Myasthenia%20gravis Signs and Symptoms
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, 15% 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