Kleppe B, Reimers C D, Altmann C, Pongratz D E
Friedrich-Baur-Institut, Ludwig-Maximilians-Universität München.
Med Klin (Munich). 1995 Nov 15;90(11):623-7.
In outpatient clinics for neuromuscular diseases sometimes patients are presented because of an raised activity of the serum creatine kinase (CK) activity, accidentally detected. The purpose of this study was to investigate, how many patients with an unexplained hyperCKemia really suffer from a neuromuscular disease, and to present an adequate procedure for evaluating pathological CK activities.
In this retrospective study, clinical, electromyographic and myopathological findings of 100 consecutive patients (38 women and 62 men, aged 19 to 78 years) with such an abnormality of unknown origin are presented.
Only 41 patients really had no muscular troubles and no signs of neuromuscular disorders at physical examination. The CK of 12 patients turned to normal at follow-up examination. In 4 patients a so-called makro-CK was found. In 50 patients, in-depth examinations including a muscle biopsy were performed. In 10 patients, the increased CK-activity could be attributed to a complication of an already known basic disease, e.g., by confirming a myositis in case of proven connective tissue disease. In 14 patients an independent muscle disease was proven: 3 degenerative, 5 metabolic or mitochondrial as well as 4-inflammatory myopathies. In 2 patients, a secondary myopathy in polyneuropathy was suspected to be the etiology of the increased CK activity. In 9 patients, well treatable disorders were discovered. In 34 patients, no muscle biopsy was performed and follow-up examinations were recommended. The ratio of the clarified diagnoses did not depend on the level of the CK activity.
Every hyperCKemia indicates a thorough internal and neurological examination independently from the level of the enzyme activity. A scheme for examination in case of abnormal CK activity is proposed.
在神经肌肉疾病门诊中,有时会有患者因血清肌酸激酶(CK)活性升高而前来就诊,这种情况是偶然发现的。本研究的目的是调查有多少不明原因的高肌酸激酶血症患者真正患有神经肌肉疾病,并提出评估病理性CK活性的适当程序。
在这项回顾性研究中,呈现了100例连续患者(38名女性和62名男性,年龄19至78岁)的临床、肌电图和肌病理检查结果,这些患者均有不明原因的此类异常。
仅41例患者在体格检查时确实没有肌肉问题且没有神经肌肉疾病的体征。12例患者的CK在随访检查时恢复正常。4例患者发现了所谓的巨CK。对50例患者进行了包括肌肉活检在内的深入检查。10例患者CK活性升高可归因于已知基础疾病的并发症,例如在确诊结缔组织病时证实有肌炎。14例患者被证实患有独立的肌肉疾病:3例为退行性、5例为代谢性或线粒体性以及4例为炎性肌病。2例患者怀疑多神经病中的继发性肌病是CK活性升高的病因。9例患者发现了可有效治疗的疾病。34例患者未进行肌肉活检,建议进行随访检查。明确诊断的比例不取决于CK活性水平。
每例高肌酸激酶血症均表明应独立于酶活性水平进行全面的内科和神经科检查。提出了CK活性异常时的检查方案。