Painful ophthalmoplegia: an unresolved clinical problem (original) (raw)
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Tolosa-Hunt syndrome - diagnostic problem of painful ophthalmoplegia
Background. Tolosa-Hunt syndrome (THS) is an uncommon disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. The disease is characterized by periorbital pain, paresis of the bulbomotor and quick response to steroid treatment. The orbital process may lead to optic nerve atrophy. According to the International Headache Society Classification of 2004, the diagnostic protocol includes magnetic resonance imaging (MRI) and biopsy. Case reports. We presented 46-year old male patient, with THS. The patient had unilateral periorbital pain, inflammatory process in the cavernous sinus, the apex of the orbit and the paranasal sinuses. Inflammatory process had spread into the fascia of the bulbomotor and performed compression to the optic nerve, causing paresis of the bulbomotor, protrusion of the eyeball and atrophy of the optic nerve. Pulse doses of corticosteroids were effective. Regarding the presented patient, diagnostic dilemmas arose from nonspecific sinusitis. The initial ophthalmological diagnosis, based on periorbital pain, drop in visual acuity and the narrow chamber angle was angular glaucoma, which resulted in a delayed diagnosis of THS and the beginning of the treatment. MRI and positive response to the treatment with corticosteroids were relevant for making the diagnosis. Conclusion. According to the International Headache Society Classification of 2004, THS is an entity that occurs rarely, its etiopathogenesis is unknown, it is manifested clinically by unilateral orbital pain associated with simple or multiple oculomotor paralyses, which resolves spontaneously but may recur. MRI orbital phlebography and biopsy are the recommended methods for making diagniosis. In our patient MRI findings and positive response to the corticosteroide treatment were relevant for making the diagnosis.
Cephalalgia, 2014
Background: Three editions of International Classification of Headache Disorders (ICHD) diagnostic criteria for Tolosa-Hunt syndrome (THS) have been published in 1998, 2004 and 2013; in ICHD-3 beta, there have been considerable changes. The validity of these new diagnostic criteria remains to be established. Methods: We retrospectively identified 77 patients with nontraumatic painful ophthalmoplegia (PO) admitted between 2003 and 2013. We reviewed patients' age at onset and gender, time courses between onset of pain and development of cranial nerve palsy, the cranial nerves involved, imaging findings, therapeutic efficacy of steroid treatment and recurrence of attacks. Results: THS was the most frequent type of PO (46/77). In THS patients, the third cranial nerve was most commonly involved (76.3%). The median time interval between pain and cranial nerve palsy was two days, although in five patients (10.9%) the interval ranged from 16 to 30 days. Definitely abnormal MRI findings were found in 24 patients (52.2%). Conclusions: It is essential to rule out other causes of PO in diagnosing THS, with MRI playing a crucial role in differential diagnosis. It may be helpful to understand and master the entity of THS for researchers and clinicians to adjust the gradation and ranking of the diagnostic criteria.
Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia
European Journal of Radiology, 2009
Purpose: (a) To assess MR features in patients with Tolosa-Hunt syndrome (THS) and to (b) correlate MR findings with criteria derived from previously reported pathologic observations. Methods: Fifteen patients with twenty episodes of painful ophthalmoplegia prospectively selected according to International Headache Society (IHS) standards underwent MR examinations focused on the cavernous sinus. Initial examinations in 20 and follow-up MR images in 17 episodes were retrospectively reviewed by 3 independent observers. Results: The primary criteria: an enhancing soft tissue lesion within the cavernous sinus, increase in size and lateral bulging of the anterior cavernous sinus contour were consistently present in 15 initial episodes and in 5 recurrences (20/20). Agreement among observers was 100%. The secondary criteria: internal carotid artery narrowing in 7 patients, extension towards the superior orbital fissure in 13 and orbital apex involvement in 8 patients were unanimously agreed upon in 87.5%, 86.6% and 80%. Complete resolution of findings was observed on follow-up studies. Conclusion: In patients with THS the MR features conform to previously reported pathologic findings. MR features are evocative of THS when an increase in size and bulging of the dural contour of the anterior CS supplemented by carotid artery involvement and extension towards the orbit are present. Resolution of findings within 6 months is required to support the diagnosis.
Cases Journal, 2009
Introduction: Painful ophthalmoplegia refers to periorbital or hemicraneal pain plus ipsilateral ocular motor nerve palsies with or without oculo-sympathetic paralysis, sensory loss in the distribution of V1 and V2 can co-occur. There are many etiologies of painful ophthalmoplegia. Tolosa-Hunt syndrome is a steroid-responsive painful ophthalmoplegia secondary to idiopatic granulomatous inflammation of the cavernous sinus or orbital apex. THS is a diagnosis of exclusion and treatment should be with high dose steroid.
Painful Ophthalmoplegia of the Right Eye in a 20-Year-Old Man
The Journal of Emergency Medicine, 2013
Background: Painful ophthalmoplegia is an infrequent but very important presentation in the acute care setting and requires a thorough neurologic evaluation to determine its cause. This clinical sign has a large differential and generally indicates a serious cerebrovascular process. Case Report: We describe a patient with a subacute presentation of painful ophthalmoplegia, ultimately diagnosed as Tolosa-Hunt syndrome, a rare inflammatory process of the cavernous sinus. Conclusion: This case highlights the importance for the Emergency Physician to quickly recognize painful ophthalmoplegia and rapidly elucidate the cause to prevent further complications for the patient.
IRJPMS, 2022
Introduction: Tolosa-Hunt syndrome (THS) is a rare, painful ophthalmoplegia associated with paresis of one or more of the third, fourth and/or sixth cranial nerves that in some uncommon cases dysfunction of the optic, trigeminal, facial, acoustic nerves and sympathetic innervation of the pupil can be seen. THS is a diagnosis of exclusion and treatment should be with high dose steroid. Case presentation: We describe the case of a 55-year-old Egyptian male that was admitted to our hospital for painful ophthalmoplegia of the right eye. After the diagnostic work-up, we concluded that the patient had an inflammatory form of Tolosa-Hunt syndrome. We initiated treatment with IV steroids and showed great response. Discussion: The etiologies of headache in association with third cranial nerve palsy-pupillary involvement are: Tolosa-Hunt syndrome (THS), recurrent painful ophthalmoplegic neuropathy (RPON) formerly named ophthalmoplegic migraine (OM), posterior communicating artery aneurysms (PCAs), pituitary apoplexy. Conclusion: Finally, we concluded that the patient had Tolosa-Hunt syndrome because he completely fulfilled HIS 2018 diagnostic criteria, and the treatment with steroids at a dose of 1 mg/kg/day tapered slowly and has been well received. I.
Painful ophthalmoplegia in a 17 months old child
A 17-month-old girl started to present recurrent episodes of headache and ophthalmoplegia. The vision of the left eye was severely impaired at the age of 39 months when she was diagnosed to have Tolosa-Hunt syndrome. Acute attacks were aborted by steroids during 18 months. The surgical correction of the residual eye deviation was followed one month later by the relapse of the disease. The particular aspect of this syndrome in childhood are discussed.