Pathology · Immunopathology (Hypersensitivity, Autoimmunity, Immunodeficiency, Amyloidosis)

Seronegative myasthenia gravis (MuSK-antibody positive) differs from classic AChR-antibody MG in which pathological mechanism?

  • A Complement-mediated destruction of the neuromuscular junction
  • B T-cell mediated destruction of motor neurons in the anterior horn
  • C Type III immune complex deposition in the NMJ basement membrane
  • D Blocking of acetylcholine binding without complement activation; also involving disruption of AChR clustering at the NMJ
Correct answer: D. Blocking of acetylcholine binding without complement activation; also involving disruption of AChR clustering at the NMJ

Explanation

Anti-MuSK (muscle-specific kinase) antibodies are predominantly IgG4, which does not fix complement. They block MuSK, a receptor tyrosine kinase essential for agrin-mediated clustering of AChRs at the NMJ, thereby disrupting NMJ architecture without complement-mediated lysis. Classic AChR antibody MG involves complement activation and AChR downregulation/destruction. MuSK MG has distinct clinical features including more prominent bulbar and facial involvement.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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