neuralgic amyotrophy

Pathology

• nerve damage
  • nerves of the brachial plexus are targeted as antigens
  • phrenic nerve may be affected
  • recurrent laryngeal may be affected
• muscle atrophy occurs in response to nerve degeneration
  • deltoid atrophy (brachial plexus)
  • diaphragm atrophy, respiratory insufficiency (phrenic nerve)
  • dysphonia (recurrent laryngeal nerve)

Genetics

• mutation in the SEPT9 gene (17q25)

Clinical manifestations

• episodes of severe pain generally lasting 1-6 weeks
• pain in the back, neck, arms, or shoulders

Management

• prednisone
• prognosis:
  • nerve regeneration occurs after an episode
  • in less severe cases, full recovery of nerves & muscles
  • in severe cases, permanent nerve damage may occur

More general terms

• neuralgia
• muscle atrophy (amyotrophy)

Additional terms

• idiopathic brachial plexitis;

Database

• OMIM: https://mirror.omim.org/entry/162100