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Neuromuscular hamartoma is a possible primary pathology of oculomotor ophthalmoplegic migraine
Jiro Akimoto1, Shinjiro Fukami, Ryo Hashimoto
1Department of Neurosurgery, Tokyo Medical University, Japan.
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.
Insights
Oculomotor ophthalmoplegic migraine (O-OPM) in a man was linked to a rare neuromuscular hamartoma. This congenital mass in the oculomotor nerve
Area of Science:
- Neurology
- Pathology
- Ophthalmology
Background:
- Oculomotor ophthalmoplegic migraine (O-OPM) is observed in children, sometimes with MRI-detected masses at the oculomotor nerve's root exit zone (REZ).
- These masses are hypothesized to stem from nerve hypertrophy due to recurrent demyelination.
Observation:
- A 51-year-old man with O-OPM since age 6 experienced worsening migraine frequency and intensity.
- Brain MRI revealed a small nodular mass in the REZ of the oculomotor nerve.
- Initial diagnosis suggested oculomotor schwannoma, but biopsy revealed a neuromuscular hamartoma due to adherence and hemorrhage.
Findings:
- Pathological diagnosis confirmed a neuromuscular hamartoma in the REZ of the oculomotor nerve.
- This hamartoma was tightly adhered to and hemorrhagic, complicating surgical intervention.
Implications:
- A small nodular mass in the REZ of the oculomotor nerve may represent a hamartoma with congenital origins.
- This hamartoma could be the primary cause of O-OPM in specific cases.
- Further research into congenital factors and hamartomas in O-OPM is warranted.
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