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首页> 外文期刊>Epilepsia: Journal of the International League against Epilepsy >Reversible pseudoatrophy of the brain and mental deterioration associated with valproate treatment.
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Reversible pseudoatrophy of the brain and mental deterioration associated with valproate treatment.

机译：与丙戊酸盐治疗相关的可逆性假性萎缩和精神退化。

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PURPOSE: To describe an 11-year-old girl with symptomatic localization-related epilepsy and normal intelligence who developed reversible mental deterioration and pseudoatrophic brain changes while receiving valproate (VPA). METHODS: Assessment of mental function using Wechsler Intelligence Scale for Children-III (WISC) and Raven's Progressive Matrices (PM), EEG recordings while awake and asleep, and brain magnetic resonance imaging (MRI), were performed at the beginning of VPA therapy, after 2 years and 8 months of treatment and following VPA discontinuation. RESULTS: After 2 years and 6 months on VPA (< or = 26 mg/kg/day) the girl insidiously developed mental deterioration (loss of 18 IQ points and drop in age-adjusted PM score from the 95th to the 50th percentile) associated with MRI-documented pseudoatrophy of the brain. Onset of severe cognitive impairment coincided with serum VPA concentrations near 100 microg/ml. There were no other manifestations of drug toxicity or hyperammonemia. Background EEG activity was normal. Reduction of VPA dosage and subsequent discontinuation 4 months later resulted in disappearance of clinical symptoms with a 20-point improvement at IQ testing and recovery of previous PM score. Repeat MRI showed disappearance of pseudoatrophic changes. CONCLUSIONS: The striking cognitive improvement and reversal of pseudoatrophic brain changes following VPA discontinuation strongly suggest a drug-induced condition. Based on this and previous reports, the syndrome of VPA-associated mental deterioration and pseudoatrophy of the brain appears to encompass different but possibly related clinical entities, which include parkinsonism with cognitive deterioration, mental deterioration with signs of VPA-toxicity, and isolated mental deterioration, as seen in our patient. A drug-induced effect should be considered whenever cognitive deterioration and imaging findings of brain atrophy occur in VPA-treated patients.
机译：目的：描述一名患有症状性局部相关性癫痫和正常智力的11岁女孩，该女孩在接受丙戊酸盐（VPA）时出现可逆的精神退化和假萎缩性脑变。方法：在VPA治疗开始时，使用韦氏儿童智力量表（WISC）和Raven进步矩阵（PM），清醒和睡着时的EEG记录以及脑磁共振成像（MRI）进行心理功能评估，经过2年8个月的治疗以及VPA停药后。结果：使用VPA 2年零6个月后（<或= 26 mg / kg /天），该女孩潜伏地发展为智力下降（智商下降18点，年龄调整后的PM得分从第95个百分点降至第50个百分点） MRI记录的大脑假性萎缩。严重的认知障碍发作与血清VPA浓度接近100微克/毫升相吻合。没有其他药物毒性或高氨血症的表现。背景脑电活动正常。降低VPA剂量并随后在4个月后停药导致临床症状消失，IQ测试和先前PM评分的恢复提高了20分。重复MRI显示假萎缩性改变消失。结论：VPA停药后，明显的认知改善和假萎缩性脑变化的逆转强烈提示药物诱发的疾病。根据此报告和先前的报告，与VPA相关的智力退化和大脑假性萎缩综合征似乎涵盖了不同但可能相关的临床实体，包括帕金森氏症伴认知能力减退，具有VPA毒性迹象的精神退化和孤立的精神退化，正如我们的患者所见。每当接受VPA治疗的患者出现认知能力下降和脑萎缩的影像学发现时，都应考虑药物引起的作用。

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