkarni, MD   more...
 
Updated: May 24, 2011
 

Overview

Amyotrophic lateral sclerosis (ALS), also known as Charcot's disease and Lou Gehrig's disease, is a disease of unknown cause characterized by slowly progressive degeneration of upper motor neurons (UMNs) and lower motor neurons (LMNs).[1]

The UMN findings include hyperreflexia and spasticity. They result from degeneration of the lateral corticospinal tracts in the spinal cord. The LMN findings include weakness, atrophy, and fasciculations. They are a direct consequence of muscle denervation via destruction of the anterior horn cell. ALS is eventually fatal because of respiratory muscle weakness. Aspiration pneumonia and medical complications of immobility contribute to morbidity.[2] Although ALS is incurable, there are treatments that can prolong meaningful quality of life; therefore, diagnosis is important to patients and families.

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Diagnosis

Emergency physicians should be familiar with amyotrophic lateral sclerosis (ALS) and should consider the diagnosis in patients with only motor syndromes.

The diagnosis of ALS is primarily clinical. Electrodiagnostic testing contributes to the diagnostic accuracy.

ALS can be differentiated from stroke or trauma due to the subacute or chronic progression of symptoms. When focal limb weakness occurs, ALS is differentiated from a root or peripheral nerve lesion by the lack of pain or sensory symptoms. While ALS is a slowly progressive disease, a precipitous event may occur to bring the patient to the emergency department (ED).

Bulbar symptoms

The patient's family first notices slurring of words or choking during a meal. An aspiration event or acute respiratory symptoms of air hunger occur.

Extremity weakness

The patient notices wrist drop interfering with his or her work performance. Or, the patient may find reduced finger dexterity, cramping, stiffness, and weakness or wasting of intrinsic hand muscles. The patient may develop foot drop resulting in a fall or sprain.

Fasciculations may present early on in the disease, particularly in the tongue.

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Emergency Department Care

Patients initially are cared for symptomatically, and emotional support should be available to the patient and family. Discussion of a new diagnosis of ALS in the ED is inappropriate before further evaluation occurs.

If a living will or a declaration for a natural death is in place, keep patients comfortable and do not intubate.

Complications, such as infections, deep vein thrombosis, or respiratory insufficiency, should be managed appropriately.

Further information on the diagnosis of and treatment for ALS is available from the European Federation of Neurological Societies.[3]

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Consultations

Refer patients to a neurologist experienced in the diagnosis and treatment of neuromuscular diseases.

Refer to a pulmonologist if respiratory failure is imminent and patient wishes allow.

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Inpatient Care

Physical and occupational therapy and speech pathology consultations assist the patient with amyotrophic lateral sclerosis (ALS) in maintaining strength, daily living activities, and communication skills (as demonstrated in the image below).

Patient with amyotrophic lateral sclerosis (ALS) uPatient with amyotrophic lateral sclerosis (ALS) using eye-gaze computer device to conduct business and communicate.

When forced vital capacity (FVC) reaches about 50% of predicted levels, supplemental respiratory support may be needed. (The first symptom may be disturbed sleep.) Use of noninvasive positive pressure ventilation improves quality of life; further respiratory deterioration may necessitate tracheostomy and ventilator support.

Patients with progressive weight loss, symptomatic dysphagia, or aspiration should be considered for percutaneous gastrostomy placement. Risk of percutaneous endoscopic gastrostomy (PEG) placement increases with declining respiratory function. PEG is best placed before FVC falls to less than 50% of predicted levels.

Advance directives may be addressed by the patient's neurologist and be discussed at regular intervals. Hospice referral may be appropriate to aid in care during the terminal phase of the disease.

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Contributor Information and Disclosures
Author

Kathleen Clem, MD, FACEP  Chair, Department of Emergency Medicine, Loma Linda University Medical Center

Kathleen Clem, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Justin T Mhoon, MD  Neuromuscular Medicine Fellow, Department of Neurology, Duke University Medical Center

Justin T Mhoon, MD is a member of the following medical societies: American Academy of Neurology and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Joel C Morgenlander, MD  Professor, Division of Neurology, Duke University School of Medicine; Clinical Chief of Service, Duke Neurology

Joel C Morgenlander, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association, and North Carolina Neurological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Roy Alson, MD, PhD, FACEP, FAAEM  Associate Professor, Department of Emergency Medicine, Wake Forest University School of Medicine; Medical Director, Forsyth County EMS; Deputy Medical Advisor, North Carolina Office of EMS; Associate Medical Director, North Carolina Baptist AirCare

Roy Alson, MD, PhD, FACEP, FAAEM is a member of the following medical societies: Air Medical Physician Association, American College of Emergency Physicians, American Medical Association, National Association of EMS Physicians, North Carolina Medical Society, North Carolina Medical Society, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: eMedicine Salary Employment

J Stephen Huff, MD  Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD 

Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: WebMD Salary Employment

References
  1. Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet J Rare Dis. Feb 3 2009;4:3. [Medline]. [Full Text].

  2. Spataro R, Lo Re M, Piccoli T, Piccoli F, La Bella V. Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand. Sep 2010;122(3):217-23. [Medline].

  3. [Guideline] Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, et al. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol. Dec 2005;12(12):921-38. [Medline]. [Full Text].

  4. [Guideline] Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. Oct 13 2009;73(15):1218-26. [Medline]. [Full Text].

  5. [Guideline] Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. Oct 13 2009;73(15):1227-33. [Medline]. [Full Text].

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Patient with amyotrophic lateral sclerosis (ALS) using eye-gaze computer device to conduct business and communicate.
 
 
 
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