朗讀老師：Jane   天津某醫(yī)院

翻譯老師：章維    昆明醫(yī)科大學第二附屬醫(yī)院

審校老師：姜春雷   青島市第九人民醫(yī)院

62-year-old man with progressive weakness, gait deficiency

62歲男性患者，進行性肌無力和步態(tài)不穩(wěn)

History: A 62-year-old alcoholic man presents with progressive gait deficiency and generalized weakness.

病史：62歲男性，長期酗酒，出現(xiàn)進行性步態(tài)不穩(wěn)和全身乏力癥狀。

A brain MRI with stroke protocol was performed.

患者接受了常規(guī)顱腦MRI檢查，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.

橫斷位液體衰減反轉(zhuǎn)恢復序列（FLAIR）、梯度回波序列（GRE）、彌散加權(quán)成像（DWI）以及表面彌散系數(shù)圖（ADC）顯示如下：

Findings

影像表現(xiàn)

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，腦橋中央彌散受限，周圍未受累及，同時，在FLAIR序列，病變呈高信號。病變無出血、占位效應或者腦積水。

Differential diagnosis

鑒別診斷

Central pontine myelinolysis (osmotic demyelination syndrome)

腦橋中央髓鞘溶解癥（滲透性脫髓鞘綜合征）

Acute infarct

急性腦梗塞

Acute carbon monoxide poisoning

急性一氧化碳中毒

Multiple sclerosis

多發(fā)性硬化

Pontine astrocytoma

腦橋星形細胞瘤

Wernicke encephalopathy

韋尼克腦病

Hypoxic ischemic encephalopathy

缺血缺氧性腦病

Cocaine overdose

可卡因過量

Hepatic encephalopathy

肝性腦病

Diagnosis: Central pontine myelinolysis (osmotic demyelination syndrome)

診斷：腦橋中央髓鞘溶解癥（滲透性脫髓鞘綜合征）

Discussion

討論

Central pontine myelinolysis (osmotic demyelination syndrome)

腦橋中央髓鞘溶解癥（滲透性脫髓鞘綜合征）

Epidemiology and pathogenesis

流行病學和發(fā)病機制

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.

腦橋中央髓鞘溶解癥于1959年首次被描述為一種見于酗酒者和營養(yǎng)不良患者的疾病，患者伴有痙攣性四肢癱瘓，并在尸檢中發(fā)現(xiàn)腦橋受損。

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.

之后研究發(fā)現(xiàn)，這種情況與快速糾正的低鈉血癥（大于12 mmol/L/天）密切相關，而且大腦的其他部分也會受到影響，因此，被稱為橋外髓鞘溶解癥，通常與腦橋受累同時發(fā)生，但是偶爾也會單獨發(fā)生。

Osmotic demyelination syndrome is used to encompass both entities.

滲透性脫髓鞘綜合征包含了腦橋和橋外髓鞘溶解癥。

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.

該病的發(fā)病機制尚不完全清楚，但認為與快速糾正血鈉水平的滲透作用引起的少突膠質(zhì)細胞的髓鞘內(nèi)分裂、空泡化和髓鞘破裂有關。

Middle-aged men are more commonly affected.

中年男性更容易受到此病影響。

Risk factors in addition to alcoholism and malnourishment include prolonged use of diuretics, liver failure, organ transplant, and extensive burns.

除了酗酒和營養(yǎng)不良外，危險因素還包括長期使用利尿劑、肝功能衰竭、器官移植和大面積燒傷。

Clinical presentation

臨床表現(xiàn)

Patients with severe hyponatremia and other electrolyte disturbances often present with seizures or encephalopathy.

嚴重低鈉血癥和其他電解質(zhì)紊亂的患者常出現(xiàn)癲癇發(fā)作或相關腦病。

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.

在快速糾正低鈉血癥后，這些癥狀可能會消失，但是隨后會迅速惡化，出現(xiàn)痙攣性四肢麻痹、假性球麻痹(頭頸部無力、吞咽困難和構(gòu)音障礙)和意識改變，通常會出現(xiàn)昏迷或譫妄。

Diagnosis

診斷

Clinical history and laboratory values are typically used to make the diagnosis.

通常依據(jù)臨床病史和實驗室檢查是可以做出診斷的。

Imaging findings can help and are often classic, but they may lag behind clinical symptoms by one to two weeks.

影像學檢查可以起到輔助診斷的作用，影像表現(xiàn)通常很典型，但是它們可能會落后于臨床癥狀一到兩周。

Imaging features

影像學特點

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.

顧名思義，這種情況通常發(fā)生在腦橋，但在更嚴重的病例中，可以在基底節(jié)、皮層下白質(zhì)、淺層皮質(zhì)和海馬看到腦橋外髓鞘溶解。

As with other metabolic/toxic sources, the effects are typically bilateral and symmetrical.

與其他代謝性或中毒性疾病一樣，其影響通常是雙側(cè)的和對稱的。

A symmetrical “trident-shaped” area of T2/FAIR hyperintensity in the central pons, sparing the periphery, is characteristic.

特征性影像表現(xiàn)為腦橋中央呈對稱的“三叉戟形”T2或FLAIR序列高信號病變，周邊不受累，比較有特征性。

The ventrolateral pons and pontine portion of the corticospinal tracts are typically spared.

腦橋部分的皮質(zhì)脊髓束以及腹外側(cè)的腦橋通常是不受累的。

Restricted diffusion usually occurs early, within 24 hours.

彌散受限通常發(fā)生在早期，24小時內(nèi)。

Enhancement is rare but has been reported in the acute phase.

對比增強后，病變罕見強化，但是有報道在急性期可見強化。

The extent of T2 signal abnormality has not been shown to be associated with clinical outcome.

病變的T2信號異常程度與臨床預后無關。

Treatment

治療

It is prevented by slowly correcting hyponatremia.

該病可以通過緩慢糾正低鈉血癥來預防。

Treatment consists of supportive care with some case reports showing that steroids and IV immunoglobulin may be helpful.

治療主要包括支持性療法，一些病例報告顯示類固醇和靜脈注射免疫球蛋白可能有效。