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
Validation

Diseases presenting "peripheral neuropathy" symptom

adrenomyeloneuropathy

esophageal squamous cell carcinoma

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

kallmann syndrome

krabbe disease

neuralgic amyotrophy

oculocutaneous albinism

pyruvate dehydrogenase deficiency

sneddon syndrome

systemic capillary leak syndrome

thoracic outlet syndrome

triple a syndrome

waldenstrÃ¶m macroglobulinemia

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated