一名 16 岁女性的神经肌张力障碍在接受 IVIG 治疗后得到显著改善:病例报告和文献综述。

IF 2.7 4区 医学 Q2 CLINICAL NEUROLOGY Neuromuscular Disorders Pub Date : 2024-11-05 DOI:10.1016/j.nmd.2024.105239
Omar Ketranji, Issa Alawneh, Asmaa Alenizi, Elisa Nigro, Michal S Zimmer, Freddy Paiz, Hernan Gonorazky
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引用次数: 0

摘要

神经肌张力障碍又称艾萨克综合征,是一种罕见的神经系统疾病,以肌肉持续活动、僵硬和自发性肌肉收缩为特征,在儿童中非常罕见。我们报告了一名患有神经肌张力障碍的 16 岁女性患者。她出现双下肢疼痛、僵硬、自主神经症状和肌肉肌强直。患者接受了短期甲基强的松龙治疗和为期 4 周的静脉注射免疫球蛋白治疗,并接受了对症治疗,结果症状得到了显著改善和缓解。我们对患有神经肌张力障碍的儿科患者进行了文献回顾,迄今为止共发现了 10 个病例。尽管采用了不同的治疗方法,但所有神经肌张力障碍儿科患者的预后均良好。虽然神经肌张力障碍与恶性肿瘤之间的关系在成人人群中已为人所知,但在已报道的儿科病例中却未发现这种情况。事实上,鉴于数据的稀缺性,我们仍然建议对神经肌张力障碍的儿科患者进行恶性肿瘤筛查。
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Neuromyotonia in a 16-year-old female with dramatic improvement after IVIG therapy: Case report and literature review.

Neuromyotonia, also known as Isaac syndrome, is a rare neurological disorder characterized by continuous muscle activity, stiffness, and spontaneous muscle contractions, it is very rare in children. We report a 16-year-old female patient with neuromyotonia. She presented with pain, stiffness, autonomic symptoms and muscle myokymia in both lower limbs. The patient was treated with a short course of methylprednisolone, IVIG over the course of 4 weeks, and symptomatic management which resulted in a dramatic improvement and relief of symptoms. A literature review for pediatric patients with neuromyotonia was conducted revealing 10 reported cases so far. All pediatric patients with neuromyotonia showed favorable prognosis despite using different treatment modalities. Although the association between neuromyotonia and malignancy is known in adult population, this has not been seen in the reported pediatric cases. Indeed, given the scarcity of data, we still do recommend screening for malignancy in pediatric patients with neuromyotonia.

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来源期刊
Neuromuscular Disorders
Neuromuscular Disorders 医学-临床神经学
CiteScore
4.60
自引率
3.60%
发文量
543
审稿时长
53 days
期刊介绍: This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.
期刊最新文献
Neuromyotonia in a 16-year-old female with dramatic improvement after IVIG therapy: Case report and literature review. Editorial Board Publisher's Note 26th Meryon Lecture St Anne's College, Oxford, 5th July 2024 FSHD: The long road to DUX4 Upper limb function changes over 12 months in untreated SMA II and III individuals: an item-level analysis using the Revised Upper Limb Module
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