[Recurrent familial brachial plexopathy as the only clinical expression of neuropathy with susceptibility to pressure].
We report a family with hereditary neuropathy with liability to pressure palsies (HNPP) and chromosome 17p11.2 deletion. This family exhibits a peculiar phenotype consisting in recurrent brachial plexopathy episodes. This phenotype has to be distinguished from hereditary neuralgic amyotrophy on clinical grounds. Although the incidence of brachial plexopathy on HNPP is relatively high it is unusual as the sole symptom of the disease. It is noteworthy that in the six published families with this peculiar phenotype most of the acute episodes became evident after sleep. A greater liability of the plexus and a greater vulnerability to mechanical factors during sleep hours are the suggested mechanisms to explain this rare clinical onset. Recurrent painless brachial plexopathy when associated to generalized conduction abnormalities should suggest a HNPP.
Gil-Néciga
E
E
Servicio de Neurología, Hospital Universitario Virgen del Rocío, Sevilla.
Franco
E
E
Sánchez
A
A
Donaire
A
A
Chinchón
I
I
Palau
F
F
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D020516:Brachial Plexus Neuropathies / Q000175:diagnosis* / Q000235:genetics*; D002648:Child; D002872:Chromosome Deletion; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D006801:Humans; D008297:Male; D010375:Pedigree; D011312:Pressure; D012008:Recurrence; D013497:Sural Nerve / Q000473:pathology*