Amyotrophic lateral sclerosis physical examination

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  • Limb onset:
    • Awkwardness when walking or running
    • Tripping or stumbling
    • Difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock** Occasionally the symptoms remain confined to one limb; this is known as monomelic amyotrophy.
  • Bulbar onset:
    • These patients first notice difficulty speaking clearly
    • Garbled and slurred speech
    • Nasality and loss of volume are frequently the first symptoms
    • Difficulty swallowing and loss of tongue mobility follow
    • Eventually total loss of speech and the inability to protect the airway when swallowing are experienced
  • Muscle weakness and atrophy spread to other parts of the body as the disease progresses
  • Increasing difficulty moving
  • Swallowing (dysphagia)
  • Speaking or forming words (dysarthria)
  • Upper motor neuron involvement:
  • Lower Motor Neuron involvement:
    • Muscle weakness and atrophy,
    • Muscle cramps, and
    • Fleeting twitches of muscles that can be seen under the skin (fasciculations)
  • 45% of patients experience pseudobulbar affect, also known as "emotional lability" attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion
    • Uncontrollable laughter
    • Crying or smiling
  • Frontotemporal dementia

References

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