第 275 届 ENMC 国际研讨会：血清阴性重症肌无力：诊断和管理的最新范例，2024 年 2 月 9-11 日，荷兰 Hoofddorp。

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2024-10-05 DOI:10.1016/j.nmd.2024.104468

Amelia Evoli , Jacqueline Palace , Gregorio Spagni , Marta Cheli , Annabel Ruiter , Jan Verschuuren , Lorenzo Maggi

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引用次数: 0

摘要

第275届ENMC血清阴性重症肌无力（SNMG）诊治研讨会于2024年2月9日至11日举行。与会者包括成人和儿童重症肌无力领域的专家以及患者代表。此次研讨会旨在根据最新的诊断和治疗方法重新定义SNMG，并确定患者尚未满足的需求。研讨会突出强调了在 SNMG 诊断工作中面临的巨大挑战。迄今为止，由于缺乏特定的诊断测试，SNMG 的确认往往存在争议；文献中没有国际专家小组的建议；肌病、先天性肌无力综合征和功能性疾病是最常见的误诊。改进疾病诊断对于避免长期拖延接受适当治疗至关重要。为此，一种全面的诊断算法已达成共识。此外，SNMG 对治疗的反应和长期预后的显著差异也得到了强调。

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275th ENMC international workshop: Seronegative myasthenia gravis: An update paradigm for diagnosis and management, 9–11 February 2024, Hoofddorp, the Netherlands

The 275th ENMC workshop on the diagnosis and management of seronegative myasthenia gravis (SNMG) was held on February 9–11, 2024. The participants included experts in the field of adult and pediatric MG together with patient representatives. This workshop aimed to redefine SNMG in view of recent diagnostic and therapeutic updates and to identify patient unmet needs. The workshop has highlighted considerable challenges in the SNMG diagnostic work-up. To date, SNMG confirmation is often controversial, given the absence of specific diagnostic tests; no recommendations from international panels of experts are available in literature; myopathies, congenital myasthenic syndromes and functional disorders are the commonest misdiagnoses. Improving the disease diagnosis is crucial to avoid long delays in receiving appropriate treatment. To this purpose, a comprehensive diagnostic algorithm achieved consensus. Moreover, a remarkable variability in SNMG response to therapy and long-term prognosis has also been highlighted.

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来源期刊

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.