eMedicine Specialties > Emergency Medicine > Neurology

Amyotrophic Lateral Sclerosis: Treatment & Medication

Author: Kathleen Clem, MD, FACEP, Chair, Department of Emergency Medicine, Loma Linda University Medical Center
Coauthor(s): Joel C Morgenlander, MD, Professor, Division of Neurology, Duke University School of Medicine; Consulting Staff, Electromyography Laboratory, Muscular Dystrophy Association Clinic, Duke University Medical Center
Contributor Information and Disclosures

Updated: Sep 15, 2009

Treatment

Emergency Department Care

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

  • 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 diagnosis and treatment for patients with ALS is available from the European Federation of Neurological Societies.5

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.

Medication

Treatment of pneumonia or urosepsis initially involves empiric use of a relatively broad-spectrum antibiotic or antibiotics effective against probable pathogens, after appropriate cultures and specimens for laboratory evaluation have been obtained. These medications may include cephalosporins, fluoroquinolones, vancomycin, penicillins, and aminoglycosides.

Amyotrophic Lateral Sclerosis Agent

These agents may inhibit the release of neurotransmitters.


Riluzole (Rilutek)

Only current disease-specific medication approved by US Food and Drug Administration for use in patients with ALS. Should only be prescribed by physician familiar with inclusion/exclusion criteria and who will be monitoring patient. Should not be prescribed in ED acutely. Blocks glutamatergic neurotransmission in CNS. Actions appear to be indirect. May activate guanosine triphosphate-binding signal transduction proteins (G-proteins) with resultant inhibition of neurotransmitter release. Glutamic acid is major excitatory neurotransmitter in CNS. Accumulation at synapses triggers excessive stimulation of excitatory amino acid receptors on postsynaptic cell with subsequent neuronal death (postulated pathogenesis of ALS).

Adult

50 mg PO q12h; no benefit can be expected from higher qd doses, but adverse events are increased

Pediatric

ALS not seen in pediatric patients

Coadministration with drugs that inhibit CYP450 1A2 (eg, caffeine, theophylline, amitriptyline, quinolones) may decrease rate of elimination of riluzole, increasing toxicity; inducers of CYP450 1A2 (eg, phenytoin, rifampin, omeprazole, cigarette smoke) may decrease blood levels by increasing rate of elimination

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in abnormal liver function; monitor liver enzymes closely; worsening of asthenia may occur

Antispasticity agents

Treatment of spasticity is not specific for ALS.


Baclofen (Lioresal)

Treats spastic muscles. May induce hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic reflexes at the spinal level.

Adult

5 mg PO tid initially, followed by gradual increase of 5 mg/d q4-7d to therapeutic level (0.08-0.4 mcg/mL); not to exceed 80 mg/d divided qid

Pediatric

ALS not seen in pediatric patients

Opiate analgesics, benzodiazepines, alcohol, tricyclic antidepressants, guanabenz, MAOIs, clindamycin, and hypertensive agents may increase effects

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in patients with history of autonomic dysreflexia and when spasticity is used to obtain increased function; autonomic dysreflexia can result from withdrawal of this medication


Tizanidine (Zanaflex)

Treats spastic muscles. Centrally acting muscle relaxant. Possesses alpha2-adrenergic agonist properties. Metabolized in liver and excreted in urine and feces.

Adult

4-8 mg PO q8h prn; not to exceed 36 mg/d

Pediatric

ALS not seen in pediatric patients

May interact with alcohol (increasing somnolence, stupor) and PO contraceptives (which decrease clearance); can cause increased hypotensive effects when administered concurrently with diuretics

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in renal impairment

Cholinergic agents

For patients with ALS who may have secondarily impaired neuromuscular junction transmission.


Pyridostigmine (Mestinon, Regonol)

Acts in smooth muscle, CNS, and secretory glands, where it blocks action of acetylcholine at parasympathetic sites and facilitates transmission of impulses across myoneural junction.

Adult

Initial: 60 mg PO tid followed by a maintenance dose of 60 mg/d to 1.5 g/d, or 2 mg IV/IM q2-3h (or 1/30th the PO dose)

Pediatric

ALS not seen in pediatric patients

Increases effects of depolarizing neuromuscular blockers; increases toxicity of edrophonium

Documented hypersensitivity; GI or GU obstruction

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in bronchial asthma and those receiving a cardiac glycoside; overdose may cause cholinergic crisis, which may be fatal; atropine IV should be readily available for treatment of cholinergic reactions

More on Amyotrophic Lateral Sclerosis

Overview: Amyotrophic Lateral Sclerosis
Differential Diagnoses & Workup: Amyotrophic Lateral Sclerosis
Treatment & Medication: Amyotrophic Lateral Sclerosis
Follow-up: Amyotrophic Lateral Sclerosis
Multimedia: Amyotrophic Lateral Sclerosis
References
Further Reading
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References

  1. Kimura F, Fujimura C, Ishida S, et al. Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology. Jan 24 2006;66(2):265-7. [Medline].

  2. Murphy J, Henry R, Lomen-Hoerth C. Establishing subtypes of the continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol. Mar 2007;64(3):330-4. [Medline].

  3. Sabel CE, Boyle PJ, Loytonen M, et al. Spatial clustering of amyotrophic lateral sclerosis in Finland at place of birth and place of death. Am J Epidemiol. May 15 2003;157(10):898-905. [Medline].

  4. Hudson AJ. The motor neuron diseases and related disorders. In: Clinical Neurology. Vol. 4. 1996:11-14.

  5. [Guideline] Andersen PM, Borasio GD, Dengler R, 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].

  6. Lo Coco D, Marchese S, Pesco MC, La Bella V, Piccoli F, Lo Coco A. Noninvasive positive-pressure ventilation in ALS: predictors of tolerance and survival. Neurology. Sep 12 2006;67(5):761-5. [Medline].

  7. Abramowitz M. Riluzole for amyotrophic lateral sclerosis. Med Lett Drugs Ther. 1995;37.

  8. Carter GT, Miller RG. Comprehensive management of amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am. Feb 1998;9(1):271-84, viii-ix. [Medline].

  9. Eisen A, Schulzer M, MacNeil M, Pant B, Mak E. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve. Jan 1993;16(1):27-32. [Medline].

  10. Goldblatt D. Caring for patients with amyotrophic lateral sclerosis. In: Smith RA, ed. Handbook of Amyotrophic Lateral Sclerosis. Marcel Dekker; 1992.

  11. Gourie-Devi M, Nalini A, Subbakrishna DK. Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis. J Neurol Sci. Sep 10 1997;150(2):167-72. [Medline].

  12. Gubbay SS, Kahana E, Zilber N, Cooper G, Pintov S, Leibowitz Y. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol. 1985;232(5):295-300. [Medline].

  13. Househam E, Swash M. Diagnostic delay in amyotrophic lateral sclerosis: what scope for improvement?. J Neurol Sci. Nov 1 2000;180(1-2):76-81. [Medline].

  14. Ince PG, Lowe J, Shaw PJ. Amyotrophic lateral sclerosis: current issues in classification, pathogenesis and molecular pathology. Neuropathol Appl Neurobiol. Apr 1998;24(2):104-17. [Medline].

  15. Jablecki CK, Berry C, Leach J. Survival prediction in amyotrophic lateral sclerosis. Muscle Nerve. Oct 1989;12(10):833-41. [Medline].

  16. Kaufmann P, Pullman SL, Shungu DC, et al. Objective tests for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS). Neurology. May 25 2004;62(10):1753-7. [Medline].

  17. [Guideline] Miller RG, Rosenberg JA, Gelinas DF, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology. Apr 22 1999;52(7):1311-23. [Medline].

  18. Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. Aug 23 2005;65(4):586-90. [Medline].

  19. Rippon GA, Scarmeas N, Gordon PH, Murphy PL, Albert SM, Mitsumoto H. An observational study of cognitive impairment in amyotrophic lateral sclerosis. Arch Neurol. Mar 2006;63(3):345-52. [Medline].

  20. [Best Evidence] Rowland LP. Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. Oct 1998;160 Suppl 1:S6-24. [Medline].

  21. Rowland LP. What's in a name? Amyotrophic lateral sclerosis, motor neuron disease, and allelic heterogeneity. Ann Neurol. Jun 1998;43(6):691-4. [Medline].

  22. Shefner JM. Amyotrophic lateral sclerosis. In: Gilchrist JM, ed. Prognosis in Neurology. Boston: Butterworth-Heinemann; 1998:135-138.

  23. Tandan R. Disorders of the upper and lower motor neurons. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds. Neurology in Clinical Practice. Boston: Butterworth-Heinemann; 1996:1823-1852.

  24. Tandan R, Bradley WG. Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol. Sep 1985;18(3):271-80. [Medline].

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Keywords

ALS, Lou Gehrig disease, Lou Gehrig's disease, amyotrophic lateral sclerosis, Charcot disease, Charcot's disease, motor neuron 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)

Joel C Morgenlander, MD, Professor, Division of Neurology, Duke University School of Medicine; Consulting Staff, Electromyography Laboratory, Muscular Dystrophy Association Clinic, Duke University Medical Center
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.

Medical Editor

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: American College of Emergency Physicians, American Medical Association, National Association of EMS Physicians, North Carolina Medical Society, Society for Academic Emergency Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

J Stephen Huff, MD, Associate Professor, Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health Sciences Center
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.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
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

 
 
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