Gamma Knife radiosurgery in steroid-intolerant Tolosa-Hunt syndrome: case report.

Tolosa-Hunt syndrome is a rare cause of painful ophthalmoplegia due to idiopathic chronic granulomatous inflammation in the cavernous sinus. Usually clinical manifestations are well controlled by corticosteroid therapy, but steroid dependency or resistance is common. We report a case of marked improvement of Tolosa-Hunt syndrome without symptom relapse after Gamma Knife radiosurgery in a patient with steroid intolerance.

Microsurgical findings of Tolosa-Hunt syndrome.

BACKGROUND: Tolosa-Hunt syndrome (THS), a nonspecific chronic inflammation of the cavernous sinus, is a rarely needed surgical manipulation, even for diagnosis, because corticosteroid therapy is markedly effective against this condition. METHODS: A 59-year-old man presented with left trigeminal neuralgia and right abducens nerve palsy 2 months after the improvement of right oculomotor nerve palsy by corticosteroid therapy. Radiological examinations showed a mass lesion in the left cavernous sinus. The clinical course indicated THS; however, hematological examination showed a positive tuberculous test. We performed a biopsy to rule out tuberculoma in the cavernous sinus. RESULTS: The biopsy was via a left frontotemporal interdural approach. The dura propria over the cavernous sinus was thickened and tightly adhered to the inner layer. The cavernous sinus was occupied by whitish elastic-hard tissue, and it was partially resected through the anterolateral triangle. Histopathological examination showed thick fibrous tissue with numerous degenerated cells and partial inflammatory cell infiltrations without any findings of tuberculoma. The final diagnosis was THS, and corticosteroid therapy was effective despite the chronic fibrous change of the cavernous sinus lesion. CONCLUSIONS: This article presents details of surgical findings of THS that have not been demonstrated in any previous reports.

Metastatic skull base tumor from thymic carcinoma mimicking Tolosa-Hunt syndrome.

A 39-year-old male without contributory medical history had sustained progressive double vision, ptosis, and trigeminal pain for 2 weeks. Physical examination revealed total ophthalmoplegia and visual field defect with normal blood examination and chest radiography. Cranial computed tomography revealed a hyperdense mass in the left frontotemporal fossae with bony erosion. Magnetic resonance imaging confirmed a broad-based, intensely enhanced extraaxial tumor of 4x4x4 cm diameter with dural tail sign. Cerebral angiography demonstrated insignificant blood supply both from the internal carotid and middle meningeal arteries. Nearly total tumor resection was achieved via orbitofrontotemporal craniotomy. Intraoperative findings revealed the extraaxial tumor with broad attachment to the dura mater and invasion to the optic and oculomotor nerves. Histological examination revealed hypercellular tumor with significant cell atypism, mitotic activity, and focal necrosis. Immunohistochemical staining was positive for AE1/3 and c-kit, but negative for glial fibrillary acidic protein. Systemic examination performed postoperatively revealed a thymic tumor without additional remote lesions. The final diagnosis was metastatic brain tumor from thymic carcinoma. Rapid progression of neurological impairment inconsistent with a benign extraaxial tumor needs prompt surgical intervention.

Parálisis dolorosa incompleta del III nervio causada por un aneurisma de la carótida interna-comunicante posterior / Painful incomplete third-nerve palsy caused by an internal carotid-communicating posterior artery aneurysm

Caso clínico: Mujer de 38 años que presentó de forma aguda una parálisis incompleta y dolorosa del III par causada por un aneurisma de la carótida interna-comunicante posterior (CI-CP). Describimos el diagnóstico diferencial con el Síndrome Tolosa-Hunt y los resultados tras tratamiento endovascular. Discusión: La presencia de una parálisis del III par sin afectación pupilar no excluye la presencia de un aneurisma de la CI-CP. La realización de una resonancia nuclear magnética orbitaria y/o angio-tomografía axial computerizada es obligada en estos casos. La rápida recuperación tras tratamiento endovascular es consistente con otros trabajos previos

Clinical case: A 38-year-old woman presented acute-onset, painful incomplete third-nerve palsy caused by an internal carotid-posterior communicating artery (IC-PC) aneurysm. We describe the differential diagnosis with Tolosa-Hunt syndrome and the results after endovascular treatment. Discussion: The presence of pupil-sparing third-nerve palsy may not exclude the presence of an IC-PC aneurysm and orbital magnetic resonance imaging and/or computed tomography imaging must be performed in these cases. The prompt recovery after endovascular treatment is consistent with previous reports (Arch Soc Esp Oftalmol 2009; 84: 43-46)

A case of Tolosa-Hunt syndrome affecting both the cavernous sinuses and the hypophysis, and associated with C3 and C4 aneurysms.

BACKGROUND: We describe the first case of Tolosa-Hunt syndrome that is associated with C3 and C4 aneurysms. CASE DESCRIPTION: The patient, a female aged 58 years, had diplopia and right retroorbital pain. Magnetic resonance imaging revealed an enlargement of the hypophysis and bilateral cavernous sinuses, particularly on the right side. Cerebral angiography demonstrated ICA aneurysms of the left C3 and right C4 portions. These symptoms were immediately alleviated after initiation of prednisolone therapy, but recurred after the dose was tapered off. Radiological examination revealed an enlargement of the hypophysis whereas the right C4 aneurysm had decreased in size and no blood flow was apparent in the ipsilateral ophthalmic artery. A biopsy was performed and the results showed a focal inflammatory change. Steroid therapy was represcribed, and after a follow-up period of 10 months without therapy, the patient has been free of symptoms. CONCLUSION: We conclude that bilateral ICA aneurysms might be directly induced by inflammatory infiltration into intracavernous ICAs.

Reidel's thyroiditis and Tolosa-Hunt syndrome, a rare association.

A previously undescribed association of Reidel's thyroiditis with Tolosa-Hunt syndrome is reported. A 33 year-old Caucasian female presented with a two-year history of painful progressive goitre, histologically a Reidel's thyroiditis. She underwent left partial thyroidectomy. Post-operatively she developed severe right retro-orbital pain, right temporal headache and a partial right occulomotor nerve palsy. A diagnosis of Tolosa-Hunt syndrome was made. Some important aspects of this case are discussed.