Supranuclear Paralysis of Upgaze

Supranuclear Paralysis of Vertical Gaze; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Third Nerve PalsyPowerPoint Presentation: Thalamic Stroke and Disordered Sleep: http://library.med.utah.edu/NOVEL/Wray/PPT/Thalamic_Stroke_and_Disordered_Sleep_guest_lecture.ppt Kenneth C. Sassower, M.D. Massachusetts General HospitalSomnolence; DiplopiaThe patient is a 58 year old man who was admitted as an emergency with hypersomnolence, gait ataxia and diplopia. On the night before Thanksgiving he went to bed feeling well. He was awoken by a phone call at 2 p.m. on Thanksgiving day having over slept by 7 hours. He got out of bed to answer the phone and found that he was markedly unsteady and tremulous reaching for the telephone. He spoke to his girlfriend who told him the time which surprised him. He did not feel well enough to dress and go to her home and instead went back to bed taking two tylenol for what he called a "boring" bitemporal headache. He fell asleep and awoke again at 4:30 p.m. and at 8 p.m. by calls from his friend. He aroused easily on each occasion only to lapse back to sleep. He was finally brought to the emergency room and admitted. Family History: Brother had had a stroke Past Medical History: Former alcohol abuser, negative drug use Migraine with visual aura since childhood. Average 2x/year. Neurological Examination: BP 125/65, pulse 70 regular Alert, oriented x3 He went to sleep if not stimulated but aroused easily. Followed two-step commands Neuro-ophthalmic Examination: Visual acuity 20/20 OU Fields and fundus examination normal Pupils anisocoria, OD 3 mm, sluggish to light, absent to near. OS 2.5 mm, normal reflexes. Partial ptosis OD Primary gaze non paretic esotropia OD> OS, (fixing alone full abduction). Ocular Motility: Supranuclear paralysis of upgaze (saccades and pursuit) Convergence retraction nystagmus on attempted upgaze Horizontal and down gaze normal. Deviation of the eyes up on forced eye closure (Bell's phenomenon) Vertical oculocephalic reflex normal Convergence normal Brain MRI: Left medial thalamic infarct. Head MRA: No abnormality Basilar and vertebral arteries normal. Echocardiogram: A transesophageal echo revealed a patent foramen ovale (PFO) with right to left shunting inducible with valsalva. Ultrasound and MRV of the lower extremities and pelvis: No deep vein thrombosis. Diagnosis: Pretectal Syndrome Unilateral thalamic infarct Treatment: IV heparin and transitioned to Coumadin prior to discharge.On day 6, this patient, with a unilateral thalamic infarct. had: • Supranuclear paralysis of upgaze (saccades and pursuit) • Convergence retraction nystagmus on attempted upgaze • Normal horizontal and downgaze • Deviation of the eyes up on forced eye closure (Bell's phenomenon) • Vertical oculocephalic reflex normal • Convergence normal • Convergence nystagmus on upgaze brought out by following the stripes of an OKN drum down • Lateral view of the eye confirmed retraction of the globe • Slight right ptosis • Anisocoria, OD > OS, sluggish to light and near. At four weeks: Full recovery of upgaze Persistent convergence retraction nystagmus Small amplitude horizontal gaze evoked nystagus At two months: Slow convergence movements on upgaze only. View ID167-11 and 207-1 alongside this case. 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 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.No neuroimaging studies are available in this patient. In another case of unilateral thalamic infarction (ID207-1) brain MRI showed: Figure 1: MRI DWI hyperintensity in the right thalamus extending into the right parasagittal midbrain surrounding the red nucleus. Figure 2A: Axial T2 MRI right medial thalamic infarct. Figure 2B: Right medial thalamic infarct. Figure 2C: Saggital MRI right medial thalamic infarct.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 posterior commissure (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.Dichgans and Dieterich reported the case of a 77 year old diabetic woman who presented with an acute, painful "isolated" pupil sparing left third nerve palsy suggestive of diabetic mononeuropathy. Measurements of ocular torsion and perception of verticality showed, however, involvement of both eyes with binocular tilts to the contralateral right side thereby indicating a left sided midbrain lesion which included the fascicle of the third nerve as well as supranuclear integration centers for torsional eye movements, the INC and the riMLF. In their case an MRI confirmed a paramedian rostral midbrain infarct within the left paramedian mesencephalic region. The lesion involved the left INC, (the neural integrater for vertical gaze) as well as a small part of the left oculomotor nucleus and a larger part of the fascicle of the oculomotor nerve which explained the ‘mixed' oculomotor disorder with the combination of a supranuclear and fascicular oculomotor nerve palsy. Additionally, very small parts of the Edinger-Westphal nucleus were affected.Embolic infarctionAnticoagulation1. Biller J. Sand JJ., Corbett JJ, Adams HPJr., Dunn V. 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