Oculomotor Abnormalities in Friedreich's Ataxia
Oculomotor Abnormalities in Friedreich's Ataxia
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弗里德赖希共济失调的动眼神经异常
DOI：
10.1017/s0317167100119584
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发表时间：
1979
期刊：
影响因子：
--
通讯作者：
E. Andermann
中科院分区：
文献类型：
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作者：
T. H. Kirkham;D. Guitton;A. Katsarkas;L. B. Kline;E. Andermann
总结对14例弗里德赖希共济失调患者进行了临床神经眼科和眼电图研究。没有证据表明有视神经功能障碍。尽管所有患者都有不同程度的眼部运动缺陷，但没有患者抱怨示波症，这似乎与疾病一般表现的严重程度有关。缺陷包括方波急动、急动追踪，无法维持偏心注视，导致凝视麻痹性眼震和反弹性眼震。未能通过固定抑制视觉-眼反射。热性眼震的慢相速度常为减速。增加刺激速度不能增加视动性眼震的慢相速度。扫视系统的异常表现为远视。这些体征结合起来提示累及小脑、小脑小叶和小脑蚓部或其脑干连接的疾病。17名父母或兄弟姐妹均未发现异常。
Summary A clinical neuro-opthalmo-logical and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestíbulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cere be I lar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.