[Recurrent familial amyotrophic neuralgia of the brachial plexus. Report of a family and review of the literature].
A family with hereditary neuralgic amyotrophy of the brachial plexus throughout three generations is described. Outstanding features are early onset occurring during childhood or adolescence, unlike the idiopatic sporadic form of the disease, and the association with a peculiar physiognomy that reminds one of the facial expression found in Modigliani's paintings. Clinical evolution is not always favorable since relapses and long-lasting sequelae are common. The disease is inherited through an autosomal dominant gene with high penetrance and the neuropathy is always associated with the phenotypic features. The possible relationship with other pathological entities of very different clinical expression, such as tomacular neuropathy, is discussed, as well as the distinguishing peculiarities between the inherited and the sporadic forms of brachial plexus neuropathy.
Zuazo Zamalloa
E
E
Departamento de Pediatría, Hospital de Cruces, Vizcaya.
Pomposo Bolumburu
I
I
Garaizár Axpe
C
C
Prats Viñas
J M
JM
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001917:Brachial Plexus / Q000503:physiopathology; D020968:Brachial Plexus Neuritis / Q000235:genetics*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D010641:Phenotype; D012008:Recurrence; D013577:Syndrome
Neuralgia amiotrófica recurrente familiar del plexo braquial. Descripción de una familia y revisión de la literatura.