Updated: 2/1/2020

Amyotrophic Lateral Sclerosis (ALS)

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  • TongueA 61-year-old male presents with left-sided hand weakness and trouble with walking. He is not sure why these symptoms occur. On physical exam, tongue fasciculations are appreciated. He has slow speech. The left upper extremity shows forearm atrophy and depressed reflexes. The right lower extremity is hypertonic, with 3+ reflexes, and positive Babinski sign.
Introduction
  • A progressive neurodegenerative disease affecting both upper and lower motor neurons 
    • there are sporadic (~90% of cases) and familial causes
    • sensation is intact
    • bowel and bladder are spared
  • Certain cases may be due to superoxide dismutase type 1 (SOD-1) mutations
    • results in the generation of free radicals, eventually leading to cell death
  • Epidemiology
    • age of onset:
      • ~60 years of age in sporadic cases
      • ~50 years of age in familial cases
Presentation
  • Symptoms
    • progressive weakness affecting any segment of the body
      • asymmetric limb weakness (most common)
      • upper and lower motor neuron signs
        • upper motor neuron (UMN) signs
          • spasticity or stiffness
          • hyperreflexia
          • dysarthria
          • dysphagia
        • lower motor neuron (LMN) signs
          • atrophy
          • fasciculations
          • hyporeflexia
          • weakness
Evaluation
  • LMN signs determined by physical exam, electrophysiologic, or neuropathologic studies
  • UMN signs determined by physical exam
  • Symptom progression spread or signs
  • No other disease process can explain the patient's findings
  • EMG shows widespread denervation and fibrillation potential
Differential
  • Benign fasciculations
  • Spinal muscular atrophy
  • Poliomyelitis
  • Multiple sclerosis
  • Syringomyelia
Treatment
  • Riluzole  
    • only medication to slow disease progression
    • may decrease excitotoxic damage to neurons
  • Supportive treatment
Prognosis, Prevention, and Complications
  • Prognosis
    • main cause of death is respiratory failure
  • Complications
    • weight loss
    • respiratory failure
    • sialorrhea
    • depression
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(M2.NE.17.4672) A 39-year-old male presents with muscle weakness in his upper and lower extremities. The patient has also noticed some trouble with swallowing. On physical exam, signs of hyperreflexia, spasticity, fasciculations, and muscle atrophy are present in an asymmetric fashion. Tongue fasciculations are also present. No sensory loss is noted. The patient does not report any abnormality with his bowel or bladder function. What will most likely be found on muscle biopsy? Tested Concept

QID: 107177
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Mitochondrial proliferation leading to ragged appearance in Gomori trichrome stain

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Perimysial CD4+ infiltration and perifascicular atrophy

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CD8+ infiltrating within the fascicle

50%

(2/4)

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Denervation and reinnervation of the muscle

25%

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Larval cysts

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M 6 C

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