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Amyotrophic Lateral Sclerosis

Ajoy Kumar, MD, FAAFP, Kellen H. Gower, MD and Andres E. Santayana, MD Reviewed 06/2019
 


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

  • Insidious onset of symptoms may result in delay in definitive diagnosis, with median time to diagnosis about 14 months.

  • Diagnosis can be established according to revised El Escorial World Fed...

TREATMENT

GENERAL MEASURES

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

  • Treatment at...

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

1
Genetics Home Reference. Amyotrophic Lateral Sclerosis. Bethesda, MD: U.S. National Library of Medicine, National Institutes of Health; 2018. https://ghr.nlm.nih.gov/condition/amyotrophic-...

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 and lower degenerative disease

  • Diagnosis: history, PE, EMG, and NCS

  • Riluzole and edaravone are the current treatments.

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

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