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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 5  |  Page : 778-780

Transient oculomotor synkinesis in a case of ophthalmoplegic migraine

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Date of Web Publication6-Oct-2015

Correspondence Address:
Subasree Ramakrishnan
Department of Neurology, NIMHANS, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.166537

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How to cite this article:
Ramakrishnan S, Kallollimath P, Kulkarni GB, Mustare V. Transient oculomotor synkinesis in a case of ophthalmoplegic migraine. Neurol India 2015;63:778-80

How to cite this URL:
Ramakrishnan S, Kallollimath P, Kulkarni GB, Mustare V. Transient oculomotor synkinesis in a case of ophthalmoplegic migraine. Neurol India [serial online] 2015 [cited 2020 Jun 6];63:778-80. Available from:


Ophthalmoplegic migraine is very rare and is characterized by more than two attacks of migraine accompanied or followed within 4 days of its onset by paresis of one or more of the third, fourth and/or sixth cranial nerves. It is also recognized as a cranial neuralgia.[1] Ophthalmoplegia may persist for several hours, weeks, months, or even permanently. The attacks of extraocular muscle weakness becomes more severe with each attack until a permanent unilateral oculomotor palsy develops.[2] Oculomotor regeneration and synkinesis that develops after oculomotor palsy in OM is extremely rare.

A 30-year-old lady presented with an eight month history of blurring of vision of left eye and a fifteen day history of drooping of the right eye with binocular double vision on a background history of infrequent migraine for 5 years. Her headache frequency and severity increased over the last 8 months. Episodes of headache lasted for 1-3 days, occurred every alternate day, and were associated with photo- and phonophobia, nausea and vomiting. The gradual onset of painless vision loss was associated with one such episode of headache. The vision loss progressed over 2–3 weeks and then remained status quo, so that she could only perceive light through the left eye at presentation.

On examination, the patient was found to have complete ptosis of the right eye with restricted adduction and elevation of the right eye. There was mild ptosis, vertical malalignment, and restricted elevation, depression, and abduction (more than adduction) in the left eye [Figure 1]. There was chorioretinal pigmentation of the right eye and optic disc pallor of the left eye. The left pupil was dilated and sluggishly reacting. On elevation of the ptotic right eyelid, there was synkinetic elevation of the left eyelid [Video 1]; and, elevation and adduction which were restricted in left eye became full range with passive elevation of right eyelid [Figure 2]. Furthermore, on adducting the left eye, there was elevation of left eyelid (reverse Duane syndrome) [Video 1]. There was retraction of the left globe on attempted vertical eye movements. This bilateral oculomotor synkinesis was considered to be due to aberrant regeneration and innervation from the right oculomotor nerve to the left levator palpebris superioris and the third nerve. There was also innervation from the left medial recti to the left levator palpebris superioris. Interestingly, on passive closure of the right eye, elevation of the left eye, which was restricted, became full.
Figure 1: Right eye complete ptosis with restriction of adduction and elevation, and vertically malaligned left eye with restriction of all movements

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Figure 2: On elevation of the ptotic right eyelid, there was synkinetic elevation of the left eyelid, and elevation and adduction which were restricted on the left side became full range with passive elevation of the right eyelid

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Her imaging (including constructive interference in steady-state three-dimensional sequence) was performed to look for any evidence of brainstem/posterior fossa/orbital pathology and was found to be normal. The patient was diagnosed to be having ophthalmoplegic migraine in view of the cranial nerve involvement with the onset of migraine. She was given intravenous valproate and a short course of intravenous steroids followed by oral steroids. She showed significant improvement in her headache and after 1-month, her oculomotor palsies and oculomotor synkinesis improved significantly [Figure 3]. During follow-up, she was prescribed tablet valproate 600 mg/day and flunarizine 10 mg/day as prophylaxis, and sumatriptan 25 mg SOS during the acute attacks. Vasculitic work-up, serum angiotensin converting enzyme levels and computed tomography of the thorax were normal.
Figure 3: Normal eye movements during follow-up

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Primary aberrant regeneration of the oculomotor nerve is a clinical syndrome involving the eyelid, pupil, and extraocular movement abnormalities following damage to the peripheral portion of the oculomotor nerve. Aberrant regeneration of the oculomotor nerve is commonly seen during the recovery from acute palsies caused by aneurysms, head injuries and rarely by tumors. It is also reported in intracavernous meningiomas, pituitary tumors with hemorrhage and in idiopathic cases. In children, it is a common sequel to congenital third nerve palsy and congenital fibrosis syndromes.[2] The proposed hypotheses for synkinesis are misdirection of regenerating third nerve fibers, ephaptic transmission, and central synaptic reorganization.[3]

Ocular findings of misdirection syndrome include elevation of the upper lid on attempted adduction (reverse Duane syndrome) or downgaze of the eye (pseudo-von Graefe sign), retraction of the globe on attempted vertical gaze and constriction of the pupil on adduction of the eye (pseudo-Argyll Robinson pupil).[4] Aberrant regeneration of nerve fibres to iris sphincter causes segmental contraction of the iris when eyes try to move in any direction guided by muscles innerved by the third nerve (Czarnecki's sign). The third feature is adduction of the affected eye on attempted elevation or depression due to the misdirected innervation of fibers from superior and inferior rectus into the medial rectus.

Oculomotor nerve ischemia or its compression, swelling of the posterior cerebral artery, vascular anomaly, viral infection, demyelinating neuropathy and trigeminovascular system activation are the various pathogenesis implicated in the genesis of OM. Initially, nerve compression by an edematous cavernous sinus, ischemia, and the combination of peripheral and intramedullary mechanisms were considered in the etiopathogenesis.[5],[6],[7] Lepore and Glaser in 1980,[8] reported a case of a young boy with transient oculomotor synkinesis after migrainous ophthalmoplegia. He postulated that ephaptic neuronal impulse transmission or chromatolysis-induced nuclear synaptic reorganization were responsible for the transient synkinesis.

Various disorders such as a vascular malformation, Tolosa-Hunt syndrome, pituitary apoplexy, diabetic ophthalmoplegia and chronic inflammatory polyneuropathies may mimic OM. Contrast enhanced magnetic resonance imaging (MRI) and magnetic resonance angiography are the investigations of choice for the diagnosis of OM, followed by spinal tap. The gadolinium enhancement of perimesencephalic third nerve can be seen during an acute attack. Steroids during the acute phase, and flunarizine, acetazolamide, propranolol, cyproheptadine, or verapamil administered prophylactically, are the various treatments prescribed.[9]

Transient oculomotor synkinesis can be seen following oculomotor palsy in ophthalmoplegic migraine, although it has rarely been reported. This interesting clinical finding helps in better understanding of the pathophysiology of ophthalmoplegic migraine. Trauma, aneurysm, tumor and other causes mimicking an OM or causing oculomotor synkinesis should be ruled out by adequate investigations like MRI and angiography.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders. 2nd ed. Cephalalgia 2004. Suppl 1: 9-160.  Back to cited text no. 1
O'Day J, Billson F, King J. Ophthalmoplegic migraine and aberrant regeneration of the oculomotor nerve. Br J Ophthalmol 1980;64:534-6.  Back to cited text no. 2
Sebag J, Sadun AA. Aberrant regeneration of the third nerve following orbital trauma. Synkinesis of the iris sphincter. Arch Neurol 1983;40:762-4.  Back to cited text no. 3
Slavin ML, Einberg KR. Abduction defect associated with aberrant regeneration of the oculomotor nerve after intracranial aneurysm. Am J Ophthalmol 1996;121:580-2.  Back to cited text no. 4
Walsh JP, O'Doherty DS. A possible explanation of the mechanism of ophthalmoplegic migraine. Neurology 1960;10:1079-84.  Back to cited text no. 5
Walsh FB, Hoyt WF. Clinical Neuro-Ophthalmology. 3rd ed. Baltimore: Williams and Wilkins; 1969. p. 2.  Back to cited text no. 6
Elston JS. Traumatic third nerve palsy. Br J Ophthalmol 1984;68:538-43.  Back to cited text no. 7
Lepore FE, Glaser JS. Misdirection revisited. A critical appraisal of acquired oculomotor nerve synkinesis. Arch Ophthalmol 1980;98:2206-9.  Back to cited text no. 8
Bharucha DX, Campbell TB, Valencia I, Hardison HH, Kothare SV. MRI findings in pediatric ophthalmoplegic migraine: A case report and literature review. Pediatr Neurol 2007;37:59-63.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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