Brachial plexus involvement as the only expression of hereditary neuropathy with liability to pressure palsies.
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant focal neuropathy characterized by recurrent entrapment neuropathies. Single members of families with HNPP and brachial plexus involvement have been reported previously. We describe a family with three affected members and in which the only symptom of entrapment was recurrent brachial plexopathy. The diagnosis of HNPP in this family was confirmed by both neurophysiological methods and DNA analysis. The distinction between HNPP and hereditary neuralgic amyotrophy is discussed.
Ørstavik
K
K
Department of Neurology, Ullevål Hospital, 0407 Oslo, Norway. kristino@medscape.com
Skard Heier
M
M
Young
P
P
Stögbauer
F
F
D000328:Adult; D020516:Brachial Plexus Neuropathies / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D004252:DNA Mutational Analysis; D004568:Electrodiagnosis; D004576:Electromyography; D005799:Genes, Dominant; D020022:Genetic Predisposition to Disease; D006801:Humans; D019656:Loss of Heterozygosity; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009185:Myelin Proteins / Q000172:deficiency / Q000235:genetics; D009408:Nerve Compression Syndromes / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D009431:Neural Conduction; D010375:Pedigree; D010543:Peroneal Nerve / Q000503:physiopathology
D009185:Myelin Proteins; C073687:PMP22 protein, human