双语病例——脑桥中央髓鞘溶解症（渗透性脱髓鞘综合征）
朗读老师：Jane 天津某医院
翻译老师：章维 昆明医科大学第二附属医院
审校老师：姜春雷 青岛市第九人民医院
62-year-old man with progressive weakness, gait deficiency
62岁男性患者，进行性肌无力和步态不稳
History: A 62-year-old alcoholic man presents with progressive gait deficiency and generalized weakness.
病史：62岁男性，长期酗酒，出现进行性步态不稳和全身乏力症状。
A brain MRI with stroke protocol was performed.
患者接受了常规颅脑MRI检查，以除外卒中。
Axial fluid-attenuated inversion-recovery (FLAIR), gradient-recalled echo (GRE), diffusion-weighted (DWI), and apparent diffusion coefficient (ADC) images are shown below.
横断位液体衰减反转恢复序列（FLAIR）、梯度回波序列（GRE）、弥散加权成像（DWI）以及表面弥散系数图（ADC）显示如下：
Findings
影像表现
There is restricted diffusion in the central pons with sparing of the periphery and with corresponding FLAIR hyperintensity. The is no hemorrhage, mass effect, or hydrocephalus.
在DWI影像上，脑桥中央弥散受限，周围未受累及，同时，在FLAIR序列，病变呈高信号。病变无出血、占位效应或者脑积水。
Differential diagnosis
鉴别诊断
Central pontine myelinolysis (osmotic demyelination syndrome)
脑桥中央髓鞘溶解症（渗透性脱髓鞘综合征）
Acute infarct
急性脑梗塞
Acute carbon monoxide poisoning
急性一氧化碳中毒
Multiple sclerosis
多发性硬化
Pontine astrocytoma
脑桥星形细胞瘤
Wernicke encephalopathy
韦尼克脑病
Hypoxic ischemic encephalopathy
缺血缺氧性脑病
Cocaine overdose
可卡因过量
Hepatic encephalopathy
肝性脑病
Diagnosis: Central pontine myelinolysis (osmotic demyelination syndrome)
诊断：脑桥中央髓鞘溶解症（渗透性脱髓鞘综合征）
Discussion
讨论
Central pontine myelinolysis (osmotic demyelination syndrome)
脑桥中央髓鞘溶解症（渗透性脱髓鞘综合征）
Epidemiology and pathogenesis
流行病学和发病机制
Central pontine myelinolysis was first described in 1959 as a condition seen in alcoholics and malnourished patients with spastic quadriplegia found to have damage to the pons upon autopsy.
脑桥中央髓鞘溶解症于1959年首次被描述为一种见于酗酒者和营养不良患者的疾病，患者伴有痉挛性四肢瘫痪，并在尸检中发现脑桥受损。
The condition was later found to be strongly associated with rapid correction of hyponatremia (greater than 12 mmol/L/day) and that other parts of the brain could be involved, which was termed extrapontine myelinolysis and typically occurred in addition to pons involvement but was occasionally found to occur in isolation.
之后研究发现，这种情况与快速纠正的低钠血症（大于12 mmol/L/天）密切相关，而且大脑的其他部分也会受到影响，因此，被称为桥外髓鞘溶解症，通常与脑桥受累同时发生，但是偶尔也会单独发生。
Osmotic demyelination syndrome is used to encompass both entities.
渗透性脱髓鞘综合征包含了脑桥和桥外髓鞘溶解症。
The mechanism is not fully understood, but it is thought to be linked to intramyelinitic splitting, vacuolization, and rupture of myelin sheaths in oligodendrocytes due to osmotic effects of rapid correction of sodium levels.
该病的发病机制尚不完全清楚，但认为与快速纠正血钠水平的渗透作用引起的少突胶质细胞的髓鞘内分裂、空泡化和髓鞘破裂有关。
Middle-aged men are more commonly affected.
中年男性更容易受到此病影响。
Risk factors in addition to alcoholism and malnourishment include prolonged use of diuretics, liver failure, organ transplant, and extensive burns.
除了酗酒和营养不良外，危险因素还包括长期使用利尿剂、肝功能衰竭、器官移植和大面积烧伤。
Clinical presentation
临床表现
Patients with severe hyponatremia and other electrolyte disturbances often present with seizures or encephalopathy.
严重低钠血症和其他电解质紊乱的患者常出现癫痫发作或相关脑病。
After rapid correction of hyponatremia, those symptoms may resolve, followed by rapid deterioration, including spastic quadriplegia, pseudobulbar palsy (head and neck weakness, dysphagia, and dysarthria), and altered consciousness. Coma or delirium typically follows.
在快速纠正低钠血症后，这些症状可能会消失，但是随后会迅速恶化，出现痉挛性四肢麻痹、假性球麻痹(头颈部无力、吞咽困难和构音障碍)和意识改变，通常会出现昏迷或谵妄。
Diagnosis
诊断
Clinical history and laboratory values are typically used to make the diagnosis.
通常依据临床病史和实验室检查是可以做出诊断的。
Imaging findings can help and are often classic, but they may lag behind clinical symptoms by one to two weeks.
影像学检查可以起到辅助诊断的作用，影像表现通常很典型，但是它们可能会落后于临床症状一到两周。
Imaging features
影像学特点
As the names implies, this condition typically occurs in the pons, but in more severe cases, extrapontine myelinolysis can be seen in the basal ganglia, subcortical white matter, peripheral cortex, and hippocampus.
顾名思义，这种情况通常发生在脑桥，但在更严重的病例中，可以在基底节、皮层下白质、浅层皮质和海马看到脑桥外髓鞘溶解。
As with other metabolic/toxic sources, the effects are typically bilateral and symmetrical.
与其他代谢性或中毒性疾病一样，其影响通常是双侧的和对称的。
A symmetrical “trident-shaped” area of T2/FAIR hyperintensity in the central pons, sparing the periphery, is characteristic.
特征性影像表现为脑桥中央呈对称的“三叉戟形”T2或FLAIR序列高信号病变，周边不受累，比较有特征性。
The ventrolateral pons and pontine portion of the corticospinal tracts are typically spared.
脑桥部分的皮质脊髓束以及腹外侧的脑桥通常是不受累的。
Restricted diffusion usually occurs early, within 24 hours.
弥散受限通常发生在早期，24小时内。
Enhancement is rare but has been reported in the acute phase.
对比增强后，病变罕见强化，但是有报道在急性期可见强化。
The extent of T2 signal abnormality has not been shown to be associated with clinical outcome.
病变的T2信号异常程度与临床预后无关。
Treatment
治疗
It is prevented by slowly correcting hyponatremia.
该病可以通过缓慢纠正低钠血症来预防。
Treatment consists of supportive care with some case reports showing that steroids and IV immunoglobulin may be helpful.
治疗主要包括支持性疗法，一些病例报告显示类固醇和静脉注射免疫球蛋白可能有效。