Casas Parera I, Fischman D, Paz L, Lehkuniec E, Muchnik S
Servicio de Neurologia, Instituo de Investigaciones Médicas Alfredo Lanari, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Medicina (B Aires). 1998;58(2):197-201.
The case of a 72-year-old woman presenting sensory neuropathy and anti-Hu antibodies is reported. She was admitted in November 1995 with a one year history of sensory neuropathy. Her first symptoms were painful numbness and dysesthesias in both feet. She experienced progression of the sensory symptoms affecting upper limbs, and clumsiness of gait. One month before admission she complained of diminished strength in both hands. The neurologic examination showed anicocoric fixed pupils, with no reaction to light; convergence miosis was evident in the right eye (Argyll-Robertson pupil). In the lower limbs she had very mild distal weakness, and tendon reflexes were universally abolished. Pin and touch sensation, position sense and pallesthesia were absent in all four limbs. Romberg test was elicited, and a tabetic gait was patent. Pseudoathetotic movements were observed in hands and feet. An ulcer was present in the fifth finger of the right foot. Routine blood biochemistry and hematology showed a ESR of 105 and an increased IgG in the immune-electrophoretic run. Neurophysiologic evaluation disclosed a mild demyelinating neuropathy. Positive anti-Hu antibodies were found in the serum (Western blot - Athena Diagnostics); CSF was normal but not tested for anit-Hu. An abdominal CT scan disclosed multiple hypodense nodules in liver, right adrenal gland and peritoneum. A chest CT scan showed a hyperdense mass in the lower right pulmonary lobe and enlarged retrocava-pretracheal lymph nodes. A biopsy of the peritoneal nodule was performed, showing a metastatic small cell carcinoma. The patient died eight days after discharge. Although multiple organs were affected, she was independent until death, showing an indolent clinical course.
报告了一例72岁女性出现感觉神经病变并伴有抗Hu抗体的病例。她于1995年11月入院,有一年的感觉神经病变病史。她最初的症状是双脚疼痛性麻木和感觉异常。她的感觉症状逐渐发展至上肢,并出现步态笨拙。入院前一个月,她抱怨双手力量减弱。神经系统检查显示双侧瞳孔不等大且固定,对光无反应;右眼可见集合性瞳孔缩小(阿-罗瞳孔)。下肢有非常轻微的远端无力,腱反射普遍消失。四肢均无针刺觉、触觉、位置觉和震动觉。引出罗姆伯格征,可见脊髓痨步态。双手和双足出现假手足徐动症。右足第五趾有一处溃疡。常规血液生化和血液学检查显示血沉为105,免疫电泳显示IgG升高。神经生理学评估显示轻度脱髓鞘性神经病变。血清中发现抗Hu抗体阳性(蛋白质印迹法 - 雅典娜诊断公司);脑脊液正常,但未检测抗Hu抗体。腹部CT扫描显示肝脏、右肾上腺和腹膜有多个低密度结节。胸部CT扫描显示右下肺叶有一个高密度肿块,腔静脉后气管前淋巴结肿大。对腹膜结节进行活检,显示为转移性小细胞癌。患者出院八天后死亡。尽管多个器官受累,但她直到死亡前都能自理,临床病程进展缓慢。