Hereditary Neuralgic Amyotrophy

Hereditary Neuralgic Amyotropy, alternatively referred to as HNA, is a neuralgic disorder that is characterized by nerve damage and muscle atrophy, preceded by severe pain. It is caused by a mutation to the gene locus 17-q25 of the septin 9 gene. While not much is known about this disorder, it has been characterized to be similar to Parsonage-Turner syndrome in prognosis. [ [http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=162100 Omim - Amyotrophy, Hereditary Neuralgic; Hna ] ]

HNA is an episodic disorder; it is characterized by episodes generally lasting 1-6 weeks. During an episode, the nerves of the brachial plexus are targeted by the body as antigens, and the body's immune system begins to degenerate the nerves of the brachial plexus. The exact order or location of the nerve degeneration cannot be deciphered before an episode, as HNA is a highly variable disorder. Other areas of the nervous system that have been targeted in past cases are the phrenic nerves and the recurrent larangyl. As the nerves lose function, the muscles associated with those nerves begin to atrophy. In brachial plexus degeneration, atrophy may occur in the deltoids. In phrenic nerve degeneration, the diaphragm may be affected. In this case, breathing can be impaired due to a lack of muscle control of the diaphragm. If the recurrent larangyl is targeted, the pharynx will begin to atrophy and voice function may be lost. [ [http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=162100 Omim - Amyotrophy, Hereditary Neuralgic; Hna ] ]

ymptoms

Symptoms of HNA may include pain in the back, neck, arms, or shoulders, nerve pulls in the arms or back, muscular atrophy, and weakness. [ [http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=162100 Omim - Amyotrophy, Hereditary Neuralgic; Hna ] ]

Treatment

The severe pain of HNA can be controlled with an anti-inflammatory drug such as prednisone, although it is unknown whether these anti-inflammatory drugs actually slow or stop the nerve degeneration process.

Nerve regeneration after an episode is normal, and in less severe cases a full recovery of the nerves and muscles can be expected. However, in a severe case permanent nerve damage may occur. [ [http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=162100 Omim - Amyotrophy, Hereditary Neuralgic; Hna ] ]

References

ee also

*Parsonage Turner Syndrome, known as Neuralgic Amyotrophy