Amyotrophic Lateral Sclerosis

Ajoy Kumar, MD, FAAFP and Justin Thompson, MD Reviewed 06/2018
 


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 (UMNs/LMNs), its progression ...

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 neuropath...

TREATMENT

GENERAL MEASURES

  • Treatment is palliative. Multidisciplinary care should be available as this may extend survival, decrease medical complications, and improve quality of life.

  • Outpatient may ne...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients should be involved in regular exercise and a physical therapy program. 

Patient Monitoring

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

REFERENCES

Genetics Home Reference. Amyotrophic Lateral Sclerosis. Bethesda, MD: U.S. National Library of Medicine, National Institutes of Health; 2018. https://ghr.nlm.gov/condition/amyotrophic-late...

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...

CLINICAL PEARLS

ALS: progressive upper & lower degenerative disease. 
  • Diagnosis: history, PE, EMG, and NCS.

  • Riluzole and edaravone are the current treatments.

  • Survival from diagnosis to 10 years is ~...

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