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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