Supranuclear Paralysis of Upgaze

Supranuclear Paralysis of Upgaze; Bilateral Lid Retraction- Collier's Sign; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Normal ConvergenceHeadacheThe patient is a 32 year old man who was admitted as an emergency with severe headache and papilledema. On examination he had signs of the Pretectal Syndrome. • Bilateral lid retraction in primary gaze - Collier's sign • Supranuclear paralysis of upgaze (saccades and pursuit) • Full horizontal and downgaze • Convergence retraction nystagmus • Absent convergence • Deviation of the eyes up under closed lids (Bell's phenomenon) • Oculocephalic reflex normal. • Light/near dissociation of the pupils CT Brain Scan: Showed a mass in the posterior third ventricle obstructing the aqueduct and hydrocephalus. Surgery: A shunt procedure was performed. A supracerebellar infra-tentorial approach was used to obtain a biopsy. Pathology: DysgerminomaThis patient with a pineal region tumor and hydrocephalus had signs of the Pretectal Syndrome: • Bilateral lid retraction in primary gaze - Collier's sign • Supranuclear paralysis of upgaze (saccades and pursuit) • Full horizontal and downgaze • Convergence retraction nystagmus • Absent convergence • Deviation of the eyes up under closed lids (Bell's phenomenon) • Oculocephalic reflex normal. • Light/near dissociation of the pupils The pretectal dorsal midbrain syndrome is known by a variety of names, Parinaud's syndrome, Koeber-Salus-Eischnig syndrome, and the Sylvian aqueduct syndrome and is due to a lesion of the posterior commissure and the nucleus of the posterior commissure (nPC). Parinaud's syndrome is characterized by: 1. Supranuclear upgaze palsy 2. Light/near dissociation of the pupils and 3. Paralysis of convergence Collier's Sign: Collier's ‘tucked lid" sign, has also been termed the posterior fossa stare, and is iinvariable associated with a supranuclear paralysis of upgaze. The lid retraction is usually symmetric and sustained so long as the patient directs his eyes straight ahead or slightly upward. It may be accompanied by excess or infrequent blinking. On downgaze, the tone of the levator decreases smoothly and the lids follow the eye down in normal fashion. When the patient again looks up, lid retraction appears as the eyes reach the horizontal, and continued upgaze increases the disparity between the position of the upper lids and the eyes. Walsh and Hoyt observed retraction of the upper lids in a boy with a pinealoma when his gaze was directed down. This is unusual. Collier's sign has been attributed to compression of levator inhibitory fibers in the posterior commissure originating in the M-group of neurons identified by Büttner Ennever and Horn as playing a major role in the control of eyelid and eye movement function. Nystagmus: Classic descriptions of nystagmus in the pretectal syndrome report : 1. Pure retraction nystagmus (nystagmus retractorius,) 2. Pure convergence nystagmus and 3. The two combined Barrani reported a case with unilateral retraction nystagmus associated with convergent nystagmus of the contralateral eye. Both phenomena have been reported to occur intermittently in the same eye with lesions in the rostral midbrain. Convergence retraction nystagmus is perhaps the most distinctive pretectal phenomenon. Convergence retraction nystagmus may be present before upward gaze becomes grossly limited. With every attempt to make an upward saccade, the eyes first jerk inward several times then diverge again. When the phenomenon is pronounced, any saccadic attempt, horizontal or vertical, causes a convergent jerk of the eyes followed by a slower divergent drift back to the parallel position. Atkin and Bender used the term lightening eye movements to describe bursts of rapid, small amplitude movements seen on attempted upgaze. Patients with such signs complain of slowness in focusing and difficulty reading. When convergent eye movements distort horizontal saccades, the result in limitation of abduction mimics partial abducens nerve palsy, so called pseudo-abducens palsy of upper midbrain lesions. Oculocephalic or caloric stimulation usually produces full abduction and thereby resolves the question of paresis. Pupillary areflexia: Gowers was the first to report pupillary areflexia and upgaze palsy from a pathologically confirmed case of pinealoma. Interestingly, in Parinaud's report two years later, the pupils were small and recorded as nonreactive to convergence, while light reactions were retained. In our experience, and in the reports of others, light-near dissociation of the pupillary light reflexes occurs frequently. The pupils are moderately dilated and poorly reactive to direct light while pupillary constriction to near is retained. This type of pupil abnormality has been called Argyll-Robertson, but it differs from classic Argyll-Robertson pupils by virtue of lack of miosis and the normal response to atropine. The afferent fibers of the pupillary light reflex pass from the optic tract to the pretectum, where they decussate, in part, through the posterior commissure before reaching reticular cells surrounding the Edinger-Westphal nucleus. Light reflex abnormality is frequently present in patients with pineal tumors. Control signals for accommodation coming from the striate and peristriate cortex, are usually spared as they transverse the posterior capsule, and reach the EW nucleus from below. Electronic infrared pupillographic studies, however, have shown that pineal region tumors impair both light and near responses and that true light-near dissociation is rare. Impaired sympathetic pupillary control is equally rare. Alternating contraction anisocoria is another pupillary syndrome of the anterior midbrain. This syndrome is characterized by greater pupillary constriction in the directly stimulated eye than in the consensually reacting pupil a subtle sign of minimal pretectal impairment. Corectopia or displaced pupil may be a permanent or transient sign. Selhorst et al in a case of midbrain corectopia with bilateral rostral midbrain infarction observed the pupils to dilate spontaneously, independently and eccentrically. Sporadic cycles of dilation and constriction, each lasting 5 to 15 minutes, occurred during the last 3 days of the patient's life. The aperture expanded irregularly to an oval shape. The pupils independently shifted off-center, upward and outward in the right eye and downward and outward in the left eye. Then the pupils reconstricted, became round, and returned to the center. Autopsy in this case disclosed isolated but intact EW nuclei and the explanation of midbrain corectopia by the authors centered on the segmental innervation of the pupil by the EW nucleus. In the presence of a paralyzed dilator muscle, select, central inhibition of sphincter tone resulted in oval and eccentric pupils. Paralysis of Convergence: Paralysis of convergence is the third sign completing the triad of Parinaud's syndrome. The syndrome of convergence palsy is characterized by failure of convergence with crossed diplopia when the eyes view a near target but with absence of paresis of the medial recti and lateral gaze. Although the anatomic substrates for convergence, vertical gaze, and pupillary reaction lie in the same vicinity in the midbrain, convergence is often retained when only supranuclear upgaze is involved. Convergence is usually absent when supranuclear downgaze palsy is added.No neuroimages studies are available in this patient.The neural structures in the mesencephalic reticular formation involved in the generation of vertical eye movements are: 1. The posterior commissure (PC) and the nucleus of the PC (nPC) 2. The interstitial nucleus of Cajal (INC) 3. The rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) The pretectal dorsal midbrain syndrome is due to a lesion of the posterior commissure and the nucleus of the posterior commissure (nPC). Cells in the nPC project through the posterior commissure to the rostral interstitial nucleus of the MLF, (riMLF), the interstitial nucleus of Cajal (INC) and the M-group of neurons, which relays to the central caudal subdivision of the oculomotor nucleus and coordinates vertical eye and lid movements. Unilateral lesions of the mesencephalic reticular formation create the same bilateral oculomotor syndrome by interrupting both projections through the posterior commissure. The interstitial nucleus of Cajal is frequently involved in lesions producing supranuclear gaze palsies and the INC lesion is not just one of vertical gaze evoked nystagmus but also of restriction in the range of vertical eye movements although saccades are not slowed. Bilateral lesions of INC affect vertical gaze whereas unilateral lesions produce the ocular tilt reaction and ipsilesional torsional nystagmus with quick phases moving the top poles of the eyes towards the side of the lesion. The rostral interstitial nucleus of the MLF (riMLF) is a cluster of neurons which lie adjacent to, but are distinct from the INC. The riMLF is situated rostral to the latter nucleus among the fibers of the MLF and ventral to the nucleus of Darkshevich within a few millimeters of the midline. It also lies near the fasciculus retroflexus and the rostral pole of the red nucleus. Büttner-Ennerver and Büttner gave this region the name rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) in the monkey, whereas Graybiel referred to it as the nucleus of the prerubral field in the cat. In the riMLF approximately equal numbers of neurons are activated by upward and downward gaze, but none are activated by horizontal movements.DysgerminomaShunt procedure Whole head and spine radiation1. Atkin A, Bender MB. "Lightning eye movements" (ocular myoclonus). J Neurol Sci 1964;1:2-12. http://www.ncbi.nlm.nih.gov/pubmed/14174044 2. Collier J. Nuclear ophthalmoplegia with special reference to retraction of the lids and ptosis and to lesions of the posterior commissure. Brain 1927;50:488-498. 3. DeGirolami U, Schmidek H. Clinicopathological study of 53 tumors of the pineal region. J Neurosurg 1973;39:455-462. http://www.ncbi.nlm.nih.gov/pubmed/4730334 4. Elschnig A. Nystagmus retractorius, ein cerebrales Herdsymptom. Med Klin 1913;9:8-11. 5. Gowers WR. Case of intracranial disease with optic neuritis and paralysis of the upward movement of both eyes. Trans. Ophthalmol Soc. U.K. 1881; 1:117-119. 6. Horn AKE, Büttner-Ennever JA, Gayde M. Messoudi A. Neuroanatomical identification of mesencephalic premotor neurons coordinating eyelid with upgaze in monkey and man. J Comp Neurol 2000;420:19-34. http://www.ncbi.nlm.nih.gov/pubmed/10745217 7. Leigh JR, Zee DS. The Diagnosis and Disorders of Eye Movements. Chp 12;598-718. In: The Neurology of Eye Movements, 4th Edition, Oxford University Press, New York, 2006. 8. Lowenstein O. Alternating contraction anisocoria: Pupillary syndrome of anterior midbrain. Arch Neurol 1954;72:742-757. http://www.ncbi.nlm.nih.gov/pubmed/13206493 9. Parinaud H. Paralysis of the movement of convergence of the eyes. Brain 1886;9:330-341. 10. Parinaud H. Paralysie des mouvements associes des yeux. arch Neurol 1883;5:145-172. 11. Salus R. Uber erworbene Retraktionsbewegungen der Augen. Arch Kinderkeilk, 1910;47:61-76. 12. Selhorst JB, Hoyt WF, Feinsod M, Hosobuchi Y. Midbrain corectopia. Arch Neurol 1976;33:193-195. http://www.ncbi.nlm.nih.gov/pubmed/766739 13. Seybold ME, Yoss RE, Hollenhorst RW, Moyer NJ. Pupillary abnormalities associated with tumors of the pineal region. Neurology 1971;21:232-237. http://www.ncbi.nlm.nih.gov/pubmed/5314863 14. Shallat RF, Pawl RP, Jerva MJ. Significance of upward gaze palsy (Parinaud's syndrome) in hydrocephalus due to shunt malfunction. J Neurosurg 1973;38:717-721. http://www.ncbi.nlm.nih.gov/pubmed/4351065 15. Smith JL, Zieper I, Gay AJ, Cogan DG. Nystagmus retractorius. Arch Ophthalmol 1959;62:864-867. http://www.ncbi.nlm.nih.gov/pubmed/13832032 16. Walsh FB, Hoyt WF. Clinical Neuro-ophthalmology. Williams and Wilkins, Baltimore 1969. 17. Wray SH. The Neuro-Ophthalmic and Neurologic Manifestations of Pinealomas. In: Pineal Tumors. Schmidek, H., ed. Mason Publishing USA, Inc. 1977; 21-59.curriculum_fellow; VPlidretraction; MTupgazepalsy; MTdorsalmidbrainsyndrome; GVSeyelidretraction; MTemspverticalgazepals