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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
Related
idiopathic brachial plexitis;
General
neuralgia
muscle atrophy (amyotrophy)
Database Correlations
OMIM 162100
References
- Wikipedia: Hereditary neuralgic amyotrophy
http://en.wikipedia.org/wiki/Hereditary_neuralgic_amyotrophy
- van Alfen N.
The neuralgic amyotrophy consultation.
J Neurol. 2007 Jun;254(6):695-704. Epub 2007 Apr 20
PMID: 17446996