Pohl K R, Pritchard J, Wilson J
Newcomen Centre, Guys Hospital, London, UK.
Eur J Pediatr. 1996 Mar;155(3):237-44. doi: 10.1007/BF01953945.
Fifty-four patients with dancing eye syndrome (DES), presenting over a 25-year period, were reviewed retrospectively. One third of them were on active follow up at the time of the study. Malignancy was uncommon, diagnosed in only 4 patients, neuroblastoma in 3 cases and acute lymphoblastic leukaemia (ALL) in 1. An intercurrent illness preceded onset of DES in 51% of the children and was of equivocal significance. There was no clear temporal relationship to immunization. The acute phase of the illness was severe and caused total immobilization in 88% of patients. A favourable initial response to treatment with prednisolone or corticotrophin gel was observed in all patients. Although corticotrophin seemed to produce a more rapid response, overall improvement was similar with both treatments. Long-term neurological morbidity was a major problem with 91% of children suffering either persisting symptoms or repeated relapses. A persisting disability was found in 88% and was considered severe in 61% of patients. No features prognostic of neurological severity or outcome were identified.
Demonstrable malignancy is uncommon in the dancing eye syndrome. The neurological legacy of DES is often evident well into adult life.
回顾性分析了25年间出现的54例舞蹈眼综合征（DES）患者。研究时，其中三分之一的患者正在接受积极随访。恶性肿瘤并不常见，仅4例患者被诊断出患有恶性肿瘤，3例为神经母细胞瘤，1例为急性淋巴细胞白血病（ALL）。51%的儿童在DES发病前有并发疾病，其意义不明确。与免疫接种没有明确的时间关系。疾病急性期严重，88%的患者完全无法活动。所有患者对泼尼松龙或促肾上腺皮质激素凝胶治疗均有良好的初始反应。虽然促肾上腺皮质激素似乎产生的反应更快，但两种治疗的总体改善情况相似。长期神经功能障碍是一个主要问题，91%的儿童有持续症状或反复复发。88%的患者存在持续残疾，61%的患者被认为残疾严重。未发现神经严重程度或预后的特征。
在舞蹈眼综合征中，可证实的恶性肿瘤并不常见。DES的神经后遗症在成年期往往仍很明显。
