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十岁女孩因罕见病因致可逆性轴索性神经病和高血压

An unusual cause of reversible axonal neuropathy and hypertension in a 10-year-old girl.

机构信息

Department of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India.

出版信息

J Clin Neurosci. 2012 Aug;19(8):1196-7. doi: 10.1016/j.jocn.2011.10.018. Epub 2012 May 20.

Abstract

A 10-year-old girl, who was referred with refractory epilepsy, had 1.5 years of episodic abnormal behavior. On examination, she also had hypertension and peripheral neuropathy. Hypoglycemia with correspondingly high insulin levels was documented during a confusional episode. MRI of the abdomen revealed an islet cell tumor in the body of the pancreas. One year after tumor excision, both the neuropathy and hypertension showed remarkable improvement. A final diagnosis of insulinoma with hypoglycemic axonal neuropathy and hypertension (reversed with tumor excision) was made. Insulinoma is the commonest cause of hyperinsulinemic hypoglycemia in adults, but is rare in childhood. To our knowledge, distal symmetrical motor-sensory axonal neuropathy has been described in only 40 patients, and hypertension has not been reported with insulinoma.

摘要

一位 10 岁女孩因难治性癫痫就诊,有 1.5 年的间歇性异常行为。检查时,她还患有高血压和周围神经病。在一次意识障碍发作期间,记录到低血糖伴相应高胰岛素水平。腹部 MRI 显示胰腺体部有胰岛细胞瘤。肿瘤切除 1 年后,周围神经病和高血压均显著改善。最终诊断为胰岛素瘤伴低血糖性轴索性神经病和高血压(肿瘤切除后逆转)。胰岛素瘤是成人高胰岛素血症性低血糖的常见原因,但在儿童中很少见。据我们所知,仅有 40 例患者描述了远端对称性运动感觉轴索性神经病,而胰岛素瘤未报道过高血压。

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