Neuromuscular hamartoma is a possible primary pathology of oculomotor ophthalmoplegic migraine
ABSTRACT 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.
SourceAvailable from: Choong Hyun Kim[Show abstract] [Hide abstract]
ABSTRACT: Benign triton tumor (BTT) or neuromuscular hamartoma is an uncommon tumor composed of mature neural and well-differentiated striated muscular elements. Its development is exceptionally rare in the adult and head region. This report describes a case of adulthood BTT that occurred in the orbit. The patient was a 53-year-old woman who presented with right periorbital swelling and pain in eyeball over 2 months. Magnetic resonance imaging revealed a well-enhancing mass surrounding optic nerve and ocular muscles in the right retrobulbar area. The tumor was subtotally removed via transcranial approach. Its pathological diagnosis was confirmed to be a neuromuscular hamartoma. She developed diplopia postoperatively. Adulthood BTT should be considered in the differential diagnosis of head and neck tumors. It is also important to make adequate therapeutic strategy to avoid postoperative neural dysfunction.Journal of Korean Neurosurgical Society 08/2014; 56(2):146-8. DOI:10.3340/jkns.2014.56.2.146 · 0.52 Impact Factor
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ABSTRACT: The International Classification of Headache Disorders classifies ophthalmoplegic migraine (OM) under "cranial neuralgias and central causes of facial pain."OM is diagnosed when all the following criteria are satisfied: At least two attacks fulfilling criterion B.Migraine-like headache accompanied or followed within four days of its onset by paresis of one or more of the III, IV and/or VI cranial nerves.Parasellar orbital fissure and posterior fossa lesions ruled out by appropriate investigations.In children the syndrome is rare and magnetic resonance (MR) shows strongly enhancing thickened nerve at the root entry zone (REZ). The authors review the literature focusing on pathogenesis theories. The authors suggest that ischemic reversible breakdown of the blood-nerve barrier is the most probable cause of OM and to include MR findings in the hallmarks of the disease. OM is the same disease in adulthood and childhood, even if in adults the MR imaging findings are negative. In the authors' opinion, OM should be classified as migraine.Cephalalgia 02/2014; 34(11). DOI:10.1177/0333102414523843 · 4.12 Impact Factor
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ABSTRACT: Benign triton tumors (BTT) are very rare lesions composed of mature skeletal muscle and neural tissue. We report a case of a 14-year-old boy who presented with asymptomatic swelling of the chin over an 18-month duration which increased gradually to involve the left side of the lower lip. Clinically, a diagnosis of neurofibroma was made. Excisional biopsy confirmed the diagnosis of a BTT. Having an affinity for large nerve trunks like the brachial and sciatic, these tumors rarely occur in the head and neck region. When they do, they may involve the large central cranial nerve trunk and present as intracranial masses or involve the smaller peripheral nerve twigs and present as asymptomatic skin nodules, of which only four cases involving peripheral nerves are reported in the English literature. Here, we report the fifth documented case of a BTT involving the mental branch of the trigeminal nerve. A brief review of the literature is also provided.The Korean Journal of Pathology 02/2013; 47(1):74-76. DOI:10.4132/KoreanJPathol.2013.47.1.74 · 0.17 Impact Factor