Department of Psychiatry, National Institute of Mental Health and Neuro Sciences, Bangalore, Karnataka, India.
Indian J Psychiatry. 2013 Jan;55(1):81-3. doi: 10.4103/0019-5545.105522.
Huntington's disease is characterized by choreic movements, psychiatric disorders, striatal atrophy with selective small neuronal loss, and autosomal dominant inheritance. The genetic abnormality is CAG expansion in Huntingtin gene. Newer therapeutic strategies are evolving to treat this progressive disorder. The neuroprotective agents are one such group of drugs being tried. Lithium has been used to treat Huntington's disease in the past due to its neuroprotective effects. Though the precise mechanism of action is not clear, Lithium can directly or indirectly modulate proteins involved in neuronal survival/differentiation which may account for its neuroprotective effects. We report three patients with Huntington's disease in whom Lithium prevented the progression of chorea and also helped stabilize mood.
亨廷顿病的特征为舞蹈样动作、精神障碍、纹状体萎缩伴选择性小神经元丧失，以及常染色体显性遗传。遗传异常是亨廷顿基因中的 CAG 扩展。目前正在开发新的治疗策略来治疗这种进行性疾病。神经保护剂是正在尝试的一类药物。过去曾使用锂来治疗亨廷顿病，因为它具有神经保护作用。尽管确切的作用机制尚不清楚，但锂可以直接或间接地调节参与神经元存活/分化的蛋白质，这可能是其神经保护作用的原因。我们报告了 3 例亨廷顿病患者，锂可预防舞蹈病的进展，也有助于稳定情绪。
