Han Min Jeong, Kim Soon Chul, Joo Chan Uhng, Kim Sun Jun
Department of Pediatrics Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea.
Medicine (Baltimore). 2016 Sep;95(36):e4393. doi: 10.1097/MD.0000000000004393.
Cerebral Salt-Wasting Syndrome (CSWS) is characterized by hyponatremia and sodium wasting in the urine. These conditions are triggered by various neurosurgical disorders such as subarachnoid hemorrhage, brain tumor, head injury, and brain surgery. To our knowledge, CSWS caused by Wernicke encephalopathy (WE) has been rarely reported.
A 2-year-old male patient presented to our hospital due to a seizure attack. He had been neglected and refused to take food for a long time (body weight < 3rd percentile). During admission, the patient showed low serum osmolality, high urine osmolality, dehydration state, increased urine output, and negative water balance, a diagnosis of CSWS was made.
DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Brain MRI displayed symmetrical lesions of T2WI and FLAIR high signal intensity in the peri-aqueductal and hypothalamic areas, which suggests Wernicke encephalopathy. For the early diagnosis of WE, neuroimaging studies can be an important marker. Thiamine hydrochloride was administered at a dose of 100 mg/day for 3 weeks. Cerebral salt-wasting syndrome was subsequently diagnosed due to persistent hyponatremia, dehydrated state, and high urine sodium with massive urination.
Wernicke encephalopathy is a very rare cause of cerebral salt-wasting syndrome in pediatrics patients. The patient had a good outcome after hypertonic solution and fludrocortisone therapy.
脑性盐耗综合征(CSWS)的特征是低钠血症和尿钠排泄。这些情况由各种神经外科疾病引发,如蛛网膜下腔出血、脑肿瘤、头部损伤和脑部手术。据我们所知,由韦尼克脑病(WE)引起的CSWS鲜有报道。
一名2岁男性患者因癫痫发作前来我院就诊。他长期被忽视且拒食(体重低于第3百分位数)。入院期间,患者表现为低血清渗透压、高尿渗透压、脱水状态、尿量增加和负水平衡,诊断为CSWS。
诊断、干预措施及结果:脑部MRI显示中脑导水管周围和下丘脑区域T2WI及FLAIR序列呈对称高信号病变,提示韦尼克脑病。对于WE的早期诊断,神经影像学检查可能是一个重要指标。给予盐酸硫胺100mg/天,持续3周。随后,由于持续性低钠血症、脱水状态以及高尿钠伴大量排尿,诊断为脑性盐耗综合征。
韦尼克脑病是小儿患者脑性盐耗综合征非常罕见的病因。经高渗溶液和氟氢可的松治疗后,患者预后良好。