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垂直注视麻痹常见类型及影像表现

 芋森遇缇慕 2020-01-08

目前对于支配眼球垂直运动的神经调控通路没有完全研究清楚。但通常认为眼球垂直凝视的皮质下中枢及其传导通路位于中脑,中脑病变及丘脑病变扩延至中脑水平均可引起垂直眼球凝视障碍。常见表现为4种综合征。

Parinaud’s syndrome

2 Vertical ‘half-and-a-half’ syndrome

3 Vertical ‘one-and-a-half’ syndrome

4 Vertical ‘one-and-a-one’ syndrome

1、Parinaud’s syndrome

Parinaud 综合征,或者叫中脑背侧综合征、顶盖前区综合征或后联合综合征。临床表现为双眼上视麻痹(如下图)。

中脑与眼球垂直运动相关结构主要有:内侧纵束头端间质核(riMLF)、Cajal间质核(interstitial nucleus of Cajal,INC)、后连合(posterior commissure,PC)。riMLF主要参与垂直扫视,INC参与垂直凝视和维持注视,PC使双眼活动同步。其结构位置如下图所示:

上视传导路径如下图:riMLF内神经元发出垂直扫视指令,INC内神经元发出垂直追踪指令,传导纤维在后联合部位交叉,投射到双侧动眼神经核的相应部分。当后联合相关区域病变时,患者出现双侧上视麻痹。

病例1:肿瘤压迫后联合

病例2:left poseromedial thalamic infarct

病例3:left paramedian midbrain lacunar stroke

2、Vertical ‘half-and-a-half’ syndrome

临床表现为病变同侧下视麻痹伴对侧上视麻痹。

正常垂直扫视传导通路为:riMLF内神经元发出垂直扫视指令后,上视扫视纤维通过后联合交叉传导至对侧动眼神经上直肌核,支配上直肌,下视扫视纤维在同侧下行至同侧动眼神经下直肌核,支配下直肌。完成垂直扫视。如下图所示:

当出现后联合交叉前病变时,出现同侧下视麻痹合并对侧上视麻痹,如图所示:

病例1:a small stroke restricted to the thalamic-mesencephalic junction

病例2:a small thalamomesencephalic stroke

3、Vertical ‘one-and-a-half’ syndrome

“垂直一个半综合征”,常表现为一眼上视麻痹,双眼下视麻痹。

当病变累及riMLF及后联合交叉时,可表现出同侧上视、下视麻痹伴有对侧上视麻痹,传导通路及病变位置如下图:

病例:left periventricular mesodiencephalic infarct

(a)axial FLAIR and (b)diffusion-weighted image showing left periventricular mesodiencephalic infarct at the bottom of the third ventricle.

4、Vertical ‘one-and-a-one’ syndrome

临床表现为双眼均不能上视或下视,影像学表现常为单侧病变,具体机制不明,有待进一步研究明确,仅将病例列举如下:

病例1:Brain magnetic resonance imaging-diffusion-weighted imaging axial view (a and b) bright signal change in the right paramedian midbrain and anteromedial thalamus (red arrow); apparent diffusion coefficient axial view (d and e) corresponding dark signal change (red arrow); magnetic resonance angiography of intracranial arteries shows thinning of right posterior cerebral artery (red arrow)(c)

病例2:Brain magnetic resonance imaging-diffusion-weighted imaging axial view (a) bright signal change in left anteromedial thalamus (red arrow); apparent diffusion coefficient axial view (b) corresponding dark signal change (red arrow)。magnetic resonance angiography of intracranial arteries shows no abnormality (c)

病例3:Brain magnetic resonance imaging-diffusion-weighted imaging axial view (a) bright signal change in the left thalamus (red arrow) and occipital cortex; magnetic resonance angiography (b) shows thinning of both  osterior cerebral artery。

病例4:(a) T2 axial and (b) diffusion MRI of the midbrain. The arrows indicate the ischemic lesion at caudal (left) and rostral (right) midbrain levels.

参考文献

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[3] Priscilla Xinhui Wang and Srinivasan Sanjay.Parinaud Syndrome in Association with Thalamic Infarct in a Young Person [J]. Ophthalmology Research: An International Journal.2014, Vol. 2 (No.5) :241-249

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[6] Deleu, Dirk.Vertical one-and-a-half syndrome with contralesional pseudo-abducens palsy in a patient with thalamomesencephalic stroke.[J].Journal of the Neurological Sciences.2012,Vol.312(No.1-2):180-183.

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