Morino Kotaro, Kita Yasusi, Yoneda Yukihiro, Tabuchi Masayasu, Nishino Ichizo, Nonaka Ikuya
Neurology Service, Hyogo Brain and Heart Center at Himeji.
Rinsho Shinkeigaku. 2003 Sep;43(9):556-9.
We reported a patient with so-called "dropped head plus syndrome", a non-progressive myopathy characterized by striking neck extensor weakness followed by progressive limb weakness which is well controlled by azathioprine. This 74-year-old man complained of neck and shoulder fatigue followed by progressive neck extensor weakness over 7 months. On physical examination, neck extensor strength was 4/5 on the MMT scale, but the remainder of the neurological examination and blood tests were normal. Needle EMG showed myopathic changes in the right trapezius and biceps brachialis muscles. Without any treatment, he developed weakness of upper and lower limb muscles about 8 months later, and Gowers' sign was positive. Blood test and needle EMG was unchanged. Muscle biopsy of the left trapezius, deltoid and supraspinatus muscles disclosed nonspecific myopathic changes consisting of variation in fiber size with disorganized intermyofibrillar networks in all the fibers, and there were rimmed vacuoles in some fibers of the deltoid muscle. There was no change on corticosteroids, but there was a dramatic improvement with azathioprine and his muscle symptoms disappeared. We, therefore, made the diagnosis of "dropped head plus syndrome". This appears to be the first such case in Japan.
我们报告了一名患有所谓“低头加综合征”的患者，这是一种非进行性肌病，其特征为显著的颈伸肌无力，随后出现进行性肢体无力，硫唑嘌呤可有效控制病情。这名74岁男性在7个月内先是出现颈部和肩部疲劳，随后颈伸肌无力逐渐加重。体格检查时，根据医学研究委员会（MMT）肌力评级标准，颈伸肌力量为4/5，但其余神经系统检查和血液检查均正常。针极肌电图显示右侧斜方肌和肱二头肌有肌病性改变。未经任何治疗，约8个月后他出现了上肢和下肢肌肉无力，且Gowers征阳性。血液检查和针极肌电图结果未变。对左侧斜方肌、三角肌和冈上肌进行肌肉活检，发现非特异性肌病性改变，表现为所有肌纤维大小不一，肌原纤维网络紊乱，三角肌的一些肌纤维中有镶边空泡。使用皮质类固醇治疗无效，但使用硫唑嘌呤后病情显著改善，其肌肉症状消失。因此，我们诊断为“低头加综合征”。这似乎是日本首例此类病例。
