Heiman-Patterson T D, Rosenberg H, Fletcher J E, Tahmoush A J
Department of Neurology, Hahnemann University, Philadelphia, PA 19102.
Muscle Nerve. 1988 May;11(5):453-7. doi: 10.1002/mus.880110507.
The association of malignant hyperthermia (MH) with neuromuscular disorders has been recognized since 1970. These disorders include central core disease, Duchenne muscular dystrophy, myotonia congenita, myotonic dystrophy, nonspecific myopathies, and King-Denborough syndrome. In order to assess the anesthetic risk of MH in the neuromuscular population, we performed halothane and caffeine contracture testing for MH susceptibility on biopsied muscle removed from 25 consecutive neuromuscular patients during diagnostic evaluation. Positive contracture tests were found in 7 of 18 patients with myopathic disorders and 3 of 7 patients with neurogenic disorders. Two of our patients had anesthetic events suggesting MH. These findings suggest that myopathic and neuropathic disorders share pathogenic mechanisms with MH, resulting in positive contracture tests and possibly leading to clinical events during anesthesia. Although there is controversy regarding the interpretation of a positive contracture test, contracture testing remains the most widely accepted test for MH susceptibility. Thus, a variety of neuromuscular disorders may be associated with MH susceptibility, and caution should be exercised during anesthesia in this group of patients.
自1970年以来,恶性高热(MH)与神经肌肉疾病之间的关联已得到认可。这些疾病包括中央轴空病、杜氏肌营养不良症、先天性肌强直、强直性肌营养不良症、非特异性肌病以及金-登博罗综合征。为了评估神经肌肉疾病患者发生MH的麻醉风险,我们对25例连续的神经肌肉疾病患者在诊断评估期间切除的活检肌肉进行了氟烷和咖啡因挛缩试验,以检测MH易感性。在18例肌病患者中有7例挛缩试验呈阳性,7例神经源性疾病患者中有3例呈阳性。我们的两名患者发生了提示MH的麻醉事件。这些发现表明,肌病和神经病变与MH具有共同的致病机制,导致挛缩试验呈阳性,并可能在麻醉期间引发临床事件。尽管对于阳性挛缩试验的解读存在争议,但挛缩试验仍然是最广泛接受的MH易感性检测方法。因此,多种神经肌肉疾病可能与MH易感性相关,对这组患者进行麻醉时应谨慎。