The patients with myotonic dystrophy (MD) show ocular motor abnormalities including strabismus, vergence deficits, and inaccurate or slow saccades. Two theories have been proposed to explain the oculomotor deficits in MD. The central theory attributes the defects of eye movements of MD to the involvement of the central nervous system while the muscular theory attributes to dystrophic changes of the extraocular muscles. A 58-year-old woman with MD showed selective slowing of horizontal saccades and reduced peak velocities for both horizontal canals in head impulse tests, while smooth-pursuit eye movements and vertical head impulse responses were normal. This case suggests that the extraocular muscles-as a final common pathway of the voluntary saccade and reflexive vestibular eye movements-may better explain the defective rapid eye movements observed in MD.

Saccades are rapid eye movements that shift the line of sight between successive points of fixation. The cerebellum calibrates saccadic amplitude (dorsal vermis and fastigial nucleus) and the saccadic pulse‐step match (flocculus) for optimal visuo‐ocular motor behavior. Based on electrophysiology and the pharmacological inactivation studies, early activity in one fastigial nucleus could be important for accelerating the eyes at the beginning of a saccade, and the later activity in the other fastigial nucleus could be critical for stopping the eye on target, which is controlled by inhibitory projection from the dorsal vermis. The cerebellum could monitor a corollary discharge of the saccadic command and terminate the eye movement when it is calculated to be on target. The fastigial nucleus and dorsal vermis also participate in the adaptive control of saccadic accuracy.

Citations

• Quadrigeminal plate arachnoid cyst presenting with eye movement related migraine: a rare case report
  Yemima Graciela, Robert Shen, Mardjono Tjahjadi
  Medical Journal of Indonesia.2023; 32(2): 129.     CrossRef

Eye movements serve vision by placing the image of an object on the fovea of each retina, and by preventing slippage of images on the retina. The brain employs two modes of ocular motor control, fast eye movements (saccades) and smooth eye movements. Saccades bring the fovea to a target, and smooth eye movements prevent retinal image slip. Smooth eye movements comprise smooth pursuit, the optokinetic reflex, the vestibulo-ocular reflex (VOR), vergence, and fixation. Saccades achieve rapid refixation of targets that fall on the extrafoveal retina by moving the eyes at peak velocities that can exceed 700?s. Various brain lesions can affect saccadic latency, velocity, or accuracy. Smooth pursuit maintains fixation of a slowly moving target. The pursuit system responds to slippage of an image near the fovea in order to accelerate the eyes to a velocity that matches that of the target. When smooth eye movements velocity fails to match target velocity, catch-up saccades are used to compensate for limited smooth pursuit velocities. The VOR subserves vision by generating conjugate eye movements that are equal and opposite to head movements. If the VOR gain (the ratio of eye velocity to head velocity) is too high or too low, the target image is off the fovea, and head motion causes oscillopsia, an illusory to-and-fro movement of the environment.

Department of Neurology, Eulji Hospital Eulji University College of Medicine, Seoul, KoreaSpinocerebellar ataxia type 2 (SCA2) is characterized by progressive cerebellar ataxia and slow saccades. A 40-year-old woman presented with progressive gait disturbance and ataxia over 15 years. Neurologic examination revealed scanning speech, ataxia, and hyporeflexia. Brain CT showed diffuse atrophy of the cerebellum. Electronystagmography demonstrated slowed saccades with normal accuracy and delayed latency. The diagnosis of SCA2 was confirmed by the genetic test. Documentation of slow saccades may help differentiation among SCA subgroups.