Amyotrophic Lateral Sclerosis

Ajoy Kumar, MD, FAAFP, Nazrah C. Franco, MD and Dylan H. Zini, MD Reviewed 06/2017
 


BASICS

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the brain and spinal cord. Characterized by loss of upper and lower motor neurons (LMNs), it is relentless in p...

DIAGNOSIS

  • Diagnosis can be established according to Revised El Escorial World Federation of Neurology criteria.

  • The presence of

    • Evidence of LMN degeneration by clinical, electrophysiologic, or neuropa...

TREATMENT

GENERAL MEASURES

  • Multidisciplinary care should be available as this may extend survival, decrease medical complications, and improve quality of life (4)[C].

  • Outpatient may need nursing home pl...

ONGOING CARE

FOLLOWUP RECOMMENDATIONS

Patients should be involved in regular exercise and a physical therapy program (8)[C]. 

MONITORING

  • Initially every 3 months; frequency to be increased as needed for ...

REFERENCES

Mehta P, Antao V, Kaye W et al. Prevalence of amyotrophic lateral sclerosis—United States, 2010–2011. MMWR Surveill Summ.  2014;63(Suppl 7):1–14. [View Abstract on OvidInsights]
Ander...

ADDITIONAL READING

Morren JA, Galvez-Jimenez N. Current and prospective disease-modifying therapies for amyotrophic lateral sclerosis. Expert Opin Investig Drugs.  2012;21(3):297–320. [View Abstr...

CODES

ICD10

  • G12.21 Amyotrophic lateral sclerosis

  • G12.22 Progressive bulbar palsy

  • G12.29 Other motor neuron disease

ICD9

  • 335.20 Amyotrophic lateral sclerosis

  • 335.22 Progressive bulbar palsy

  • 335.23 Pseudobulba...

PEARLS

  • ALS is a progressive upper and LMN degenerative disease.

  • Diagnosis is made by history, physical exam, EMG, and nerve conduction study.

  • Riluzole is the only available treatment that might increase...

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