oculomotor nerve schwannoma associated with ophthalmoplegic migraine

3
sustained an avulsion of the optic nerve and only two were from the golf club; the remainder were from the golf ball. A 10-year-old male was struck in the left eye by a swinging golf club and was initially seen in the emergency department with visual acuity of RE: 20/20 and LE: no light perception. A strong (31) relative afferent pupillary defect was present in the left eye. A 5-mm superficial skin laceration was present just inferior to the temporal left eyebrow with minimal ecchymosis. The rims of the orbit were normal to palpation, and the patient did not complain of pain. Slit-lamp exami- nation revealed a normal right eye and a deep anterior chamber in the left eye with a mild diffuse hyphema. The lens was intact and clear. Fundus examination revealed avulsion of the optic nerve, with hemorrhage extending from the optic disk (Figure 1). Marked ischemia of the posterior pole was apparent, but no retinal detachment was observed. The patient was treated initially with 500 mg of intravenous methylprednisolone in an attempt to reverse any component of traumatic optic neuropathy, but he failed to recover any vision. Computed tomographic imaging of the brain and orbits revealed no abnormalities and an intact optic nerve bilaterally. The left optic nerve was slightly thicker than the right (Figure 2). Partial and complete optic nerve avulsions have been previously described, usually in the setting of blunt trauma. 3 Severe head trauma or orbital trauma can also lead to optic nerve avulsion. 4 In cases of significant vitreous hemorrhage obscuring the fundus, echography can be instrumental in making the diagnosis of optic nerve avulsion. 5 In golf-related ocular injuries, the golf ball or golf club usually strikes the eye directly, resulting in rupture of the globe or severe intraocular damage. In our case, the site of impact was probably between the globe and the temporal orbital rim, as noted by the superficial skin laceration and ecchymosis over that area. The rotational force impacting the globe at this point most likely leads to a shearing force between the optic nerve and the globe. A similar mechanism of optic nerve stretching and shearing is likely to occur in finger-stabbing injuries that lead to avulsion of the optic nerve. 3 This case of optic nerve avulsion highlights the danger associated with standing too close to a golfer swinging a club and delineates the different mechanisms by which these injuries can occur. When the impact site is between the globe and the orbital rim, as in this case, optic nerve avulsion is more likely to occur. Rupture of the globe is more likely to occur with direct impact to it. REFERENCES 1. United States Eye Injury Registry Annual Report. Birming- ham, Alabama, 1994. 2. Burnstine MA, Elner VM. Golf-related ocular injuries. Am J Ophthalmol 1996;121:437– 438. 3. Foster BS, March GA, Lucarelli MJ, Samiy N, Lessell S. Optic nerve avulsion. Arch Ophthalmol 1997;115:623– 630. 4. Chang M, Eifrig DE. Optic nerve avulsion. Arch Ophthalmol 1987;322–323. 5. Talwar D, Kumar A, Verma L, Tewari HK, Khosla PK. Ultrasonography in optic nerve head avulsion. Acta Ophthal- mol 1991;69:121–123. Oculomotor Nerve Schwannoma Associated With Ophthalmoplegic Migraine Aki Kawasaki, MD Accepted for publication June 11, 1999. From the Midwest Eye Institute, Clarian Hospitals of Indiana, Depart- ment of Ophthalmology and Neurology, Indiana University Medical Center, Indianapolis, Indiana. Inquiries to Aki Kawasaki, MD, 201 Pennsylvania Pkwy, Indianapolis, IN 46260; fax: (317) 817-1027. FIGURE 2. Computed tomography of the orbits reveals soft tissue swelling around the left globe without radiographic evidence of optic nerve avulsion or bony fractures. Note the thickening of the left optic nerve (arrow). AMERICAN JOURNAL OF OPHTHALMOLOGY 658 NOVEMBER 1999

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sustained an avulsion of the optic nerve and only two werefrom the golf club; the remainder were from the golf ball.

A 10-year-old male was struck in the left eye by a swinginggolf club and was initially seen in the emergency departmentwith visual acuity of RE: 20/20 and LE: no light perception. Astrong (31) relative afferent pupillary defect was present inthe left eye. A 5-mm superficial skin laceration was presentjust inferior to the temporal left eyebrow with minimalecchymosis. The rims of the orbit were normal to palpation,and the patient did not complain of pain. Slit-lamp exami-nation revealed a normal right eye and a deep anteriorchamber in the left eye with a mild diffuse hyphema. The lenswas intact and clear. Fundus examination revealed avulsionof the optic nerve, with hemorrhage extending from the opticdisk (Figure 1). Marked ischemia of the posterior pole wasapparent, but no retinal detachment was observed. Thepatient was treated initially with 500 mg of intravenousmethylprednisolone in an attempt to reverse any componentof traumatic optic neuropathy, but he failed to recover anyvision. Computed tomographic imaging of the brain andorbits revealed no abnormalities and an intact optic nervebilaterally. The left optic nerve was slightly thicker than theright (Figure 2).

Partial and complete optic nerve avulsions have beenpreviously described, usually in the setting of blunt trauma.3Severe head trauma or orbital trauma can also lead to opticnerve avulsion.4 In cases of significant vitreous hemorrhageobscuring the fundus, echography can be instrumental inmaking the diagnosis of optic nerve avulsion.5

In golf-related ocular injuries, the golf ball or golf clubusually strikes the eye directly, resulting in rupture of theglobe or severe intraocular damage. In our case, the site ofimpact was probably between the globe and the temporalorbital rim, as noted by the superficial skin laceration andecchymosis over that area. The rotational force impacting theglobe at this point most likely leads to a shearing force

between the optic nerve and the globe. A similar mechanismof optic nerve stretching and shearing is likely to occur infinger-stabbing injuries that lead to avulsion of the opticnerve.3

This case of optic nerve avulsion highlights the dangerassociated with standing too close to a golfer swinging aclub and delineates the different mechanisms by whichthese injuries can occur. When the impact site is betweenthe globe and the orbital rim, as in this case, optic nerveavulsion is more likely to occur. Rupture of the globe ismore likely to occur with direct impact to it.

REFERENCES

1. United States Eye Injury Registry Annual Report. Birming-ham, Alabama, 1994.

2. Burnstine MA, Elner VM. Golf-related ocular injuries. Am JOphthalmol 1996;121:437–438.

3. Foster BS, March GA, Lucarelli MJ, Samiy N, Lessell S. Opticnerve avulsion. Arch Ophthalmol 1997;115:623–630.

4. Chang M, Eifrig DE. Optic nerve avulsion. Arch Ophthalmol1987;322–323.

5. Talwar D, Kumar A, Verma L, Tewari HK, Khosla PK.Ultrasonography in optic nerve head avulsion. Acta Ophthal-mol 1991;69:121–123.

Oculomotor Nerve SchwannomaAssociated With OphthalmoplegicMigraineAki Kawasaki, MD

Accepted for publication June 11, 1999.From the Midwest Eye Institute, Clarian Hospitals of Indiana, Depart-

ment of Ophthalmology and Neurology, Indiana University MedicalCenter, Indianapolis, Indiana.

Inquiries to Aki Kawasaki, MD, 201 Pennsylvania Pkwy, Indianapolis,IN 46260; fax: (317) 817-1027.

FIGURE 2. Computed tomography of the orbits reveals soft tissue swelling around the left globe without radiographic evidence ofoptic nerve avulsion or bony fractures. Note the thickening of the left optic nerve (arrow).

AMERICAN JOURNAL OF OPHTHALMOLOGY658 NOVEMBER 1999

PURPOSE: To describe a patient with an oculomotor nerveschwannoma who had symptoms of ophthalmoplegicmigraine.METHODS: Case report.RESULTS: A 23-year-old woman had a history of recur-rent headache accompanied by transient right oculomotorpalsy since age 7 years. Ophthalmoplegic migraine wasdiagnosed. She was subsequently found to have a struc-tural lesion of her right oculomotor nerve on magneticresonance imaging. The magnetic resonance image char-acteristics were consistent with schwannoma originatingfrom the oculomotor nerve.CONCLUSIONS: This case illustrates that an intrinsiclesion of the oculomotor nerve (schwannoma) may beassociated with a painful relapsing-remitting oculomo-tor palsy mimicking the clinical syndrome of ophthal-moplegic migraine. (Am J Ophthalmol 1999;128:658 – 660. © 1999 by Elsevier Science Inc. All rightsreserved.)

OPHTHALMOPLEGIC MIGRAINE IS A RARE SYNDROME

in which episodic headaches are associated withophthalmoplegia, most commonly a unilateral oculomotorpalsy. Onset is typically before age 10 years. The headacheresolves quickly, but the oculomotor palsy recovers gradu-ally over days to weeks.

This 23-year-old woman had a severe throbbingheadache accompanied by nausea, diplopia, right uppereyelid blepharoptosis and a dilated nonreactive rightpupil. She had a history of “ophthalmoplegic migraines”since age 7 years. Her typical ophthalmoplegic migraineconsisted of an acute headache and complete or near-complete right oculomotor (third nerve) palsy thatspontaneously resolved within 1 week. Neurologic ex-amination between episodes of ophthalmoplegic mi-graine were documented as normal. She had fivestereotypic episodes of ophthalmoplegic migraine be-tween age 7 and 18 years and then was symptom freeuntil the onset of the present episode.

A magnetic resonance image (MRI) taken at the time ofher last ophthalmoplegic migraine had revealed a 4-mmnodular enhancing lesion along the cisternal segment ofthe right oculomotor nerve (Figure 1). The radiographiccharacteristics were consistent with a schwannoma origi-nating from the oculomotor nerve.1

Her present headache resolved promptly with analge-sic medication. However, her oculomotor palsy wasincompletely recovered after 3 weeks, so a follow-upMRI was performed. It showed no change in the lesionnoted 6 years earlier. She was referred for managementof her persistent blepharoptosis and diplopia. On exam-ination, she had visual acuity of 20/20 in each eye, withnormal Goldmann visual fields. There was 3-mm rightupper lid blepharoptosis with incomplete adduction,supraduction, and infraduction of her right eye (Figure

2). Pupils were 6 mm in room light, but the right pupilwas poorly reactive to light and near stimulation. Noother neurologic deficits were found.

The patient was treated with oral prednisone, starting at60 mg daily and tapered to discontinuation in 5 weeks.Within 3 weeks, her blepharoptosis and diplopia resolved.Repeat examination confirmed that her eyelid and eyemovements were completely normal; her right pupil re-mained poorly reactive. Visual acuity and visual field werestill normal in each eye. Headache or progression ofoculomotor palsy had not recurred in 3 years.

Schwannomas, also known as neurinomas and neurilem-momas, make up 6.8% of all intracranial tumors.1,2 Mostinvolve the vestibuloauditory or trigeminal cranial nerves.An isolated schwannoma originating from the oculomotornerve occurs rarely and typically produces a chronic orprogressive third nerve palsy.1–4 Headache is a commonfeature. Large schwannomas may cause additional signsand symptoms related to compression of adjacent struc-tures such as the midbrain, third ventricle, or otherintracavernous nerves.

This patient is distinctive because for 18 years she hadrecurrent episodes of a transient right oculomotor palsyassociated with acute headache and was subsequentlyfound to have an oculomotor nerve schwannoma. Al-though the schwannoma may have been an incidentalfinding, it did correspond to the side of the patient’soculomotor palsy. This case illustrates that an intrinsicstructural lesion of the oculomotor nerve may be associated

FIGURE 1. Magnetic resonance T1-weighted axial image withgadolinium shows a 4-mm nodular enhancing lesion (whitearrow) along the course of the right oculomotor nerve withinthe interpeduncular cistern. The radiographic appearance ofthis lesion is consistent with a schwannoma.

BRIEF REPORTSVOL. 128, NO. 5 659

with a painful relapsing-remitting oculomotor palsy mim-icking ophthalmoplegic migraine.

REFERENCES

1. Katsumata Y, Maehara T, Noda M, Shirouzu I. Neurinoma ofthe oculomotor nerve: CT and MRI features. J Comput AssistTomogr 1990;14:658–661.

2. Leunda G, Vaquero J, Cabezudo J, Garcia-Uria J, Bravo G.

Schwannoma of the oculomotor nerves: report of four cases.J Neurosurg 1982;57:563–565.

3. Niazi W, Boggan JE. Schwannoma of extraocular nerves:survey of literature and case report of an isolated third nerveschwannoma. Skull Base Surg 1994;4:219–226.

4. Abdul-Rahim AS, Savino PJ, Zimmerman RA, Sergott RC,Bosley TM. Cryptogenic oculomotor nerve palsy: the need forrepeated neuroimaging studies. Arch Ophthalmol 1989;107:387–390.

FIGURE 2. (Top) In primary gaze position, patient has equal pupil size and partial right upper lid blepharoptosis. (Middle left) Onattempted left gaze, patient demonstrates limited adduction of her right eye. (Middle right) Patient has normal right abduction. (Bottomleft and right). On attempted upgaze and downgaze, patient demonstrates limited supraduction and infraduction of her right eye.

AMERICAN JOURNAL OF OPHTHALMOLOGY660 NOVEMBER 1999