Dropped head syndrome can be the presenting feature of a wide spectrum of neurological conditions. In this study, we aimed to define the clinical characteristics and treatment outcomes of 107 patients, where head drop was the presenting or predominant clinical feature of a myopathy. Median age at presentation was 68 years (range 42-88). A specific diagnosis was reached in 53% of patients: Inflammatory myopathy (n = 16), myopathy with rimmed vacuoles (n = 10), radiation-induced myopathy (n = 8), sporadic late-onset nemaline myopathy (n = 7), myofibrillar myopathy (n = 4), facioscapulohumeral dystrophy (n = 3), inclusion body myositis (n = 2), mitochondrial myopathy (n = 2), scleroderma-associated myopathy (n = 2), and single cases of necrotizing autoimmune myopathy, drug-induced myopathy, and B-cell chronic lymphocytic leukemia-myopathy. Splenius capitis had the highest diagnostic yield for a muscle biopsy (67%). When tested, 31/35 (89%) of patients had abnormal pulmonary function tests, 15/30 (50%) abnormal swallow evaluation, 24/65 (37%) abnormal electrocardiogram and 5/38 (13%) abnormal transthoracic echocardiogram. 23/43 (53%) treated patients responded to treatment. Patient-reported limb weakness and neck flexion weakness on physical examination were associated with good response to treatment. A wide spectrum of acquired and hereditary myopathies can present with head drop, some of which are potentially treatable. Establishing a diagnosis is crucial for timely treatment administration, screening for swallowing and cardiorespiratory involvement, and counseling regarding prognosis.

中文翻译：

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表现为头下垂的肌病：临床谱和治疗结果

低头综合征可能是多种神经系统疾病的表现特征。在这项研究中，我们旨在确定 107 名患者的临床特征和治疗结果，其中头部下垂是肌病的主要临床特征。就诊时的中位年龄为 68 岁（范围 42-88）。53% 的患者获得了特定诊断：炎症性肌病 (n = 16)、边缘空泡的肌病 ​​(n = 10)、辐射诱发的肌病 (n = 8)、散发性迟发性线状体肌病 (n = 7) , 肌原纤维肌病 (n = 4), 面肩肱型营养不良 (n = 3), 包涵体肌炎 (n = 2), 线粒体肌病 (n = 2), 硬皮病相关肌病 (n = 2), 和坏死性自身免疫病的单个病例肌病、药物性肌病和 B 细胞慢性淋巴细胞白血病-肌病。头脾的肌肉活检诊断率最高（67%）。测试时，31/35 (89%) 的患者肺功能检查异常，15/30 (50%) 吞咽评估异常，24/65 (37%) 异常心电图和 5/38 (13%) 异常经胸超声心动图。23/43 (53%) 接受治疗的患者对治疗有反应。体格检查时患者报告的肢体无力和颈部屈曲无力与对治疗的良好反应有关。广泛的获得性和遗传性肌病可表现为低头，其中一些可能是可以治疗的。建立诊断对于及时治疗管理、筛查吞咽和心肺受累以及预后咨询至关重要。31/35 (89%) 患者肺功能检查异常，15/30 (50%) 吞咽评估异常，24/65 (37%) 异常心电图和 5/38 (13%) 异常经胸超声心动图。23/43 (53%) 接受治疗的患者对治疗有反应。体格检查时患者报告的肢体无力和颈部屈曲无力与对治疗的良好反应有关。广泛的获得性和遗传性肌病可表现为低头，其中一些可能是可以治疗的。建立诊断对于及时治疗管理、筛查吞咽和心肺受累以及预后咨询至关重要。31/35 (89%) 患者肺功能检查异常，15/30 (50%) 吞咽评估异常，24/65 (37%) 异常心电图和 5/38 (13%) 异常经胸超声心动图。23/43 (53%) 接受治疗的患者对治疗有反应。体格检查时患者报告的肢体无力和颈部屈曲无力与对治疗的良好反应有关。广泛的获得性和遗传性肌病可表现为低头，其中一些可能是可以治疗的。建立诊断对于及时治疗管理、筛查吞咽和心肺受累以及预后咨询至关重要。23/43 (53%) 接受治疗的患者对治疗有反应。体格检查时患者报告的肢体无力和颈部屈曲无力与对治疗的良好反应有关。广泛的获得性和遗传性肌病可表现为低头，其中一些可能是可以治疗的。建立诊断对于及时治疗管理、筛查吞咽和心肺受累以及预后咨询至关重要。23/43 (53%) 接受治疗的患者对治疗有反应。体格检查时患者报告的肢体无力和颈部屈曲无力与对治疗的良好反应有关。广泛的获得性和遗传性肌病可表现为低头，其中一些可能是可以治疗的。建立诊断对于及时治疗管理、筛查吞咽和心肺受累以及预后咨询至关重要。