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Neuralgic amyotrophy is a distinct clinical syndrome with acute severe pain and patchy paresis in the shoulder and arm region. The clinical phenotype was recently found to be more comprehensive and the long-term prognosis less optimistic than usually assumed for many patients. The disorder can be idiopathic or hereditary in an autosomal dominant fashion, with only few phenotypical variations between the two. This article provides a practical overview of current knowledge on the clinical presentation, diagnosis, pathogenesis and the treatment of pain and complications.
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PMID:The neuralgic amyotrophy consultation. 1744 96

Neuralgic amyotrophy (NA) is characterised by neuropathic pain and patchy paresis of the upper or lower limbs, usually involving the upper and middle trunks of the brachial plexus. The aetiology of NA is varied, with precipitating factors that include trauma, surgery, pregnancy, inoculations and infections. Deranged liver enzymes have been noted in previous NA reports but no cause identified. We describe a case of bilateral NA in a 53-year-old man who presented with peripheral neuropathy and isolated derangement of liver enzymes. Serology was positive for hepatitis E infection and negative for other infections previously described to be associated with NA. The diagnosis was supported by electrophysiological findings. This case report suggests that hepatitis E is a potential cause of NA that had not previously been described in the literature, and further supports a proposed immune pathogenesis underlying the condition.
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PMID:Neuralgic amyotrophy associated with hepatitis E virus. 1898 57

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 SEPT9 gene on chromosome 17q25. 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.
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PMID:Clinical and pathophysiological concepts of neuralgic amyotrophy. 2155 32

A 40-year-old man presented with sudden onset of severe left buttock pain that radiated down the thigh to the leg. On examination, he showed moderate weakness and atrophy in the left quadriceps and tibialis anterior muscles, and absence of a left patellar tendon reflex. Needle electromyography revealed an active denervation pattern in the left quadriceps muscles, suggesting neuralgic amyotrophy. Contrast-enhanced MRI showed abnormal enhancement of the left cauda equina. Steroid pulse therapy relieved pain, and subsequent high-dose intravenous immunoglobulin prevented progression of muscle atrophy and weakness. Neuralgic amyotrophy is characterized by attacks of severe neuropathic pain and subsequent patchy paresis in the upper or lower extremities. Since overall recovery is less favourable than usually assumed, early diagnosis is very important. This case was remarkable in that contrast-enhanced MRI revealed abnormal enhancement and thickening of the cauda equina, which may help in achieving early diagnosis and treatment.
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PMID:[Lumbar radiculopathy presenting neuralgic amyotrophy with gadolinium-enhanced MRI lesions: a case report]. 2235 36

Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a distinct peripheral nervous system (PNS) disorder, characterized by sudden attacks of severe neuropathic pain usually in the shoulder and/or arm. The neuralgia commonly disappears after a few days to weeks, and consequently patchy paresis with amyotrophy appears. The available evidence suggests that NA is essentially idiopathic immune-mediated neuritis of the brachial plexus, and also has a complex pathogenesis that includes an underlying predisposition, susceptibility to dysfunction of some PNS structure, and a trigger for the attacks, such as viral infection, vaccination, trauma, surgery, and strenuous exercise. Genetic factors also contribute to the pathogenesis of NA, and thus, this disorder occurs in both idiopathic and hereditary forms, but hereditary one is considered to be 10 times less common than idiopathic one. NA has been considered to be self-limiting, benign disorder showing good recovery without specific treatments. However, recent studies have indicated that the long-term prognosis of NA is less favorable than has been assumed. In 2009, a Cochrane review identified one open label, retrospective series, the results of which suggested that administration of corticosteroids in the acute phase of NA could shorten the duration of painful symptoms and also accelerate recovery in some patients. We recently have reported that intravenous immunoglobulin (IVIg) with methylpredonisolone pulse therapy is effective for motor impairment of NA.
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PMID:[Pathogenesis and treatment of brachial plexus neuritis]. 2429 49

Neuralgic Amyotrophy (NA) is a rare disturb of the peripheral nervous system that can include extreme pain, multifocal paresis and atrophy of the muscles of the upper limbs. When the nerves located outside of the brachial plexus are involved, the term Neuralgic Amyotrophy Extended (ANE) is used. Diagnosis of NA is clinical and has a series of inclusion and compatibility criteria established by the European CMT Consortium. On this study the clinical history, multidimensional vocal assessment data and the vocal techniques used in five-weeks voice therapy for one patient, professional voice, with ANE are presented. In this case, sudden and recurrent paralysis of his right vocal fold was the only manifestation of the disease. At the end of the fifth week the patient's voice was normal, the spoken and sung vocal ranges were same as before the current episode of ANE and scores of his vocal self-assessment were appropriate.
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PMID:Extended Neuralgic Amyotrophy Syndrome: voice therapy in one case of vocal fold paralysis. 2491 13

Neuralgic amyotrophy (NA) is a distinct peripheral nervous system (PNS) disorder characterized by sudden attacks of neuropathic pain, usually in a unilateral upper extremity, and patchy paresis with atrophy in the glenohumeral muscles. The lesion sites of NA are commonly considered to be upper brachial plexus (BP) and/or individual branches of the BP. The cause of NA remains unknown. Some evidence suggests a complex pathogenesis in NA that includes predisposition and susceptible PNS structures, and it can be triggered by infection, trauma, and strenuous exercise. NA is considered to be broad and encompasses a spectrum of atypical presentations, including involvement of the lower part of the BP, isolated nerves (anterior interosseous nerve or posterior interosseous nerve), or lumbosacral plexuses. Functional prognosis of NA is less favorable than previously assumed; however, specific therapy for patients with NA remains to be established.
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PMID:[Neuralgic amyotrophy]. 2547 29

We report the case of a 49-years-old patient who presented to the accident and emergency department with sudden onset dyspnea associated with acute shoulder pain. He was breathless at rest with supine hypoxemia. He had an amyotrophic left shoulder with localized paresis of the shoulder. Both hemi-diaphragms were elevated on chest X-rays. Pulmonary function tests showed a restrictive pattern and both phrenic nerve conduction velocities were decreased. At night, alveolar hypoventilation was evidenced by elevated mean capnography (PtcCO2: 57mmHg). Neuralgic amyotrophy, Parsonage-Turner syndrome was the final diagnosis. This syndrome is a brachial plexus neuritis with a predilection for the suprascapular and axillary nerves. Phrenic nerve involvement is rare but where present can be the most prominent clinical feature as in our case report.
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PMID:[Bilateral diaphragmatic paralysis due to Parsonage-Turner syndrome]. 2553 71

Neuralgic amyotrophy (NA) is a distinct peripheral nervous system (PNS) disorder characterised by sudden attacks of neuropathic pain, usually in a unilateral upper extremity, and patchy paresis with amyotrophy. Under-recognition of NA patients may be frequent because symptoms of NA can be similar to those of common orthopedic disorders. The lesion sites of NA are commonly considered to be brachial plexus (BP) and/or individual branches of the BP. The cause of NA remains unknown. Some evidence support the concept of a complex pathogenesis in NA that includes underlying predisposition and susceptible PNS structures, and it can be triggered by infection, trauma, and strenuous exercise. Typical presentation of NA is characterized by patchy paresis of the periscapular and periglenohumeral muscles. In such cases, STIR-MRI often shows hyperintense signal abnormalities on the affected side of the proximal upper BP. NA is considered to be broad and encompasses a spectrum of atypical presentations, including involvement of lower part of BP, isolated nerves (anterior interosseous nerve or posterior interosseous nerve), or lumbosacral plexuses. Functional prognosis of NA is less favorable than previously assumed. Administration of corticosteroids and intravenous immunoglobulin was described as potential therapeutics for NA, although their efficacy remains unestablished.
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PMID:[Clinical features and MRI characteristics in neuralgic amyotrophy]. 2567 7

Neuralgic amyotrophy (NA), even known as Personage-Turner's syndrome (PTS), is a neurologic condition, affecting the lower motor neurons of brachial plexus and/or individual nerves or nerve branches, characterized by pain, muscle weakness/atrophy, and sensory symptoms. NA has an acute/subacute onset, after an infection or vaccination; it is more common in male and is rare in the pediatric population. The etiology remains uncertain, being considered heterogeneous and multifactorial. A severe acute neurologic pain around the shoulder girdle is the classic presenting symptom at onset. As the pain subsides, weakness and paresis develop. NA is usually unilateral, but sometimes, a subclinical contralateral limb involvement could be present and bilateral affection has been described. The diagnosis is clinical, through a comprehensive history and neurological examination. However, electrophysiological testing and imaging are critical, because there is no diagnostic test for PTS and it remains a diagnosis of exclusion. Upper brachial plexus peripheral involvement with weakness of periscapular and perihumeral muscles is the classic presentation, associated with electrophysiological evidence of denervation in the affected muscles. Imaging, laboratory, and genetic testing can be useful for the differential diagnosis. NA is in most cases a self-limiting condition, and it is characterized by good recovery. Treatment of NA usually involves a combination of corticosteroids, analgesics, immobilization, and physical therapy, even if limited data are available in children. Physiotherapy is required to maintain muscle strength.
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PMID:Clinico-diagnostic features of neuralgic amyotrophy in childhood. 3214 Sep 11


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