Hoffmann L A, Jarius S, Pellkofer H L, Schueller M, Krumbholz M, Koenig F, Johannis W, la Fougere C, Newman T, Vincent A, Voltz R
Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University, Munich, Germany.
J Neurol Neurosurg Psychiatry. 2008 Jul;79(7):767-73. doi: 10.1136/jnnp.2007.118588. Epub 2008 Jan 25.
Paraneoplastic neurological syndromes (PNS) are indirect remote effects of cancer on the nervous system, often associated with the presence of specific serum antibodies. The most recently described PNS defining reactivity is anti-Ma/anti-Ta. Here we present 22 newly diagnosed patients with anti-Ma or anti-Ta reactivity, refine the associated clinical picture and review all published patients to date.
Patients were identified by testing for PNMA1 and PNMA2 antibodies by western blotting and indirect immunofluorescence. Clinical data were obtained either by referral of the patient or from the referring physicians.
Analysis of 22 new patients (14 anti-Ma, eight anti-Ta) confirmed that anti-Ta are usually found in young men with limbic encephalitis and testicular germ cell tumours who stabilise neurologically with long term survival after tumour treatment. Patients with anti-Ma were of either sex, middle-aged, presented with a range of tumours and neurological symptoms and had a limited response to treatment. Furthermore, we expanded the range of associated clinical features: (1) the peripheral nervous system may be involved; (2) an overlap with anti-Hu is possible; and (3) testicular tumour manifestation can be extragonadal or detectable only at orchiectomy.
Refining and expanding the range of anti-Ma/anti-Ta associated neurological presentations and tumours clearly demonstrated that the distinction between anti-Ma and anti-Ta associated PNS is of high clinical relevance.
副肿瘤性神经系统综合征(PNS)是癌症对神经系统产生的间接远隔效应,常与特定血清抗体的存在相关。最近描述的定义PNS反应性的是抗-Ma/抗-Ta。在此,我们报告22例新诊断的具有抗-Ma或抗-Ta反应性的患者,细化相关临床表现,并回顾迄今所有已发表的患者。
通过蛋白质免疫印迹法和间接免疫荧光法检测PNMA1和PNMA2抗体来识别患者。临床数据通过患者转诊或从转诊医生处获得。
对22例新患者(14例抗-Ma,8例抗-Ta)的分析证实,抗-Ta通常见于患有边缘叶脑炎和睾丸生殖细胞肿瘤的年轻男性,他们在肿瘤治疗后神经功能稳定且长期存活。抗-Ma患者性别不限,为中年,有多种肿瘤和神经症状,对治疗反应有限。此外,我们扩大了相关临床特征的范围:(1)外周神经系统可能受累;(2)可能与抗-Hu重叠;(3)睾丸肿瘤表现可为性腺外的,或仅在睾丸切除术中可检测到。
细化和扩大抗-Ma/抗-Ta相关神经系统表现和肿瘤的范围清楚地表明,抗-Ma和抗-Ta相关PNS之间的区分具有高度临床相关性。