| Identifier |
921-1 |
| Title |
Supranuclear Paralysis of Upgaze |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
Ray Balhorn, Video Compressionist; Steve Smith, Videographer |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Supranuclear Paralysis of Upgaze Hemorrhage; Bilateral Lid Retraction - Collier's Sign; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Normal Convergence; Light/Near Dissociation of the Pupils; Pretectal Syndrome; Thalamic Hemorrhage; Thalamus Hemorrhage |
| History |
The patient is a 33 year old man who called the police to his apartment at 3 a.m. on the morning of admission. They found his apartment in a state of disarray and realized that he had been down on the floor for some hours and only just able to open the door to let them in. He was admitted to the local hospital, oriented x3, just able to walk but very somnolent. He had bilateral ankle clonus, extensor plantar responses and extensive bruising over large areas of the right side of his body. Hematological studies: CPK markedly elevated- 19,700 White blood cell count up 25.7 CT non-contrast showed a left thalamic hemorrhage with rupture into the ventricles. Treatment: He was treated with charcoal, narcan, decadron, manitol and thiamin and transferred immediately to the Massachusetts General Hospital (MGH). 3/12/85 MGH admission BP 180/70, pulse 88 regular. Neurologic examination: Somnolent but arousable to loud voice and tactile stimuli. Oriented x2. Speech fluent, no dysarthria. Markedly decreased attention span. Remote memory appeared intact. Cranial Nerves: Pupils 3 mm OU, sluggish to light. Marked limitation of upgaze Impaired abduction right eye Convergence normal No nystagmus Right corneal abrasion and decreased corneal reflex Motor System: Mild right hemiplegia, face, arm and leg Motor strength 4/5 Decreased rapid alternating movements, right hand Reflexes 3+ on the right, 2+ on the left Bilateral extensor plantar responses Hoffman's present on the right No frontal release signs. Sensory System: Right hemi-sensory loss to light touch and pin prick Co-ordination: Normal Electrocardiogram: Normal, no evidence of ischemia or arrhythmia. Chest x-ray: Normal. CT non-contrast: Left thalamic hemorrhage with minimal midline shift and slight surrounding edema. High density blood within the lateral, third and fourth ventricles Hydrocephalus of the lateral and third ventricles. Neurosurgery Consult: Because of the patient's markedly decreased level of consciousness, alternating between periods of extreme somnolence and sleep-like behavior, with periods of alertness, the patient was taken immediately to the operating room and a right ventriculostomy was placed that drained bloody ventricular fluid under normal pressure. Neurosurgery ICU: He received decadron and manitol. Ventriculostomy removed on day 4 Continued to gradually improve. 3/26/85 Cerebral angiogram: Left thalamic hemorrhage and mass effect Enlarged posterior choroidal artery with early venous drainage. Impression: Resolving hematoma Arteriovenous malformation (AVM) could not be ruled out. RE: Rhabdomyolysis: The patient was treated with alkaline diuresis and manitol No renal dysfunction developed. Neuro-ophthalmology consult: Visual acuity 20/25 OU Supranuclear paralysis of upgaze (saccades and pursuit) Bilateral lid retraction - Collier's Sign Convergence retraction nystagmus Vertical oculocephalic reflex normal Normal convergence Deviation of the eyes up under closed lids (Bell's phenomenon) Light/near dissociation of the pupils Diagnosis: Left thalamic hemorrhage rupturing into the ventricles Mild hydrocephalus Global supranuclear upgaze palsy Pretectal syndrome Hospital Course: The patient made a good recovery. The right hemiplegia resolved but the constellation of ocular motor signs persisted. On 4/2/85 he was discharged to rehab. 2/12/97 he was readmitted to the MGH, twelve years after the acute left thalamic hemorrhage, for stereotactic resection of a thalamic AVM. Neurological examination: Normal Ocular Motility: Unchanged. 1/27/95 Cerebral angiography: Injection of the left vertebral artery showed, in multiple projections, a small tortuous vessel directed superiorly towards a region of the left thalamus. This vessel probably represented a medial posterior choroidal vessel or a posterior thalamoperforator which fed a small prominent tangle of vessels lateral to the third ventricle, and considered to be the site of an AVM. 6/17/95 Brain MRI: Showed a focal signal abnormality with susceptibility effect in the medial left thalamus adjacent to the left wall of the third ventricle with ventricular contour distortion consistent with an AVM with prior hemorrhage. Brain MRA: One enlarged subappendable vein was identified No abnormal feeding vessels were seen. Stereotactic Craniotomy: A stereotactic craniotomy was performed with resection of the thalamic AVM. Post operatively the patient developed a right hemiplegia involving face, arm and leg and initially had marked weakness of the right leg with inability to walk. Over the course of 4 to 5 days, the weakness recovered so that he was able to walk with a cane. Motor strength returned to 4/5 right upper extremity with resolution of the facial palsy. The neuro-ophthalmic signs were unchanged. |
| Anatomy |
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. (For further discussion of the anatomy of vertical gaze see reference |
| Pathology |
Thalamic arteriovenous malformation |
| Disease/Diagnosis |
Thalamic hemorrhage; Pretectal Syndrome |
| Clinical |
This patient with signs of the pretectal syndrome due to a unilateral thalamic hemorrhage had a constellation of eye signs: • Supranuclear paralysis of upgaze (saccades and pursuit) • Bilateral lid retraction - Collier's Sign • Convergence retraction nystagmus • Vertical oculocephalic reflex normal • Normal convergence • Deviation of the eyes up under closed lids (Bell's phenomenon) • Light/near dissociation of the pupils • Optokinetic nystagmus (OKN) with the stripes down provoked bursts of convergence retraction nystagmus Viewing the patient from the side confirmed: • Retraction of the globe 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. |
| Presenting Symptom |
Headache |
| Ocular Movements |
Supranuclear Paralysis of Upgaze; Bilateral Lid Retraction - Collier's Sign; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Normal Convergence |
| Neuroimaging |
No neuroimaging studies are available in this patient. |
| Treatment |
Stereotactic resection of the AVM |
| Etiology |
Thalamic hemorrhage |
| Date |
1985 |
| References |
1. Castaigne P, Lhermitte F, Buge A, Escourolle R, Hauw JJ, Lyon-Caen O. Paramedian thalamic and midbrain infarcts; clinical and neuropathological study. Ann Neurol 1981;10:127-148. http://www.ncbi.nlm.nih.gov/pubmed/7283400 2. Collier J. Nuclear ophthalmoplegia with especial reference to retraction of the lids and ptosis and to lesions of the posterior commissure. Brain 1927;50:488-498. 3. Fisher A, Knezevic W. Ocular and ocular motor aspects of primary thalamic hemorrhage. Clin Exp Neurol 1985;21:129-139. http://www.ncbi.nlm.nih.gov/pubmed/3843212 4. Fisher CM. The pathologic and clinical aspects of thalamic hemorrhage. Trans Am Neurological Assoc 1959;84:56-59. http://www.ncbi.nlm.nih.gov/pubmed/13823193 5. Galetta SL, Raps EC, Liu GT, Saito NG, Kline LB. Eyelid lag without eyelid retraction in pretectal disease. J Neuro-ophthalmol 1996;16:96-98. http://www.ncbi.nlm.nih.gov/pubmed/8797164 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. Rambold H, Kompf B, Helmchen C. Convergence retraction nystagmus: a disorder of vergence ? . Ann Neurol 2001;50:677-681. http://www.ncbi.nlm.nih.gov/pubmed/11706977 9. Sand JJ, Biller J, Corbett JJ, Adams HP Jr., Dunn V. Partial dorsal mesencephalic hemorrhages: report of three cases. Neurology 1986;36:529-533. http://www.ncbi.nlm.nih.gov/pubmed/3960328 10. Thames PB, Trobe JD, Balinger WE. Upgaze paralysis caused by lesion of the periaqueductal gray matter. Arch Neurol 1984;41:437-440. http://www.ncbi.nlm.nih.gov/pubmed/6703947 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
3/4" Umatic master videotape |
| Relation is Part of |
167-11, 926-1, 939-6, 943-3. 946-3 |
| Collection |
Neuro-Ophthalmology Virtual Education Library - Shirley H. Wray Neuro-Ophthalmology Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6km28df |
| Setname |
ehsl_novel_shw |
| ID |
188541 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6km28df |
