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迟发泰-萨克斯病的神经眼科学

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世界核心医学期刊文摘（神经病学分册） 2005年第5期1卷 32-33页

作者： Rucker J.C. Shapiro B.E. R.J. Leigh 李一明 Department of Neuro logy University Hospitals 11100 Euclid Ave. Cleveland OH 44106- 5040 Unite d States Dr.

Background: Late-onset Tay-Sachs disease (LOTS) is an adult-onset, auto somal recessive, progressive variant of GM2 gangliosidosis, characterized by inv olvement of the cerebellum and anterior horn cells. Abstract Objective: To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluati ng the effectiveness of novel therapies. Methods: Fourteen patients with biochem ically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age-matched control subjects were studied. Snellen visual acuit y, contrast sensitivity, color vision, stereopsis, and visual fields were measur ed, and optic fundi were photographed. Horizontal and vertical eye movements (se arch coil) were recorded, and saccades, pursuit, vestibulo-ocular reflex (VOR) , vergence, and optokinetic (OK) responses were measured. Results: All patients showed normal visual functions and optic fundi. The main eye movement abnormalit y concerned saccades, which were “ multistep,” consisting of a series of smal l saccades and larger movements that showed transient decelerations. Larger sacc ades ended earlier and more abruptly (greater peak deceleration) in LOTS patient s than in control subjects; these changes can be attributed to premature termina tion of the saccadic pulse. Smooth-pursuit and slow-phase OK gains were redu ced, but VOR, vergence, and gaze holding were normal. Conclusions: Patients with late-onset Tay-Sachs disease (LOTS) show characteristic abnormalities of sa ccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a “ latch circuit” that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.

关键词：泰-萨克斯病神经眼科学眼球运动异常探察线圈前庭眼球反射神经节苷脂例生脊髓前角细胞循环通路对比敏感性

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单眼分离性眼颤和眼部追踪运动病例

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世界核心医学期刊文摘（眼科学分册） 2005年第1期1卷 3-3页

作者：张少娟 Onofrj M Iacono D Luciano A.L Neurophysiopatholog y Dep t.of Oncolog y and NeuroscienceUniv.G D 'Annunzio Chieti-PescaraVia Fonte Romana65124PescaraItaly.Prof

A patient affected by an ischaemic le sion of the right me-dial thalamic nucleus presented wit h a uniocular dissocia-tion of upward vertical saccades and pursuit movement,with absent upward vertical saccades in the left ***-ical observations were confirmed by magnetic field scleral search coils *** the ve rtical eye movement the patient denied any diplopia,thus su ggesting a transient visual suppression in the left eye.

关键词：分离性探察线圈丘脑中央中核垂直性

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鸡蛋自动进瓶又出瓶实验的另一种方法

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教学仪器与实验 1997年第3期17卷 28-28页

作者：李允义北京师大附属实验中学

鸡蛋自动进瓶又出瓶实验的另一种方法北京师大附属实验中学李允义鸡蛋自动进瓶的实验，常见方法是：先把蘸有酒精的棉团（或纸片）点燃，迅速投入广口瓶内，给瓶内空气加热。然后，把剥皮的熟鸡蛋及时堵在瓶口。少时，由于瓶内空气变冷... 详细信息

鸡蛋自动进瓶又出瓶实验的另一种方法北京师大附属实验中学李允义鸡蛋自动进瓶的实验，常见方法是：先把蘸有酒精的棉团（或纸片）点燃，迅速投入广口瓶内，给瓶内空气加热。然后，把剥皮的熟鸡蛋及时堵在瓶口。少时，由于瓶内空气变冷，外部大气压强大于瓶内气体压强，鸡...

关键词：冲击电流计地磁感应器鸡蛋气体压强探察线圈电子积分器法拉第定律实验中学感应电荷广口瓶