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Supranuclear and Internuclear Ocular Motility Disorders CHAPTER 19 Supranuclear and Internuclear Ocular Motility Disorders David S. Zee and David Newman-Toker OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN OCULAR MOTOR SYNDROMES CAUSED BY LESIONS OF THE MEDULLA THE SUPERIOR COLLICULUS Wallenberg’s Syndrome (Lateral Medullary Infarction) OCULAR MOTOR SYNDROMES CAUSED BY LESIONS OF Syndrome of the Anterior Inferior Cerebellar Artery THE THALAMUS Skew Deviation and the Ocular Tilt Reaction OCULAR MOTOR ABNORMALITIES AND DISEASES OF THE OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN BASAL GANGLIA THE CEREBELLUM Parkinson’s Disease Location of Lesions and Their Manifestations Huntington’s Disease Etiologies Other Diseases of Basal Ganglia OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN THE PONS THE CEREBRAL HEMISPHERES Lesions of the Internuclear System: Internuclear Acute Lesions Ophthalmoplegia Persistent Deficits Caused by Large Unilateral Lesions Lesions of the Abducens Nucleus Focal Lesions Lesions of the Paramedian Pontine Reticular Formation Ocular Motor Apraxia Combined Unilateral Conjugate Gaze Palsy and Internuclear Abnormal Eye Movements and Dementia Ophthalmoplegia (One-and-a-Half Syndrome) Ocular Motor Manifestations of Seizures Slow Saccades from Pontine Lesions Eye Movements in Stupor and Coma Saccadic Oscillations from Pontine Lesions OCULAR MOTOR DYSFUNCTION AND MULTIPLE OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN SCLEROSIS THE MESENCEPHALON OCULAR MOTOR MANIFESTATIONS OF SOME METABOLIC Sites and Manifestations of Lesions DISORDERS Neurologic Disorders that Primarily Affect the Mesencephalon EFFECTS OF DRUGS ON EYE MOVEMENTS In this chapter, we survey clinicopathologic correlations proach, although we also discuss certain metabolic, infec- for supranuclear ocular motor disorders. The presentation tious, degenerative, and inflammatory diseases in which su- follows the schema of the 1999 text by Leigh and Zee (1), pranuclear and internuclear disorders of eye movements are and the material in this chapter is intended to complement prominent. Details about these conditions can be found in that of Chapters 17 and 23. We emphasize an anatomic ap- the appropriate chapters in other volumes of this text. OCULAR MOTOR SYNDROMES CAUSED BY LESIONS IN THE MEDULLA Many structures within the medulla are important in the intercalatus nucleus, and ventrally the nucleus of Roller. control of eye movements: the vestibular nuclei, perihypog- These nuclei are interconnected with other ocular motor lossal nuclei, and inferior olive and its outflow pathway structures in the brain stem and cerebellum. The NPH and through the inferior cerebellar peduncle. The perihypoglos- the adjacent medial vestibular nuclei (MVN) are critical for sal nuclei consist of the nucleus prepositus hypoglossi holding horizontal positions of gaze (the neural integrator) (NPH), which lies in the floor of the fourth ventricle, the (2). These structures also participate in vertical gaze-hold- 907 908 CLINICAL NEURO-OPHTHALMOLOGY ing, although more rostral structures, especially the intersti- neurons and their dendrites contain increased acetylcholines- tial nucleus of Cajal (INC), also contribute. With lesions in terase reaction product (14). Guillain and Mollaret (15) sug- the paramedian structures of the medulla, nystagmus—com- gested that disruption of connections between the dentate monly upbeat—is the most common finding (3). Upbeat and the contralateral olivary nucleus (which run via the red nystagmus may also reflect involvement of a ventral tegmen- nucleus and central tegmental tract) causes this syndrome. tal pathway for the upward vestibulo-ocular reflex (VOR) Another hypothesis is that the ocular oscillations are caused producing a downward vestibular bias and a consequent up- by instability in circuits that include the projection from the beat nystagmus (4). Wernicke’s disease commonly affects inferior olive to the flocculus, which is thought to be impor- the region of NPH and MVN, which may account for the tant in the adaptive control of the VOR (10). horizontal gaze-evoked nystagmus and spontaneous vertical Occasionally, lesions are restricted to the vestibular nu- nystagmus and loss of vestibular responses that occur with clei. For example, vertigo may be the sole symptom of an this disease. exacerbation of multiple sclerosis (MS) (16,17) and of brain Lesions of the inferior olivary nucleus or its connections stem ischemia (18–21). Nystagmus caused by lesions in the may produce the oculopalatal myoclonus (oculopalatal vestibular nuclei may be purely horizontal, vertical, or tor- tremor) syndrome (5,6). This condition usually develops sional, or mixed. Moreover, nystagmus from a central vestib- weeks to months after a brain stem or cerebellar infarction, ular lesion can mimic that caused by peripheral vestibular although it may also occur with degenerative conditions disease (22,23). Dolichoectasia of the basilar artery may pro- (7,8). The term myoclonus is misleading because the move- duce a variety of combinations of central and peripheral ves- ments of affected muscles are to and fro and are approxi- tibular syndromes (24,25). Microvascular compression of mately synchronized, typically at a rate of 2–4 cycles/sec. the vestibulocochlear nerve may produce paroxysmal ver- Ocular palatal tremor is the better term (6). The abnormal tigo (26,27). Brandt and Dieterich (28), Bu¨ttner et al. (29), ocular movements consist of pendular oscillations that are and Dieterich (30) provide useful topographic schemes for often vertical but may have a horizontal or torsional compo- localizing central vestibular syndromes and central vestibu- nent. Predominantly vertical oscillations are usually associ- lar nystagmus within the brain stem and cerebellum. ated with symmetric bilateral palatal tremor. Mixed vertical and torsional movements, sometimes disconjugate and with WALLENBERG’S SYNDROME (LATERAL a seesaw quality, are associated with unilateral or asymmet- MEDULLARY INFARCTION) ric palatal tremor (9,10). Occasionally, patients develop the eye oscillations without movements of the palate. Closing Lesions of the vestibular nuclei commonly affect neigh- the eyes may bring out the vertical ocular oscillations (11). boring structures, in particular the cerebellar peduncles and The nystagmus sometimes disappears with sleep, but the perihypoglossal nuclei. The best-recognized syndrome in- palatal movements usually persist. Occasionally, the oscilla- volving the vestibular nuclei is caused by a lateral medullary tions resolve spontaneously. Gabapentin may partially ame- infarction (Wallenberg’s syndrome) (Fig. 19.2). The typical liorate the eye oscillations (12). findings of Wallenberg’s syndrome are impairment of sensa- The main pathologic finding with palatal tremor is hyper- tion of pain and temperature over the ipsilateral face, ipsi- trophy of the inferior olivary nucleus, which is often seen on lateral Horner’s syndrome and limb ataxia, and dysarthria magnetic resonance (MR) imaging (13). The olivary nucleus and dysphagia. Sensation of pain and temperature are im- contains enlarged, vacuolated neurons with expanded den- paired over the contralateral trunk and limbs. The ipsilateral drites and enlarged astrocytes (Fig. 19.1). The hypertrophic facial nerve may also be affected if the infarct extends more Figure 19.1. Pathology of palato-ocular myoclonus. A sec- tion through the cerebellum and medulla shows marked demy- elination of the right dentate nucleus and restiform body (dou- ble arrows). The left inferior olive is hypertrophic and shows mild demyelination (arrow). (From Nathanson M. Palatal my- oclonus: further clinical and pathophysiological observations. Arch Neurol Psychiatry 1956;75Ϻ285–296.) SUPRANUCLEAR AND INTERNUCLEAR OCULAR MOTILITY DISORDERS 909 and unusual sensations of body and environmental tilt, often so bizarre as to suggest a psychogenic origin (35,36). Pa- tients may report that the whole room appears tilted on its side or even upside down; with their eyes closed, they may feel themselves to be tilted. Such symptoms are occasionally reported in patients without signs of lateral medullary infarc- tion and may be caused by transient brain stem or cerebellar ischemia (37–40) and occasionally with lesions in the thala- mus (41), cerebral hemispheres (42), or peripheral vestibular apparatus (43). Lateropulsion, a compelling sensation of being pulled to- ward the side of the lesion, is often a prominent symptom in patients with Wallenberg’s syndrome and is also reflected Figure 19.2. Wallenberg’s syndrome. Transverse section through the me- in the ocular motor system (44–46). If the patient is asked dulla oblongata showing a unilateral infarction in its dorsolateral region. to fix straight ahead and then gently close the lids, the eyes (From Ongerboer de Visser BW, Kuypers HGJM. Late blink reflex changes deviate conjugately toward the side of the lesion. This is in lateral medullary lesions: an electrophysiological and neuroanatomical reflected in the corrective saccades that the patient must study of Wallenberg’s syndrome. Brain 1978;101Ϻ285–294.) make on eye opening to reacquire the target. Lateropulsion may appear with a blink. Saccadic eye movements are also affected by lateropul- rostrally (Fig. 19.3). Wallenberg’s syndrome is most com- sion (Fig. 19.5) (44,46–49). Horizontal saccades directed monly caused by occlusion of the ipsilateral vertebral artery; toward the side of the lesion usually overshoot the target, occasionally, the posterior inferior cerebellar
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