Drain Joseph P, Virk Sohrab S, Jain Nikhil, Yu Elizabeth
Department of Orthopaedics, The Ohio State University Wexner Medical Center, Columbus, OH.
Clin Spine Surg. 2019 Dec;32(10):423-429. doi: 10.1097/BSD.0000000000000811.
This study was a systematic review.
To reveal demographic factors, etiologies, response to treatment, and to propose a novel treatment algorithm for dropped head syndrome (DHS).
DHS is a rare condition defined by weakness of the cervical paraspinal muscles resulting in passively correctable flexion of the cervical spine. Patients present with neck pain, difficulty eating, and impaired horizontal gaze. Because of the rarity of DHS, a paucity of information exists with regard to demographics, etiology, and relative superiority of medical and surgical treatment.
We conducted a systematic literature review by searching PubMed for "dropped head syndrome," "chin on chest," "isolated neck extensor myopathy" (INEM), and "camptocephalia." Inclusion criteria were English-language articles that applied a specific treatment regimen with outcome data. A binomial logistic regression analysis was then performed to determine which covariates (age, sex, and treatments) were predictive of a positive response to treatment.
A total of 129 patients were described in 74 studies. Mean age was 63.6 and 63% were female. More than two-thirds of all patients fell into just 4 diagnostic categories (isolated neck extensor myopathy, 31.8%; Parkinson's, 20.2%; myasthenia gravis, 12.4%; amyotrophic lateral sclerosis, 7.0%). Overall positive response to treatment was 64.3%; primary medical treatment (73.5%), immune suppression (78.9%), and a combination of both (87.5%). Surgery was 93.8% successful. A treatment algorithm focused on appropriate diagnosis, initial medical management, with surgical evaluation only after failure of medical treatment was proposed.
Treatment for DHS starts with accurate diagnosis of the underlying etiology and is often associated with neuromuscular disease. A treatment algorithm for appropriate management of these patients was proposed. A trial of medical management and/or immunomodulators is warranted. Failing nonoperative management, surgery is predictive of a positive outcome.
Level V.
本研究为一项系统评价。
揭示人口统计学因素、病因、治疗反应，并为低头综合征（DHS）提出一种新的治疗算法。
DHS是一种罕见疾病，其定义为颈旁脊柱肌肉无力导致颈椎被动可矫正性屈曲。患者表现为颈部疼痛、进食困难和水平凝视受损。由于DHS罕见，关于人口统计学、病因以及药物和手术治疗的相对优势的信息匮乏。
我们通过在PubMed上搜索“低头综合征”“下巴抵胸”“孤立性颈伸肌肌病”（INEM）和“头后仰畸形”进行系统文献综述。纳入标准为应用特定治疗方案并具有结局数据的英文文章。然后进行二项逻辑回归分析以确定哪些协变量（年龄、性别和治疗方法）可预测治疗的阳性反应。
74项研究共描述了129例患者。平均年龄为63.6岁，63%为女性。超过三分之二的患者仅属于4种诊断类别（孤立性颈伸肌肌病，31.8%；帕金森病，20.2%；重症肌无力，12.4%；肌萎缩侧索硬化，7.0%）。总体治疗阳性反应率为64.3%；主要药物治疗（73.5%）、免疫抑制（78.9%）以及两者联合（87.5%）。手术成功率为93.8%。提出了一种治疗算法，重点在于准确诊断、初始药物治疗，仅在药物治疗失败后进行手术评估。
DHS的治疗始于对潜在病因的准确诊断，且常与神经肌肉疾病相关。提出了一种对这些患者进行适当管理的治疗算法。有必要进行药物管理和/或免疫调节剂试验。非手术治疗失败后，手术可预测阳性结局。
V级
