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Anti-Ri-associated paraneoplastic cerebellar degeneration. Report of a case and revision of the literature.
Archives Italiennes De Biologie ( IF 0.8 ) Pub Date : 2011-10-27 , DOI: 10.4449/aib.v149i3.1232
Michelangelo Mancuso 1 , Daniele Orsucci , Andrea Bacci , Elena Caldarazzo Ienco , Gabriele Siciliano
Affiliation  

Paraneoplastic cerebellar degeneration associated with anti-Ri antibodies mainly presents with opsoclonus-myoclonus-ataxia. We report here the case of a patient with anti-Ri-antibody paraneoplastic syndrome, who presented four years after treatment for small-cell lung cancer (SCLC) with oscillopsia and gait disorder. On neurological examination vertical nystagmus, ataxic gait and postural tremor of all four limbs was detected. He died one year after the onset of the symptoms because of a acute exacerbation of his severe chronic obstructive pulmonary disease. No SCLC relapse or new cancer has been detected during the one-year follow-up period.To our knowledge, our patient is the first case of anti-Ri associated disorder with oscillopsia and vertical nystagmus as the initially prominent clinical features. The findings of this case study support the variability of anti-Ri-antibody-associated paraneoplastic syndrome. Further studies must be directed to better characterize the mechanisms underlying this syndrome. Finally, paraneoplastic neurological syndromes should be kept in mind also when a neoplastic disease is not demonstrated.

中文翻译:

抗Ri相关性副肿瘤小脑变性。病例报告和文献修订。

与抗Ri抗体相关的副肿瘤性小脑变性主要表现为轻度阵挛-肌阵挛-共济失调。我们在此报告一例抗Ri抗体副肿瘤综合征的患者,该患者在患有视神经系统疾病和步态障碍的小细胞肺癌(SCLC)治疗四年后就诊。在神经系统检查中,检测到所有四个肢体的垂直眼球震颤,共济失调步态和姿势性震颤。由于严重的慢性阻塞性肺疾病的急性加重,他在症状发作一年后死亡。一年的随访期间未发现SCLC复发或新发癌症。据我们所知,我们的患者是首例抗Ri相关疾病,以巩膜和垂直眼球震颤为最初突出的临床特征。该病例研究的结果支持抗Ri抗体相关的副肿瘤综合征的变异性。必须进一步研究以更好地表征该综合征的潜在机制。最后,当未显示出肿瘤性疾病时,也应牢记副肿瘤神经综合症。
更新日期:2020-08-21
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