Parkin基因(PG)突变引起青少年的常染色体隐性遗传的帕金森综合征(AR-JP)，AR-JP的特征为同质性，包括早发(40岁以前)的静止性震颤、强直、运动过缓、足肌张力障碍，每日症状波动，左旋多巴有效。作者曾报道了PG突变的AR-JP患者表现为不典型的帕金森综合征，并有小脑和锥体束损害。在此，本文再报道帕金森综合征伴小脑性共济失调患者，他们也同时具有纯合子的外显子3和4的缺失突变， Parkin gene (PG) mutations cause autosomal recessive Parkinsonism (AR-JP) in adolescents. AR-JP is characterized by homogeneity including resting onset tremor (premature 40 years), ankylosis, Excessive exercise, foot muscle dystonia, daily symptoms, levodopa effective. The authors have reported that patients with PG-mutated AR-JP presented with atypical Parkinson’s disease with cerebellar and pyramidal tract damage. Here again, we report patients with Parkinson’s disease accompanied by cerebellar ataxia, they also have homozygous exon 3 and 4 deletion mutations,