Khaleeli A A, Levy R D, Edwards R H, McPhail G, Mills K R, Round J M, Betteridge D J
J Neurol Neurosurg Psychiatry. 1984 Sep;47(9):1009-15. doi: 10.1136/jnnp.47.9.1009.
A study of the neuromuscular features of acromegaly was performed in six patients. Clinical assessment was supplemented by quadriceps force measurements, plasma creatine kinase (CK) activities, electromyography (EMG) and nerve conduction studies. Muscle mass was measured by urinary creatinine/height indices (CHI) and cross sectional area (CSA) of thighs and calves on computed tomography. Quadriceps force/unit cross sectional area was derived. Needle biopsies of vastus lateralis were studied by histochemical and ultrastructural methods. Mean fibre area (MFA) and fibre type proportions were measured. Most of the subjects studied had muscle pain and proximal muscle weakness confirmed by quadriceps force measurements. This occurred in the absence of muscle wasting, as shown by cross sectional area measurements and normal or raised creatinine/height indices. "Myopathic" features were demonstrated by needle biopsy in half the patients and occasionally by electromyography and raised plasma creatine kinase activity. Abnormalities on needle biopsy included variation in fibre size, type 2 fibre atrophy and large type 1 MFA relative to type 2 MFA. Electronmicroscopy showed the non-specific findings of increased glycogen accumulation, excess lipofuscin pigment and myofilament loss.
对6例肢端肥大症患者的神经肌肉特征进行了研究。通过股四头肌力量测量、血浆肌酸激酶(CK)活性、肌电图(EMG)和神经传导研究对临床评估进行补充。通过尿肌酐/身高指数(CHI)以及计算机断层扫描测量大腿和小腿的横截面积来测定肌肉质量。计算股四头肌力量/单位横截面积。通过组织化学和超微结构方法对股外侧肌进行针吸活检。测量平均纤维面积(MFA)和纤维类型比例。大多数研究对象存在肌肉疼痛和近端肌无力,股四头肌力量测量证实了这一点。如横截面积测量以及肌酐/身高指数正常或升高所示,这一情况发生在无肌肉萎缩的情况下。半数患者通过针吸活检显示出“肌病性”特征,偶尔通过肌电图和血浆肌酸激酶活性升高显示出来。针吸活检异常包括纤维大小变异、2型纤维萎缩以及相对于2型MFA而言1型MFA增大。电子显微镜显示出糖原积累增加、脂褐素色素过多和肌丝丢失等非特异性表现。