Bilateral Ptosis

Update item information
Identifier 919-2
Title Bilateral Ptosis
Ocular Movements Bilateral Ptosis
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 Nuclear Third Nerve Palsy; Bilateral Ptosis; Central Caudal Nucleus; Midbrain Ptosis; Oculomotor Nerve; Thalamic Infarct
Presenting Symptom Ptosis
History Patient is a 65 year old woman who presented with acute onset of bilateral ptosis. She awoke one day and found her eyelids half shut and she was unable to see. The lids completely shut and she came to the Massachusetts General Hospital Emergency Room and was admitted. Past History: Negative for ptosis, diplopia or generalized symptoms to suggest myasthenia gravis Hypertension and hyperlipidemia Myocardial infarct years ago General examination: Mildly obese lady BP 160/90, pulse regular. Ocular Motility: Head tilted back Complete bilateral ptosis Overaction of the frontalis muscle Full vertical and horizontal gaze OS slightly exo deviated OD straight Convergence normal Anisocoria, left pupil slightly larger than the right, equally brisk to light and near. Diagnosis: Nuclear third nerve palsy involving the central caudal nucleus Midbrain infarct The key to localizing the infarct to the central caudal subnucleus is the presence of full upgaze with no paresis of the superior rectus muscle in either eye. Full eye movements indicated that the oculomotor subnuclei for the extraocular muscles, which extend rostrocaudally were spared. Similarly the Edinger-Westphal nuclei which lie rostrally were also spared. The infarct destroyed only the caudal end of the oculomotor nucleus. Differential Diagnosis: 1. Bilateral cerebral ptosis, a supranuclear disorder of eyelid opening is accompanied by conjugate gaze deviation or gaze preference ipsilateral to an acute hemispheric lesion. 2. Ocular myasthenia gravis, in the absence of pupillary involvement, must be ruled out by performing an intravenous tensilon (edrophonium chloride) test.
Clinical This 65 year old woman, five weeks after the acute onset of bilateral ptosis, due to infarction of the central caudal nucleus of the oculomotor nucleus complex had:. • Complete bilateral ptosis • Overaction of the frontalis muscles • Head back posture • Full eye movements • Diplopia on looking up (possibly due to a V-pattern exotropia) • Normal convergence • Anisocoria with equally brisk reactive pupils
Neuroimaging No neuroimaging studies are available in this patient. MRI findings are reported but precise differentiation between nuclear and fascicular lesions of the third nerve can be difficult.
Anatomy The topographical diagnosis of lesions of the oculomotor nucleus is fully discussed in reference (11) The oculomotor nucleus complex lies in the midbrain and consists of: 1. A midline unpaired structure called the central caudal nucleus that supplies the levator palpebrae muscle on both sides. 2. Four lateral paired subnuclei that innervate the superior, inferior, and medial rectus, as well as the inferior oblique muscles. 3. The Edinger-Westphal nucleus, which contains preganglionic, parasympathetic neurons whose axons project to the ciliary ganglion and ultimately control pupillary constriction and accommodation. A third nerve palsy caused by a nuclear lesion is rare. When it occurs, it produces specific deficits in both eyes because of the anatomy of the nucleus complex: 1. Superior rectus subnucleus: Axons from one superior rectus (SR) subnucleus cross and pass through the opposite SR subnucleus; thus, a lesion of one SR subnucleus results in bilateral superior rectus palsy 2. Central caudal nucleus: • Unpaired and supplies both levator palpebrae muscles, thus, a lesion in the nucleus causes bilateral ptosis • Located in the most caudal part of the oculomotor nuclear complex, so it may be selectively affected (i.e., bilateral ptosis may be the only manifestation of a nuclear third nerve palsy), or it may be selectively spared. 3. Medial rectus subnuclei: lie in three different locations; thus, an isolated medial rectus palsy (unilateral or bilateral) without other muscle involvement cannot be a nuclear third nerve palsy. 4. Edinger-Westphal nucleus: spread throughout the rostral half of the oculomotor nucleus complex; thus, the pupil may be spared in lesions affecting the caudal half of the complex, but when the pupil is involved, both pupils are affected (i.e. bilateral internal ophthalmoplegia). This summary was taken from Chp 12, Nuclear and Infranuclear Ocular Motor Disorders. In: Wong AM (Ed) Eye Movement Disorders, Oxford University Press, 2008.
Pathology Autopsy cases of isolated bilateral complete ptosis are rare. Case 1. In 1974 we reported a case of midbrain ptosis with clinicopathological correlation (9). The patient was a 78 year old woman with cardiovascular disease and atrial fibrillation. She presented with dysarthria, somnolence, bilateral ptosis and conjugate deviation of the eyes to the right and no voluntary eye movements. The eyes moved fully to the right with oculocephalic reflex and ice water caloric stimulation. The left eye abducted fully to the left, but the right eye did not adduct across the midline. There was no vertical gaze or nystagmus. The pupils were normal. She has a left hemiplegia involving face, arm and leg and brisk symmetrical reflexes. Her ptosis started to recover after 15 days and at 5 months on examination showed she had fully recovered. She died of a myocardial infarct 9 months later. At autopsy, sections through the midbrain showed a cavitary infarct had destroyed portions of the right fourth nerve nucleus and caudal end of the third nerve nucleus with extensive neuronal destruction on the right side and in the dorsal midline. The middle of the third nerve nucleus was destroyed with preservation of neurons on the left side. The rostral end of the third nerve nuclear complex was virtually intact. There was minimal encroachment on the right Edinger-Westphal complex Bilateral ptosis is the most striking ocular sign in the present case. According to Warwick, this could result from a single lesion damaging the central caudal nucleus. It is difficult to identify individual neurons innervating specific muscles within the oculomotor nuclear mass. The central caudal nucleus, however, has a more restricted topography than other cell groups. Its location coincides precisely with the midline lesion in the present case, at the caudal end of the third nerve nuclear complex just ventral to the cerebral aqueduct. The recovery is best explained by the model of columnar organization, in which the nuclear groups are parallel cylinders oriented in a rostrocaudal direction. Here the surviving rostral cells within a column of neurons innervating a single extraocular muscle would compensate for those cells damaged at more caudal levels. The findings in this case then complement Warwick's studies of oculomotor organization in monkey and extend their validity to man. Case 2. Another report is of a 45 year old man who had bilateral complete ptosis attributed to damage to the central caudal nucleus in the nuclear complex of the third cranial nerve. At autopsy sections of the midbrain showed in the midline at the level of the central caudal nucleus a perivascular collection of lymphocytes surrounded by an astrocytic and microglial reaction. The seventh nerve nuclei and some of the descending and ascending tracts contained many discreet foci of perivascular collections of lymphocytes and nodules of rod-shaped microglial cells. The inflammatory foci in the midbrain and brainstem resembled the lesions seen in subacute viral brainstem encephalitis.
Etiology Ischemia is the commonest cause from embolic or thrombotic occlusion of small, dorsal perforating branches of the mesencephalic portion of the basilar artery. Occlusion of the distal portion of the basilar artery (top of the basilar syndrome) is rarely associated with a nuclear third nerve palsy. Additional etiologies include hemorrhage, metastatic disease, inflammation and compression.
Disease/Diagnosis Nuclear Third Nerve Palsy involving only the Central Caudal; Nucleus; Midbrain Infarct
References 1. Akdal G, Kutluk K, Men S, Yaka E. Benedikt and "plus -minus lid" syndromes arising from posterior cerebral artery branch occlusion. J Neurol Sci 2005;228:105-107. http://www.ncbi.nlm.nih.gov/pubmed/15607218 2. Bienfang DC. Crossing axons in the third nerve nucleus. Invest Ophthalmol 1975;14:927-931. http://www.ncbi.nlm.nih.gov/pubmed/1193807 3. Bogousslavsky J, Maeder P, Regli F, Meuli R. Pure midbrain infarction: clinical syndromes MRI and etiologic patterns. Neurology 1994;44:2032-2040. http://www.ncbi.nlm.nih.gov/pubmed/7969955 4. Bryan JS, Hamed LM. Levator-sparing nuclear oculomotor palsy. Clinical and magnetic resonance imaging findings. J Clin Neuro-ophthalmol 1992;12:26-30. http://www.ncbi.nlm.nih.gov/pubmed/1532597 5. Caplan LR. Ptosis. J Neurol Neurosurg Psychiatry 1974;37:1-7. http://www.ncbi.nlm.nih.gov/pubmed/4813425 6. Conway VH, Rozdilsky B, Schneider RJ, Sundaram M. Isolated bilateral complete ptosis. Can J Ophthalmol 1983;18:37-40. http://www.ncbi.nlm.nih.gov/pubmed/6839203 7. Gaymard B, Lafitte C, Gelot A. deToffol B. Plus-minus lid syndrome. J Neurol Neurosurg Psychiatry 1992;55:846-848. http://www.ncbi.nlm.nih.gov/pubmed/1402980 8. Gaymard B, Larmande P, deToffol B, Autret A. Reversible nuclear oculomotor nerve paralysis. Eur Neurol 1990;30:128-131. http://www.ncbi.nlm.nih.gov/pubmed/2358005 9. Growdon JH, Winkler GF, Wray SH. Midbrain Ptosis. A case with clincopathologic correlation. Arch Neurol 1974;30:179-181. http://www.ncbi.nlm.nih.gov/pubmed/4810897 10. Kwon JH, Kwon SU, Ahn HS, Sung KB, Kim JS. Isolated superior rectus palsy due to contralateral midbrain infarction. Arch Neurol 2003;60:1633-1635. http://www.ncbi.nlm.nih.gov/pubmed/14623739 11. Leigh JR. Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Chp 9, 385-474. In: The Neurology of Eye Movements 4th Edition, Oxford University Press, New York 2006. 12. Lepore FE. Bilateral cerebral ptosis. Neurology 1987;37:1043-1046. http://www.ncbi.nlm.nih.gov/pubmed/3587627 13. Liu GT, Carrazana EJ, Charness ME. Unilateral oculomotor palsy and bilateral ptosis from paramedian midbrain infarction. Arch Neurol 1991;48:983-986. http://www.ncbi.nlm.nih.gov/pubmed/1953423 14. Martin TJ, Corbett JJ, Babikian PV, Crawford SC, Currier RD. Bilateral ptosis due to mesencephalic lesions with relative preservation of ocular motility. J Neuro-ophthalmol 1992;16:258-263. http://www.ncbi.nlm.nih.gov/pubmed/8956161 15. Masucci EF. Bilateral ophthalmoplegia in basilar-vertebral artery disease. Brain 1965;88:97-106. http://www.ncbi.nlm.nih.gov/pubmed/14280280 16. Meienberg O, Mumenthaler M, Karbowski K. Quadriparesis and Nuclear Oculomotor Palsy with Total Bilateral Ptosis Mimicking Coma. A Mesencephalic ‘locked-in Syndrome'? Arch Neurol 1979;36:708-710. http://www.ncbi.nlm.nih.gov/pubmed/508130 17. Moncayo J, Bogousslavsky J. Vertebro-basilar syndromes causing oculomotor disorders. Curr Opin Neurol 2003;16:45-50. http://www.ncbi.nlm.nih.gov/pubmed/12544856 18. Nutt JG. Lid abnormalities secondary to cerebral hemisphere lesions. Ann Neurol 1977;1:149-151. http://www.ncbi.nlm.nih.gov/pubmed/889298 19. Pratt DV, Orengo-Nania S, Horowitz BL, Oram O. Magnetic resonance imaging findings in a patient with nuclear oculomotor palsy. Ann Ophthalmol 1995;113:141-142. http://www.ncbi.nlm.nih.gov/pubmed/7864742 20. Saeki N, Yamaura A, Sunami K. Bilateral ptosis with pupil sparing because of a discrete midbrain lesion: magnetic resonance imaging evidence of topographic arrangement within the oculomotor nerve. J Neuro-ophthalmol 2000;20:130-134. http://www.ncbi.nlm.nih.gov/pubmed/10870930 21. Stevenson GC, Hoyt WF: Metastasis to midbrain from mammary carcinoma: Cause of bilateral ptosis and ophthalmoplegia. JAMA 1963;186:514-516. http://www.ncbi.nlm.nih.gov/pubmed/14053360 22. Warwick R. Representation of the extraocular muscles in the oculomotor nuclei of the monkey. J Comp Neurol 1953;98:449-504. http://www.ncbi.nlm.nih.gov/pubmed/13069631 23. Wong AM. Nuclear and Infranuclear Ocular Motor Disorders. Ch 12. In: Eye Movement Disorders, Oxford University Press, New York 2008.
Relation is Part of 163-21, 166-26, 932-6
Contributor Secondary Ray Balhorn, Video Compressionist; Steve Smith, Videographer
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1984
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: https://NOVEL.utah.edu/about/copyright
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 http://NOVEL.utah.edu
Language eng
ARK ark:/87278/s6rn65dm
Setname ehsl_novel_shw
Date Created 2008-09-23
Date Modified 2017-02-23
ID 188648
Reference URL https://collections.lib.utah.edu/ark:/87278/s6rn65dm
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