Supranuclear Paralysis of Upgaze

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Identifier 943-3
Title Supranuclear Paralysis of Upgaze
Ocular Movements Supranuclear Paralysis of Upgaze; Bilateral Lid Retraction- Collier's Sign; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Skew Deviation
Creator Shirley H. Wray, M.D., Ph.D., FRCP, Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Contributor Primary Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Subject Supranuclear Paralysis of Upgaze; Bilateral Lid Retraction- Collier's Sign; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Skew Deviation; Pretectal Syndrome; Brainstem Encephalitis; Acquired Immune Deficiency Syndrome; CNS Infection; Supranuclear Paralysis of Upgaze Infection AIDS-Encephalitis
Presenting Symptom Headache
History The patient is a 37 year old man with a history of intravenous drug abuse, AIDS, and tuberculosis. He presented with a chief complaint of unsteadiness walking for two weeks and vertical diplopia for four days. Past History: Human immunodeficiency Virus (HIV) positive When his CD4 lymphocyte count became sufficiently depleted, less than 30, in January 1992, he developed opportunistic infections including: Cytomegalovirus retinitis Pneumocystitis carinii pneumonia and Candida esophagitis During admission to the Massachusetts General Hospital he was found to have tuberculosis with left parahilar lymphadenopathy. Sputum positive for acid fast bacilli. Prescribed: isoniazid and ethambutol Social History: 15 packs/year smoker Past alcohol abuse Neurological/ Neuro-ophthalmological Examination: Cognitive function normal Speech normal Cranial Nerves: Visual acuity 20/30 OD, 20/25 OS Visual fields monocular: left inferior quadrantopsia OS (consistent with cytomegalovirus retinopathy) Pupils equal, normal reflexes. Ocular Motility: Supranuclear upgaze palsy (saccades and pursuit) Bilateral lid retraction - Collier's sign Convergence retraction nystagmus Convergence normal Skew deviation with a right hypotropia Horizontal and downgaze normal Vertical oculocephalic reflex normal Deviation of the eyes up under forced eye closure (Bell's phenomenon) The remainder of the cranial nerves normal Motor System: Strength 5/5 bilaterally Reflexes 2+ throughout with flexor plantar responses Sensory System: Normal Co-ordination: No dysmetria, finger-nose-finger or heel-to-shin test Wide based unsteady gait, leaning to the right Blood Studies: Notable for a white blood count of 2.7 Hematocirt 30.7 mg/dl Platelet count 105,000 Coagulation studies normal Serum VDRL and FTA-ABS test negative. Lumbar Puncture: Opening pressure 100 Total protein 38 mg/dl Sugar 47 mg/dl White blood cells 2 Cryptococcal antigen and VDRL tests were negative. Microscopic examination showed no malignant cells, fungi, acid-fast bacilli, or other micro-organisms and cultures were negative. Brain CT with and without contrast: Normal study Brain MRI with and without gadolinium: Normal study Diagnosis: Pretecal syndrome Mild brainstem encephalitis Medications: Fluconazole 400 mg b.i.d. Bactrim one double strength every other day Foscarnet 4500 mg daily Gancyclovir Epogen and G-CSF On completion of his workup, the patient was discharged home with follow-up in the Infectious Disease Clinic. He was lost to follow-up.
Clinical This 37 year old man with AIDS encephalitis had subtle ocular motility signs localized to the midbrain: • A partial supranuclear saccadic and pursuit paralysis of upgaze with upbeat nystagmus • Lid retraction in primary gaze - Collier's sign • Convergence retraction nystagmus elicited by rotating the stripes of an optokinetic drum down • Skew deviation with a right hypotropia • Light/near dissociation of the pupils. Confirmation that the gaze disorder is supranuclear is the presence of: 1. Upward deviation of the eyes on forced eye closure (intact Bell's) and 2. Full upward eye movements when the head is bent forward with the eyes fixating on a stable target (oculocephalic or doll's eye reflex normal). 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. Skew deviation has been attributed to unilateral or incomplete bilateral destruction of the medial longitudinal fasciculus (MLF) and the disruption of the otolith-ocular pathway from the utricle in the inner ear to the interstitial nucleus of Cajal (INC) in the midbrain. Lesions in the midbrain and INC may present with a sustained contralesional ocular tilt reaction (OTR). Eye movement abnormalities in OTR are: 1. Skew deviation (e.g. hypotropia of the right eye in right OTR) 2. Ocular torsion with upper poles of the eyes rotated towards the lower ear (e.g. in right OTR, excyclotorsion of the right eye and incyclotorsion of the left eye) and 3. Head tilt (e.g. right head tilt in right OTR). Acutely, there may be associated torsional nystagmus. Deviation of the subjective visual vertical (e.g. tilting of the subjective visual vertical to the right in right OTR). Skew deviation was present in 5 of 22 of my cases of pinealoma and in one reported by Dr. Cogan.
Neuroimaging The neuroimaging studies in this patient were normal.
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.
Pathology Although HIV most frequently affects the nervous system indirectly via opportunistic infections and lymphomas secondary to the immunodeficiency state it produces, it is well recognized that HIV itself directly infects the CNS. HIV encephalitis is characterized microscopically by reactive astrocytosis with frequent multinucleated giant cells. Multinucleated giant cells are the microscopic hallmark of this disorder. Immunohistochemistry with anti-HIV antibodies reveals HIV antigens within the giant cells' cytoplasm. These lesions are usually multiple and can be disseminated to any region of the CNS but are most common in the deep white matter and basal ganglia.
Disease/Diagnosis Acquired immune deficiency syndrome
Treatment See text
References 1. Collier, J. Nuclear ophthalmoplegia with a special reference to retraction of the lids and ptosis and to lesions of the posterior commissure. Brain. 1927. 50:488-498. 2. Currie J, Benson E, Ramsden B, Perdices M, Cooper D. Eye movement abnormalities as a predictor of the acquired immunodeficiency syndrome dementia complex. Arch Neurol. 1988 Sep;45(9):949-953. 3. Hamed LM, Schatz NJ, Galetta SL. Brainstem ocular motility defects and AIDS. Am J Ophthalmol. 1988 Oct 15;106(4):437-442. 4. Hedges TR 3rd. Ophthalmoplegia associated with AIDS. Surv Ophthalmol. 1994 Jul-Aug;39(1):43-51. 5. Keane JR. Neuro-ophthalmologic signs of AIDS: 50 patients. Neurology. 1991 Jun;41(6):841-845. 6. 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. 7. Page NG, Lean JS, Sanders MD. Vertical supranuclear gaze palsy with secondary syphilis. J Neurol Neurosurg Psychiatry. 1982 Jan;45(1):86-88.
Relation is Part of 167-11, 921-1, 926-1, 939-6, 946-3
Contributor Secondary Ray Balhorn, Video Compressionist; Steve Smith, Videographer
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1993
Type Image/MovingImage
Format video/mp4
Source 3/4" Umatic master videotape
Rights Management Copyright 2002. For further information regarding the rights to this collection, please visit:
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL
Language eng
ARK ark:/87278/s6fn43rq
Setname ehsl_novel_shw
Date Created 2006-09-05
Date Modified 2021-05-06
ID 188573
Reference URL
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