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Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Progressive, incurable disease of adults
  • Neurodegenerative disease of the motor system at all levels
  • Some patients have associated dementia
  • Manifestations:
    • Muscle weakness
    • Wasting
    • Fasciculations
    • Babinski sign
    • Hyperreflexia
  • Variants with predominately upper or lower motor neuron manifestations also occur
  • May begin with bulbar symptoms of dysphagia and dysarthria
  • Also known as “Lou Gehrig Disease” after the famous baseball player who was affected
  • Eventually leads to respiratory compromise secondary to weakness of diaphragm and other muscles of respiration
  • 80% of cases begin between ages 40 and 70 yr
  • Death (usually from respiratory paralysis) typically occurs within 3–5 yr of the diagnosis
  • 50% die within 3 yr
  • ∼10% ALS patients live 10 yr or more
  • Males > females

Etiology

  • Etiology of amyotrophic lateral sclerosis (ALS) is unknown
  • ∼10% of affected patients have another affected family member
  • Cigarette smoking and heavy metal exposure may be risk factors
  • There is a disease cluster in the western Pacific
  • Pathologically, there is loss of both upper and lower motor neuron cells
  • Predilection for the motor system and sparing of other neurons

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Citation

Rosen, Peter, et al., editors. "Amyotrophic Lateral Sclerosis." 5-Minute Emergency Consult, 5th ed., Lippincott Williams & Wilkins, 2016. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis.
Amyotrophic Lateral Sclerosis. In: Rosen P, Shayne P, Barkin AZ, et al, eds. 5-Minute Emergency Consult. 5th ed. Lippincott Williams & Wilkins; 2016. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis. Accessed April 24, 2019.
Amyotrophic Lateral Sclerosis. (2016). In Rosen, P., Shayne, P., Barkin, A. Z., Wolfe, R. E., Hayden, S. R., Barkin, R. M., & Schaider, J. J. (Eds.), 5-Minute Emergency Consult. Available from https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis
Amyotrophic Lateral Sclerosis [Internet]. In: Rosen P, Shayne P, Barkin AZ, Wolfe RE, Hayden SR, Barkin RM, Schaider JJ, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2016. [cited 2019 April 24]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Amyotrophic Lateral Sclerosis ID - 307487 ED - Rosen,Peter, ED - Shayne,Philip, ED - Barkin,Adam Z, ED - Wolfe,Richard E, ED - Hayden,Stephen R, ED - Barkin,Roger M, ED - Schaider,Jeffrey J, BT - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis PB - Lippincott Williams & Wilkins ET - 5 DB - Emergency Central DP - Unbound Medicine ER -