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      Four cases of pediatric neuralgic amyotrophy treated with immunotherapy: one-year follow-up and literature review

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          Abstract

          Neuralgic amyotrophy (NA) is a neurological disease that occurs across all age groups, but its prognosis in children is controversial. The present report adds to the knowledge about its prognosis by describing four cases of pediatric NA in which the patients were treated with immunotherapy and followed up for 1 year. We also present a summary of relevant cases of pediatric NA treated with immunotherapy. The clinical features of the four present cases were similar to those of previously reported cases, and their symptoms improved after immunotherapy. At the 1-year follow-up, three of the children gained near complete recovery, and their improvement was significantly better than that observed at the 2-month follow-up. A review of the literature showed that most previously reported children with NA showed improvement after immunotherapy, but no more than half of the patients recovered fully. These findings indicate that in children with NA, immunotherapy is fairly effective and its benefits improve with time. Thus, long-term follow-up is needed in these patients to determine their prognosis.

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          Most cited references25

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          The clinical spectrum of neuralgic amyotrophy in 246 cases.

          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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            Neuralgic amyotrophy: An update on diagnosis, pathophysiology, and treatment

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              Mutations in SEPT9 cause hereditary neuralgic amyotrophy.

              Hereditary neuralgic amyotrophy (HNA) is an autosomal dominant recurrent neuropathy affecting the brachial plexus. HNA is triggered by environmental factors such as infection or parturition. We report three mutations in the gene septin 9 (SEPT9) in six families with HNA linked to chromosome 17q25. HNA is the first monogenetic disease caused by mutations in a gene of the septin family. Septins are implicated in formation of the cytoskeleton, cell division and tumorigenesis.
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                Author and article information

                Journal
                J Int Med Res
                J. Int. Med. Res
                IMR
                spimr
                The Journal of International Medical Research
                SAGE Publications (Sage UK: London, England )
                0300-0605
                1473-2300
                31 March 2020
                March 2020
                : 48
                : 3
                : 0300060520912082
                Affiliations
                [1 ]Department of Neurology, Children’s Hospital of Soochow University, Suzhou, Jiangsu Province, China
                [2 ]Department of Neurology, Wuxi Children’s Hospital, Wuxi, Jiangsu Province, China
                Author notes
                [*]Jihong Tang, Department of Neurology, Children’s Hospital of Soochow University, 303 Jingde Road, Suzhou, Jiangsu Province 215003, China. Email: tjhzsh@ 123456126.com
                Author information
                https://orcid.org/0000-0001-5656-4348
                Article
                10.1177_0300060520912082
                10.1177/0300060520912082
                7132571
                32228355
                47e532c6-44e5-40ea-9174-d13945586880
                © The Author(s) 2020

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 14 November 2019
                : 17 February 2020
                Funding
                Funded by: Science and Technology Project of the Health and Health Committee of Jiangsu Province;
                Award ID: H2018010
                Funded by: Wuxi Young Medical Talents;
                Award ID: QNRC021
                Funded by: Wuxi maternal and child health research project;
                Award ID: FYKY201904
                Funded by: Wuxi Medical Development Discipline;
                Award ID: FZXK001
                Funded by: Science and Technology Program of Suzhou ;
                Award ID: SS201866
                Categories
                Case Report
                Custom metadata
                corrected-proof
                ts2

                neuralgic amyotrophy,pediatric,immunotherapy,prognosis,review,case report

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