| 胡纪源,王共强,程楠,王训,洪铭范,韩咏竹,杨任民.肝豆状核变性合并癫痫的表现和中西医结合治疗的临床研究[J].,2004,(9):793-797 |
| 肝豆状核变性合并癫痫的表现和中西医结合治疗的临床研究 |
| Clinical Study on Manifestation of Hepatolenticular Degeneration Complicated with Epilepsy and Therapeutic Effect of Integrative Chinese and Western Medicine Treatment |
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| DOI： |
| 中文关键词: 肝豆状核变性 癫痫 颅脑影像 大脑损害 脑萎缩 二巯基丙磺酸钠 肝豆汤 |
| 英文关键词:hepatolenticular degeneration epilepsy cranial image cerebral lesion brain atrophy sodium dimercaptosulfonate Gandou Decoction |
| 基金项目:国家自然科学基金资助课题 (No .39570 878) |
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| 中文摘要: |
| 目的探讨 15 5例肝豆状核变性 (hepatolenticulardegeneration ,HLD)合并癫痫发作患者的临床表现和中西医结合治疗的效果。方法观察HLD合并癫痫发作临床表现及其与颅脑CT或 (及 )MRI异常所见的关系 ;采用二巯基丙磺酸钠 2 0mg/kg·d静脉滴注和中药肝豆汤加味口服综合驱铜 8～ 10个疗程 ,同时应用抗癫痫药 ,对临床疗效、尿铜及血清铜等结果进行比较。结果 15 5例HLD中 ,合并部分性癫痫发作96例、全身性发作 5 9例 ;72例行颅脑CT或 (及 )MRI检查者均有异常 (10 0 % ) ,除了HDL常见的基底节区对称性损害灶 (6 5例次 ,90 3% )及程度不等的脑萎缩 (6 1例次 ,84 7% )外 ,尚观察到 5 4例 (74 % )有大脑白质为主累及皮质的大片状损害灶 ,病灶多为双侧对称性或累及相邻的两个脑叶 ,其发生频率依次为额叶、顶叶、颞叶及扣带回 ,无上述病灶的患者均见明显的脑萎缩。 2 9例 (4 0 2 % )有脑电图异常 ,主要表现为中～高电位θ波或 (及 )短阵棘 -慢或尖 -慢综合波发放。患者治疗前尿铜为 (4 4 9± 1 93) μmol/2 4h ,治疗后尿铜为 (34 5 0± 2 1 6 0 ) μmol/2 4h ,较治疗前显著增高 (P <0 0 1) ,血清铜亦有所下降 (P <0 0 1) ,患者在锥体外系等症状逐渐改善的同时 ,癫痫发作亦得到完全控制。结论HLD合并癫痫最常 |
| 英文摘要: |
| ObjectiveTo observe the clinical manifestation of 155 patients with hepatolenticular degeneration (HLD) complicated with epilepsy and the therapeutic effect of integrative Chinese and Western medicine treatment on them. MethodsClinical manifestation of patients and its relationship with abnormalities in cranial CT and/or MRI were observed. Patients were treated by combined treatment of copper repellent with sodium dimercaptosulfonate 20 mg/kg per day by intravenous dripping, and modified Gandou Decoction (GDD) by oral intake and antiepileptics as well, after treatment for 8 10 courses, the clinical effect, copper levels in urine and serum were compared between groups. ResultsIn the 155 HLD patients, 96 were complicated with petit mal and 59 with grand mal. In the CT and/or MRI conducted in 72 patients, all showed abnormal images, besides such frequently met images as bilateral symmetrical basal ganglia focal lesion in 65 case episode (90.3%) and brain atrophy of various degrees in 61 case episode (84.7%), the massive lesions in cerebral white matter as principal, with the cortex involved, were also found in 54 patients (74%), which were mostly bilateral and symmetric or located in 2 adjecent lobes of brain, the sites of damage, in sequence of occurrence, were frontal lobe, parietal lobe, temporal lobe and callosal gyrus. Brain atrophy was found in all the remained patients without above mentioned lesions. Abnormal EEG was shown in 29 patients (40.2%), which mainly manifested as θ wave of moderate to high potential and/or short paroxysmal spike slow or sharp slow complex wave evoked. The urinary copper level in patients after treatment was 34.5±21 6 μmol/24 hrs, significantly higher than that before treatment, 4 49±1 93 μmol/24 hrs (P<0 01). And the serum copper level in patients also lowered significantly (P<0 01). Epileptic seizure was controlled completely along with the gradually improving of extrapyramidal symptoms. ConclusionPartial seizure was the most common type of seizure of HLD patient complicated with epilepsy, the next is systemic seizure. Cerebral damage lesion and obvious brain atrophy could be the main etiological factors of HLD complicated with epilepsy, combined copper repellent therapy of integrative Chinese and Western medicine, and antiepileptics produced good clinical effect on the patients. |
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