[Hereditary neuralgic amyotrophy: a paediatric and familial presentation of Parsonage-Turner syndrome]
Article in French
Husson M(1), Goizet C, Rivera S, Lacombe D, Pedespan JM.
Author information:
(1)Unité de neuropédiatrie, centre hospitalier universitaire Pellegrin, hôpital
des enfants, place Amélie-Raba-Léon, 33076 Bordeaux cedex, France.
Hereditary neuralgic amyotrophy is a rare disorder, characterized by recurrent
attacks of pain in a brachial plexus distribution. We report the case of a
12-year-old boy with several attacks of pain and atrophy of the muscles of the
shoulders. The age of onset of this disease is variable, most frequently in the
second or third decade. Pediatric onsets, during the first decade are rare. The
differences between the hereditary neuralgic amyotrophy and the sporadic
Parsonage-Turner syndrome are painful recurrent episodes of weakness and similar
familial cases. The analysis of several families has shown that hereditary
neuralgic amyotrophy phenotype is heterogeneous and two different clinical
courses can be discerned. Recent evidence indicates that HNA is genetically
heterogeneous. Pathophysiology of the disease remains unclear, so the treatment
is not clearly established.
DOI: 10.1016/j.arcped.2004.06.023
PMID: 15519832 [Indexed for MEDLINE]