• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

弗里德赖希共济失调中的固定不稳定性和眼球运动异常。

Fixation instability and oculomotor abnormalities in Friedreich's ataxia.

作者信息

Spieker S, Schulz J B, Petersen D, Fetter M, Klockgether T, Dichgans J

机构信息

Department of Neurology, University of Tübingen, Germany.

出版信息

J Neurol. 1995 Aug;242(8):517-21. doi: 10.1007/BF00867423.

DOI:10.1007/BF00867423
PMID:8530980
Abstract

Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnormalities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients showed various combinations of cerebellar, vestibular and brain-stem oculomotor signs. Our patients did not comprise a homogeneous group with regard to their oculomotor findings. There was no correlation between the severity of any of the so-called cerebellar oculomotor disturbances and the number of SWJ. We tried to correlate the extent of oculomotor disturbances with floccular atrophy and atrophy of the dorsal vermis on MRI in seven of the patients. None of the oculomotor features (including SWJ) correlated with flocculus or dorsal vermis size. Furthermore, floccular and vermal measurements on MRI were normal. Accordingly, we think it unlikely that the oculomotor disturbances, including SWJ, are attributable to cerebellar pathology per se.

摘要

对13例弗里德赖希共济失调患者的眼球运动进行了研究,并与MRI结果相关联,以调查在这种疾病中眼球运动异常是否可追溯到小脑功能障碍。最突出的眼部体征之一是注视不稳定(方波急跳,SWJ)。除SWJ外,患者还表现出小脑、前庭和脑干眼球运动体征的各种组合。就其眼球运动结果而言,我们的患者并非一个同质群体。任何所谓的小脑眼球运动障碍的严重程度与SWJ的数量之间均无相关性。我们试图将7例患者眼球运动障碍的程度与MRI上的绒球萎缩和小脑蚓部背侧萎缩相关联。没有任何眼球运动特征(包括SWJ)与绒球或小脑蚓部大小相关。此外,MRI上的绒球和蚓部测量结果正常。因此,我们认为包括SWJ在内的眼球运动障碍不太可能归因于小脑本身的病变。

相似文献

1
Fixation instability and oculomotor abnormalities in Friedreich's ataxia.弗里德赖希共济失调中的固定不稳定性和眼球运动异常。
J Neurol. 1995 Aug;242(8):517-21. doi: 10.1007/BF00867423.
2
(1)H MR spectroscopy in Friedreich's ataxia and ataxia with oculomotor apraxia type 2.(1)弗里德里希共济失调和眼动运动不能型 2 型的 H 磁共振波谱。
Brain Res. 2010 Oct 28;1358:200-10. doi: 10.1016/j.brainres.2010.08.030. Epub 2010 Aug 14.
3
Oculomotor abnormalities in Friedreich's ataxia.弗里德赖希共济失调中的动眼神经异常。
Can J Neurol Sci. 1979 May;6(2):167-72. doi: 10.1017/s0317167100119584.
4
Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich's ataxia.脊髓小脑共济失调3型、脊髓小脑共济失调6型及弗里德赖希共济失调中小脑皮质和核团的结构与功能磁共振成像异常
Brain. 2015 May;138(Pt 5):1182-97. doi: 10.1093/brain/awv064. Epub 2015 Mar 28.
5
Vestibular, saccadic and fixation abnormalities in genetically confirmed Friedreich ataxia.基因确诊的弗里德赖希共济失调患者的前庭、扫视和注视异常
Brain. 2008 Apr;131(Pt 4):1035-45. doi: 10.1093/brain/awm323. Epub 2008 Jan 30.
6
Eye movements in Friedreich's ataxia.弗里德赖希共济失调中的眼球运动。
Arch Neurol. 1983 Jun;40(6):343-6. doi: 10.1001/archneur.1983.04050060043006.
7
Structural and functional MRIs disclose cerebellar pathologies in idiopathic downbeat nystagmus.结构和功能磁共振成像揭示了特发性下跳性眼球震颤中的小脑病变。
Neurology. 2007 Sep 11;69(11):1128-35. doi: 10.1212/01.wnl.0000276953.00969.48.
8
Oculomotor and Vestibular Deficits in Friedreich Ataxia - Systematic Review and Meta-Analysis of Quantitative Measurements.Friedreich 共济失调的眼球运动和前庭功能障碍——定量测量的系统评价和荟萃分析。
Cerebellum. 2024 Dec;23(6):2269-2284. doi: 10.1007/s12311-024-01716-8. Epub 2024 Jul 27.
9
[Eye movement disorders in hereditary degenerative ataxia. Electro-oculographic study of 11 cases].[遗传性退行性共济失调中的眼球运动障碍。11例的眼电图研究]
Acta Neurol (Napoli). 1992 Aug-Dec;14(4-6):440-50.
10
Square wave jerks in Friedreich's ataxia.弗里德赖希共济失调中的方波急跳
Am J Ophthalmol. 1978 Mar;85(3):400-6. doi: 10.1016/s0002-9394(14)77738-4.

引用本文的文献

1
Best Oculomotor Endpoints for Clinical Trials in Hereditary Ataxias: A Systematic Review and Consensus by the Ataxia Global Initiative Working Group on Digital‑Motor Biomarkers.遗传性共济失调临床试验的最佳动眼神经终点:共济失调全球倡议数字运动生物标志物工作组的系统评价与共识
Cerebellum. 2025 Aug 13;24(5):141. doi: 10.1007/s12311-025-01894-z.
2
Assessing inter-ocular fixational eye movements throughout the lifespan.评估整个生命周期中的眼固视运动。
Exp Brain Res. 2024 Dec;242(12):2749-2763. doi: 10.1007/s00221-024-06936-2. Epub 2024 Oct 12.
3
Oculomotor and Vestibular Deficits in Friedreich Ataxia - Systematic Review and Meta-Analysis of Quantitative Measurements.

本文引用的文献

1
Magnetic resonance imaging in hereditary and idiopathic ataxia.遗传性和特发性共济失调的磁共振成像
Neurology. 1993 Feb;43(2):318-25. doi: 10.1212/wnl.43.2.318.
2
Oculomotor abnormalities and MRI findings in idiopathic cerebellar ataxia.特发性小脑共济失调的动眼神经异常与磁共振成像表现
J Neurol. 1994 Feb;241(4):234-41. doi: 10.1007/BF00863774.
3
Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features.弗里德赖希共济失调:对90个家庭的临床与遗传学研究,分析早期诊断标准及临床特征的家族内聚集情况。
Friedreich 共济失调的眼球运动和前庭功能障碍——定量测量的系统评价和荟萃分析。
Cerebellum. 2024 Dec;23(6):2269-2284. doi: 10.1007/s12311-024-01716-8. Epub 2024 Jul 27.
4
Ocular myasthenia gravis saccades as a measure of extraocular muscle function.重症肌无力性眼肌病的扫视运动作为眼外肌功能的一项指标
Front Ophthalmol (Lausanne). 2022 Oct 12;2:938088. doi: 10.3389/fopht.2022.938088. eCollection 2022.
5
A Review of Ocular Movement Abnormalities in Hereditary Cerebellar Ataxias.遗传性小脑共济失调的眼球运动异常综述。
Cerebellum. 2024 Apr;23(2):702-721. doi: 10.1007/s12311-023-01554-0. Epub 2023 Mar 31.
6
Quantitative Oculomotor Assessment in Hereditary Ataxia: Discriminatory Power, Correlation with Severity Measures, and Recommended Parameters for Specific Genotypes.遗传性共济失调的定量眼动评估:鉴别能力、与严重度测量的相关性,以及特定基因型的推荐参数。
Cerebellum. 2024 Feb;23(1):121-135. doi: 10.1007/s12311-023-01514-8. Epub 2023 Jan 14.
7
Neuro-Ophthalmological Findings in Friedreich's Ataxia.弗里德赖希共济失调的神经眼科表现
J Pers Med. 2021 Jul 23;11(8):708. doi: 10.3390/jpm11080708.
8
Vestibular impact of Friedreich ataxia in early onset patients.早发型弗里德赖希共济失调的前庭影响
Cerebellum Ataxias. 2020 May 28;7:6. doi: 10.1186/s40673-020-00115-z. eCollection 2020.
9
Ocular Involvement in Friedreich Ataxia Patients and its Relationship with Neurological Disability, a Follow-up Study.弗里德赖希共济失调患者的眼部受累情况及其与神经功能障碍的关系:一项随访研究
Diagnostics (Basel). 2020 Jan 29;10(2):75. doi: 10.3390/diagnostics10020075.
10
A Cortical Substrate for Square-Wave Jerks in Progressive Supranuclear Palsy.进行性核上性麻痹中方形波急跳的皮质基质
J Clin Neurol. 2020 Jan;16(1):37-45. doi: 10.3988/jcn.2020.16.1.37.
Brain. 1981 Sep;104(3):589-620. doi: 10.1093/brain/104.3.589.
4
Cerebellar control of fixation eye movements.小脑对注视眼动的控制。
Neurology. 1982 Jan;32(1):31-6. doi: 10.1212/wnl.32.1.31.
5
Eye movements in Friedreich's ataxia.弗里德赖希共济失调中的眼球运动。
Arch Neurol. 1983 Jun;40(6):343-6. doi: 10.1001/archneur.1983.04050060043006.
6
Neuro-otological abnormalities in Friedreich's ataxia.弗里德赖希共济失调的神经耳科学异常
J Neurol Neurosurg Psychiatry. 1984 Jan;47(1):26-32. doi: 10.1136/jnnp.47.1.26.
7
Follow-up study of electronystagmographic findings in Friedreich's ataxia patients and evaluation of their relatives.弗里德赖希共济失调患者眼震电图检查结果的随访研究及其亲属评估
Can J Neurol Sci. 1984 Nov;11(4 Suppl):570-3. doi: 10.1017/s031716710003506x.
8
Macrosaccadic oscillation, saccadic dysmetria and motor error in spinocerebellar degeneration.脊髓小脑变性中的巨眼跳振荡、眼跳辨距不良和运动误差
Jpn J Ophthalmol. 1985;29(4):369-77.
9
Reversible brain shrinkage in abstinent alcoholics, measured by MRI.通过磁共振成像(MRI)测量发现,戒酒的酗酒者存在可逆性脑萎缩。
Neuroradiology. 1988;30(5):385-9. doi: 10.1007/BF00404102.
10
Significance of MRI-confirmed atrophy of the cranial spinal cord in Friedreich's ataxia.MRI确诊的弗里德赖希共济失调患者颅脊髓萎缩的意义
Eur Arch Psychiatry Neurol Sci. 1989;238(4):225-30. doi: 10.1007/BF00381470.