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

The authors report a case of a patient with giant, invasive skull base tumor extending to the parasellar area discovered incidentally during the work-up for decreased memory. The patient's neurological exam was otherwise unremarkable. Endocrine evaluation performed at a local hospital showed a moderate hyperprolactinemia 103 ng/ml (normal up to 20 ng/ml). Given the large size of the tumor, the elevated prolactin (PRL) was interpreted to be secondary to stalk effect and patient underwent debulking surgery through a transcranial approach. Immunostaining of the excised tumor tissue was strongly positive for prolactin. His prolactin was found to be 13,144 ng/ml in our lab after surgery confirming the diagnosis of invasive giant prolactinoma. The patient developed a complete right third, fourth and sixth nerve palsy postoperatively. He was started on Cabergoline with normalization of his prolactin level and more than 50% decrease in residual tumor size over 9 months periods. There has been no clinically significant improvement in his right eye ophthalmoplegia since surgery. This case highlights the importance of 'Hook Effect' resulting in falsely low prolactin level, which may have significant therapeutic implication.
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PMID:Giant invasive pituitary prolactinoma with falsely low serum prolactin: the significance of 'hook effect'. 1659 25

We report a rare skull base neurocytoma. A 44-year-old female with a history of focal seizure and progressive right-sided weakness sought treatment at an outside institution, where she underwent total resection of a "left medial sphenoid wing paraganglioma" in 1984. In 1995 after experiencing intense left-sided headaches for 3 weeks, the patient presented to our institution. Magnetic resonance imaging revealed a large local recurrence. She had deficits dating to her initial surgery, including moderate right-sided hemiparesis, complete left ophthalmoplegia, and left facial numbness.The patient underwent a craniotomy with extensive removal of the involved sphenoid bone and a subtotal resection of the tumor. Neurocytoma was diagnosed based on strong immunohistochemical staining for synaptophysin and no reactivity for glial fibrillary acidic protein. Postoperatively, her headaches resolved completely and her neurologic status remained at baseline. The residual tumor was treated with radiation therapy. After 5 years, she remains clinically and radiographically stable.Although typically located adjacent to the foramen of Monro, neurocytomas have now been reported in almost every subcompartment of the craniospinal axis. Finding neurocytomas in extraventricular locations may require revisiting the current theory that subependymal progenitor cells are the cells of origin for these tumors.
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PMID:Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas. 1716 46

The endoscopic approach has gained an increased popularity in recent years for the biopsy and, in selected cases, the removal of tumors of the posterior third ventricle and pineal region. The authors report their experience on a series of 20 patients discussing also the technical limitations and complication avoidance. This is a prospective study of 20 patients with posterior third ventricle and pineal region tumors surgically managed by endoscopic biopsy and/or excision and simultaneous third ventriculostomy. The removal of the lesion could be achieved in 12 cases whereas in 8, only a biopsy could be performed. A histological diagnosis could be obtained in all cases. No delayed third ventricular stoma failures were recorded in any patient at the latest follow-up (mean follow-up, 39 months). Severe postoperative complications were recorded in 2 out of 12 cases of tumor removal attempt and in zero out of eight cases of biopsy. A delayed (3 weeks) postoperative mortality occurred in a patient harboring a GBM that developed an intratumoral hematoma 48 h postoperatively, one patient was in a vegetative state. Transient postoperative complications included: nausea and vomiting (five cases) and diplopia (two cases). One patient developed a bilateral ophthalmoplegia that recovered within 6 months due to residual tumor hemorrhage. Higher rate of complications was found in the case of vascularized and/or larger lesions. Endoscopic management of posterior third ventricle lesions may represent an effective option. However, though biopsies remain often a safe procedure, tumor excision should be limited to highly selected cases (cystic, poorly vascularized, and/or smaller than 2.5-cm lesions).
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PMID:Neuroendoscopic management of posterior third ventricle and pineal region tumors: technique, limitation, and possible complication avoidance. 2225 94

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