Ophthalmoplegic Migraine Paediatric Oculomotor Schwannoma

Ophthalmoplegic Migraine Paediatric third cranial heart and soul SchwannomaCorresponding AuthorDr.R.SubasreeTitle Ophthalmoplegic Migraine and Paediatric third cranial typeface organization Schwannoma experience or Co-Incidence?Structured AbstractObjective To proclaim a persona of Ophthalmoplegic migraine with Pediatric nervus nervus oculomotoriusius Schwannoma which is very r atomic number 18.Methods A 16 stratum old boy admitted as inpatient at our tertiary referral sum of money and University hospital in S break throughh India, with history of recurrent headaches and oculomotor paralyze of 14 divisions duration was evaluated in detail to command out tramp fossa, orbital fissure, parasellar lesions, granulomatous disorders and aneurysms.Results Initial CT wag revealed a nodulated non-enhancing lesion in the interpeduncular cistern, MR Imaging along with CISS 3D sequence through with(p) cardinal years later after CT, revealed a meek enhancing nodular lesion a t the level of midbrain in the interpeduncular cistern at fondness go forth level suggestive of schwannoma of third slipperiness.Practice Patient was inured with analgesics, nimodipine and valproate with which there was a partial response. During his subsequent 2 year follow-up, his frequency and severity of firings had reduced. destination nervus oculomotorius nerve schwannomas are extremely idealistic. yet 12 children under the age of 18, without von Recklinghausens disease have been sufficiently documented. The co knowence of OM and Oculomotor schwannoma suggests that it is not a coincidence. Awareness and suspicion is required to identify cranial cephalalgia /OM and it warrants thorough investigation to rule out intrinsic lesions mimicking OM.Implications.Controversies exist till date regarding etiology, pathophysiology, imaging findings and management guidelines of OM. The sublime association account in our report gives insight into better understanding of the patho physiology and clinico-radiological correlations in OM.Keywords Ophthalmoplegic migraine, Oculomotor SchwanommaIntroductionOphthalmoplegic migraine is very rare with annual incidence being 0.7 per million. It around often occurs in infancy or childhood. There are recurrent attacks of headache in association with ophthalmoplegia due to paresis of cranial nerve III, IV, or VI .The episodes of ophthalmoplegia may persist for several hours to several weeks, months, or aeonianly. Most often it is self-limited condition. Ophthalmoplegic migraine is also recognized as a cranial neuralgia according to 2004 edition of the world-wide smorgasbord of Headache Disorders. 1 .Paediatric Oculomotor Schwannoma is extremely rare and it can mimic OM. We name a young boy with recurrent ophthalmoplegic migraine and oculomotor schwannoma in MR imaging.Clinical ObservationA 16 year old boy presented with history of left(a)(a) hemicranial headache of 14year duration. The headache was throbbing, st ark(a) associated with drooping of left shopping center, blurring of left eye vision, photo-phonophobia, nausea and vomiting. The frequency of attacks was around 15 per month each lasting for 3-48 hours. During the attack, he was found to have left sided ptosis, disadvantageously responsive normal sized pupil and mild left t solelyness and adduction restriction. (Figure 1).There was persistent residual vision loss in left eye with acuity of 6/60. Fundus was normal. There were no other deficits.Investigations were carried out to rule out posterior fossa, orbital fissure and parasellar lesions. Initial CT Brain revealed a nodular non-enhancing lesion in the interpeduncular cistern , MR Imaging along with CISS 3D sequence done two years later (Figure 3a, 3b) revealed a small enhancing nodular lesion at the level of midbrain in the interpeduncular cistern at nerve exit level suggestive of schwannoma of third nerve. MR Angiography was normal. (Figure 3c). Patient was hard-boiled with analgesics, nimodipine and valproate with which there was a partial response. Steroids were not administered. During his subsequent 2 year follow-up, his frequency and severity of attacks had reduced.Paediatric Oculomotor Schwanomma is present as painless oculomotor deficit or may be asymptomatic and detected incidentally. Its presence with OM poses a question whether it was a mere coincidence or the cause of OM.DiscussionOphthalmoplegic migraine is a rare distinct neurologic syndrome characterized by recurrent headache and ophthalmoplegia. The third cranial nerve is most commonly affected. Most patients recover completely within days to weeks, only when a minority are left with persistent neurologic deficits. 1 .As per the international Classification of Headache, ophthalmoplegic migraine is defined as at least 2 attacks of migraine- akin headache followed within 4 days by paresis of the third, fourth, and/or 6th cranial brace, including ophthalmoparesis, ptosis, or mydriasi s .2 Gap betwixt the onset of headache and the cranial nerve palsy has varied between 2 days and10 weeks. 3The lease aetiology of this condition remains unknown. Oculomotor nerve compression, ischemia, swelling of the posterior cerebral artery, pituitary gland swelling, vascular anomaly, benign viral infection, demyelinating neuropathy, activation of trigemino-vascular remains are the various pathogenesis implicated.Diseases such as vascular malformation, granulomatous infections, pituitary apoplexy, sarcoidosis and chronic inflammatory, demyelinating polyneuropathies may have similar clinical presentation like OM. So contrast enhanced magnetic resonance imaging and magnetic resonance angiography should be the investigations of first choice for the diagnosis of OM, followed by a careful clinical examination and spinal tap. Sometimes, conventional angiogram may be necessary to draw an aneurysm. 4. secern et al. 1998.,5found focal lymph gland of the nerve in non-contrast studie s, and further thickening was present on the contrast-enhanced images in the area of the exit zone of the nerve in the inter peduncular cistern. Carlow studied the magnetic resonance scans in half-dozen patients diagnosed with OM and did a retrospective literature survey in 17 patients with OM, all of whom showed thickened ipsilateral oculomotor nerves at the midbrain exit in noncontrast T 1 -weighted images. Contrast T 1 -weighted magnetic resonance scans showed enhancement of the ipsilateral oculomotor nerves. 6.Many cases show improvement in the enhancement of cranial nerve III with resolution of the symptoms, but the clock and ground level of resolution has not been consistent in reports. Contrast enhancement on MRI is not a sine qua non for the diagnosis of OM.Gelfand AA et al., 2011 1 consistently reviewed all cases of OM in literature between1995 to 2010. There were a total of 80 cases .The median age at the time of the first ophthalmoplegic migraine attack was 8 years (3- 16 years) .The third cranial nerve was involved in the vast majority of cases (83%), sixth cranial nerve was involved in 20% and the fourth nerve in 2% of cases. The interval between headache onset and ophthalmoparesis ranged from immediate to up to 14 days. The ophthalmoplegia tended to last time-consuming (2 to 3 weeks to 2 to 3 months) .In 54%, persistent deficits were observed. Of 52 patients who had a contrast brain MRI during an acute attack, 75% had contrast enhancement of the third nerve and 76% had nerve thickening. There was a attain from corticosteroid treatment in 54%.Schwannomas are benign computer peripheral nerve sheath tumours with great propensity to arise from vestibular nerves. Oculomotor nerve schwannomas are extremely rare. There are only 40 cases reported in the literature. Only 12 children under the age of 18, without neurofibromatosis have been sufficiently documented. 7In 1982, Leunda et al. 8 reported a case in an 11-year-old boy whose tumour was resecte d en bloc and ranked as the largerst oculomotor nerve schwannoma documented at that time, with a 55-mm diameter. Since then, another 11 histologically proven paediatric cases have been draw successively in the literature. The median(a) diameters of the paediatric tumours size is 19.5 mm. Oculomotor nerve paresis was the most common neurological sign and a variable degree of oculomotor nerve dysfunction, including ptosis, diplopia, or dilated pupil, was present in all but one . Duration of operative symptoms and signs ranged from 2 weeks to 12 years. Authors opine that lesion size did not correlate with the degree of oculomotor nerve deficit. Ipsilateral ophthalmoplegic migraine was found in two cases of cisternal microlesions involving the initial prepontine segment of oculomotor nerve. 7.Total removal of schwannoma usually resulted in severe postoperative parent nerve paresis. Surgical treatment was indicated only for large tumours that presented in association with consciousne ss disturbance, other cranial nerve signs, or hemiparesis due to mass effect, or in cases where the lesion showed malignant features with rapid enlargement.Murakami et al., 2005 9 described a case of an 11-year-old daughter with oculomotor nerve schwannoma who had been suffering from symptoms mimicking OM. Her attacks became more than frequent and were not controlled by medication. After surgery, the frequency of OM attacks reduced. This was the first report to describe a pathologically confirmed case of oculomotor nerve schwannoma mimicking OM. Riahi A et al., 201410 described a 12 year old girl with recurrent raw ophthalmoplegia, who on examination was found to have left oculomotor palsy. Her third MRI focusing on third nerve revealed schwannoma of the oculomotor nerve in left cisternal portion. Kawasaki et al., 1999 11 reported a case of the coexistence of OM and ipsilateral 3rd nerve schwannoma .The authors had an acute and a follow-up MRI during a recovery phase and noticed no difference. The coexistence of two rare conditions suggests that a mere coincidence is unlikely. It has been proposed that repeated inflammation could lead to a demyelination/ remyelination process with Schwann cell proliferation and onion bulb formation.There are no published treatment trials for ophthalmoplegic migraine. Oral steroids may be of possible improvement in treating acute exacerbations based on available case series. stir up steroid therapy at the time of attack might minimize permanent sequelae of OM, including residual weakness of the third cranial nerve and pupillary dysfunction. Bharucha et al., 2007 4 disk operating system that various treatments have been described, including steroids acutely and flunarizine, acetazolamide, propranolol, cyproheptadine, or verapamil prophylactically. OM is a conglomerate disorder that needs adequate scrutiny, periodic magnetic resonance monitoring, and clinical correlation before we can conclude and draw management guideline s.Conclusion Awareness and suspicion is required to identify cranial cephalalgia /OM as it is a rare entity and diagnosis even by headache experts is make by exclusion. It warrants thorough investigation to rule out intrinsic lesions mimicking OM. The rare association reported in our report gives insight into pathophysiology and clinico-radiological correlations in OM.Bibliography1. Gelfand AA, Gelfand JM, Prabakhar P et al. Ophthalmoplegic Migraine or Recurrent Ophthalmoplegic Cranial Neuropathy innovative Cases and a taxonomic Review. Journal of Child Neurology.2011 27(6)759-7662. Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders 2nd edition. Cephalalgia. 2004 24(suppl 1)9160.3. Ravi Shankar. K. Ophthalmoplegic Migraine Still a Diagnostic Dilemma? Current Pain and Headache writings. 2008 12 285 2914. Diana X. Bharucha, herds grass B. Campbell, Ignacio Valencia. MRI Findings in Pediatric Ophthalmop legic Migraine A Case Report and Literature Review. Pediatric Neurology .2007 37 59635. Mark AS, Casselman J, Brown D, et al. 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