The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain

Department of Neurology, Neuromuscular Centre Nijmegen, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Brain (Impact Factor: 9.2). 03/2006; 129(Pt 2):438-50. DOI: 10.1093/brain/awh722
Source: PubMed


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 approximately 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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    • "Trauma is the most common cause of brachial plexopathy[5]. Other causes of brachial plexopathy are paralytic brachial neuritis (Parsonage–Turner syndrome)[6], hereditary brachial plexopathy (hereditary neuralgic amyotrophy)[7], neoplasms especially small cell lung cancer , breast cancer, and lymphoma[8], radiation-induced brachial plexopathy[8], thoracic outlet syndrome[9], dia- betes[10], and iatrogenic brachial plexopathies[5]. Rarely inflammatory and autoimmune diseases cause brachial plexopathy. "
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    ABSTRACT: Microscopic polyangiitis is a small-vessel necrotizing vasculitis associated with anti-neutrophil cytoplasmic antibodies and presents itself with glomerulonephritis and hemorrhagic pulmonary capillaritis. Peripheral nervous system involvement is common in anti-neutrophil cytoplasmic antibodies-associated vasculitis, but brachial plexopathy is unusual. We present the case of a 22-year-old man with known microscopic polyangiitis who was under maintenance therapy with prednisolone and cyclophosphamide and developed cough, dyspnea, and hemoptysis which increased in 6 days accompanying pain and paresthesia in the upper limbs. His physical examination revealed hypoesthesia, absence of deep tendon reflexes, and decreased muscle strength in the upper limbs. His chest computed tomography scan showed ground glass pattern in the lower and middle lobes. Electromyography and nerve conduction study showed bilateral brachial plexopathy with involvement of all of the cervical roots that were more severe in the lower roots and left side.
    Full-text · Article · Jan 2016 · Rheumatology International
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    • "La récupération de la force est obtenue dans 36 %, 75 % et 89 % des cas, respectivement à 1, 2 et 3 ans d'évolution [18]. Des séquelles peuvent persister, et associent douleurs et incapacité fonctionnelle, liées probablement à des altérations de la cinématique de la scapula [17] [19]. Environ 25 % des patients ont une gêne fonctionnelle nécessitant une intervention chirurgicale [5]. "
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    ABSTRACT: Dyskinesia of the scapula is a clinical diagnosis and includes all disorders affecting scapula positioning and movement whatever its etiology. Scapular winging is a subtype of scapular dyskinesia due to a dynamic prominence of the medial border of the scapula (DSW) secondary to neuromuscular imbalance in the scapulothoracic stabilizer muscles. The two most common causes of DSW are microtraumatic or idiopathic lesions of the long thoracic nerve (that innerves the serratus anterior) or the accessory nerve (that innerves the trapezius). Diagnosis of DSW is clinical and electromyographic. Use of magnetic resonance imaging (MRI) could be of interest to distinguish lesion secondary to a long thoracic nerve from accessory nerve and to rule out scapular dyskinesia related to other shoulder disorders. Causal neuromuscular lesion diagnosis in DSW is challenging. Clinical examinations, combined with scapular MRI, could help to their specific diagnosis, determining their stage, ruling out differential diagnosis and thus give raise to more targeted treatment.
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    • "The anterior interosseus and suprascapular motor nerves and the lateral antebrachial cutaneous and superficial radial sensory nerves are frequently involved. Sensory symptoms are usually moderate, but almost 80% of the patients can recall hypaesthesia [2,4]. "
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    ABSTRACT: Background The ‘Parsonage-Turner syndrome’ (PTS) is a rare but distinct disorder with an abrupt onset of shoulder pain, followed by weakness and atrophy of the upper extremity musculature, and a slow recovery requiring months to years. To our best knowledge, this is the first case describing symptoms and signs of PTS following the administration of a post-exposure prophylaxis (PEP) regimen against possible human immunodeficiency virus (HIV) and hepatitis B virus (HBV) infection. Case presentation A 25-year-old Caucasian man presented with pain and unilateral scapular winging following PEP against possible HIV and HBV infection. Although atrophy and weakness were observed for the right supraspinatus muscle, a full range of motion was achievable. Neurological examination, plain radiography of the right shoulder and electromyography showed no additional abnormalities. The patient was diagnosed with post-vaccination PTS and treated non-operatively. During the following 15 months the scapular winging receded and full muscle strength was regained. Conclusion Parsonage-Turner syndrome is a rare clinical diagnosis. The precise pathophysiological mechanism of PTS remains unclear, but it seems to involve an interaction between genetic predisposition, mechanical vulnerability and an autoimmune trigger. An immunological event, such as – in this case – a vaccination as part of PEP treatment, can trigger the onset of PTS. The clinical presentation is distinctive with acute severe pain followed by patchy paresis, atrophy and sensory symptoms that persist for months to years. No currently available tests can provide a definite confirmation or exclusion of PTS. Routine blood examination, electromyography (EMG), and computed tomography (CT) or magnetic resonance imaging (MRI) serve mainly to exclude other disorders. The recovery can be quite lengthy, non-operative treatment is the accepted practice. Supplementary administration of oral prednisolone could shorten the duration of pain. Although the outcome is typically preferable, a substantial amount of patients are left with some residual paresis and functional impairment.
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