Dalmau Josep, Rosenfeld Myrna R
Division of Neuro-oncology, Department of Neurology, University of Pennsylvania, Philadelphia 19104, USA.
Handb Clin Neurol. 2011;100:315-21. doi: 10.1016/B978-0-444-52014-2.00024-0.
Paraneoplastic neurological disorders are an extensive group of neurological syndromes that occur in patients with cancer and often present before the presence of an underlying tumor is known. Many of these disorders appear to be immune-mediated, with targets throughout the nervous system, including basal ganglia and brainstem, in which case they may result in movement disorders. Recent descriptions of new immune-mediated encephalitides in children and adults have substantially increased the number of patients with paraneoplastic movement disorders. There are several key concepts that assist in the identification of a movement disorder as likely paraneoplastic, including a rapid onset, the presence of cerebrospinal fluid inflammatory changes, and in some patients the identification of specific serum and cerebrospinal fluid antineuronal antibodies. Once identified, prompt diagnosis and treatment of the tumor can result in neurological symptom improvement or stabilization, although some patients may require immunotherapy only. Understanding the natural course of each immune-mediated paraneoplastic movement disorder minimizes unnecessary testing and the use of potentially toxic therapies.
副肿瘤性神经系统疾病是一组广泛的神经系统综合征,发生于癌症患者,且常在潜在肿瘤被发现之前就已出现。其中许多疾病似乎是免疫介导的,其靶点遍布整个神经系统,包括基底神经节和脑干,在这种情况下可能导致运动障碍。近期对儿童和成人新的免疫介导性脑炎的描述大幅增加了副肿瘤性运动障碍患者的数量。有几个关键概念有助于将运动障碍识别为可能的副肿瘤性疾病,包括起病迅速、脑脊液有炎症变化,以及在一些患者中发现特定的血清和脑脊液抗神经元抗体。一旦确诊,及时诊断和治疗肿瘤可使神经症状改善或稳定,不过有些患者可能仅需免疫治疗。了解每种免疫介导的副肿瘤性运动障碍的自然病程可减少不必要的检查和使用潜在有毒的治疗方法。