Review Article

Recurrent Painful Ophthalmoplegic Neuropathy and Oculomotor Nerve Schwannoma: A Pediatric Case Report with Long-Term MRI Follow-Up and Literature Review

Table 1

A literature review of symptoms and magnetic resonance imaging findings in 43 patients under the age of 18 years with ophthalmoplegic migraine or recurrent painful ophthalmoplegic neuropathy.

AuthorReferenceCaseM/FAge of onsetAge of observationHeadacheOculomotor involvementMRIMRI follow-upDiagnosis

Aers et al.[16]1F812Severe right unilateral headache with photophobia and vomitingDiplopia and ptosis of the right upper eyelidMRI: marked thickening and gadolinium enhancement of the right oculomotor nerve along its subarachnoid course in the interpeduncular fossaSix months later: slightly thickened but no longer enhancing cisternal portion of the right oculomotor nerveOM
Wong and Wong[32]2M66Acute onset of headache with photophobia and vomitingDiplopia, acute periorbital pain with droopy eyelidsNo contrast MRI scan: slight asymmetry in size of the oculomotor nerveNAOM
Mark et al.[17]3FNot reported8HeadacheOculomotor nerve palsyAcute MRI: focal thickening and enhancement7 to 9 weeks later: resolution of the enhancementOM
Mark et al.[17]4MNot reported12HeadacheOculomotor nerve palsyAcute MRI: focal thickening7 to 9 weeks later: resolution of the enhancementOM
Mark et al.[17]5FNot reported5HeadacheOculomotor nerve palsyAcute MRI: focal thickening and enhancement7 to 9 weeks later: resolution of the enhancementOM
Mark et al.[17]6MNot reported3HeadacheOculomotor nerve palsyAcute MRI: whole thickening and enhancement7 to 9 weeks later: resolution of the enhancementOM
Prats et al.[18]7F1112Throbbing headacheDefective elevation and adduction of the left eye and mild dilation of the left pupil and diplopiaMRI: enlargement of the cisternal portion of the left III cranial nerve along the first 3 mm of its course, unilateral enhancement after intravenous gadolinium administrationA second MRI with contrast administration: normalOM
Prats et al.[18]8M3.55Throbbing headacheComplete III cranial nerve palsy of the left sideMRI: enlargement and enhancement of the cisternal portion of the left III cranial nerve40 days later: similar findings; 4.5 years later: normalOM
Prats et al.[18]9F66Headache in the left orbital regionModerate left ptosis of the eyelid, mild mydriasis, and displacement of the eye upward and outwardMRI: normal5 years later: normalOM
Del Toro et al.[33]10M1010Ocular painComplete right third nerve palsyMRI: right cavernous sinus enlargement, more obvious after intravenous gadolinium administration1 month later: the normal caliber of the right carotid artery similar to the left with normal appearance of the ipsilateral cavernous sinusTolosa–Hunt syndrome
O’hara et al.[21]11F47Acute onset of severe headache, nausea, and vomitingComplete right III nerve palsyRight parasagittal T1-weighted MRI scan postcontrast: enhancing cisternal portion of the right CN III with nodular mass-like thickening of the proximal portion adjacent to the brainstem6 weeks later, follow-up postcontrast: marked improvement in enhancement and thickening of the right CN IIIOM
O’hara et al.[21]12M23HeadacheComplete left CN III palsyLeft parasagittal T1-weighted MRI scan postcontrast: thickened enhancing cisternal portion of CN III8 months later: coronal T1-weighted scan postcontrast again: mass-like thickening and enhancement of the proximal cisternal portion of the left CN IIIOM
Lance and Zagami[22]13F316Sharp pain behind the left eye, followed by drooping of left eyelid; nausea, vomiting, and sensitivity to light, sound, and smellLeft ptosis with paresis of upward deviation in the left eye which turned outwards as she attempted to look upAcute MRI: enhancement of the intracisternal portion of the oculomotor nerve with gadoliniumRepeat MRI: enhancement of the oculomotor nerve present but less intenseOM
Lance and Zagami[22]14M55Right-sided headache accompanied by vomitingDrooping of right eyelid and difficulty in looking upwards and inwards with his right eyeUnenhanced MRI normalNAOM
Lance and Zagami[22]15M313Left frontal headache, vomiting, and photophobiaPtosisUnenhanced MRI normalNAOM
Shin et al.[25]16M78Severe, throbbing, left periorbital headache with associated nausea, vomiting, and photophobiaDiplopia and drooping of the upper left eyelidContrasted MRI: no abnormal findingsNAOM
Shin et al.[25]17F611Right periorbital headacheDrooping of the right eyelid and diplopiaContrasted MRI: no abnormal findingsNAOM
Weiss and Phillips[26]18M27Left supraorbital pain, nausea, and vomitingLeft ptosis, exodeviation of the left eye with horizontal and vertical diplopiaBrain MRI scan: normal, no contrast enhancement of the oculomotor nerve at its exit from the midbrainNAOM
Farage et al.[34]19M1616Pulsatile migraine associated with nauseaPtosis, mydriasis, and divergent strabismusMRI weighted in T1 with contrast: enhanced signal at left oculomotor nerve in cisternal portion18 months later: no remarkable lesionOM
Huang et al.[19]20F99Acute-onset right frontal and periorbital eye pain with migrainous characteristicsPtosis of right eyelid, a dilated pupil with slow pupillary response to light and a extraocular motor weakness compatible with partial oculomotor palsyMRI: thickening of the oculomotor nerve with abnormal enhancement at the exit of brainstem on the right sideNAOM
Murakami et al.[35]21F46Severe throbbing headacheDiplopia, left blepharoptosis, and nonreactive left midriasisMRI after contrast: a thickened oculomotor nerve in its course from the brainstem through the prepontine cistern continuouslyNASchwannoma
Choi et al.[36]22F1313Blurred vision, photophobia, and pulsating headacheComplete right side internal ophthalmoplegiaMRI: gadolinium enhancement on the cisternal portion of right oculomotor cranial nerve8 months later: gadolinium enhancement at the proximal part of the left oculomotor nerveOM
Bharucha et al.[9]23F1.516Right-sided headacheComplete palsy of the right cranial nerve IIIAcute MRI: Thickening and enhancement of cranial nerve III on the right side3 and 7 months later: reversal of abnormalitiesOM
Mcmillan et al.[20]24F912Right eye painAbrupt onset right pupil dilatation, diplopia, and ptosisAcute MRI: an area of prominent thickening and contrast enhancement at the cisternal portion of the right oculomotor nerve10 weeks later: resolution of abnormal enhancement of the right third cranial nerve, but persistent thickening of the previously enhancing segmentOM
Mcmillan et al.[20]25M11Not describedAbrupt onset left ptosis and eye deviationMRI: a small area of increased enhancement on the anterior surface of the left peduncle at the site of exit of the left oculomotor nerve17 months later: enhancement and thickened of the cistern portion of left oculomotor nerveOM
Mcmillan et al.[20]26F1616Throbbing, right-sided headache with photophobiaRight pupil dilatation, no change in visual acuityMRI: normal2 weeks later: definite enhancement of the cistern portion of the right oculomotor nerveOM
Orssaud et al.[28]27F1414Supraorbital and left ocular pain in upward movementsComplete III nerve palsyMRI: normalN ATolosa–Hunt syndrome
Orssaud et al.[28]28M55MigraineComplete III nerve palsyMRI: normalRepeat MRI 2 years later (relapse): normalOM
Vieria et al.[37]29M0.87Frontotemporal and orbital pain always on the right side, photophobia, and phonophobiaPtosis, external ophthalmoplegia and mydriasisInfundibular dilatation of a perforating branch of the posterior cerebral artery adiacent to the III nerveNAOM
Arasho[38]30M515Left hemicranial, more retro-orbital, throbbing/aching headache; photophobia; and phonophobiaComplete III nerve palsyAcute MRI: no mass lesionNAOM
Borade et al.[29]31F56Bilateral throbbing headache with photophobia and intollerance to loud sounds; vomitingPtosis on the right side and diplopiaAcute MRI: thickened and enhancing right oculomotor nerve in the suprasellar cistern regionNAOM
Vecino-Lopez et al.[30]32F0.532 months later: headache, moderate intensity; episodes <24 hoursRight eye ptosis with mydriasis, complete III nerve palsyMRI: enlargement and enhancement with contrast of the cisternal portion of the oculomotor nerveFollow-up MRI: reduced enlargement and enhancement with contrast of the cisternal portion of the oculomotor nerveOM
Miglio et al.[39]33M88Headache on the right supraorbital side; photophobia; and vomitingPtosis, outward deviation of the right eye and diplopiaMRI: focal enlargement and marked enhancement in the cisternal portion of the third right cranial nerve at the root exit zone3 months later: reduced thickening on the third right cranial nerve and resolution of the enhancementOM
Lierly et al.[40]34F310Ipsilateral, throbbing headacheAcute onset of right ptosis with lateral/inferior eye deviationMRI: isolated enhancement of the cisternal segment of the right oculomotor nerveNAOM
Da Rocha et al.[41]35M5Severe frontal headacheRight cranial nerve paresisT1-weighted postcontrast MRI: typical focal thickening and enhancement of the proximal cisternal segment of the 3 cranial nerve3 years later: persistent focal thickening without evident enhancementOM
Gelfand et al.[23]36M519Left periorbital, sharp, throbbing headache, photophobia, and nauseaNot reportedAcute MRI: enhancement of the cisternal portion of the third nerve1 year later: normalOM
Gelfand et al.[23]37M413Right, diffuse, throbbing headache, photophobia, phonophobia, nausea, and vomitingNot reportedNormalNormalOM
Gelfand et al.[23]38F916Right frontal throbbing headacheNot reportedNormalOM
Gelfand et al.[23]39M310Left, periorbital, sharp headache, nausea, and vomitingNot reportedAcute MRI thickening and enhancement of cisternal portion of third nerveNonacute MRI: persistent thickening but no enhancementOM
Verma et al.[24]40F69Right hemicranial, largely retro-orbital throbbing/pulsatile headache with photophobia, phonophobia, and vomitingDrooping of right eyelid with difficulty in moving the eyeball and diplopiaAcute MRI: thickened, enhancing right oculomotor nerve in the cisternal segment3 months later: resolution of oculomotor nerve enhancementOM
Riahi et al.[42]41F3 episodes:
2008
2010
2011
12Left side headache with vomitingLeft eye ptosis and diplopiaTwo cerebral MRI (2008–2010) normal3 years later (third episode): tissular mass in the cavernous sinus, suggesting a third nerve schawannomaSchwannoma
Jibia et al.[43]42F713Migraine with shimmering scotomasIncomplete right ptosis with semi-midriasis, diplopiaMRI: a right nodular schwannoma located within the cisternal segment of the oculomotor nerve2 months later: no changes (absence of neuroradiological variation)Schwannoma
Hurd and Sabo[27]43M412Severe, ipsilateral, pulsatile headache with photophobia, phonophobia, and nauseaLeft eye ptosis, corneal injection, mydriasis, lacrimation, exotropia, and diplopiaInitial MRI without contrast: no abnormalitiesMRI with contrast: enhancing lesion along the cisternal segment of the left oculomotor nerveRPON