Third Nerve Palsy

Ptosis; Third Nerve Palsy; Aberrant Reinnervation of the Third Nerve; Paresis of Abduction; Sixth Nerve PalsyPowerPoint Presentation: Cavernous Sinus Meningioma: http://novel.utah.edu/Wray/PPT/Cavernous_Sinus_Meningioma.pdf Shirley H. Wray, M.D., Ph.D., FRCP, Harvard Medical SchoolDiplopiaThis patient is a 46 year old woman from Portugal who was admitted to the Massachusetts General Hospital in September 1986 with ophthalmoplegia of the left eye (OS) and signs of aberrant reinnervation of the third nerve. She presented, in August 1985, with an episode of diplopia. The diplopia was sudden in onset, and on awakening in the morning she had diplopia in all directions of gaze for approximately 15 minutes. When the diplopia resolved in primary gaze, she noted persistent vertical diplopia looking up. By July 1986, she had diplopia in all directions of gaze and drooping of the left eyelid. She consulted an ophthalmologist and was referred to the Neurovisual Clinic at the Massachusetts General Hospital and admitted. Past History: Negative for additional episodes of diplopia, transient ptosis, or strabismus as a child. No headaches, face or eye pain, seizures, syncope or vertigo. Neuro-ophthalmological examination: Visual acuity 20/20, J1OU. Visual fields, color vision and fundoscopic examination normal. Pupils anisocoria. OD 3 mm brisk to light and near OS 4 mm sluggish to light and near Eyelid OS: Subtle signs of aberrant reinnervation of the left third nerve present with changes in the position of the ptotic eyelid on abduction and adduction. Partial ptosis in primary gaze Palpebral fissure OD 9 mm, OS 7 mm Complete ptosis on abduction Elevation of the ptotic lid on adduction No constriction of the pupil OS on adduction No exophthalmos Normal orbital resilience Normal corneal reflex No ocular pulsation or bruit Sense of smell intact Ocular motility OS: The pattern of weakness of the ocular muscles innervated by the third nerve was characteristic of a partial paresis of the trunk of the third nerve with paresis of the: Levator superioris Superior rectus (SR) Inferior oblique (IO) Mild weakness of the medial rectus and inferior rectus Superior oblique (cranial nerve 4) normal Abduction weakness (cranial nerve 6) Absent Bell's OS (elevation of the eye under closed lids) Diagnosis: Compressive lesion of the left third nerve trunk within the cavernous sinus Primary aberrant reinnervation of the third nerve. Primary aberrant reinnervation of the third nerve is a well recognized sign of long standing compression of the nerve from a cavernous sinus meningioma or an aneurysm of the internal carotid artery (ICA). It has also been reported with a compressive lesion of the third nerve due to a basilar artery aneurysm. Brain MRI Pre-contrast axial T1 W1 sequence shows a mass expanding the left cavernous sinus (Figure 1). Post-contrast axial T1 W1 showed the mass enhanced strongly and uniformly. A dural tail extended posteriorly along the tentorium. The left ICA was occluded. (Figure 2) Coronal post-contrast T1 W1 showed the mass expanded the cavernous sinus and completely obliterated Meckel's cave. (Figure 3) MRA study showed: 1. No flow related enhancement in the left intracranial ICA. 2. Cross filling of the left middle cerebral artery via the anterior communicating artery. 3. A retrograde left posterior communicating artery providing inflow from the vertebral basilar system. 2-D time of flight MRA through the neck revealed flow related enhancement in the left ICA which tapered to a pointed stump just above the bulb. This finding was thought to reflect greatly slowed flow or frank occlusion of the cervical ICA related to distal encasement. The right common carotid, bifurcation and cervical ICA were normal. Diagnosis: Left cavernous sinus meningioma Partial third nerve (trunk) palsy involving the pupil Primary aberrant reinnervation of the third nerve Partial cavernous sinus syndrome Partial 6th nerve palsy Neurosurgical Consultation: Conservative management without a biopsy was recommended. Treatment: Patient elected conservative management. In 1990, with the onset of dull retro-orbital pain OS, the patient returned for focal radiation therapy. Brain MRI showed no significant change at that time. Follow-Up: The patient was last seen in 2003 with no significant change in her clinical findings.This patient with a cavernous sinus meningioma had subtle eyelid signs of aberrant reinnervation of the left third nerve: • Partial ptosis OS • Complete ptosis on abduction • Elevation of the lid on adduction and • Paresis of the inferior oblique and superior rectus (double elevator palsy) • Paresis of the medial and inferior rectus • Superior oblique normal • Lateral rectus weakness of full abduction • Absent Bell's Sixth Nerve Palsy: In this patient, the weakness of full abduction OS was attributed to a simultaneous, partial 6th nerve palsy due to compression of the 6th nerve in the cavernous sinus. Slavin and Einberg found, however, in a patient with aberrant reinnvervation of the third nerve and delayed-onset of ipsilateral abduction weakness, as in this case, failure of relaxation of the ipsilateral medial rectus muscle on attempted abduction on ocular electromyography suggesting co-contraction of horizontal recti muscles as the origin of the abduction defect. Primary aberrant reinnervation of the third nerve is a common sequel of long standing third nerve lesions which include compression from a slow growing intracavernous meningioma or carotid aneurysm. This unusual synkinesis is also reported following trauma, as a complication of neurosurgery, and with congenital third nerve palsy, Aberrant reinnervation almost never occurs with diabetic third nerve palsy. The abnormal eyelid movements are due to co-contraction of muscles innervated by the third nerve. The regenerating fibers no longer follow their previous paths but innervate different muscles supplied by the third nerve. This mechanism may not apply in every case since anomalous synkinesis can occur transiently after an acute third nerve palsy. It is noteworthy that primary aberrant reinnervation has also been reported following a mesencephalic lesion affecting the third nerve fascicles.View Cavernous Sinus Meningioma to see imaging studies.The diagnosis of a third nerve palsy is straight forward but it is important to consider whether it is: 1. A nuclear lesion 2. A complete or partial lesion of the nerve trunk or a 3. Superior division of the third nerve or an 4. Inferior division of the third nerve A lesion involving the superior division of the third nerve results in paresis of the levator palpebrae muscle and the superior rectus so that the patient will have partial ptosis and paresis of elevation in the line of action of the superior rectus. A lesion involving the inferior division of the third nerve, involves all the extraocular muscles innervated by the third nerve, except the levator palpabrae and the superior rectus with or without pupil involvement.Meningiomas have a variable histological appearance but are composed of epithelial-like cells that form whorl patterns.MeningiomaFocal radiation therapy1. Balkan R, Hoyt CS. Associated neurological abnormalities in congenital third nerve palsies. Am J Ophthalmol 1984;97:319. http://www.ncbi.nlm.nih.gov/pubmed/6199980 2. Boghen D, Chartrand JP, Laflamme P, Kirkham T, Hardy J, Aube M. Primary aberrant third nerve regeneration. Ann Neurol 1979;6(5);415-418. http://www.ncbi.nlm.nih.gov/pubmed/518037 3. Braunstein JB, Vick NA. Meningiomas: the decision not to operate. Neurology 1997;48:1459-1462. http://www.ncbi.nlm.nih.gov/pubmed/9153494 4. Cox TA, Wurster JB, Godfrey WA. Primary aberrant oculomotor regeneration due to intracranial aneurysm. 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Am J Ophthalmol 1994;118:83-87. http://www.ncbi.nlm.nih.gov/pubmed/8023880curriculum_fellow; KBDneurodiscavernoussinussyndrome; VBneurodiscavernoussinusmeningioma; GVSptosis; MTemspthirdsubnuclea