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儿童眼阵挛性脑病(金斯伯恩综合征)

[Opsoclonic encephalopathy in childhood (Kinsbourne syndrome)].

作者信息

Corrias A, Nurchi A M, Rossi G, Sorcinelli R, Pusceddu G, Corda R

出版信息

Pediatr Med Chir. 1985 May-Jun;7(3):437-41.

PMID:3837205
Abstract

Kinsbourne's syndrome (ataxia--opsoclonus--myoclonus) is described. The characteristics of this syndrome are: infections of the upper respiratory tract and enteric system and behavioural disturbances leading to the typical symptomatological triad. Various tests were carried out to determine the presence of neuroblastoma which is often associated (46%) to this syndrome. These tests resulted negative. Differential diagnosis was considered in relation to other conditions which present a characteristic symptomatology similar to Kinsbourne's syndrome. Synthetic ACTH (Synacthen) treatment produced a regression of the clinical symptoms. Based on the examinations carried out, which always resulted negative even after 36 months, and on the drug dependence, the present case was diagnosed as primitive opsoclonus.

摘要

本文描述了金氏综合征(共济失调 - 眼阵挛 - 肌阵挛)。该综合征的特征为:上呼吸道和肠道系统感染以及行为障碍,进而导致典型的三联征症状。进行了各种检查以确定是否存在神经母细胞瘤,因为该综合征常与之相关(46%)。这些检查结果为阴性。针对其他具有与金氏综合征相似特征症状的病症进行了鉴别诊断。合成促肾上腺皮质激素(辛纳科)治疗使临床症状有所缓解。基于所进行的检查(即使在36个月后结果始终为阴性)以及药物依赖性,本病例被诊断为原发性眼阵挛。

相似文献

1
[Opsoclonic encephalopathy in childhood (Kinsbourne syndrome)].儿童眼阵挛性脑病(金斯伯恩综合征)
Pediatr Med Chir. 1985 May-Jun;7(3):437-41.
2
[Myoclonic encephalopathy (Kinsbourne syndrome)].
Padiatr Padol. 1984;19(3):279-85.
3
[Ataxia-opsoclonus-myoclonus syndrome].[共济失调-眼阵挛-肌阵挛综合征]
Sem Hop. 1981;57(9-10):488-94.
4
[Kinsbourne's myoclonic encephalopathy in the light of our clinical observations].
Pol Tyg Lek. 1984 May 28;39(22):743-6.
5
[Kinsbourne's disease. Study of four cases (author's transl)].
An Esp Pediatr. 1978 Jun-Jul;11(6-7):461-70.
6
[Benign brainstem encephalopathy with truncal ataxia--a clinical study of 3 cases].[伴有躯干共济失调的良性脑干脑病——3例临床研究]
No To Shinkei. 1992 Oct;44(10):893-8.
7
[Opsoclonus-myoclonus syndrome in children with neuroblastoma: description of 3 cases].[神经母细胞瘤患儿的眼阵挛-肌阵挛综合征:3例病例描述]
Przegl Lek. 2010;67(6):427-9.
8
Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae.儿童神经母细胞瘤所致眼阵挛-共济失调:发育及神经后遗症
Pediatrics. 2002 Jan;109(1):86-98.
9
[Ataxia-myoclonus-opsoclonus syndrome (author's transl)].共济失调-肌阵挛-眼阵挛综合征(作者译)
Ann Pediatr (Paris). 1980 May;27(5):269-75.
10
Cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma.小脑共济失调和眼球阵挛作为神经母细胞瘤相关肌阵挛性脑病的初始表现。
Childs Nerv Syst. 1987;3(4):245-7. doi: 10.1007/BF00274060.