Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
Center for Sensorimotor Research, Department of Neurology, Ludwig-Maximilian University Munich, Munich, Germany.
Brain. 2018 Mar 1;141(3):762-775. doi: 10.1093/brain/awx381.
Here we characterize persistent apogeotropic type of central positional nystagmus, and compare it with the apogeotropic nystagmus of benign paroxysmal positional vertigo involving the lateral canal. Nystagmus was recorded in 27 patients with apogeotropic type of central positional nystagmus (22 with unilateral and five with diffuse cerebellar lesions) and 20 patients with apogeotropic nystagmus of benign paroxysmal positional vertigo. They were tested while sitting, while supine with the head straight back, and in the right and left ear-down positions. The intensity of spontaneous nystagmus was similar while sitting and supine in apogeotropic type of central positional nystagmus, but greater when supine in apogeotropic nystagmus of benign paroxysmal positional vertigo. In central positional nystagmus, when due to a focal pathology, the lesions mostly overlapped in the vestibulocerebellum (nodulus, uvula, and tonsil). We suggest a mechanism for apogeotropic type of central positional nystagmus based on the location of lesions and a model that uses the velocity-storage mechanism. During both tilt and translation, the otolith organs can relay the same gravito-inertial acceleration signal. This inherent ambiguity can be resolved by a 'tilt-estimator circuit' in which information from the semicircular canals about head rotation is combined with otolith information about linear acceleration through the velocity-storage mechanism. An example of how this mechanism works in normal subjects is the sustained horizontal nystagmus that is produced when a normal subject is rotated at a constant speed around an axis that is tilted away from the true vertical (off-vertical axis rotation). We propose that when the tilt-estimator circuit malfunctions, for example, with lesions in the vestibulocerebellum, the estimate of the direction of gravity is erroneously biased away from true vertical. If the bias is toward the nose, when the head is turned to the side while supine, there will be sustained, unwanted, horizontal positional nystagmus (apogeotropic type of central positional nystagmus) because of an inappropriate feedback signal indicating that the head is rotating when it is not.
我们对持续性向地性中枢性位置性眼球震颤进行了特征描述,并将其与涉及外侧半规管的良性阵发性位置性眩晕的向地性眼震进行了比较。记录了 27 例向地性中枢性位置性眼球震颤患者(22 例单侧病变,5 例弥漫性小脑病变)和 20 例良性阵发性位置性眩晕向地性眼震患者的眼震。他们在坐位、仰卧位头直位和右耳低位及左耳低位进行检查。向地性中枢性位置性眼球震颤患者仰卧位与坐位时自发性眼球震颤强度相似,但良性阵发性位置性眩晕向地性眼震患者仰卧位时更强。在中枢性位置性眼球震颤中,当病灶为局灶性病变时,病变主要重叠于前庭小脑(小结、垂乳突和扁桃体)。我们根据病变部位和使用速度储存机制的模型提出了向地性中枢性位置性眼球震颤的机制。在倾斜和平移过程中,耳石器官可以传递相同的重感惯性加速度信号。这种固有的模糊性可以通过“倾斜估计器电路”来解决,该电路将关于头部旋转的半规管信息与关于线性加速度的耳石信息通过速度储存机制相结合。这一机制在正常受试者中的作用的一个例子是,当正常受试者以偏离真实垂直的轴(偏离垂直轴旋转)以恒定速度旋转时,会产生持续的水平眼球震颤。我们提出,当倾斜估计器电路出现故障时,例如前庭小脑病变,重力方向的估计会错误地偏离真实垂直。如果偏差指向鼻子,当仰卧位时头部转向一侧,由于指示头部旋转的不当反馈信号,会出现持续的、不想要的水平位置性眼球震颤(向地性中枢性位置性眼球震颤),而头部实际上并未旋转。