Gómez-Lado C, Martinón-Torres F, Alvarez-Moreno A, Eirís-Puñal J, Carreira-Sande N, Rodriguez-Nuñez A, Castro-Gago M
Hospital Clinico Universitario de Santiago de Compostela, Santiago de Compostela, Spain.
Rev Neurol. 2007;44(8):475-8.
Posterior reversible leukoencephalopathy syndrome (PRLS) is characterised by the sudden and usually transient onset of headaches, visual disorders, decreased awareness and convulsions associated with cerebral oedema in the posterior areas of the brain. Different reports have associated it with numerous processes and possibly with haemolytic-uremic syndrome (HUS). We describe a new case of PRLS within the context of HUS.
We describe the case of a 4-year-old child with HUS who developed arterial hypertension that did not respond well to treatment and kidney failure that required renal replacement therapy. During the course of the disease the patient presented several transient episodes of headache, a lowered level of consciousness and seizures. Magnetic resonance imaging revealed multiple lesions in the white matter and the cortex that were hyperintense in T2 and hypointense in T1 situated in the bilateral occipital and right frontotemporoparietal regions. The patient made a full clinical recovery in a few days, although an magnetic resonance imaging scan performed at six weeks after the last episode showed partial remission of the lesions and a small cerebral infarction.
The pathogenesis of PRLS is still not fully understood. Its multifactorial origin suggests that several different mechanisms may be involved. In the case reported here, it is possible that the arterial hypertension, retention of fluids and even the sessions of dialysis played a role in its development.
后部可逆性白质脑病综合征(PRLS)的特征是突然且通常为短暂性发作的头痛、视觉障碍、意识减退以及与脑后部区域脑水肿相关的惊厥。不同报告将其与多种病症相关联,可能还与溶血尿毒综合征(HUS)有关。我们在此描述一例HUS背景下的PRLS新病例。
我们描述了一名患有HUS的4岁儿童病例,该患儿出现对治疗反应不佳的动脉高血压以及需要肾脏替代治疗的肾衰竭。在疾病过程中,患者出现了几次短暂的头痛发作、意识水平降低和癫痫发作。磁共振成像显示双侧枕叶和右侧额颞顶叶区域的白质和皮质有多个T2高信号、T1低信号的病变。患者在数天内实现了完全临床康复,尽管在最后一次发作六周后进行的磁共振成像扫描显示病变部分缓解且有一个小的脑梗死。
PRLS的发病机制仍未完全明确。其多因素起源表明可能涉及几种不同的机制。在本报告的病例中,动脉高血压、液体潴留甚至透析治疗可能在其发展过程中起了作用。