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Review
. 2014 Sep;261 Suppl 2(Suppl 2):S542-58.
doi: 10.1007/s00415-014-7385-9.

Central Ocular Motor Disorders, Including Gaze Palsy and Nystagmus

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Free PMC article
Review

Central Ocular Motor Disorders, Including Gaze Palsy and Nystagmus

M Strupp et al. J Neurol. .
Free PMC article

Abstract

An impairment of eye movements, or nystagmus, is seen in many diseases of the central nervous system, in particular those affecting the brainstem and cerebellum, as well as in those of the vestibular system. The key to diagnosis is a systematic clinical examination of the different types of eye movements, including: eye position, range of eye movements, smooth pursuit, saccades, gaze-holding function and optokinetic nystagmus, as well as testing for the different types of nystagmus (e.g., central fixation nystagmus or peripheral vestibular nystagmus). Depending on the time course of the signs and symptoms, eye movements often indicate a specific underlying cause (e.g., stroke or neurodegenerative or metabolic disorders). A detailed knowledge of the anatomy and physiology of eye movements enables the physician to localize the disturbance to a specific area in the brainstem (midbrain, pons or medulla) or cerebellum (in particular the flocculus). For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fascicle, with impaired vertical saccades only, the interstitial nucleus of Cajal or the posterior commissure; common causes with an acute onset are an infarction or bleeding in the upper midbrain or in patients with chronic progressive supranuclear palsy (PSP) and Niemann-Pick type C (NP-C). Isolated dysfunction of horizontal saccades is due to a pontine lesion affecting the paramedian pontine reticular formation due, for instance, to brainstem bleeding, glioma or Gaucher disease type 3; an impairment of horizontal and vertical saccades is found in later stages of PSP, NP-C and Gaucher disease type 3. Gaze-evoked nystagmus (GEN) in all directions indicates a cerebellar dysfunction and can have multiple causes such as drugs, in particular antiepileptics, chronic alcohol abuse, neurodegenerative cerebellar disorders or cerebellar ataxias; purely vertical GEN is due to a midbrain lesion, while purely horizontal GEN is due to a pontomedullary lesion. The pathognomonic clinical sign of internuclear ophthalmoplegia is an impaired adduction while testing horizontal saccades on the side of the lesion in the ipsilateral medial longitudinal fascicule. The most common pathological types of central nystagmus are downbeat nystagmus (DBN) and upbeat nystagmus (UBN). DBN is generally due to cerebellar dysfunction affecting the flocculus bilaterally (e.g., due to a neurodegenerative disease). Treatment options exist for a few disorders: miglustat for NP-C and aminopyridines for DBN and UBN. It is therefore particularly important to identify treatable cases with these conditions.

Figures

Fig. 1
Fig. 1
Cover and uncover test examination: examination to detect misalignments of the visual axes (modified from [3])
Fig. 2
Fig. 2
Clinical examination using a Fresnel-based device as an alternative to Frenzel’s goggles. The lenses prevent gaze fixation, which may suppress peripheral vestibular spontaneous nystagmus, for example. In addition, they make it easier to study the patient’s eye movements. When these lenses are used to examine a patient, attention should be paid to possible spontaneous nystagmus, GEN, head-shaking nystagmus (to this end, the patient should be asked to turn his/her head quickly from right to left and back, about 20 times; subsequently the eye movements should be studied), positional nystagmus, and hyperventilation-induced nystagmus. Positioning nystagmus indicates a muscle tonus imbalance of the VOR; if this originates from a peripheral vestibular lesion—as occurs, for example, in vestibular neuritis—then the nystagmus can be typically suppressed by visual fixation. Head-shaking nystagmus indicates a latent asymmetry of the so-called velocity storage; this may be due to peripheral or central vestibular functional disorders
Fig. 3
Fig. 3
Clinical examination of eye position and eye movements with an examination flashlight. The advantage of this examination is that the images reflected on the retina can be observed and ocular misalignments identified. It is important that the examiner looks at the retinal images from the direction of the light and that the patient is instructed to fixate his/her gaze on the target object. GEN to all sides is usually caused by medication (such as antiepileptic drugs or benzodiazepines) or intoxication (e.g., alcohol). DBN increases when looking sideways and when looking downwards (modified from [3])
Fig. 4
Fig. 4
Clinical examination of saccades. Spontaneous saccades that are triggered by visual or acoustic stimuli should be studied first. Then the patient should be asked to switch his/her gaze between two horizontal and two vertical targets. The velocity and accuracy of the saccades should be observed, and whether they are conjugate. In healthy subjects, the target will be reached immediately or will be made by one correctional saccade. Slow saccades in all directions typically occur in neurodegenerative disorders. Slowed horizontal saccades are usually observed in pontine brainstem lesions and slowed vertical saccades in midbrain lesions. Hypermetric saccades, which are recognized by a corrective saccade back to the target, are found in cerebellar lesions. The pathognomonic sign of internuclear ophthalmoplegia is a slowed adducent saccade ipsilaterally to the defect of the medial longitudinal fasciculus. (Modified from [3])
Fig. 5
Fig. 5
Video-oculography (VOG) allows the recording of all types of eye movements: a VOG device; b examination of a child, sitting in front of a screen, fixating and following the targets presented. VOG is particularly relevant to measuring the velocity of saccades to detect mild to moderate slowing as found in the initial stages of PSP or NP-C. Therefore, it could be a sensitive tool for an early diagnosis of these diseases
Fig. 6
Fig. 6
Examination of eye movements with an optokinetic drum: a vertical direction; b horizontal direction (modified from [3])
Fig. 7
Fig. 7
The supranuclear centers for control of eye movements. These centers allow exact topographical determination: lesions in the region of the interstitial nucleus of Cajal (INC) lead to a vertical gaze-holding defect; lesions in the region of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) lead to impairments of vertical saccades; lesions of the PPRF result in impairments of the horizontal saccades and; lesions of the nucleus prepositus hypoglossi (NPH) are characterized by a horizontal gaze-holding defect (adapted from [7, 37] and modified from [2])
Fig. 8
Fig. 8
Recording of: a vertical and b horizontal saccades in a patient with NP-C. There is a significant slowing of the velocity of vertical saccades (upward 26°/s, downward 11°/s). The velocity of horizontal saccades is more than 350°/s which is within the lower normal range. Typically, there is first a slowing of vertical downward, then upward and finally also horizontal saccades in patients with NP-C

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References

    1. Leigh RJ, Zee D. The neurology of eye movements. Oxford: Oxford University Press; 2006.
    1. Strupp M, Hufner K, Sandmann R, Zwergal A, Dieterich M, Jahn K, Brandt T. Central oculomotor disturbances and nystagmus: a window into the brainstem and cerebellum. Dtsch Arztebl Int. 2011;108:197–204. - PMC - PubMed
    1. Brandt T, Dieterich M, Strupp M. Vertigo and dizziness—common complaints. 2. London: Springer; 2013.
    1. Cnyrim CD, Newman-Toker D, Karch C, Brandt T, Strupp M. Bedside differentiation of vestibular neuritis from central “vestibular pseudoneuritis”. J Neurol Neurosurg Psychiatry. 2008;79:458–460. doi: 10.1136/jnnp.2007.123596. - DOI - PubMed
    1. Kattah JC, Talkad AV, Wang DZ, Hsieh YH, Newman-Toker DE. HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging. Stroke. 2009;40:3504–3510. doi: 10.1161/STROKEAHA.109.551234. - DOI - PMC - PubMed

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