A nutritional Vitamin B12 deficiency (infantile tremor syndrome) presenting as hemiconvulsion hemiplegia epilepsy syndrome

V.K. Gowda, V. R. Challa, Varunvenkat M Srinivasan, Dhananjaya K. Vamyanmane
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Abstract

Hemiplegia Hemiconvulsion Epilepsy syndrome (HHE syndrome) is a condition characterized by the acute onset of unilateral seizures, which progress to cortical atrophy, hemiplegia and later develop epilepsy over weeks to years. Various aetiologies include infectious causes, especially meningitis, encephalitis, trauma, vascular insults, and sometimes idiopathic. Here, we present a 13-month-old female child, first in birth order, born to a non-consanguineous marriage, who presented with mild global developmental delay with acute onset right focal seizures followed by right-sided weakness and drug-resistant epilepsy. The child was found to have clinical and biochemical features of vitamin B12 deficiency with right-sided weakness. MRI of the brain initially showed edema of the left cerebral hemisphere and later atrophy on the left side. The electroencephalogram showed low voltage activity over the left cerebral hemisphere. Post-treatment with cobalamin, improvement in development was noted, with partial improvement in weakness and persistent epilepsy. In the current report, we highlight Hemiplegia Hemiconvulsion Epilepsy (HHE) syndrome on the background of nutritional vitamin B12 deficiency.
营养性维生素 B12 缺乏症(婴儿震颤综合征)表现为偏瘫偏瘫癫痫综合征
偏瘫半抽搐癫痫综合征(HHE 综合征)是一种以单侧癫痫急性发作为特征的疾病,经过数周至数年的发展,会出现大脑皮层萎缩、偏瘫,随后发展为癫痫。病因多种多样,包括感染性原因,尤其是脑膜炎、脑炎、外伤、血管损伤,有时也可能是特发性的。在此,我们介绍一名 13 个月大的女婴,她出生时排行第一,非近亲结婚,出现轻度全身发育迟缓,右侧局灶性癫痫急性发作,随后出现右侧肢体无力和耐药性癫痫。该患儿被发现具有维生素B12缺乏症的临床和生化特征,并伴有右侧肢体无力。脑部核磁共振成像最初显示左侧大脑半球水肿,后来显示左侧大脑半球萎缩。脑电图显示左侧大脑半球有低电压活动。使用钴胺素治疗后,患者的发育有所改善,乏力和癫痫持续状态也得到部分改善。在本报告中,我们重点介绍了以营养性维生素 B12 缺乏为背景的偏瘫抽搐癫痫(HHE)综合征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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