Golbe L I, Miller D C, Duvoisin R C
Department of Neurology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, New Brunswick 08903.
Mov Disord. 1989;4(2):147-52. doi: 10.1002/mds.870040206.
A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. Inflammation, Lewy bodies, and tumor were absent. Cerebellar Purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism.
一名42岁女性出现不明原因体重减轻，随后出现动作性震颤及起步步态困难。症状出现三个月后，被诊断为乳腺浸润性导管癌伴肝和淋巴结转移，并接受了环磷酰胺、甲氨蝶呤和5-氟尿嘧啶（5FU）的短期治疗。之后出现了严重的对称性动作性和姿势性震颤，并伴有肌阵挛成分，静止性震颤轻微，同时伴有严重构音障碍和吞咽困难，步态小步且稍显共济失调，逐渐发展至卧床状态，还出现了严重的全身性肌张力障碍姿势。后者起初表现为典型的帕金森式躯干和上肢姿势，进而发展为肘部和腕部固定性疼痛性屈曲以及手指和颈部伸展。左旋多巴-卡比多巴、抗胆碱能药物、巴氯芬、地西泮以及血浆置换均未见疗效。患者在神经症状出现5个月后死于长期卧床并发症。尸检显示黑质中有许多含色素巨噬细胞，色素神经元中度缺失。未见炎症、路易小体及肿瘤。小脑浦肯野细胞中度减少。苍白球和小脑皮质存在轻度神经元丢失及胶质细胞增生。抗人IgG、IgM、κ和λ染色均为阴性。据我们所知，这是黑质副肿瘤性变性或副肿瘤性帕金森综合征的首例报告。
