We present the case of a 46-year-old woman with gradually progressive ataxia, parkinsonism, and neuropathy. Symptoms started at age 29 years with speech impairment and difficulty walking. At presentation, she had mild midline ataxia and dysarthria. Left hand rest tremor began at age 33 years. Over the next decade, she developed parkinsonism and lower extremity weakness, which was more prominent than ataxia (Videos 1 and 2 ). Her parkinsonism was responsive to levodopa, and over time, she developed motor fluctuations. She also developed urinary retention and constipation. Medical history was otherwise notable for lumbar radiculopathy, ankylosing spondylitis, elevated anti-nuclear antibodies, migraine, and depression. Current examination reveals appendicular and gait ataxia, dysarthria, parkinsonism (left predominant), bilateral hip flexor weakness and foot drop, length-dependent decrease in pinprick sensation, and hyperreflexia (bilateral Babinski and jaw jerk reflexes). She is adopted and has 2 healthy teenaged sons. She is apparently of European descent but does not know her family history.

我们介绍了一名 46 岁女性逐渐进行性共济失调、帕金森症和神经病变的病例。症状始于 29 岁，伴有语言障碍和行走困难。就诊时，她有轻度中线共济失调和构音障碍。左手休息性震颤开始于 33 岁。在接下来的十年中，她出现了帕金森综合征和下肢无力，这比共济失调更为突出（视频 1 和 2）。她的帕金森症对左旋多巴有反应，随着时间的推移，她出现了运动波动。她还出现尿潴留和便秘。其他值得注意的病史包括腰神经根病、强直性脊柱炎、抗核抗体升高、偏头痛和抑郁症。目前的检查显示阑尾和步态共济失调、构音障碍、帕金森综合征（左侧为主）、双侧髋屈肌无力和足下垂，针刺感觉的长度依赖性降低和反射亢进（双侧巴宾斯基反射和下颌反射）。她被收养并有两个健康的十几岁的儿子。她显然是欧洲血统，但不知道她的家族史。