Lieberman Frank S, Schold S Clifford
University of Pittsburgh, Cancer Institute, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Oncology (Williston Park). 2002 Nov;16(11):1539-48; discussion 1550-1, 1554-6.
Paraneoplastic disorders of the nervous system are important to the practicing oncologist, because these syndromes, although uncommon, produce significant neurologic dysfunction and disability. The neurologic disorder may be the first manifestation of an unrecognized systemic malignancy, and appropriate diagnosis of the paraneoplastic disorder can lead to a focused search for an underlying cancer. Paraneoplastic disorders may involve any component of the central or peripheral nervous system, and diagnosis requires careful neurologic assessment. The diagnosis is made by recognition of clinical neurologic syndromes and the use of selected laboratory studies as indicated by the clinicalpicture. Over the past 10 years, the application of molecular biologic techniques to the study ofthese disorders has elucidated much about the mechanisms that cause neurologic injury. In most cases, disordered humoral and cellular immunity has been demonstrated, and the role of novel targets for autoimmune attack is being clarified. For some paraneoplastic disorders, treatment of the underlying tumor may lead to improvement of the neurologic disorder. For others, various forms of immunosuppressive therapy may be indicated. Unfortunately, for several of the more common paraneoplastic syndromes such as paraneoplastic cerebellar degeneration or limbic encephalitis, treatment is still unsatisfactory, and further research into the exact pathophysiology is clearly needed.
神经系统副肿瘤综合征对于肿瘤专科医生来说很重要,因为这些综合征虽然不常见,但会导致严重的神经功能障碍和残疾。神经疾病可能是未被识别的全身性恶性肿瘤的首发表现,而对副肿瘤综合征的正确诊断可以促使有针对性地寻找潜在癌症。副肿瘤综合征可能累及中枢或周围神经系统的任何部分,诊断需要仔细的神经学评估。诊断是通过识别临床神经综合征,并根据临床表现进行特定的实验室检查来做出的。在过去10年里,分子生物学技术在这些疾病研究中的应用,已经阐明了许多导致神经损伤的机制。在大多数情况下,已证实存在体液和细胞免疫紊乱,自身免疫攻击新靶点的作用也正在得到阐明。对于一些副肿瘤综合征,治疗潜在肿瘤可能会使神经疾病得到改善。对于其他一些综合征,则可能需要进行各种形式的免疫抑制治疗。不幸的是,对于一些较常见的副肿瘤综合征,如副肿瘤性小脑变性或边缘叶脑炎,治疗仍然不尽人意,显然需要对确切的病理生理学进行进一步研究。