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.
| Author | Reference | Case | M/F | Age of onset | Age of observation | Headache | Oculomotor involvement | MRI | MRI follow-up | Diagnosis |
|---|---|---|---|---|---|---|---|---|---|---|
| Aers et al. | [16] | 1 | F | 8 | 12 | Severe right unilateral headache with photophobia and vomiting | Diplopia and ptosis of the right upper eyelid | MRI: marked thickening and gadolinium enhancement of the right oculomotor nerve along its subarachnoid course in the interpeduncular fossa | Six months later: slightly thickened but no longer enhancing cisternal portion of the right oculomotor nerve | OM |
| Wong and Wong | [32] | 2 | M | 6 | 6 | Acute onset of headache with photophobia and vomiting | Diplopia, acute periorbital pain with droopy eyelids | No contrast MRI scan: slight asymmetry in size of the oculomotor nerve | NA | OM |
| Mark et al. | [17] | 3 | F | Not reported | 8 | Headache | Oculomotor nerve palsy | Acute MRI: focal thickening and enhancement | 7 to 9 weeks later: resolution of the enhancement | OM |
| Mark et al. | [17] | 4 | M | Not reported | 12 | Headache | Oculomotor nerve palsy | Acute MRI: focal thickening | 7 to 9 weeks later: resolution of the enhancement | OM |
| Mark et al. | [17] | 5 | F | Not reported | 5 | Headache | Oculomotor nerve palsy | Acute MRI: focal thickening and enhancement | 7 to 9 weeks later: resolution of the enhancement | OM |
| Mark et al. | [17] | 6 | M | Not reported | 3 | Headache | Oculomotor nerve palsy | Acute MRI: whole thickening and enhancement | 7 to 9 weeks later: resolution of the enhancement | OM |
| Prats et al. | [18] | 7 | F | 11 | 12 | Throbbing headache | Defective elevation and adduction of the left eye and mild dilation of the left pupil and diplopia | MRI: enlargement of the cisternal portion of the left III cranial nerve along the first 3 mm of its course, unilateral enhancement after intravenous gadolinium administration | A second MRI with contrast administration: normal | OM |
| Prats et al. | [18] | 8 | M | 3.5 | 5 | Throbbing headache | Complete III cranial nerve palsy of the left side | MRI: enlargement and enhancement of the cisternal portion of the left III cranial nerve | 40 days later: similar findings; 4.5 years later: normal | OM |
| Prats et al. | [18] | 9 | F | 6 | 6 | Headache in the left orbital region | Moderate left ptosis of the eyelid, mild mydriasis, and displacement of the eye upward and outward | MRI: normal | 5 years later: normal | OM |
| Del Toro et al. | [33] | 10 | M | 10 | 10 | Ocular pain | Complete right third nerve palsy | MRI: right cavernous sinus enlargement, more obvious after intravenous gadolinium administration | 1 month later: the normal caliber of the right carotid artery similar to the left with normal appearance of the ipsilateral cavernous sinus | Tolosa–Hunt syndrome |
| O'hara et al. | [21] | 11 | F | 4 | 7 | Acute onset of severe headache, nausea, and vomiting | Complete right III nerve palsy | Right 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 brainstem | 6 weeks later, follow-up postcontrast: marked improvement in enhancement and thickening of the right CN III | OM |
| O'hara et al. | [21] | 12 | M | 2 | 3 | Headache | Complete left CN III palsy | Left parasagittal T1-weighted MRI scan postcontrast: thickened enhancing cisternal portion of CN III | 8 months later: coronal T1-weighted scan postcontrast again: mass-like thickening and enhancement of the proximal cisternal portion of the left CN III | OM |
| Lance and Zagami | [22] | 13 | F | 3 | 16 | Sharp pain behind the left eye, followed by drooping of left eyelid; nausea, vomiting, and sensitivity to light, sound, and smell | Left ptosis with paresis of upward deviation in the left eye which turned outwards as she attempted to look up | Acute MRI: enhancement of the intracisternal portion of the oculomotor nerve with gadolinium | Repeat MRI: enhancement of the oculomotor nerve present but less intense | OM |
| Lance and Zagami | [22] | 14 | M | 5 | 5 | Right-sided headache accompanied by vomiting | Drooping of right eyelid and difficulty in looking upwards and inwards with his right eye | Unenhanced MRI normal | NA | OM |
| Lance and Zagami | [22] | 15 | M | 3 | 13 | Left frontal headache, vomiting, and photophobia | Ptosis | Unenhanced MRI normal | NA | OM |
| Shin et al. | [25] | 16 | M | 7 | 8 | Severe, throbbing, left periorbital headache with associated nausea, vomiting, and photophobia | Diplopia and drooping of the upper left eyelid | Contrasted MRI: no abnormal findings | NA | OM |
| Shin et al. | [25] | 17 | F | 6 | 11 | Right periorbital headache | Drooping of the right eyelid and diplopia | Contrasted MRI: no abnormal findings | NA | OM |
| Weiss and Phillips | [26] | 18 | M | 2 | 7 | Left supraorbital pain, nausea, and vomiting | Left ptosis, exodeviation of the left eye with horizontal and vertical diplopia | Brain MRI scan: normal, no contrast enhancement of the oculomotor nerve at its exit from the midbrain | NA | OM |
| Farage et al. | [34] | 19 | M | 16 | 16 | Pulsatile migraine associated with nausea | Ptosis, mydriasis, and divergent strabismus | MRI weighted in T1 with contrast: enhanced signal at left oculomotor nerve in cisternal portion | 18 months later: no remarkable lesion | OM |
| Huang et al. | [19] | 20 | F | 9 | 9 | Acute-onset right frontal and periorbital eye pain with migrainous characteristics | Ptosis of right eyelid, a dilated pupil with slow pupillary response to light and a extraocular motor weakness compatible with partial oculomotor palsy | MRI: thickening of the oculomotor nerve with abnormal enhancement at the exit of brainstem on the right side | NA | OM |
| Murakami et al. | [35] | 21 | F | 4 | 6 | Severe throbbing headache | Diplopia, left blepharoptosis, and nonreactive left midriasis | MRI after contrast: a thickened oculomotor nerve in its course from the brainstem through the prepontine cistern continuously | NA | Schwannoma |
| Choi et al. | [36] | 22 | F | 13 | 13 | Blurred vision, photophobia, and pulsating headache | Complete right side internal ophthalmoplegia | MRI: gadolinium enhancement on the cisternal portion of right oculomotor cranial nerve | 8 months later: gadolinium enhancement at the proximal part of the left oculomotor nerve | OM |
| Bharucha et al. | [9] | 23 | F | 1.5 | 16 | Right-sided headache | Complete palsy of the right cranial nerve III | Acute MRI: Thickening and enhancement of cranial nerve III on the right side | 3 and 7 months later: reversal of abnormalities | OM |
| Mcmillan et al. | [20] | 24 | F | 9 | 12 | Right eye pain | Abrupt onset right pupil dilatation, diplopia, and ptosis | Acute MRI: an area of prominent thickening and contrast enhancement at the cisternal portion of the right oculomotor nerve | 10 weeks later: resolution of abnormal enhancement of the right third cranial nerve, but persistent thickening of the previously enhancing segment | OM |
| Mcmillan et al. | [20] | 25 | M | 1 | 1 | Not described | Abrupt onset left ptosis and eye deviation | MRI: a small area of increased enhancement on the anterior surface of the left peduncle at the site of exit of the left oculomotor nerve | 17 months later: enhancement and thickened of the cistern portion of left oculomotor nerve | OM |
| Mcmillan et al. | [20] | 26 | F | 16 | 16 | Throbbing, right-sided headache with photophobia | Right pupil dilatation, no change in visual acuity | MRI: normal | 2 weeks later: definite enhancement of the cistern portion of the right oculomotor nerve | OM |
| Orssaud et al. | [28] | 27 | F | 14 | 14 | Supraorbital and left ocular pain in upward movements | Complete III nerve palsy | MRI: normal | N A | Tolosa–Hunt syndrome |
| Orssaud et al. | [28] | 28 | M | 5 | 5 | Migraine | Complete III nerve palsy | MRI: normal | Repeat MRI 2 years later (relapse): normal | OM |
| Vieria et al. | [37] | 29 | M | 0.8 | 7 | Frontotemporal and orbital pain always on the right side, photophobia, and phonophobia | Ptosis, external ophthalmoplegia and mydriasis | Infundibular dilatation of a perforating branch of the posterior cerebral artery adiacent to the III nerve | NA | OM |
| Arasho | [38] | 30 | M | 5 | 15 | Left hemicranial, more retro-orbital, throbbing/aching headache; photophobia; and phonophobia | Complete III nerve palsy | Acute MRI: no mass lesion | NA | OM |
| Borade et al. | [29] | 31 | F | 5 | 6 | Bilateral throbbing headache with photophobia and intollerance to loud sounds; vomiting | Ptosis on the right side and diplopia | Acute MRI: thickened and enhancing right oculomotor nerve in the suprasellar cistern region | NA | OM |
| Vecino-Lopez et al. | [30] | 32 | F | 0.5 | 3 | 2 months later: headache, moderate intensity; episodes <24 hours | Right eye ptosis with mydriasis, complete III nerve palsy | MRI: enlargement and enhancement with contrast of the cisternal portion of the oculomotor nerve | Follow-up MRI: reduced enlargement and enhancement with contrast of the cisternal portion of the oculomotor nerve | OM |
| Miglio et al. | [39] | 33 | M | 8 | 8 | Headache on the right supraorbital side; photophobia; and vomiting | Ptosis, outward deviation of the right eye and diplopia | MRI: focal enlargement and marked enhancement in the cisternal portion of the third right cranial nerve at the root exit zone | 3 months later: reduced thickening on the third right cranial nerve and resolution of the enhancement | OM |
| Lierly et al. | [40] | 34 | F | 3 | 10 | Ipsilateral, throbbing headache | Acute onset of right ptosis with lateral/inferior eye deviation | MRI: isolated enhancement of the cisternal segment of the right oculomotor nerve | NA | OM |
| Da Rocha et al. | [41] | 35 | M | — | 5 | Severe frontal headache | Right cranial nerve paresis | T1-weighted postcontrast MRI: typical focal thickening and enhancement of the proximal cisternal segment of the 3 cranial nerve | 3 years later: persistent focal thickening without evident enhancement | OM |
| Gelfand et al. | [23] | 36 | M | 5 | 19 | Left periorbital, sharp, throbbing headache, photophobia, and nausea | Not reported | Acute MRI: enhancement of the cisternal portion of the third nerve | 1 year later: normal | OM |
| Gelfand et al. | [23] | 37 | M | 4 | 13 | Right, diffuse, throbbing headache, photophobia, phonophobia, nausea, and vomiting | Not reported | Normal | Normal | OM |
| Gelfand et al. | [23] | 38 | F | 9 | 16 | Right frontal throbbing headache | Not reported | — | Normal | OM |
| Gelfand et al. | [23] | 39 | M | 3 | 10 | Left, periorbital, sharp headache, nausea, and vomiting | Not reported | Acute MRI thickening and enhancement of cisternal portion of third nerve | Nonacute MRI: persistent thickening but no enhancement | OM |
| Verma et al. | [24] | 40 | F | 6 | 9 | Right hemicranial, largely retro-orbital throbbing/pulsatile headache with photophobia, phonophobia, and vomiting | Drooping of right eyelid with difficulty in moving the eyeball and diplopia | Acute MRI: thickened, enhancing right oculomotor nerve in the cisternal segment | 3 months later: resolution of oculomotor nerve enhancement | OM |
| Riahi et al. | [42] | 41 | F | 3 episodes: 2008 2010 2011 |
12 | Left side headache with vomiting | Left eye ptosis and diplopia | Two cerebral MRI (2008–2010) normal | 3 years later (third episode): tissular mass in the cavernous sinus, suggesting a third nerve schawannoma | Schwannoma |
| Jibia et al. | [43] | 42 | F | 7 | 13 | Migraine with shimmering scotomas | Incomplete right ptosis with semi-midriasis, diplopia | MRI: a right nodular schwannoma located within the cisternal segment of the oculomotor nerve | 2 months later: no changes (absence of neuroradiological variation) | Schwannoma |
| Hurd and Sabo | [27] | 43 | M | 4 | 12 | Severe, ipsilateral, pulsatile headache with photophobia, phonophobia, and nausea | Left eye ptosis, corneal injection, mydriasis, lacrimation, exotropia, and diplopia | Initial MRI without contrast: no abnormalities | MRI with contrast: enhancing lesion along the cisternal segment of the left oculomotor nerve | RPON |