威尔逊氏病的孤立神经症状表现为局灶性癫痫发作而不累及肝脏:来自病例报告的见解。

IF 1.4 4区 医学 Q4 MEDICINE, RESEARCH & EXPERIMENTAL
Journal of International Medical Research Pub Date : 2025-04-01 Epub Date: 2025-04-11 DOI:10.1177/03000605251328574
Ayham Qatza, Ahmad Almohamed, Rima Alassaad, Saja Karaja, Ghina Hamsho, Ghiath Alassad
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引用次数: 0

摘要

Wilson病是一种由ATP7B突变引起的常染色体隐性遗传病,患者表现为肝脏和神经系统症状,包括震颤、舞蹈病、人格改变,以及神经病变、自主神经功能障碍、头痛和癫痫等罕见症状。本报告描述的情况下,一个14岁的男子出生的近亲父母谁提出局灶性癫痫发作和口腔下颌肌张力障碍。神经学检查发现左上肢张力不足。脑电图显示右半球癫痫样活动,磁共振成像显示双侧基底节区高信号。眼科检查发现一个不完整的凯瑟-弗莱舍环。实验室检查证实威尔逊病血清铜蓝蛋白低(3毫克/分升)和尿铜排泄量升高(1226微克/24小时)。治疗包括青霉胺(250毫克/天)和锌(50毫克,每天两次),以及氯硝西泮用于癫痫发作。建议进行常规随访。本病例强调了识别威尔逊氏病患者的神经学表现对及时诊断和治疗的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Isolated neurological symptoms of Wilson's disease manifesting as focal epileptic seizures without hepatic involvement: Insights from a case report.

Patients with Wilson's disease, an autosomal recessive disorder caused by ATP7B mutations, present with hepatic and neurological symptoms, including tremors, chorea, personality changes, and rare manifestations such as neuropathy, autonomic dysfunction, headache, and epilepsy. This report describes the case of a 14-year-old man born to consanguineous parents who presented with focal seizures and oromandibular dystonia. A neurological exam revealed left upper limb hypotonia. An electroencephalogram showed right hemisphere epileptiform activity, and magnetic resonance imaging indicated bilateral basal ganglia hyperintensities. An ophthalmological exam revealed an incomplete Kayser-Fleischer ring. Laboratory tests confirmed Wilson's disease with low serum ceruloplasmin (3 mg/dL) and elevated urinary copper excretion (1226 mcg/24 h) levels. Treatment included penicillamine (250 mg/day) and zinc (50 mg bi-daily), along with clonazepam for seizures. Routine follow-ups were recommended. This case highlights the importance of recognizing neurological presentations in patients with Wilson's disease for timely diagnosis and management.

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来源期刊
CiteScore
3.20
自引率
0.00%
发文量
555
审稿时长
1 months
期刊介绍: _Journal of International Medical Research_ is a leading international journal for rapid publication of original medical, pre-clinical and clinical research, reviews, preliminary and pilot studies on a page charge basis. As a service to authors, every article accepted by peer review will be given a full technical edit to make papers as accessible and readable to the international medical community as rapidly as possible. Once the technical edit queries have been answered to the satisfaction of the journal, the paper will be published and made available freely to everyone under a creative commons licence. Symposium proceedings, summaries of presentations or collections of medical, pre-clinical or clinical data on a specific topic are welcome for publication as supplements. Print ISSN: 0300-0605
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