Dorban S, Gille M, Kessler R, Piéret F, Declercq I, Sindic C J-M
Service de Neurologie, Cliniques Universitaires UCL, St-Luc, Bruxelles, Belgique.
Rev Neurol (Paris). 2004 Jan;160(1):126-9. doi: 10.1016/s0035-3787(04)70863-2.
Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis.
A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles.
A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.
副肿瘤性舞蹈手足徐动症较为罕见。我们报告一例伴有舞蹈手足徐动症的抗Hu综合征病例。
一名48岁女性因抗Hu综合征被诊断出患有小细胞肺癌。神经学特征包括以上肢为主的舞蹈手足徐动症、慢性感觉运动性轴索性多神经病和眼阵挛。脑脊液无细胞成分，含有几条寡克隆IgG带，在相应血清中未发现。磁共振成像显示放射冠在T2/液体衰减反转恢复序列上有双侧高强度病变。在几个卡铂-依托泊苷疗程后，观察到副肿瘤性神经综合征有中度短暂改善。
所有不明原因的舞蹈手足徐动症病例均须考虑副肿瘤起源。副肿瘤性舞蹈手足徐动症常与其他神经症状相关。最常见的相关肿瘤是伴有抗CRMP5和/或抗Hu抗体的小细胞肺癌。我们的患者在没有抗CRMP5/CV2抗体的情况下出现了与抗Hu综合征相关的副肿瘤性舞蹈手足徐动症。副肿瘤性舞蹈手足徐动症可能由中枢病变和/或周围神经病变继发的本体感觉传入缺失引起。
