Parkinsonism in cirrhosis: pathogenesis and current therapeutic options
Parkinsonism in cirrhosis: pathogenesis and current therapeutic options
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DOI：
10.1007/s11011-012-9341-7
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发表时间：
2013-06-01
影响因子：
3.6
通讯作者：
Butterworth, Roger F.
中科院分区：
文献类型：
--
作者：
Butterworth, Roger F.
获得性肝豆状核变性，也称为“肝硬化中的帕金森症”，其特征在于锥体外系症状，包括运动功能减退、肌张力障碍和强直，这些症状迅速进展，并且可能与认知功能障碍的严重程度无关。磁共振成像显示苍白球和黑质T1加权高信号。据报道，帕金森综合征在肝硬化中的患病率估计高达21%。肝硬化帕金森氏症的原因是基底神经节结构中的锰沉积，导致多巴胺能神经递质系统功能障碍。特别是，有证据表明，从光谱和生物化学研究损害（或功能障碍）突触前多巴胺转运蛋白连同损失的突触后多巴胺受体在基底神经节的受影响的患者。治疗选择是有限的;降低氨的策略是没有实质性的好处，有效的锰螯合剂是不可用的。在许多患者中，左旋多巴替代疗法和多巴胺受体激动剂溴隐亭是有益的，肝移植通常有效。然而，移植后残留的锥体外系症状的报告表明，在某些情况下，不可逆的元素。
Acquired hepatolenticular degeneration, also known as "Parkinsonism in cirrhosis" is characterized by extrapyramidal symptoms including hypokinesia, dystonia and rigidity that are rapidly progressive and may be independent of the severity of cognitive dysfunction. Magnetic resonance imaging reveals T1-weighted hyperintense signals in both globus pallidus and substantia nigra. Estimates of the prevalence of Parkinsonism in cirrhosis have been reported as high as 21 %. The cause of Parkinsonism in cirrhosis has been attributed to manganese deposition in basal ganglia structures, leading to the dysfunction of the dopaminergic neurotransmitter system. In particular, there is evidence from both spectroscopic and biochemical investigations for damage to (or dysfunction of) presynaptic dopamine transporters together with a loss of post-synaptic dopamine receptors in basal ganglia of affected patients. Therapeutic options are limited; ammonia-lowering strategies are without substantial benefit, and an effective manganese chelator is not available. In many patients, L-Dopa replacement therapy and the dopamine receptor agonist bromocriptine are beneficial, and liver transplantation is generally effective. However, reports of post-transplant residual extrapyramidal symptoms suggest an element of irreversibility in some cases.