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一例慢性肾衰竭儿童患者的脑桥和脑桥外髓鞘溶解症罕见病例。

A rare case of pontine and extrapontine myelinolysis in a pediatric patient with chronic renal failure.

机构信息

Department of Pediatric Neurology, Adana City Education and Research Hospital, Adana, Turkey.

Department of Nephrology, Adana City Education and Research Hospital, Adana, Turkey.

出版信息

Childs Nerv Syst. 2021 Mar;37(3):1025-1027. doi: 10.1007/s00381-020-04720-5. Epub 2020 Jun 6.

Abstract

Osmotic demyelination syndrome (ODS) is a very rare condition in childhood occurring usually secondary to the rapid increase of serum sodium levels. This situation occurring secondary to the rapid correction of hyponatremia can be seen more rarely in the form of extrapontine myelinolysis and even the coexistence of these two conditions besides central pontine demyelinolysis. However, osmotic demyelination syndrome due to the rapid correction of hyponatremia in chronic renal failure (CRF) patients is very rare depending on existing uremia. In this article, we present an extremely rare case of pontine and extrapontine myelinolysis, which occurred in a pediatric patient with chronic renal failure, secondary to the rapid correction of hyponatremia. In the diffusion and cranial magnetic resonance imaging (MRI), bilateral symmetrical caudate, putamen, and thalamus involvements and hyperintense linear lesions at the pons, cortical, and subcortical areas were revealed. It was evaluated as pontine and extrapontine myelinolysis. This clinical situation presents that the presence of severe hyponatremia and extremely rapid correction of it can develop pontine and extrapontine myelinolysis even though it is very rare in uremic patients.

摘要

渗透性脱髓鞘综合征(ODS)是一种在儿童中非常罕见的疾病,通常继发于血清钠水平的快速升高。这种继发于低钠血症快速纠正的情况在桥外髓鞘溶解症中更为少见,甚至这两种情况同时存在,除了桥脑中心性脱髓鞘。然而,在慢性肾衰竭(CRF)患者中,由于存在尿毒症,低钠血症的快速纠正导致的渗透性脱髓鞘综合征非常罕见。在本文中,我们报告了一例非常罕见的儿童慢性肾衰竭患者,由于低钠血症的快速纠正,发生了桥脑和桥外髓鞘溶解症。在弥散和颅磁共振成像(MRI)上,双侧尾状核、壳核和丘脑对称性受累,桥脑、皮质和皮质下区呈现高信号线性病变。评估为桥脑和桥外髓鞘溶解症。这种临床情况表明,严重的低钠血症和极其快速的纠正,即使在尿毒症患者中也可能导致桥脑和桥外髓鞘溶解症。

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