Effect of fetal neural transplants inpatients with Huntington's disease 6 years after surgery:: a long-term follow-up study
Effect of fetal neural transplants inpatients with Huntington's disease 6 years after surgery:: a long-term follow-up study
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DOI：
10.1016/s1474-4422(06)70381-7
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发表时间：
2006-04-01
期刊：
影响因子：
48
通讯作者：
Peschanski, M
中科院分区：
文献类型：
--
作者：
Bachoud-Lévi, AC;Gaura, V;Peschanski, M
背景虽然我们已经在五分之三的亨廷顿病患者中发现，脑内胚胎神经移植2年后运动和认知能力的改善与移植纹状体区和大脑皮层相连区域的脑代谢活动的恢复相关，但尚不清楚神经移植物本身对宿主脑具有保护作用。我们进行了长期的后续行动，以前报道的患者的疾病，以确定的性质和程度的任何二次下降后grafts.Methods从我们的试点研究5例亨廷顿舞蹈病进行了评估，每年与统一的亨廷顿舞蹈病评定量表，神经心理学测试，MRI，长达6年后神经移植。静息脑活动记录在2年和6 years.Findings临床改善平台后2年，然后消失了14 -6年手术后。肌张力障碍持续恶化，而舞蹈病没有。认知能力在非定时测试中保持稳定，而运动障碍的进展在定时测试中表现为恶化。低代谢也影响了大脑的异质性，保留了额叶皮层和移植物精确位置的益处，但在其他区域显示出进行性恶化。两名患者在2年内没有从移植中获益，继续以与非移植patients. Interpretation相同的方式下降，神经元移植在亨廷顿病中提供了几年的改善和稳定期，但不是疾病的永久治愈。手术方式和患者选择的改进可以提高治疗价值，但神经保护治疗似乎是不可避免的疾病。
Background Although we have shown in three out of five patients with Huntington's disease that motor and cognitive improvements 2 years after intracerebral fetal neural grafts are correlated with recovery of brain metabolic activity in grafted striatal areas and connected regions of the cerebral cortex, neural grafts are not known to have Protective effects on the host brain per se. We undertook long-term follow-up of previously reported patients with the disease to ascertain the nature and extent of any secondary decline after grafting.Methods Five patients with Huntington's disease from our pilot study were assessed annually with the unified Huntington's disease rating scale, neuropsychological tests, and MRI, for up to 6 years after neural grafting. Resting cerebral activity was recorded at 2 and 6 years.Findings Clinical improvement plateaued after 2 years and then faded off variably 4-6 years after surgery. Dystonia deteriorated consistently, whereas chorea did not. Cognitive performance remained stable on non-timed tests, whereas progression of motor disability was shown by deterioration on timed tests. Hypometabolism also affected the brain heterogeneously, sparing the benefits in the frontal cortex and at the precise location of the grafts, but showing a progressive deterioration in other areas. Two patients who had no benefit from grafting at 2 years continued to decline in the same way as non-grafted patients.Interpretation Neuronal transplantation in Huntington's disease provides a period of several years of improvement and stability, but not a permanent cure for the disease. Improvement of the surgical procedure and in patient selection could improve the therapeutic value, but neuroprotective treatment seems to be unavoidable in the disease.