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脂质沉积性肌病是肌肉中长链脂肪酸代谢障碍致脂质沉积在肌纤维中而引起的一组肌病。该病少见,国内报道甚少,50岁以上发病者更为少见。本文报告一例老年男性脂质沉积性肌病。肌肉活检显示肌纤维中有大量的脂肪颗粒沉积,以Ⅰ型纤维为主,经地塞米松,辅酶Q_(10)和低脂饮食治疗病情好转。 1 病例报告 患者,陈XX,男,56岁,已婚,住院号191729。因颈部无力5年,四肢无力2年于1996年3月12日入院。该患于1991年起无诱因出现颈部无力,抬头困难,曾在本单位医院诊断为“颈椎病”,经治疗无好转。近年2年来出现双下肢无力,每行几十米后双下肢发颤,早晚无差异,冬季加重,天气转暖时症状略有减轻。近3年月来,咀嚼肌无力,无吞咽困难及饮水呛咳,无肌肉疼痛感,活动后自觉心慌、气短。既往无特殊病史,无阳性家族吏。
Lipid-depositional myopathy is a group of myopathies caused by the deposition of lipid deposits in the muscle fibers by long-chain fatty acid metabolism disorders in the muscles. The disease is rare, rarely reported in the country, the incidence of more than 50 years of age are even more rare. This article reports an elderly male lipid deposition myopathy. Muscle biopsy showed a large number of muscle fibers in the deposition of fat particles, the main type Ⅰ fibers, dexamethasone, coenzyme Q_ (10) and low-fat diet condition improved. 1 case report patients, Chen XX, male, 56 years old, married, hospital number 191729. 5 years due to neck weakness, limb weakness 2 years in March 12, 1996 admission. The suffering from no incentive in 1991 appeared neck weakness, rise in difficulty, had a diagnosis in the unit of hospital “cervical spondylosis”, after treatment did not improve. Two years in recent years, the emergence of lower extremity weakness, tens of meters after each line of the lower extremities tremble, no difference in the morning and evening, winter increased, the weather warming slightly alleviate the symptoms. In recent 3 years, masticatory muscle weakness, no difficulty swallowing and drinking cough, no muscle pain, consciously palpitation after activity, shortness of breath. No previous history of a particular history, no positive family officials.