累及边缘系统-丘脑-脊髓-周围神经的副肿瘤综合征的临床、电生理与影像学表现一例

目的探讨以周围神经病变、脊髓及中枢神经系统病变为表现的副肿瘤综合征的临床、电生理及影像学改变特点。方法对1例累及边缘系统-丘脑-脊髓-周围神经的神经系统副肿瘤综合征(PNS)患者的临床表现、电生理与影像学资料进行分析研究。结果本例患者女性,65岁,半年前开始出现双下肢麻木,电生理检查提示周围神经损伤,神经活检示慢性轴索性周围神经病,脑脊液抗Hu抗体滴度1:10。以后逐渐出现行走不稳和认知障碍。头颅MRI及DWI可见双侧丘脑及海马异常信号,头颅及全身PET双侧颞叶、海马、丘脑、颈髓、下胸髓条状葡萄糖代谢异常增高灶;左肺下叶背段结节及葡萄糖代谢异常增高,纵膈及左肺门多发肿大淋巴结葡萄糖代谢增高。临床诊断左肺癌伴肺内淋巴结转移,累及边缘系统-丘脑-脊髓-周围神经的PNS。结论本例是少见的PNS,出现神经系统多个部位的损害,不但有中枢神经系统受累边缘叶脑炎表现,也有周围神经受累。而且还出现了以往少有的脊髓及丘脑同时受累,因此早期进行脑DWI及全身PET-CT扫描极其必要。

One rare case of neurological paraneplastic syndrome refered to spinal cord and thalamus

Objective To investigate the clinical,electrophysiological and imaging characteristics of a case of paraneoplastic neurological syndrome.Methods Electrophysiological,muscle histochemical,MRDW and PET-CT were used in a patient with numbness of both lower extremities associated with lung cancer.Results The clinical features showed progressive numbness in lower extremities,gait ataxia and cognitive impairment.Electrophysiological examination and biopsy of peripheral nerve showed axonal lesions in verves,and the CSF anti-Hu antibody titer was 1:10.Abnormal signals were found in bilateral thalamus and hippocampus on both routine cranial MRI and DWI scanning.PET-CT examination on CNS showed abnormal high metabolism signals in cervical spinal cord and lower thoracic spinalcord and in bilateral temporal lobe,thalamus and hippocampus.PET-CT examination on the whole body except CNS tissues indicated abnormal image of the left lung,hilum of left lung,mediastinum,lymph nodes within the lung and thyroid gland.So the clinical diagnosis for this patient was lung lymph nodees metastasis with lung cancer involving the PNS of limbic system-the thalamus-the spinal cord-peripheral nerve.Conclusion This is a rare case of paraneoplastic neurological syndrome with damage to several parts of the nervous system involving limbic encephalitis,peripheral nerve,the spinal cord and thalamus lesion on PET-CT scans,which has not been reported in the lastest literature about paraneoplastic neurological syndrome case.So PET-CT scanning for the whole body of paraneoplastic neurological syndrome patient in advance is important and necessary.