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[同一患者出现多种副肿瘤综合征:临床、影像学及神经病理学记录]

[Multiple paraneoplastic syndromes occurring in the same patient: clinical, imaging and neuro-pathological documentation].

作者信息

Sá Goreti, Correia Carlos, Pires Melo, Lopes Gabriela

机构信息

Serviços de Neurorradiologia, Neurologia e Neuropatologia, Hospital Geral de Santo António, Porto, Portugal.

出版信息

Acta Med Port. 2006 Nov-Dec;19(6):489-93. Epub 2007 May 14.

Abstract

Paraneoplastic neurological syndromes (PNS) are remote effects of cancer, which are not caused by invasion of the tumour or its metastasis, but presumably immunologic mediated. They developed in less than 1% of patients with systemic cancers, most Limbic encephalitis (LE) is the most common clinical paraneoplastic syndrome attainting the CNS (Central Nervous System), and it is characterized by involvement of hippocampus and amygdala; LE is also the only one with clearly defined imaging features. We report a 64 year old man, former smoker, which presented multiple paraneoplastic syndromes, namely LE, opsoclonus-myoclonus, subacute cerebellar degeneration, brainstem encephalitis, sensory-motor neuropathy and inappropriate antidiuretic hormone secretion syndrome (SIADH); these syndromes were identifiable in the clinical and imaging examination, confirmed by the neuro-pathological study. Magnetic Resonance Imaging disclosed on T2 weighted images bilateral hyperintensity within medial temporal lobes, hypothalamus, thalamus, brain stem, upper and medium cerebellar peduncles and upper cervical spinal cord. The underlying tumour was not found in imaging studies or in the autopsy examination. A mediastinal adenopathy depicted a metastasis from low differentiated neoplasm cells, with some signs of neuroendocrine differentiation. With this case we provide a comprehensive illustration of the PNS, from a clinical, imaging and pathological point of view. This report also emphasises the importance of a diagnosis based on early clinical and imaging findings, given that, in most cases the cancer is not apparent.

摘要

副肿瘤性神经系统综合征(PNS)是癌症的远隔效应,并非由肿瘤侵袭或转移所致,推测是由免疫介导的。它们在不到1%的全身性癌症患者中发生,大多数边缘叶脑炎(LE)是最常见的累及中枢神经系统(CNS)的临床副肿瘤综合征,其特征是海马体和杏仁核受累;LE也是唯一具有明确影像学特征的综合征。我们报告一名64岁男性,既往吸烟者,出现多种副肿瘤综合征,即LE、眼阵挛-肌阵挛、亚急性小脑变性、脑干脑炎、感觉运动性神经病和抗利尿激素分泌不当综合征(SIADH);这些综合征在临床和影像学检查中可识别,并经神经病理学研究证实。磁共振成像在T2加权图像上显示双侧内侧颞叶、下丘脑、丘脑、脑干、中小脑脚和上颈髓内高信号。影像学研究或尸检检查均未发现潜在肿瘤。纵隔淋巴结肿大显示为低分化肿瘤细胞转移,有一些神经内分泌分化迹象。通过这个病例,我们从临床、影像学和病理学角度全面阐述了PNS。本报告还强调了基于早期临床和影像学发现进行诊断的重要性,因为在大多数情况下癌症并不明显。

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