Nuclear Third Nerve Palsy

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Identifier 163-21
Title Nuclear Third Nerve Palsy
Ocular Movements Nuclear Third Nerve Palsy; Bilateral Ptosis; Bilateral Superior Rectus Palsy
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; Bilateral Superior Rectus Palsy; Normal Pupils; Midbrain Infarct; Oculomotor Nerve; Nuclear; Thalamic Infarct
Presenting Symptom Bilateral ptosis
History The following case is the first patient I saw with a nuclear third nerve palsy. The patient is a 52 year old man with hypertension and hyperlipidemia. He awoke one morning unable to open his eyes. To walk he tilted his head backwards and looked down. He came to the emergency room of the Massachusetts General Hospital and was admitted. Neurological Examination: BP 210/92, pulse regular. Alert and cooperative, no headache. Marked head tilt backwards Marked overaction of the frontalis muscles Asymmetrical bilateral ptosis greater in the right eye (OD) Pupils equal and constricted normally to light and near. Ocular Motility OS: Third nerve palsy Exo deviation of the eye Marked paresis of elevation, superior rectus greater than inferior oblique Marked paresis medial rectus with absent convergence Mild paresis of the inferior rectus muscle Cranial nerves 4 and 6 normal Ocular Motility OD: Eye central in primary gaze Marked weakness of elevation, superior rectus palsy The remainder of the muscles innervated by the 3rd nerve were normal Cranial nerves 4 and 6 normal Diagnosis: Nuclear third nerve palsy. Lesions of the oculomotor nucleus are rare. Almost all of the cases that I have seen have had the exact pattern of paresis of the extraocular muscles as documented in this patient. Only one of my cases had the pattern described by Leigh and Zee which is a bilateral third nerve palsy with spared levator function with bilateral internal ophthalmoplegia. (The pupils can be normal). Tensilon test: As the pupils were normal in my patient an intravenous tensilon test (edrophonium chloride) was done which ruled out ocular myasthenia gravis. Diagnosis: Nuclear Third Nerve Palsy Midbrain infarct
Clinical This patient with a nuclear third nerve palsy from a midbrain infarct had bilateral ocular motility signs. OS: • Third nerve palsy • Exo deviation of the eye • Marked paresis of elevation, superior rectus greater than inferior oblique • Marked paresis medial rectus with absent convergence • Mild paresis of the inferior rectus muscle • Cranial nerves 4 and 6 normal OD: • Eye central in primary gaze • Marked weakness of elevation, superior rectus palsy • The remainder of the muscles innervated by the 3rd nerve • were normal • Cranial nerves 4 and 6 normal
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 are fully discussed in reference ( 9) The oculomtor 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: (CCN) • Unpaired and supplies both levator palpebrae muscles, thus, a lesion of the CCN 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 midbrain hemorrhage, metastatic disease, inflammation and compression.
Disease/Diagnosis Nuclear Third Nerve Palsy; Midbrain Infarct
Treatment Anti-hypertensive medication and treatment of hyperlipidemia
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. 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 6. 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 7. 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 8. 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 9. 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. 10. 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 11. 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 12. Pratt DV, Orengo-Nania S, Horowitz BL, Oram O. Magnetic resonance imaging findings in a patient with nuclear oculomotor palsy. Arch Ophthalmol 1995;113:141-142. http://www.ncbi.nlm.nih.gov/pubmed/7864742 13. Wong AM. Nuclear and Infranuclear Ocular Motor Disorders. Ch 12. In: Eye Movement Disorders, Oxford University Press, New York 2008.
Relation is Part of 166-26, 932-6, 939-2
Contributor Secondary Ray Balhorn, Video Compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1972
Type Image/MovingImage
Format video/mp4
Source 16 mm Film
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/s60g6gqt
Setname ehsl_novel_shw
Date Created 2007-03-09
Date Modified 2017-11-22
ID 188592
Reference URL https://collections.lib.utah.edu/ark:/87278/s60g6gqt