Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy: report of two cases and review of the literature.

BACKGROUND: In recurrent painful ophthalmoplegic neuropathy (RPON) that was previously termed as ophthalmoplegic migraine, enhancement of the ocular motor cranial nerves could be seen in the cisternal segment during the acute phase. However, various tumors involving the oculomotor nerve may mimic RPON. METHODS: We report two patients with MRI findings of oculomotor nerve schwannoma who initially presented with RPON, and found through the literature review five more patients with oculomotor nerve tumors that masqueraded as RPON. RESULTS: All patients showed an involvement of the oculomotor nerve. The radiological or pathological diagnosis included schwannoma in five, venous angioma in one, and neuromuscular harmatoma in another one. MRIs with gadolinium documented an enhancing nodule involving the cisternal portion of the oculomotor nerve in six of them, which was also observed on follow-up MRIs without an interval change. CONCLUSIONS: It should be recognized that an incomplete recovery may occur during future attacks in patients with otherwise uncomplicated RPON. Follow-up MRIs are required to detect tumors involving the ocular motor cranial nerves, especially in patients with suspected RPON when the recovery is incomplete.

The triad of luteal phase ocular migraines, interstitial cystitis, and dyspareunia as a result of sympathetic nervous system hypofunction.

PURPOSE: To evaluate whether ocular migraines can be related to sympathetic nervous system hypofunction, especially when associated with interstitial cystitis and dyspareunia. MATERIALS AND METHODS: Dextroamphetamine sulfate was administered to a 34-year-old woman with a history of long-term interstitial cystitis, dyspareunia, and ocular migraines that were resistant to all other therapies. RESULTS: In a short length of time the sympathomimetic amine therapy almost completely abrogated all of her symptoms and they have remained controlled while she continues on the drug. CONCLUSIONS: This is the first report of effectively treating ocular migraines with dextroamphetamine sulfate. The gynecologist should not be afraid to initiate therapy without referral to other specialists, especially if other symptoms of the sympathetic neural hyperalgesia edema syndromes exist, e.g., bladder pain of pelvic origin and dyspareunia.

Neuromuscular hamartoma is a possible primary pathology of oculomotor ophthalmoplegic migraine.

BACKGROUND: Oculomotor ophthalmoplegic migraine (O-OPM) occurs in many children, and in some cases MRI shows a small mass in the root exit zone (REZ) of the oculomotor nerve. This mass is considered to result from nerve hypertrophy caused by repeated demyelination. CASE RESULTS: A 51-year-old man has been on oral medication for O-OPM, which he had from 6 years of age. However, the frequency and intensity of his migraine attacks have gradually increased. Brain magnetic resonance imaging (MRI) revealed a small nodular mass in the REZ of the oculomotor nerve. The mass was initially diagnosed as oculomotor schwannoma and tumor resection was attempted. However, as the mass was tightly adhered to the oculomotor nerve and hemorrhagic, biopsy was performed. The pathological diagnosis was neuromuscular hamartoma. CONCLUSION: The small nodular mass in the REZ of the oculomotor nerve may be a hamartoma associated with congenital factors and may possibly be the primary pathology of O-OPM in this case.

Recurrent painful ophthalmoplegic neuropathy: a report of two new pediatric cases.

BACKGROUND: Recurrent painful ophthalmologic neuropathy (RPON), formerly known as ophthalmoplegic migraine, is characterized by repeated attacks of one or more ocular cranial nerve palsies with an ipsilateral headache. While steroid therapy has been reported to be beneficial for attacks, no clear consensus on prophylactic treatments exists. We present two cases emphasizing the diagnostic significance of the loss of enhancement during the symptom-free period and valproate as a beneficial option in prophylaxis. CASE 1: A 4-year-old girl presented with a one-week right frontal headache, vomiting and photophobia. Neurological examination revealed ptosis, oculomotor nerve paresis, and delay in light reflex in the right eye. Brain magnetic resonance imaging (MRI) revealed a 5.5 mm nodular enhancement in the cisternal part of the 3rd cranial nerve in the right premesencephalic area. The enhancement regressed after a 6-month symptom-free period. While propranolol, topiramate and flunarizine were inefficacious in prophylaxis, the patient responded to valproate prophylaxis and benefited from the administration of steroids for one week during the attacks. CASE 2: A 7-year-old girl presented with a ten-day right-sided, throbbing headache in the frontal region, oneday eye deviation and double vision. Neurological examination revealed inward gaze restriction and ptosis in the ipsilateral eye to the headache. Brain MRI revealed a 4.5 mm, enhancing, nodular lesion in the 3rd cranial nerve lodge in the right perimesencephalic area. Her symptoms regressed in one week with dexamethasone and she received prophylactic propranolol. Neuroimaging findings disappeared after a 3-month symptom-free period. After valproate was added because of a relapse, she did not experience any further attacks. CONCLUSIONS: RPON is an uncommon disease in childhood with unknown etiology. On brain MRI with contrast during the symptom-free period, regression of the enhancement or complete resolution of the lesion are guiding features in the diagnosis. Valproate may have beneficial effects on RPON treatment.

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

Background: Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology. Case presentation: We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings. Conclusions: This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma.

Ophthalmoplegic migraine with isolated third cranial nerve palsy in a known case of juvenile myoclonic epilepsy.

Among the various forms of migraine headaches, ophthalmoplegic migraine is an uncommon and rare form, the incidence of which is approximately 0.7 per million. It presents predominantly with headache and ophthalmoplegia. One of more cranial nerves can be affected, however the third cranial nerve is most often affected. As a result, symptoms wise, mydriasis and ptosis are commonly seen. Patients generally recover completely within a few days or weeks, however residual deficits are known to occur in a minority of patients. One of the common generalised epilepsy syndromes is the juvenile myoclonic epilepsy (JME), its prevalence being roughly up to 10% of all patients with epilepsy. It usually begins in the second decade of life. Generalised tonic-clonic seizures myoclonic jerks absences constitute the main seizure types in JME. Studies indicate a definite association of epilepsy with migraine headaches and a significant number of migraneurs are found to be epileptic. Conversely, patients with epilepsy are two times more likely to have migraine, as compared to their first degree relatives without migraine. We report a known case of a female patient of JME having a history of classical migraine with aura presenting to us with headache and ophthalmoplegia. She was extensively evaluated to rule out other causes of isolated third cranial nerve palsy, with all the investigations being negative for any obvious cause. She was treated with non-steroidal anti-inflammatory drugs for the acute attack and was subsequently put on antimigraine medication, propranolol during her hospital stay, with which her ptosis recovered completely after 2 weeks. The patient was later started on tablet divalproex sodium, which the patient continues to take on a long-term basis, especially because of its efficacy as an antimigraine prophylaxis agent and a potent drug against JME.