Abstract
We present a 10-year-old female diagnosed having hereditary neuropathy with liability
to pressure palsies (HNPP). She had suffered from acute, recurrent monoplegic episodes
affecting both the sciatic nerves and the left brachial plexus since the age of 7
years. The paresis seemed to be triggered by hiking and athletic training. Electrophysiological
studies showed a conduction block in the proximal portions of affected nerves. The
FISH method disclosed a deletion of the peripheral myelin protein 22 gene. This school
child having HNPP is considered to be susceptible to the influence of abundant physical
training, rather than minor trauma or compression at sites of entrapment of peripheral
nerves.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Spectrum of clinical and electrophysiologic features in HNPP patients with the 17p11.2 deletion.Neurology. 1999; 52: 1440-1446
- Electrodiagnostic features of hereditary neuropathy with liability to pressure palsies.Neurology. 2000; 54: 40-44
- Diagnostic strategy for familial and sporadic cases of neuropathy associated with 17p11.2 deletion.Muscle Nerve. 2001; 24: 1149-1155
- Hereditary recurrent focal neuropathies: clinical and molecular features.Neurology. 2000; 54: 546-551
- Hereditary neuropathy with liability to pressure palsies in childhood.Neuropediatrics. 1992; 23: 138-143
- Hereditary neuropathy with tendency to pressure palsies in a child: clinical and biological diagnosis.Arch Pediatr. 2000; 7: 271-273
- Brachial plexus involvement as the only expression of hereditary neuropathy with liability to pressure palsies.Muscle Nerve. 2001; 24: 1093-1096
- Steroid responsive familial neuropathy with liability to pressure palsies.Neuropediatrics. 1990; 21: 191-192
- Intravenous immunoglobulin therapy in multifocal motor neuropathy.Brain. 2001; 124: 145-153
- Gene dosage effects in hereditary peripheral neuropathy: expression of peripheral myelin protein 22 in Charcot-Marie-Tooth disease type 1A and hereditary neuropathy with liability to pressure palsies nerve biopsies.Neurology. 1997; 49: 1635-1640
Article info
Publication history
Accepted:
December 19,
2003
Received in revised form:
November 11,
2003
Received:
July 24,
2003
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
