ophthalmoplegic migraine ( om ) is characterized by recurrent attacks of migrainous headache , associated with paresis of one or more ocular cranial nerves with absence of demonstrable intracranial lesions , other than magnetic resonance imaging ( mri ) of the brain changes within the affected nerve . from the recent observations ,
international headache classification ( ihcd - ii ) has reclassified om from a variant of migraine to the category of neuralgia .
we report an adolescent girl with om , who had been treated with steroid and showed dramatic improvement .
an 11-year - old girl presented with abrupt unilateral ( right ) ptosis and diplopia for 3 weeks [ figure 1 ] . at the beginning , it was associated with severe throbbing headache , pain in the right eye , nausea and recurrent vomiting . within 7 days , except ptosis and diplopia , all other symptoms had spontaneously subsided .
she had denied having trauma , fever , diurnal variation of ptosis , tinnitus , transient visual blurring or limb weakness .
past history revealed that she had experienced similar attacks 56 times in the past 4 years . in each episode
, ptosis was unilateral and involved mostly right eyelid , but left eyelid was involved in one occasion .
image of face showing right sided ptosis ophthalmologic examination revealed incomplete ptosis of the right eyelid and paresis of the right , upward and downward gaze .
pupillary constriction was normal in the left eye and sluggish in the right eye to both direct and consensual light stimulation .
acuity of vision without correction and intraocular pressure in both eyes were within normal limit .
anterior and posterior segment examination as well as macula , vessels and periphery were normal .
investigations including complete blood counts , erythrocyte sedimentation rate ( esr ) and c - reactive protein , fasting blood sugar ( fbs ) , thyroid function tests , antinuclear antibody ( ana ) were within normal limits . mri brain ( plain and contrast ) was also normal .
om was taken as diagnosis and oral prednisolone ( 2 mg / kg / day ) was started , which resulted in resolution of painful ophthalmoplegia within 7 days .
steroid was continued for weeks and then tapered off and antimigraine prophylaxis ( flunarizine ) was started . on follow - up , she is now attack free for the last 18 months .
ophthalmoplegic migraine was named by charcot in 1890 ; since then , it was being considered as a variant of migraine .
clinical criteria for oculomotor om are : 1 ) childhood onset , 2 ) headache preceding and ipsilateral to the third nerve paresis , 3 ) a dilated pupil , 4 ) ophthalmoplegia that may be permanent and rarely accompanied by aberrant oculomotor regeneration , 5 ) a minimum of two episodes and 6 ) no evidence for a structural lesion . recently
, different pathogenic mechanisms , which include compressive , ischemic and inflammatory , have been suggested . in some cases , mri shows gadolinium uptake in the cisternal part of the affected cranial nerve .
the recent revision of the ihcd - ii has reclassified om from a subtype of migraine to the category of neuralgia .
however , other varieties of migraine like  common  or  classic  are invariably found in family .
onset of disease in our patient was about 7 years of age , but majority of the patients experience initial attack in the first decade , usually before 5 years of age .
thus , the first attack is incorrectly attributed to aneurysm , trauma , infection or recent immunization ; only when the condition resolves and recurs again , correct diagnosis is made . rarely , patients experience their first attack in adulthood , but such patients mostly have a history of typical migraine headaches with or without aura since childhood or a family history of migraine or both . approximately , 86% of patients of om have transient , reversible mri contrast enhancement of the affected cranial nerve .
contrast - enhanced studies also showed focal thickening at the exit of the nerve in the interpeduncular cistern without enhancement of the cavernous sinus or adjacent dura .
although enhancement is associated with om , it is not a sine qua non for the diagnosis of om , as in our patient normal neuroimaging may be seen in om .
the differential diagnoses of om include intracranial aneurysms or tumors , oculomotor nerve schwannoma , sphenoidal sinus mucoceles , raised intracranial tension ( ict ) with brain herniation , tolosa hunt syndrome ( ths ) , myasthenia gravis ( mg ) , and diabetic neuropathy .
although reported in a young child , intracranial aneurysm is extremely rare below 14 years of age . in our patient
, the normal mri brain excludes that diagnosis . in the case of oculomotor nerve schwannoma , slowly progressive weakness of the third nerve over the years
that is also a less likely diagnosis because the patient 's diplopia was recurrent and she was absolutely normal in between episodes .
mucocele of the sphenoidal sinus or tumors or inflammatory lesions that invade the cavernous sinus can cause painful ophthalmoplegia . however , the headache in such disorders is of rather gradual onset and is usually accompanied by systemic symptoms or signs of other cranial nerve palsies . in the index case ,
raised ict can cause herniation of hippocampal gyrus , producing an oculomotor paresis which may be transient , recurrent and associated with severe headache . in our patient ,
normal fundoscopic examination and absence of findings of brain herniation in mri ruled out that diagnosis .
evidence of inflammation in the superior orbital apex and cavernous sinus region supports a diagnosis of ths .
however , proximal enlargement with gadolinium enhancement of the oculomotor nerve within the prepontine cistern has been reported both in om and ths .
the girl had normal mri brain ; so , it was difficult to differentiate om from ths on the basis of neuroimaging in our patient . however , ths is a disease of adults , associated with retro - orbital pain and is slow to resolve , especially without prednisone . in our patient ,
the previous histories of a recurrent third nerve palsy which resolved spontaneously and whose onset was associated with headache also made om a more likely diagnosis .
ocular mg was a less likely diagnosis because there was absence of diurnal variations of ptosis and presence of pupil abnormality .
ophthalmoparesis due to uncontrolled diabetes mellitus ( dm ) rarely occurs in children . besides dm , patients with hypertension
although differential diagnosis is rather large , most other possible causes of ophthalmoplegia and headache have distinctive clinical presentations . as in our case ,
optimal prophylactic and acute treatment is still unclear , but migraine prophylactic medications such as beta blockers and calcium channel blockers have been proposed .
prognosis is good because symptoms almost always resolve , but after several episodes , some deficits may persist .
 ophthalmoplegic migraine ( om ) is characterized by recurrent attacks of headache with paresis of ocular cranial nerves . 
 previously , it was classified as a variant of migraine , but recently , international headache classification ( ihcd - ii ) has reclassified om to the category of neuralgia . 
 presently , om is considered a type of recurrent demyelinating cranial neuropathy . 
 we report an adolescent girl with om , who had been treated with steroid and showed dramatic improvement . 
Introduction
Case Report
Discussion