So-called ophthalmoplegic “migraine” has now been changed in the official Classification of Headache of the International Headache Society.¹  This is because many reports have shown abnormalities of the ocular motor nerve using magnetic resonance imaging (MRI) in children with recurrent painful ophthalmoplegia fulfilling the previous criteria for ophthalmoplegic “migraine.”^108,  It is now believed that all modern cases show MRI enhancement of the third nerve which may represent a type of inflammation and, therefore, more characteristic of what is seen with facial nerve palsy than with any form of migraine.¹¹⁰  In this rare variety of what was formerly termed “complicated migraine,” the headaches were associated with ocular motor nerve palsies.^111,112 Usually the ophthalmoplegia is transient; however, it can become permanent especially after repeated attacks.  Major controversy has surrounded the diagnostic and nosologic position of ophthalmoplegic “migraine” since its initial recognition in the mid 1880s.  Until the l930s and 1940s when angiography was introduced and practiced, it was impossible in many cases to rule out aneurysms and other lesions in the vicinity of the cavernous sinus. Multiple etiologies were cited as underlying causes of ophthalmoplegic “migraine," including aneurysm, basilar arachnoiditis, and tumors; indeed, many physicians believed that no separate clinical syndrome of ophthalmoplegic migraine existed, but  that all patients had specific organic lesions. 

              Woody and Blaw¹¹³ reported two cases of ophthalmoplegic “migraine” occurring in infants five and seven months old.  The infants had recurrent attacks with almost complete clearing between episodes. Both children were treated with prednisone during subsequent attacks which seemed to shorten the duration of the episodes. 

               In ophthalmoplegic “migraine” the third nerve is most frequently involved.  Walsh and Hoyt⁶⁷ state that abducens palsy occurs 1:10 as frequently as third nerve palsy, with even rarer affliction of the fourth nerve.  In most cases positive family history is not present.  Thus, a typical clinical syndrome emerges:  a child or young adult with periodic headache has ophthalmoplegia involving all functions of the third nerve, beginning at the height of an attack of cephalgia, which is primarily unilateral and in the orbital region; the paresis lasts for days to weeks following the cessation of headache; recovery is gradual and tends to be less complete after repeated attacks.  The following case report is considered exemplary: 

A 3‑year‑old boy presented with a complete left oculomotor palsy.  The day before he had complained of headache, was lethargic, and went to bed early.  The following morning he awakened with complete ptosis of the left upper lid, but his headache was gone. On examination the left pupil was 6 mm and slightly reactive to light; all muscles supplied by the left third nerve were profoundly affected. (Fig 10) 

 The neurologic examination and plain skull x‑rays were entirely normal.  The child recovered completely in 3 weeks' time.  An exactly similar episode occurred 20 months later, also with rapid spontaneous resolution, and a third episode occurred 1 year after that.  The child is now well and suffers only occasional headaches.

             In the differential diagnosis, consideration should be given to aneurysm, tumor, diabetes, and sphenoid sinus mucocele.  The age at onset, negative glucose tolerance test, and radiologic studies will usually rule out the listed possibilities.  Other clinical entities confused with ophthalmoplegic “migraine” have included myasthenia gravis and the Tolosa‑Hunt syndrome.  The former condition is ruled out if the pupil is involved (and actually should not be considered in the presence of pain) and with response to edrophonium chloride (Tensilon); the latter possibility should be considered if pain persists.  On rare occasions only limited involvement of the third nerve occurs. 

Reports of transient, otherwise unexplained unilateral pupillary mydriasis have been tentatively attributed to migraine in young patients.^114,115  One should be careful to exclude intermittent angle‑closure glaucoma with mydriasis as pointed out by Sarkies and colleagues.¹¹⁶  They reported a 31 year old man with an 18 month history of episodic periorbital pain who, during an attack, noted blurred vision and a dilated pupil.  He was found to have a sector palsy in the upper nasal quadrant of the left iris, an intraocular pressure of 16 mm Hg between attacks and on gonioscopy and narrow angle with a plateau‑type iris.  During an attack his intraocular pressure increased to 26 mm Hg.  Following a provocative dark‑room test, the patient developed a typical headache and was found to have an intraocular pressure of 45 mm Hg and a closed angle on gonioscopy. This report is important because it  points out a condition which must be eliminated before considering episodic mydriasis with ocular pain to be a part of ophthalmoplegic “migraine.”  

Rarely, ophthalmoplegic “migraine” may occur without headache. Durkan et al¹¹⁷ described two children with isolated recurrent painless oculomotor palsy in whom neurodiagnostic investigations were all normal. 

 In the differential diagnosis, suspicion would be raised by 1) the absence of a migraine history; 2) severe persistent headache with total ophthalmoplegia;  3) onset after age 20; and 4) symptoms and signs of subarachnoid hemorrhage. Angiography is not warranted in a young patient strictly fulling the clinical criteria.