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本文报告1例临床表现与面肌痛功能紊乱综合征(MPDS)咀嚼肌症候群相似的McArdle氏病。McArdle氏病是一种V型糖原沉积病,由于磷酸化酶缺乏引起的一种典型的肌糖原沉积紊乱,属罕见的常染色体隐性遗传病。磷酸化酶是骨骼肌中糖原转为1-磷酸葡萄糖的基本酶,由于此酶的缺乏,一方面糖原过多地沉积在肌肉中;另一方面糖原不能进行正常的糖酵解合成三磷酸腺甙(ATP)提供给肌收缩的能量。临床表现为运动性无耐受力、肌软弱、肌痛和触痛,此时如果继续运动,肌红蛋白从受损的肌组织中释放进入血流中,导致肌红蛋白尿和肾损害。 患者为21岁男性,双侧颞下颌关节痛7年,疼痛以颞下颌关节为中心向颞区和嚼肌区放射,其特点是
This article reports a case of McArdle disease with clinical manifestations similar to those of the Masticatory Muscle Syndrome of Facial Myalgia Syndrome (MPDS). McArdle's disease is a V-type glycogen deposition disease, due to a lack of phosphorylase caused by a typical muscle glycogen deposition disorders, is a rare autosomal recessive disease. Phosphorylase is the basic enzyme of glycogen in skeletal muscle converted to glucose 1-phosphate. Due to the lack of this enzyme, on the one hand, excessive deposition of glycogen in the muscle; on the other hand, glycogen can not undergo normal glycolysis Adenosine triphosphate (ATP) provides muscle contractile energy. Clinical manifestations are athletic intolerance, muscular weakness, myalgia and tenderness, when myoglobin is released from impaired muscle tissue into the bloodstream if it continues to move, leading to myoglobinuria and renal damage. The patient was 21 years old and had bilateral TMJ pain for 7 years. The pain radiates to the temporal and chewing muscles with the temporomandibular joint as its center.