Marois C, Quirins M, Hermann B, Mouri S, Bouzbib C, Rudler M, Thabut D, Weiss N
Unité de réanimation neurologique, département de neurologie, Sorbonne université, groupement hospitalier Pitié-Salpêtrière Charles-Foix, Assistance publique des hôpitaux de Paris (AP-HP), 75013 Paris, France.
Service de neurologie adulte, hôpital Bicêtre, Assistance publique des hôpitaux de Paris (AP-HP), 94250 Le Kremlin-Bicêtre, France.
Rev Med Interne. 2019 Feb;40(2):88-97. doi: 10.1016/j.revmed.2018.11.006. Epub 2019 Jan 10.
Metabolic encephalopathies (ME) are a common cause of admission to emergency rooms, to hospitalization wards or to intensive care units. They could account for 10 to 20% of causes of comatose states in ICU and could be associated to a poor outcome especially in older patients. Nevertheless, they are often reversible and are associated with a favorable outcome when diagnosed and rapidly treated. They correspond to an altered brain functioning secondary to the deficiency of a substance that is mandatory for the normal brain functioning or to the accumulation of a substance that can be either endogenous or exogenous. It preferably occurs in co-morbid patients, complicating its diagnosis and its management. Altered brain functioning, going from mild neuropsychological impairment to coma, movement disorders especially myoclonus and the absence of any obvious differential diagnosis are highly suggestive of the diagnosis. Whereas some biological samplings and brain MRI are essential to rule out differential diagnosis, some others, such as electroencephalogram, may be able to propose important clues in favor of the diagnosis. Once simple symptomatic measures are introduced, the treatment consists mainly in the correction of the cause. Specific treatment options are only seldom available for ME; this is the case for hepatic encephalopathy and some drug-induced encephalopathies. We will successively describe in this review the main pathophysiological mechanisms, the main causes, favoring circumstances of ME, the differential diagnosis to rule out and the etiological work-up for the diagnosis. Finally, a diagnostic and therapeutic strategy for the care of patients with ME will be proposed.
代谢性脑病（ME）是急诊室、住院病房或重症监护病房常见的收治原因。它们可能占重症监护病房昏迷状态病因的10%至20%，尤其在老年患者中可能与不良预后相关。然而，它们通常是可逆的，若能得到诊断并迅速治疗，则预后良好。它们是指由于正常脑功能所需物质缺乏或内源性或外源性物质蓄积继发的脑功能改变。其好发于合并其他疾病的患者，这使其诊断和管理变得复杂。脑功能改变，从轻度神经心理损害到昏迷、运动障碍尤其是肌阵挛，以及缺乏任何明显的鉴别诊断，都高度提示该病的诊断。虽然一些生物学检查和脑部磁共振成像对于排除鉴别诊断至关重要，但其他一些检查，如脑电图，可能能够提供支持诊断的重要线索。一旦采取了简单的对症措施，治疗主要在于病因的纠正。代谢性脑病很少有特异性治疗方案；肝性脑病和一些药物性脑病就是这种情况。在本综述中，我们将依次描述代谢性脑病的主要病理生理机制、主要病因、诱发因素、需要排除的鉴别诊断以及诊断的病因学检查。最后，将提出针对代谢性脑病患者的诊断和治疗策略。
