The cerebellum is a brain structure that is highly interconnected with other brain regions. There are many contributing factors to cerebellar-related brain disease, such as altered afferent input, local connectivity, and/or cerebellar output. Purkinje cells are the principle cells of the cerebellar cortex, and fire intrinsically; that is, they fire spontaneous action potentials at high frequencies. This review paper focuses on Purkinje cell intrinsic firing activity, which is altered in multiple neurological diseases, including ataxia, Huntington Disease and autism spectrum disorder. Notably, there are several cases where interventions that restore or rescue Purkinje cell intrinsic activity also improve impaired behavior in these mouse models of disease. These findings suggest that rescuing Purkinje cell firing deficits themselves may be sufficient to improve impairment in cerebellar-related behavior in disease. We propose that restoring Purkinje cell intrinsic firing represents a good target for drug development that might be of therapeutic use for several disorders.

小脑是与其他大脑区域高度互连的大脑结构。小脑相关性脑疾病有许多促成因素，例如传入输入改变，局部连接性和/或小脑输出改变。浦肯野细胞是小脑皮层的主要细胞，其内在起火。也就是说，它们以高频率激发自发动作电位。这篇综述集中在浦肯野细胞的内在激发活性，这种活性在多种神经系统疾病中发生了改变，包括共济失调，亨廷顿病和自闭症谱系障碍。值得注意的是，在某些情况下，恢复或挽救浦肯野细胞内在活性的干预措施也可以改善这些小鼠疾病模型中受损的行为。这些发现表明，挽救浦肯野细胞射击缺陷本身可能足以改善疾病中小脑相关行为的损害。我们建议恢复浦肯野细胞内源性放电代表药物开发的良好目标，这种药物可能可用于治疗多种疾病。