Brain Advance Access originally published online on December 21, 2005
Brain 2006 129(2):438-450; doi:10.1093/brain/awh722
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
The clinical spectrum of neuralgic amyotrophy in 246 cases
1 Department of Neurology, Neuromuscular Centre Nijmegen and 2 Clinical Neurophysiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Correspondence to: Nens van Alfen, MD, Neuromuscular Centre Nijmegen, Department of Clinical Neurophysiology, 920 Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB, Nijmegen, The Netherlands E-mail: n.vanalfen{at}neuro.umcn.nl
We investigated the symptoms, course and prognosis of neuralgic amyotrophy (NA) in a large group of patients with idiopathic neuralgic amyotrophy (INA, n = 199) and hereditary neuralgic amyotrophy (HNA, n = 47) to gain more insight into the broad clinical spectrum of the disorder. Several findings from earlier smaller-scale studies were tested, and for the first time the potential differences between the hereditary and idiopathic phenotypes and between males and females were explored. Generally, the course of the pain manifests itself in three consecutive phases with an initial severe, continuous pain lasting for 
4 weeks on average. Sensory involvement was quite common and found in 78.4% of patients but was clinically less impairing than the initial pain and subsequent paresis. As a typically patchy disorder NA can affect almost any nerve in the brachial plexus, although damage in the upper and middle trunk distribution with involvement of the long thoracic and/or suprascapular nerve occurred most frequently (71.1%). We found no correlation between the distribution of motor and sensory symptoms. In INA recurrent attacks were found in 26.1% of the patients during an average 6 year follow-up. HNA patients had an earlier onset (28.4 versus 41.3 years), more attacks (mean 3.5 versus 1.5) and more frequent involvement of nerves outside the brachial plexus (55.8 versus 17.3%) than INA patients, and a more severe maximum paresis, with a subsequent poorer functional outcome. In males the initial pain tended to last longer than it did in females (45 versus 23 days). In females the middle or lower parts of the brachial plexus were involved more frequently (23.1 versus 10.5% in males), and their functional outcome was worse. Overall recovery was less favourable than usually assumed, with persisting pain and paresis in approximately two-thirds of the patients who were followed for 3 years or more.
Key Words: brachial plexus neuropathy; corticosteroid treatment; neuralgic amyotrophy; Parsonage–Turner syndrome
Abbreviations: HNA = hereditary neuralgic amyotrophy; HNPP = hereditary neuropathy with liability to pressure palsies; INA = idiopathic neuralgic amyotrophy; NRS = Numerical Rating Scale
Received August 29, 2005. Revised October 21, 2005. Accepted November 12, 2005.
CiteULike 
Connotea 
Del.icio.us What's this?
Related articles in Brain:
- Editorial
- Alastair Compston
Brain 2006 129: 283-284.[Extract] [FREE Full Text]
This article has been cited by other articles:
|
|
 |
|
  J J J van Eijk, N van Alfen, M Berrevoets, G J van der Wilt, S Pillen, and B G M van Engelen Evaluation of prednisolone treatment in the acute phase of neuralgic amyotrophy: an observational study J. Neurol. Neurosurg. Psychiatry, October 1, 2009; 80(10): 1120 - 1124. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. C. Hannibal, E. K. Ruzzo, L. R. Miller, B. Betz, J. G. Buchan, D. M. Knutzen, K. Barnett, M. L. Landsverk, A. Brice, E. LeGuern, et al. SEPT9 gene sequencing analysis reveals recurrent mutations in hereditary neuralgic amyotrophy Neurology, May 19, 2009; 72(20): 1755 - 1759. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. L. Landsverk, E. K. Ruzzo, H. C. Mefford, K. Buysse, J. G. Buchan, E. E. Eichler, E. M. Petty, E. A. Peterson, D. M. Knutzen, K. Barnett, et al. Duplication within the SEPT9 gene associated with a founder effect in North American families with hereditary neuralgic amyotrophy Hum. Mol. Genet., April 1, 2009; 18(7): 1200 - 1208. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. J. Klein, Y. Wu, J. M. Cunningham, A. J. Windebank, P. J. B. Dyck, S. M. Friedenberg, D. M. Klein, and P. J. Dyck SEPT9 Mutations and a Conserved 17q25 Sequence in Sporadic and Hereditary Brachial Plexus Neuropathy Arch Neurol, February 1, 2009; 66(2): 238 - 243. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Sathasivam, B. Lecky, R. Manohar, and A. Selvan Neuralgic amyotrophy J Bone Joint Surg Br, May 1, 2008; 90-B(5): 550 - 553. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Kalluri, J. T. Huggins, and C. Strange A 56-Year-Old Woman With Arm Pain, Dyspnea, and an Elevated Diaphragm Chest, January 1, 2008; 133(1): 296 - 299. [Full Text] [PDF]
|
|
|
|
 |
|
 N. van Alfen The trouble with neuralgic amyotrophy Practical Neurology, October 1, 2006; 6(5): 298 - 307. [Full Text] [PDF]
|
|
|
|
 |
|
 The Clinical Spectrum of Neuralgic Amyotrophy Journal Watch Neurology, June 8, 2006; 2006(608): 7 - 7. [Full Text]
|
|