Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029089 (ophthalmoplegia)
3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sphenoorbital meningiomas require extensive bone removal around the superior and lateral orbital walls, superior orbital fissure, and anterior middle fossa floor. Incomplete resection can lead to recurrence or growth into the cavernous sinus (CS). A 46-year-old woman with a history of childhood leukemia treated with chemotherapy and whole-body radiotherapy had presented to an outside institution in 2004 with headache and vision changes and undergone subtotal resection for right sphenoorbital meningioma. Residual tumor growth caused progressive optic neuropathy, and she underwent multiple orbital decompressions and fractionated radiotherapy. In 2017, she underwent another craniotomy for repeat resection. Additional tumor growth causing neuropathic facial pain syndrome and progressive ophthalmoplegia was treated with orbital enucleation. On referral to our institution, magnetic resonance imaging demonstrated right sphenoorbital and CS meningioma extending into the sella and nearly to the medial border of the contralateral CS. Given her complete ophthalmoplegia and recent orbital enucleation, she underwent revision right frontotemporal craniotomy for radical resection of invasive meningioma, including right internal carotid artery occlusion and CS resection (Video 1). The skull-base defect was repaired with autologous fascia and a free muscle flap. Postoperative transient aphasia and left hemiparesis resolved over several days. At the 1-month follow-up examination, she was neurologically intact, with moderate improvement of facial pain syndrome (preoperative pain score, 9 of 10; postoperative pain score, 6 of 10). Magnetic resonance imaging demonstrated gross total resection. Pathological tissue analysis was consistent with grade 1 meningioma with an increased MIB-1 proliferative index, although, clinically, the tumor behaved more malignantly. The patient provided consent.
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PMID:Resection of Invasive Sphenoorbital and Cavernous Sinus Meningioma via Frontotemporal Craniotomy. 3241 38

Recurrent painful ophthalmoplegic neuropathy (RPON) is a very rare disease characterized by recurrent attacks (at least two) of unilateral headache associated with ipsilateral ophthalmoplegia due to paresis of one or more cranial motor nerves, not due to any orbital, parasellar, or posterior fossa lesions. The differential diagnoses for this condition are broad. In addition to disability during an acute attack, this disease could also cause a permanent neurologic deficit. The understanding of RPON pathogenesis has changed over time, leading to a change in the classification of this disorder between editions of the International Classification of Headache Disorders, in which the condition was moved from the chapter on migraine to the chapter on cranial neuralgias and central causes of facial pain. There is no consensus on the pathogenesis of RPON. It is possible that multiple pathogenic mechanisms underlie various clinical forms of the disease. A depiction of pathologic analyses of patients with radiologically confirmed changes in the affected nerves during and outside of attacks would significantly contribute to knowledge of its pathogenesis. Brain imaging should be performed in each patient during an acute RPON attack and at a regular schedule between attacks. Further case reports and case series are required before further conclusions can be made regarding RPON pathogenesis and proposals for treatment options.
J Oral Facial Pain Headache
PMID:Recurrent Painful Ophthalmoplegic Neuropathy: Migraine, Neuralgia, or Something Else? 3329 Apr 43


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