Liu S, Tunkel R, Lachmann E, Nagler W
New York Presbyterian Hospital, New York, USA.
Arch Phys Med Rehabil. 2000 Jun;81(6):834-6. doi: 10.1016/s0003-9993(00)90121-7.
Paraneoplastic cerebellar degeneration (PCD) is the most frequently seen paraneoplastic syndrome affecting the brain. PCD is most commonly associated with cancers of the ovary, breast, and lung. The anti-Purkinje cell antibodies (anti-Yo) that specifically damage the Purkinje cells of the cerebellum are found in the patient's serum and cerebrospinal fluid. The typical presentation of PCD includes limb and truncal ataxia, often along with dysarthria. This report describes the case of a 47-year-old woman without significant medical history who developed new onset of unsteady gait, headache, and vertigo. The imaging studies suggested rhombencephalitis. The patient initially responded to corticosteroid treatment. Unfortunately, her gait ataxia worsened and she developed dysarthria, neither of which responded to increasing dosages of corticosteroids. Extensive imaging studies showed no evidence of tumor, but the patient was found to have positive anti-Yo antibodies and elevated cancer antigen 125 (CA-125). Pathology results from exploratory laparotomy revealed stage III C adenocarcinoma of the ovary. This case demonstrates that PCD may be the presenting symptom of an occult malignancy. The pathogenesis, diagnosis, and treatment of PCD, and its rehabilitation implications, are reviewed.
副肿瘤性小脑变性(PCD)是最常见的累及脑部的副肿瘤综合征。PCD最常与卵巢癌、乳腺癌和肺癌相关。在患者的血清和脑脊液中可发现特异性损害小脑浦肯野细胞的抗浦肯野细胞抗体(抗Yo)。PCD的典型表现包括肢体和躯干共济失调,常伴有构音障碍。本报告描述了一名47岁无重大病史的女性,出现了新发的步态不稳、头痛和眩晕。影像学检查提示为菱脑炎。患者最初对皮质类固醇治疗有反应。不幸的是,她的步态共济失调加重,并且出现了构音障碍,增加皮质类固醇剂量对此均无反应。广泛的影像学检查未发现肿瘤证据,但发现患者抗Yo抗体阳性且癌抗原125(CA-125)升高。剖腹探查的病理结果显示为卵巢III C期腺癌。该病例表明PCD可能是隐匿性恶性肿瘤的首发症状。本文对PCD的发病机制、诊断、治疗及其康复意义进行了综述。