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儿童费希尔综合征

Fisher syndrome in childhood.

作者信息

Becker W J, Watters G V, Humphreys P

出版信息

Neurology. 1981 May;31(5):555-60. doi: 10.1212/wnl.31.5.555.

DOI:10.1212/wnl.31.5.555
PMID:7194971
Abstract

Three children, age 2, 7, and 12, developed Fisher syndrome: inability to walk because of ataxia, complete areflexia, and ophthalmoplegia. Ptosis was prominent in all the children, but the pupillary response to light was affected only in one child. Limb weakness was never present, and sensation normal. One patient was obtunded for several days. Two had prodromal upper respiratory tract illnesses, and the third patient was bitten by an insect 2 days before her symptoms began. Cerebrospinal fluid (CSF) protein content was moderately increased in all patients, but only one child's CSF had a pleocytosis. CSF gamma globulin levels were normal. In the acute phase, F waves and H responses were absent in two cases but returned to normal with clinical recovery. All three patients had marked electroencephalographic (EEG) abnormalities that later improved. The EEG pattern suggested a brainstem disorder. These findings plus the obtundation, gaze paralysis, and ataxia indicate that in Fisher syndrome there is parenchymal involvement of the central nervous system with or without nerve root involvement.

摘要

三名儿童,年龄分别为2岁、7岁和12岁,患上了费舍尔综合征:因共济失调而无法行走、完全性反射消失以及眼肌麻痹。所有儿童均有明显的上睑下垂,但只有一名儿童的瞳孔对光反应受到影响。从未出现肢体无力,感觉正常。一名患者昏迷了几天。两名患者前驱有上呼吸道疾病,第三名患者在症状出现前2天被昆虫叮咬。所有患者的脑脊液(CSF)蛋白含量均中度升高,但只有一名儿童的脑脊液有细胞增多。脑脊液γ球蛋白水平正常。急性期,两例患者的F波和H反射消失,但随着临床恢复而恢复正常。所有三名患者均有明显的脑电图(EEG)异常,随后有所改善。脑电图模式提示脑干疾病。这些发现加上昏迷、凝视麻痹和共济失调表明,在费舍尔综合征中,中枢神经系统存在实质性受累,伴或不伴有神经根受累。

相似文献

1
Fisher syndrome in childhood.儿童费希尔综合征
Neurology. 1981 May;31(5):555-60. doi: 10.1212/wnl.31.5.555.
2
Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases.米勒·费雪综合征:10例源于外周的临床及电生理学证据
Neurology. 1984 Jul;34(7):953-6. doi: 10.1212/wnl.34.7.953.
3
[Syndrome of ophthalmoplegia, ataxia and areflexia of Fisher in a 14 year old boy (author's transl)].一名14岁男孩的费希尔眼肌麻痹、共济失调和无反射综合征(作者译)
Klin Padiatr. 1981 Jul;193(4):341-2. doi: 10.1055/s-2008-1034491.
4
Syndrome of ophthalmoplegia, ataxia and areflexia.眼肌麻痹、共济失调和反射消失综合征
Clin Exp Neurol. 1977;14:75-82.
5
Acute ophthalmoplegia, ataxia, and areflexia (Fisher syndrome) in childhood.儿童急性眼肌麻痹、共济失调和反射消失(费希尔综合征)
Cleve Clin Q. 1978 Summer;45(2):247-52. doi: 10.3949/ccjm.45.2.247.
6
[The Miller Fisher syndrome: ophthalmoplegia, ataxia and areflexia].[米勒-费雪综合征:眼肌麻痹、共济失调和无反射]
Ugeskr Laeger. 1985 Feb 4;147(6):515-7.
7
[Ophthalmoplegia, ataxia and areflexia: the Miller Fisher syndrome].
Tidsskr Nor Laegeforen. 1988 Aug 20;108(23):1781-2.
8
The not-so-benign Miller Fisher syndrome: a variant of the Guilain-Barré syndrome.并非良性的米勒-费希尔综合征:吉兰-巴雷综合征的一种变异型
Arch Neurol. 1980 Jun;37(6):384-5. doi: 10.1001/archneur.1980.00500550086016.
9
Fisher syndrome in childhood.儿童费希尔综合征
Neurology. 1982 Jul;32(7):786-7. doi: 10.1212/wnl.32.7.786-b.
10
Electrophysiological study of four patients with Fisher syndrome: a mechanism of areflexia.4例费舍尔综合征患者的电生理研究:无反射机制
Brain Dev. 1992 Jul;14(4):235-8. doi: 10.1016/s0387-7604(12)80236-1.

引用本文的文献

1
Instructive case report.指导性病例报告。
Infect Dis Newsl (N Y). 1982;1(10):77-78. doi: 10.1016/S0278-2316(82)80014-5. Epub 2007 Jan 17.
2
Miller Fisher syndrome in infancy.
Childs Nerv Syst. 1996 Sep;12(9):559-61. doi: 10.1007/BF00261612.
3
"Mitochondrial myopathy" or mitochondrial disease? EEG, ERG, VEP studies in 13 children.“线粒体肌病”还是线粒体疾病?13例儿童的脑电图、视网膜电图、视觉诱发电位研究
J Neurol Neurosurg Psychiatry. 1982 Jul;45(7):627-32. doi: 10.1136/jnnp.45.7.627.
4
The nosological position of Fisher's syndrome (ophthalmoplegia, ataxia and areflexia).费希尔综合征(眼肌麻痹、共济失调和无反射)的疾病分类地位。
J Neurol. 1983;229(1):33-44. doi: 10.1007/BF00313494.
5
Demyelinating optic neuropathy with Miller-Fisher syndrome. The case for overlap syndromes with central and peripheral demyelination.伴米勒-费雪综合征的脱髓鞘性视神经病变。中枢与周围脱髓鞘重叠综合征病例。
J Neurol. 1987 Jun;234(5):353-8. doi: 10.1007/BF00314295.
6
A case of Miller Fisher syndrome: atypical findings and therapeutic considerations.
Ital J Neurol Sci. 1992 May;13(4):365-7. doi: 10.1007/BF02223105.