Clinical and pathophysiological concepts of neuralgic amyotrophy.

[neuralgic amyotrophy]

Neuralgic amyotrophy --also known as Parsonage-Turner syndrome or brachial plexus neuritis --is a distinct and painful peripheral neuropathy that causes episodes of multifocal paresis and sensory loss in a brachial plexus distribution with concomitant involvement of other PNS structures (such as the lumbosacral plexus or phrenic nerve ) in a large number of patients . The phenotype can be limited or extensive and the amount of disability experienced also varies between patients , but many are left with residual disabilities that affect their ability to work and their everyday life . Both idiopathic and hereditary forms exist . The latter form is genetically heterogeneous , but in 55 % of affected families , neuralgic amyotrophy is associated with a point mutation or duplication in the SEPT 9 gene on chromosome 17 q 25 . The disease is thought to result from an underlying genetic predisposition , a susceptibility to mechanical injury of the brachial plexus (possibly representing disturbance of the epineurial blood -nerve barrier ), and an immune or autoimmune trigger for the attacks . The precise pathophysiological mechanisms are still unclear ; treatment is empirical , and preventive measures are not yet available . This Review provides an overview of the current clinical and pathophysiological concepts and research topics in neuralgic amyotrophy .

Diseases
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Diseases presenting "autoimmune trigger for the attacks" symptom

neuralgic amyotrophy

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