Incidence of Neuralgic Amyotrophy (Parsonage Turner Syndrome) in a Primary Care Setting - A Prospective Cohort Study

ObjectiveNeuralgic amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic amyotrophy in a primary care setting.MethodsIn a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic amyotrophy. Neuralgic amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation.ResultsOf the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic amyotrophy of 1 per 1000.ConclusionsOur findings suggest that neuralgic amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.研究目的：神经痛性肌萎缩（Neuralgic Amyotrophy）被认为是一种罕见的周围神经系统疾病，但实际临床中却存在严重的识别不足问题，这对该类患者的诊疗造成了负面影响。本研究旨在前瞻性统计基层医疗环境下典型神经痛性肌萎缩的一年发病率。 研究方法：本研究于2012年开展前瞻性队列研究，纳入两家服务人口达14118人的大型基层医疗中心的所有新发颈、肩或上肢不适病例。研究开展前，全科医生已接受关于典型神经痛性肌萎缩诊断方法的短期培训。本研究参照已发表的诊断标准定义神经痛性肌萎缩，不考虑家族病史。仅具有典型表型的患者才被计为确诊病例。纳入研究后，疑似罹患神经痛性肌萎缩且尚未就诊神经内科的患者，将被安排接受神经内科评估以确认诊断。 研究结果：在492名新发颈、肩或上肢不适的患者中，共有34名被疑似罹患神经痛性肌萎缩。经神经内科评估后，14名患者的诊断得到确认。据此计算，典型神经痛性肌萎缩的一年发病率为1‰。 研究结论：本研究结果显示，神经痛性肌萎缩的发病率较此前认知高出30~50倍。对该疾病及其临床表现缺乏认知，是造成这一差异的最可能原因。鉴于其发病率可达1‰，且多数患者会遗留长期后遗症，临床应提高对该疾病的诊断警惕性，以便为及时治疗铺平道路并预防并发症。