Brain Advance Access published online on December 21, 2005
Brain, doi:10.1093/brain/awh722
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1 Department of Neurology, Neuromuscular Centre Nijmegen, The Netherlands; Clinical Neurophysiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
* To whom correspondence should be addressed. 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
Received August 29, 2005
Revised October 21, 2005
Accepted November 12, 2005
Article
The clinical spectrum of neuralgic amyotrophy in 246 cases
Nens van Alfen 1 *
and
Baziel G. M. van Engelen 2
2 Department of Neurology, Neuromuscular Centre Nijmegen, The Netherlands
Nens van Alfen, E-mail: n.vanalfen{at}neuro.umcn.nl
Abstract 
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.
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