Amyotrophic Lateral Sclerosis

Matthew A. Rushing, MEd, MD Reviewed 05/2023

BASICS

A progressive neurodegenerative disease characterized by muscle weakness and wasting induced from a loss of upper and lower motor neurons in the motor cortex, the brain stem nuclei, and the ant...

DIAGNOSIS

Diagnosis can be established according to revised El Escorial World Federation of Neurology criteria (1):

The presence of:

Evidence of LMN degeneration by clinical, electrophysiologic, or n...

TREATMENT

GENERAL MEASURES

Multidisciplinary care may extend survival, decrease medical complications, and improve quality of life.
Complete Advanced Directives and clarify code status.
Discuss the futur...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Serial pulmonary function tests (PFT) or a maximal sniff nasal inspiratory force (SNIF) are usually performed every 3 months to monitor progress...

REFERENCES

Siddique N, Siddique T. Amyotrophic lateral sclerosis overview. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle...

CODES

ICD10

G12.21 Amyotrophic lateral sclerosis

SNOMED

86044005 Amyotrophic lateral sclerosis
718555006 Juvenile amyotrophic lateral sclerosis
230258005 Amyotrophic lateral sclerosis with dementia
72298700...

CLINICAL PEARLS

ALS: progressive degeneration of upper and lower motor neurons.
Asymmetric limb weakness is the most common presentation.
Diagnosis is based upon clinical criteria, progressive features,...

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