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[肢端肥大症中的肌病。两例报告]

[Myopathy in acromegaly. Report of two cases].

作者信息

Abe M, Tabuchi K, Fujii K, Oda K, Ishimoto S

机构信息

Department of Neurosurgery, Saga Medical School, Japan.

出版信息

No To Shinkei. 1990 Oct;42(10):923-7.

PMID:2288772
Abstract

Acromegaly is often associated with neuromuscular disorders. Most of them are caused by compression of nerves with hypertrophic bone and soft tissues or complications of diabetes mellitus. Myopathy has rarely been reported in the Japanese literature. We report two cases with myopathy out of 14 cases of acromegaly. Case 1 is a 62-year-old woman who developed muscle weakness and atrophy in the shoulder girdle, pelvic girdle and femoral regions after a 10-year history of acromegaly. She showed positive Gowers' sign and normal DTRs. Basal growth hormone (GH) level in plasma was 1076 ng/ml. Electromyograms (EMG) obtained from the deltoid and rectus femoris muscles revealed typical myopathic abnormalities; an excess of small-amplitude, short-duration, polyphasic motor unit potentials. Histological examinations of the rectus femoris muscle showed diffuse atrophy of both type I and type II fibers. She also had bilateral carpal tunnel syndrome and bilateral tarsal tunnel syndrome, which were confirmed by nerve conduction studies of median nerves and posterior tibial nerves. A cranial computed tomography (CT) scan demonstrated sellar mass with suprasellar extension. She underwent transsphenoidal adenomectomy and radiation therapy. GH level lowered to 29 ng/ml, however, myopathy remained unchanged for 3 years after the surgery. Case 2 is a 38-year-old woman who had undergone partial removal of a pituitary adenoma 9 years after the onset of acromegaly. Basal GH level in plasma before the surgery had been 1694 ng/ml and was still high after the surgery (100-505 ng/ml). The patient developed proximal muscle weakness and atrophy 4 years after the surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

肢端肥大症常与神经肌肉疾病相关。其中大多数是由肥厚的骨骼和软组织压迫神经或糖尿病并发症引起的。日本文献中很少报道肌病。我们报告了14例肢端肥大症患者中的2例肌病病例。病例1是一名62岁女性,在患肢端肥大症10年后,出现肩胛带、骨盆带和股部肌肉无力和萎缩。她表现出阳性Gowers征,腱反射正常。血浆基础生长激素(GH)水平为1076 ng/ml。从三角肌和股直肌获取的肌电图(EMG)显示典型的肌病异常;大量小振幅、短时限、多相运动单位电位。股直肌的组织学检查显示I型和II型纤维均弥漫性萎缩。她还患有双侧腕管综合征和双侧跗管综合征,经正中神经和胫后神经的神经传导研究证实。头颅计算机断层扫描(CT)显示蝶鞍肿块并向上延伸至鞍上。她接受了经蝶窦腺瘤切除术和放射治疗。GH水平降至29 ng/ml,但术后3年肌病仍无变化。病例2是一名38岁女性,在肢端肥大症发病9年后接受了垂体腺瘤部分切除术。手术前血浆基础GH水平为1694 ng/ml,术后仍很高(100 - 505 ng/ml)。患者在手术后4年出现近端肌肉无力和萎缩。(摘要截断于250字)

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