Isolated idiopathic hypomagnesemia presenting as aphasia and seizures
Isolated idiopathic hypomagnesemia presenting as aphasia and seizures
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DOI：
10.1016/j.pediatrneurol.2005.02.005
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发表时间：
2005-07-01
影响因子：
3.8
通讯作者：
Carney, PR
中科院分区：
文献类型：
--
作者：
Dharnidharka, VR;Carney, PR
孤立性低镁血症的特发性形式是一种罕见的条件，是目前已知的全面运动癫痫发作的儿童。本报告描述一位4岁非裔美国男性，以突发性失语为主要症状，无明显的初始运动性癫痫发作。评价显示孤立的重度低镁血症（初始血清镁水平< 1.0 mg/dL）和镁的肾脏处理不当（在低镁血症条件下，镁排泄分数> 40%）。该儿童随后出现全身强直阵挛性癫痫发作，经丙戊酸治疗和镁补充剂控制。诊断后6个月，他恢复了50-60%的语言能力，在首次发作后没有进一步的凝视或运动性癫痫发作。孤立性和特发性低镁血症所造成的缺陷肾重吸收镁是一种罕见的家族性疾病与可变的遗传。失语症作为一种孤立性的临床表现，以前没有被描述过。低镁性失语症和癫痫发作的确切病理生理学尚不清楚，但可能与特定类型谷氨酸受体的去抑制有关。在本例中，神经元去极化可能局限于颞叶的语言区域。(c)2005年，Elsevier Inc. All rights reserved.
Isolated hypomagnesemia of the idiopathic form is a rare condition that is known to present as generalized motor seizures in children. This report describes a 4-year-old African-American male who presented with a predominant symptom of sudden onset aphasia and no clear initial motor seizure activity. An evaluation revealed an isolated and severe hypomagnesemia (initial serum magnesium levels < 1.0 mg/dL) and inappropriate renal handling of magnesium (fractional excretion of magnesium > 40% under conditions of hypomagnesemia). The child had subsequent generalized tonic-clonic seizures that were brought under control with valproic acid therapy and magnesium supplementation. Six months after the diagnosis, he had regained 50-60% of his speech and had no further staring spells or motor seizure activity after the initial episode. Isolated and idiopathic hypomagnesemia caused by defective renal reabsorption of magnesium is a rare familial condition with variable inheritance. Aphasia as the solitary presenting symptom has not been described before. The exact pathophysiology of hypomagnesemic aphasia and seizures is not known but may relate to disinhibition of specific types of glutamate receptors. In the present case, neuronal depolarization may have been localized to language areas in the temporal lobes. (c) 2005 by Elsevier Inc. All rights reserved.