Terkawi Abdullah Sulieman, Wani Tariq M, Al-Shuaibi Khalid M, Tobias Joseph D
Department of Anesthesiology, King Fahad Medical City, Riyadh, Saudi Arabia.
Saudi J Anaesth. 2012 Apr;6(2):181-5. doi: 10.4103/1658-354X.97037.
Leigh disease is an extremely rare disorder, characterized by a progressive neurodegenerative course, with subacute necrotizing encephalomyelopathy. It usually presents in infancy with developmental delay, seizures, dysarthria, and ataxia. These patients may also develop episodes of lactic acidosis that usually lead to respiratory failure and death. Due to the rarity of the condition, the most appropriate anesthetic plan remains unclear. We present a patient with Leigh disease, who required general anesthesia. The pathogenesis of the disease is discussed and previous reports of perioperative care from the literature are reviewed.
Leigh病是一种极其罕见的疾病,其特征为进行性神经退行性病程,并伴有亚急性坏死性脑脊髓病。它通常在婴儿期出现,表现为发育迟缓、癫痫发作、构音障碍和共济失调。这些患者还可能出现乳酸酸中毒发作,通常会导致呼吸衰竭和死亡。由于该疾病罕见,最合适的麻醉方案仍不明确。我们报告一例需要全身麻醉的Leigh病患者。本文讨论了该疾病的发病机制,并回顾了文献中先前关于围手术期护理的报道。