Koh P S, Raffensperger J G, Berry S, Larsen M B, Johnstone H S, Chou P, Luck S R, Hammer M, Cohn S L
Northwestern University Medical School, Chicago, Illinois.
J Pediatr. 1994 Nov;125(5 Pt 1):712-6. doi: 10.1016/s0022-3476(94)70062-1.
We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.
我们回顾了10例患有眼阵挛-肌阵挛（“跳舞眼综合征”）和神经母细胞瘤的儿童的神经学和发育过程。经过8个多月至111个多月的随访，所有患者均存活，无肿瘤疾病证据。所有患者均为局限性疾病，50%有腹外肿瘤。9名儿童的神经母细胞瘤具有良好的岛田组织学特征，所有肿瘤均有N-myc癌基因的单拷贝。神经母细胞瘤切除术后，所有患者均有持续性眼阵挛-肌阵挛或共济失调，对促肾上腺皮质激素治疗有反应。9名儿童出现神经症状复发。切除术后三年，7名有足够随访时间的患者中有6名无症状且已停止治疗。然而，9名儿童有慢性神经功能缺损，包括认知和运动发育迟缓、语言缺陷和行为异常。参加教育项目的所有6名患者都需要特殊帮助。5名儿童需要物理、职业或言语治疗。对于患有眼阵挛-肌阵挛或共济失调或两者皆有的神经母细胞瘤患者，应预期会出现长期发育和认知问题，应尽早进行干预以尽量减少这些缺陷。
