Spinal Anesthesia in MELAS Syndrome: A Case with Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis and Stroke-Like Episodes

MELAS症候群是一種典型的粒腺體腦肌病，它侵犯身體各系統而在每個病人有不同表現。通常會有肌病變、腦病變、乳酸血症、及中風狀發作等症狀。會報告對肌肉松弛劑及其他麻醉藥品有延長作用時間、增加敏感性的效果。另琥珀膽酸(succinylcholine)亦易引起高血鉀。惡性高熟也曾被報告過。為避免這些反應，我們對一名行蘭尾切除術病人探取脊髓麻醉，并得到良好結果，沒有任何神經學方面的後遺症。但基於多神經病變(polylleuropathy)及脊髓脫髓鞘病變(demyelination)曾被報告在MELAS病人身上出現過，我們建議在脊髓麻醉施行前應經詳細神經學檢查，審慎排除此類病人，以防造成神經學方面後遺症。

關鍵字

MELAS症候群；脊髓麻醉

並列摘要

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the classic mitochondrial encephalomyopathies with variable clinical presentation and multisystem involvement. Enhanced sensitivity to neuromuscular blockade or anesthetic agents and susceptibility to malignant hyperthermia in these patients have ever been reported, all of which complicate the management of general anesthesia. To avoid these appaffing troubles in general anesthesia, we chose spinal anesthesia for a patient with MELAS syndrome receiving appendectomy. The patient obtained adequate anesthesia and good recovery without neurologic sequelae. Although there is little information about the application of regional anesthesia in MELAS patients, we demonstrate that it may be a satisfactory choice. However, it is suggested that regional anesthesia is performed only when neurological abnormalities of spinal cord or peripheral nerves are definitely ruled out.

並列關鍵字

MELAS syndrome ； Anesthesia: spinal

國際替代計量

Spinal Anesthesia in MELAS Syndrome: A Case with Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis and Stroke-Like Episodes

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