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Show Journal of Clinical Neuro-ophthalmology 13(4): 221-224, 1993. Unilateral Ptosis and Contralateral Eyelid Retraction from a Thalamic-Midbrain Infarction Magnetic Resonance Imaging Correlation Steven L. .Galetta, M.D., Lawrence G. Gray, 0.0./ Eric C. Raps, M.D., Robert 1. Grossman, M.D., and Norman J. Schatz, M.D. © 1993 Raven Press, Ltd., New York We report a patient with a third nerve palsy and contralateral eyelid retraction from a thalamic-midbrain infarction. Magnetic resonance imaging confirms that this unusual clinical combination, previously termed "the plusminus lid syndrome," results from a lesion in the region of the nucleus of the posterior commissure with extension to the third nerve fascicle. Key Words: Ptosis-Lid retraction-Midbrain-Magnetic resonance imaging. From the Departments of Neurology (S.L.G., L.G.G., E.eR., N.J.S.) and Radiology (R.I.G.) at the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, and the Department of Ophthalmology (N.J.S.) at the B~~com ~alm.er Eye Institute, University of Miami School of Medlcme, MIamI, florida, U.S.A. Address correspondence and reprint requests to Dr. Steven L. Galetta, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, U.S.A. 221 Unilateral ptosis and contralateral eyelid retraction most commonly occurs with myasthenia gravis, but may be observed in patients with third nerve palsy, thyroid eye disease, orbital myositis, congenital ptosis, and trauma (1-5). Gaymard and colleagues (6,7) have observed this unusual clinical finding in two patients with "nuclear" third nerve palsies and lesions of the paramedian mesencephalon as documented by computed tomography. This combination of unilateral ptosis and contralateral lid retraction has been termed the plus-minus lid syndrome (7). Based on their study of prior clinicopathologic reports, Schmidtke and Buttner-Ennever have attributed bilateral eyelid retraction to a unilateral lesion of the nucleus of the posterior commissure (NPC) (8). Since the NPC may not directly project onto the central caudal subnucleus, an intermediary region called the supraoculomotor area (supra III) also has been implicated in the premotor control of the lids (7-10). The precise function and consequences of a lesion to this loosely defined territory of supra III have not been fully elucidated (7,8). Thus, extension of a lesion from the region of the NPC, inferiorly and ventrally to involve the third nerve fascicle, would presumably be required to produce the ipsilateral ptosis and contralateral eyelid retraction observed. We report a patient who had a third nerve palsy and contralateral eyelid retraction from a magnetic resonance (MR) image-documented infarction in the region of NPC and the oculomotor fascicle. 222 S. L. CALETTA ET AL. FIG. 1. External photograph demonstrates right ptosis and contralateral hypotropic eye with persistent eyelid retraction. CASE HISTORY A 48-year-old man with a history of poorly controlled hypertension and recurrent vertigo developed sudden left-sided weakness and lethargy. Evaluation at an outside institution included brain MR imaging that revealed bilateral cerebellar infarctions and a right thalamic-midbrain infarction. An angiogram showed a left vertebral artery occlu-sion with a small, but patent, right vertebral artery. The patient was transferred to the Hospital of the University of Pennsylvania where examination revealed dysarthric speech, severe right ptosis, and persistent left eyelid retraction, even though the left eye was displaced downward (Fig. 1). Manual elevation of the ptotic lid did not alter the contralateral eyelid retraction. Pupils were 3 mm in FIG. 2. T2-weighted axial magnetic resonance images (TR = 3,000; TE = 90). A: High-signal abnormality is apparent rostral and dorsal to the right red nucleus (arrow). B: The lesion extends superiorly to the medial thalamus (arrow). This portion of the lesion (A and B) is associated with eyelid retraction (18). C: The lesion extends ventrally and inferiorly to involve the third nerve fascicle. JClin Neuro-ophthalmol, Vol. 13, No.4, 1993 PTOSIS AND EYELID RETRACTION 223 size and briskly reactive to light. The right eye would not elevate or adduct. Depression was limited to 10% while abduction was full. The left eye was hypotropic with full infraduction, 80% normal adduction, and 40% abduction. The left eye would elevate to the midline by command or Bell's phenomenon. Oculocephalic maneuvers did not further improve the eye movements. There was a partial left peripheral seventh nerve palsy with preserved eyelid closure. Facial sensation and hearing were also impaired on the left. The gag reflex was depressed, but the palate elevated in the midline. The tongue deviated to the left upon protrusion. A left flaccid hemiparesis, hemisensory loss, hyperreflexia, and plantar extensor response were present. A repeat MR showed bilateral middle cerebellar peduncle infarctions with extension into the pons bilaterally. There was a right medial thalamicmidbrain infarction involving the region dorsal and rostral to the red nucleus with extension ventrally and inferiorly to involve the area of the third nerve fascicle (Fig. 2). DISCUSSION Movement of the eyes and lids is exquisitely controlled and coordinated. This is most evident with eye descent as the lids normally follow the globes downward (1,11). This lid-eye synkinesis implies a tight relationship between the vertical gaze center and the neural network that controls the lids (Fig. 3) (8). In disorders involving the midbrain, lid function may become dissociated from vertical eye movements. For example, eyelid retraction and impaired upgaze are hallmark findings of the dorsal midbrain syndrome (12,13). In other patients with downgaze paralysis, the eyelids will descend normally creating a "pseudoptosis" (14,15). Rarely, the eyes will descend appropriately with impaired lid descent producing the phenomenon of lid lag (13,16-18). Our patient had motility and lid findings similar to those described by Gaymard and colleagues (6,7) and others (19). An ipsilateral third nerve palsy with a supraduction deficit of the fellow eye suggests a nuclear lesion (3,4) or a superimposed vertical gaze palsy from involvement of the rostral interstitial nucleus of the medial longitudinal fasciculus and interstitial nucleus of Cajal (20). Ptosis implies extension to the third nerve fascicle. Finally, contralateral lid retraction and lag demonstrates failed inhibition from the NPC or supra III FIG. 3. Theoretical scheme of lid-eye coordination based on data from Schmidtke and Buttner-Ennever (8). The rostral interstitial nucleus of the medial longitudinal fasciculus (RiMLF) is interconnected to the nucleus of the posterior commissure (NPC) (black arrows) to coordinate lid and vertical eye movements. The inhibitory pathway from the NPC may pass thru an intermediary region called the supraoculomotor area (supra III) to influence the central caudal nucleus (CCN). The RiMLF also projects to the subnuclei of the third nerve to mediate up and downgaze. IR, inferior rectus; SR, superior rectus; 10, inferior oblique (white arrows); MR, medial rectus; SO, superior oblique; RN, red nucleus. with sparing of the central caudal subnucleus (Fig. 4). Our patient also had left fifth, seventh, and eighth cranial nerve palsies. These deficits together with impairment of left eye horizontal movements localize to the left pons. However, the persistent left eyelid retraction observed is not a consequence of the partial facial palsy. A facial palsy may produce lagophthalmos, but eyelid retraction and lag are the result of impaired relaxation of the levator muscles. Evinger and colleagues (11) have demonstrated that the orbicularis oculi are not significantly involved in the production of downward lid saccades in normal subjects or those with Bell's palsy. Rather, the downward lid saccades that follow downward saccadic eye movements result primarily from passive relaxation of the levator palpebrae muscle (11). We have previously reported two patients who had eyelid retraction and lag with minimal impairment of vertical gaze (18). Both patients had circumscribed infarctions in the region of the NPC. 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