Alternating recurrent painful ophthalmoplegia is caused by various neurological conditions including Tolosa-Hunt syndrome, sellar mass, and parasagittal meningioma. We experienced a rare case of recurrent painful ophthalmoplegia occurring on the contralateral side as a manifestation of idiopathic hypertrophic tentorial pachymeningitis. We propose that idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of alternating recurrent painful ophthalmoplegia.
Diagnosis, Differential ; Meningioma ; Meningitis* ; Ophthalmoplegia* ; Tolosa-Hunt Syndrome

Aged ; Animals ; Diabetes Complications ; Diagnosis, Differential ; Female ; Humans ; Ophthalmoplegia ; diagnosis ; etiology ; Tolosa-Hunt Syndrome ; diagnosis

Cavernous sinus syndrome (CSS) is defined as the involvement of two or more of the third, fourth, fifth (V1, V2) or sixth cranial nerves or involvement of only one of them in combination with a neuroimaging-confirmed lesion in the cavernous sinus. Some cases of CSS are attributed to Tolosa-Hunt syndrome (THS), an idiopathic inflammatory disease of the cavernous sinus. THS is characterized by painful ophthalmoplegia due to granulomatous inflammation in the cavernous sinus. THS is a diagnosis of exclusion that requires a vigorous series of differential diagnoses, and corticosteroid therapy is known to dramatically resolve clinical findings of THS. We report a case of a patient with painful ophthalmoplegia associated with vision loss, which was suspected to be THS. This patient followed a relatively typical clinical course of THS on steroid pulse therapy. We emphasize the differential diagnosis of THS, its presentation, and treatment.
Abducens Nerve ; Cavernous Sinus ; Diagnosis ; Diagnosis, Differential ; Headache* ; Humans ; Inflammation ; Ophthalmoplegia ; Tolosa-Hunt Syndrome*

To diagnosis Tolosa-Hunt syndrome (TSH) used to be difficult because some other diseases like tumors, sarcoids, vasculitises or other inflammatory masses simulate the clinical manifestations, including steroid responsiveness and radiologic findings. According to the international classification of headache disorders 2nd edition, the diagnosis of THS requires demonstration of granuloma by MRI or biopsy. We performed MRI 4 times during 6 months in a patient who seemed to have THS. Initial MRI was normal but the second MRI showed granuloma which decreased gradually in size with steroid treatment.
Biopsy ; Classification ; Diagnosis ; Granuloma ; Headache Disorders ; Humans ; Magnetic Resonance Imaging* ; Tolosa-Hunt Syndrome* ; Vasculitis

To diagnosis Tolosa-Hunt syndrome (TSH) used to be difficult because some other diseases like tumors, sarcoids, vasculitises or other inflammatory masses simulate the clinical manifestations, including steroid responsiveness and radiologic findings. According to the international classification of headache disorders 2nd edition, the diagnosis of THS requires demonstration of granuloma by MRI or biopsy. We performed MRI 4 times during 6 months in a patient who seemed to have THS. Initial MRI was normal but the second MRI showed granuloma which decreased gradually in size with steroid treatment.
Biopsy ; Classification ; Diagnosis ; Granuloma ; Headache Disorders ; Humans ; Magnetic Resonance Imaging* ; Tolosa-Hunt Syndrome* ; Vasculitis

Although isolated cranial nerve palsies are common in diabetic patients, multiple, simultaneous cranial neuropathies are rare. We describe the second case of a complete superior orbital fissure syndrome including the optic nerve in a middle-aged Papuan man with newly diagnosed diabetes mellitus. The differential diagnosis included septic cavernous sinus thrombosis and Tolosa Hunt syndrome, and management was initially directed at excluding these serious, treatable conditions.
Cavernous Sinus Thrombosis - diagnosis ; Diabetes Mellitus, Type 2 - complications ; Diabetes Mellitus, Type 2 - diagnosis ; New Guinea ; Oculomotor Nerve Diseases - diagnosis ; Oculomotor Nerve Diseases - etiology ; Tolosa-Hunt Syndrome - diagnosis

Tolosa-Hunt syndrome is a rare disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. It is characterized by ophthalmoplegia, unilateral headache or periorbital pain. Brain magnetic resonance imaging (MRI) usually shows an inflammation of cavernous sinus. It is known that steroid pulse therapy is effective. In this case, we diagnosed a 12-year-old female with Tolosa-Hunt syndrome suffered from left ophthalmoplegia and left sided headache. Her Brain MRI showed mild enlargement of left cavernous sinus. After methylprednisolone pulse therapy and following dexamethasone treatment, her symptoms were markedly improved. In children who suffer from periorbiral pain and ophthalmoplegia, proper brain MRI could be helpful in differential diagnosis including Tolosa-Hunt syndrome.
Brain ; Cavernous Sinus ; Child* ; Dexamethasone ; Diagnosis, Differential ; Eye Pain ; Female ; Headache ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Methylprednisolone ; Ophthalmoplegia ; Orbit ; Rare Diseases ; Tolosa-Hunt Syndrome*

Cavernous sinus thrombophlebitis is characterized by edema of the eyelids and the conjunctivae, and paralysis of the cranial nerves. It is rare, often misdiagnosed or underestimated by neurosurgeons, and is generally related with high rates of mortality and morbidity. High rate of suspicion is required for early diagnosis, and aggressive managements can lead to better outcomes. With recent advancements in antibiotic therapies, the mortality rate has been decreased to less than 30%. However, the morbidity remains high and complete recovery is rare. A 69-year-old woman presented with headache and right ocular pain with sixth cranial nerve palsy for one week. Under the suspicion of Tolosa-Hunt syndrome, steroid treatment was done. On discharge, there was improvement in right ocular pain, but the cranial nerve palsy remained. Subsequently, the patient returned with left ocular pain, lid swelling, redness, ecchymosis of conjunctiva, and ptosis. Enhanced brain magnetic resonance imaging revealed an asymmetrically enlarged left cavernous sinus with heterogeneously enhancing contour. Intravenous antibiotics and oral steroids along with anticoagulant medications were administered.
Abducens Nerve Diseases ; Aged ; Anti-Bacterial Agents ; Brain ; Cavernous Sinus ; Cavernous Sinus Thrombosis ; Caves ; Conjunctiva ; Cranial Nerve Diseases ; Cranial Nerves ; Early Diagnosis ; Ecchymosis ; Edema ; Exophthalmos ; Eyelids ; Female ; Headache ; Humans ; Magnetic Resonance Imaging ; Paralysis ; Steroids ; Tolosa-Hunt Syndrome

PURPOSE: To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome. CASE SUMMARY: A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months. CONCLUSIONS: Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.
Adrenal Cortex Hormones ; Angiography ; Blepharoptosis ; Cavernous Sinus* ; Central Nervous System Vascular Malformations* ; Cerebral Angiography ; Diagnosis ; Diplopia ; Embolization, Therapeutic ; Exophthalmos ; Exotropia ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Inflammation ; Magnetic Resonance Imaging ; Middle Aged ; Oculomotor Nerve Diseases ; Orbit ; Pupil ; Recurrence ; Strabismus ; Tolosa-Hunt Syndrome*