Myasthenia Gravis Signs and Symptoms

Last updated: 15 July 2024

Definition

  • An uncommon neurological disorder caused by autoantibodies against the acetylcholine receptor (AChR), against a receptor-associated protein, muscle-specific tyrosine kinase (MuSK-Ab), or occasionally to the low-density lipoprotein receptor-related protein 4 (LPR4)
    • The autoimmune attack at the muscle endplate leads to failure of neuromuscular transmission and eventually muscle weakness

Signs and Symptoms

  • Fatigability and weakness of voluntary muscles
    • Weakness tends to increase during repeated use
    • May improve with rest or sleep
  • Diplopia and ptosis
    • Common initial complaints
    • Generally asymmetrical
    • Cogan’s lid twitch or excessive lid elevation
  • Facial weakness which results in snarl-like expression when smiling
  • Nasal or slurred speech
  • Difficulty in swallowing
  • If generalized, there will be weakness in the limb muscles
    • Often asymmetric and proximal
  • Deep tendon reflexes and sensation are preserved
  • If severe, there will be shortness of breath

Clinical Features

  • Acetylcholine Receptor Myasthenia Gravis (ACh-MG)
    • Muscle weakness predominant in the limb than bulbar, neck extensor than neck flexor, prominent ptosis, and external ocular muscle weakness
    • Wasting of proximal limb and ocular muscles in long-standing disease (myasthenic myopathy)
    • Thymus pathology: 65% hyperplasia, 10% thymoma
    • Risk of recurrent crisis is low
  • Muscle-Specific Tyrosine Kinase Myasthenia Gravis (MuSK-MG)
    • Muscle weakness predominant in the bulbar than the limb, neck flexor than neck extensor, mild ptosis, and external ocular muscle weakness
    • Early wasting of facial and tongue muscles
    • Thymus pathology: 10% hyperplasia
    • High-risk of recurrent crisis