Nystagmus Syndromes

Nystagmus is defined as a repetitive or cyclical ocular movement in which the slow phase of the motion is abnormal. Nystagmus can be further delineated by the specific movement of the eyes. Nystagmus characterized by repetitive cyclical bidirectional slow phases is termed pendular nystagmus, whereas jerk nystagmus has alternating slow and fast phases in which the fast phase is a normal saccade returning the visual axis to its original position. A feature common to all forms of nystagmus is that the eyes repeatedly return to the starting position of the cycle. There are three basic functional types of nystagmus: (1) unfettered, cyclical eye movement command generators; (2) tonic bias; and (3) inadequate holding power in eccentric gaze. The first and second types are characterized by abnormal positive influences on the conjugate gaze mechanisms. The third type results from a deficit of function in the gaze-holding mechanism and was originally termed gaze paretic nystagmus, but now gaze-evoked nystagmus is the preferred term.

The most frequent clinical example of the first type of mechanism is congenital nystagmus, in which the eyes are pulled off the object of regard by cyclical abnormal signals that enter the conjugate gaze-generating systems from an unknown site or sites. The return fast phase is a normal saccade bringing about re-foveation whence the cycle resumes. It has been proposed that the variation of slow-phase trajectory from one patient to the next with congenital nystagmus reflects various strategies employed by the CNS as a whole to maintain fixation or foveation. The variable visual acuity in these patients attests to the varied success of these strategies. Patients who keep foveation longer can achieve better levels of visual acuity.

The second mechanism, bias nystagmus, is most frequently encountered with vestibular system disorders and with lesions causing asymmetry of smooth pursuit conjugate gaze systems. The factor in common is that both the pursuit mechanism and the vestibular system are organized into two bilaterally symmetrical, tonically active systems in which the right and left sides oppose each other. Each system feeds commands into the horizontal and vertical gaze-generating systems, and in health, each side is equally active so there is no net driving force in any direction away from primary gaze. When disease affects one side more than the other, a net tonic drive or bias develops. The function of the vestibular system is to keep the visual axis on a fixed point in external visual space despite movement of the head in all planes (see Chapter.12 ). For instance, as the head rotates rightward at 10 degrees per second, an induced leftward eye movement at 10 degrees per second occurs, so that the external world remains fixed on the fovea. The VOR, however, does not supply the entire eye movement drive and works together with the pursuit system to optimize function.

Tonic vestibular system imbalance passes a tonic directional bias to the brain stem gaze centers, which causes the eyes to drift toward the side with less activity. The tonic influence of each side is contraversive, and a lesion creates underactivity of the ipsilateral system, with relative overactivity of the opposite system. The unopposed contraversive tone of the system opposite the lesion imposes eye drift toward the lesion side. This drift is checked by rhythmically occurring saccades in the opposite direction. The ensemble effect is rhythmical jerk-type nystagmus with slow-phase movements toward the side with the lesion and fast phases away from the lesion. This nystagmus is rhythmical because the slow-phase drift is constant velocity, determined by the degree of bias or imbalance between the two lateral vestibular subsystems, and the corrective saccades occur whenever a certain fixed amount of retinal position error between object of regard and fovea occurs.

The third mechanism, or gaze-evoked nystagmus, occurs when the patient is unable to maintain eye deviation away from primary gaze. When neural integration in the brain stem circuits is faulty, an inadequate step or position command results, and saccades made to eccentric gaze positions are followed by a backward drift of the eyes toward primary position. When a sufficiently large retinal error signal is generated (the object of regard is now on a peripheral retinal point), another saccade is generated to refixate the eccentric point. This results in a series of saccades and back drifts that constitute gaze-evoked nystagmus. This third type of nystagmus differs from vestibular

and other bias forms of nystagmus in several ways. First, in gaze-evoked nystagmus, the velocity of the slow phase is determined by the difference between the oculomotor force being generated and the resisting forces that are tending to bring the eye back to primary position. These forces are largely contributed by elastic elements in the extraocular muscles and tendons and are greater when the eye is in a more eccentric position than when it is near primary gaze. This means that as the eyes drift back, the pulling force lessens as the elastic elements are less stretched, and the velocity of the slow phase declines exponentially. In vestibular nystagmus, on the other hand, the slow phase has constant velocity based on a fixed imbalance between the tonic forces of the two opposing vestibular systems. Second, gaze-evoked nystagmus depends on the continued effort of the subject to look in the direction of deficient holding power. As the urge to maintain the eccentric position wanes, the nystagmus slows down and may even stop with the eyes in a position close to primary gaze. Then if the subject is exhorted to look at the stimulus target, the nystagmus resumes. In general, vestibular nystagmus is faster than gaze-evoked nystagmus and keeps a fixed rate and rhythm. Third, gaze-evoked nystagmus is by definition not present in the primary position of gaze because it requires activation of a gaze shift. Vestibular nystagmus may be present in primary gaze if the intensity of the imbalance is great enough. Vestibular nystagmus is classified as first degree when it is present only with gaze in the direction of fast phase, second degree when it is present in primary gaze, and third degree when it is present in all positions of gaze.

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