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

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TY - ELEC T1 - Amyotrophic Lateral Sclerosis ID - 307487 Y1 - 2016 PB - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307487/all/Amyotrophic_Lateral_Sclerosis ER -