Hammer M S, Larsen M B, Stack C V
Division of Pediatric Neurology, Children's Memorial Hospital, Chicago, IL 60614, USA.
Pediatr Neurol. 1995 Jul;13(1):21-4. doi: 10.1016/0887-8994(95)00083-r.
Within the past 11 years, 11 patients with opsoclonus and myoclonus, with or without a history of neuroblastoma, have been admitted to Children's Memorial Hospital. Eight of the 11 children had an occult neuroblastoma. Eight children have had subsequent delayed development with motor incoordination and speech delay (7 with neuroblastoma, 1 without). Nine of 11 children initially were treated with ACTH, 1 child was treated with prednisone, and 1 was not treated. Nine of the 10 children who were treated had recurrences of symptoms during the gradual withdrawal or discontinuation of ACTH. Often the ACTH had to be restarted or increased, although several times the episodes were self-limited, not requiring treatment after ACTH was withdrawn. We found prednisone was ineffective in controlling opsoclonus-myoclonus regardless of etiology. The majority of children with opsoclonus-myoclonus, regardless of etiology, have developmental delay, more severe and at a higher rate than previously reported. When a neuroblastoma was present, tumor removal did not improve symptoms. Although limited in size, our study indicates patients with opsoclonus-myoclonus without an associated neuroblastoma have a better chance for normal neurologic development (2/3 versus 1/8).
在过去11年中，11例有或无神经母细胞瘤病史的眼阵挛-肌阵挛患者被收治入儿童纪念医院。11名儿童中有8名患有隐匿性神经母细胞瘤。8名儿童随后出现发育迟缓，伴有运动不协调和语言发育迟缓（7名有神经母细胞瘤，1名无）。11名儿童中有9名最初接受促肾上腺皮质激素（ACTH）治疗，1名儿童接受泼尼松治疗，1名未接受治疗。在逐渐减量或停用ACTH期间，10名接受治疗的儿童中有9名症状复发。尽管有几次发作是自限性的，在停用ACTH后无需治疗，但通常必须重新开始使用ACTH或增加剂量。我们发现，无论病因如何，泼尼松在控制眼阵挛-肌阵挛方面均无效。大多数眼阵挛-肌阵挛患儿，无论病因如何，均有发育迟缓，比以前报道的更为严重，发生率更高。当存在神经母细胞瘤时，切除肿瘤并不能改善症状。尽管规模有限，但我们的研究表明，无相关神经母细胞瘤的眼阵挛-肌阵挛患者神经功能正常发育的机会更大（2/3比1/8）。
