Steroid-responsive painful ophthalmoplegia: Tolosa–Hunt syndrome, Eales disease, or both?

Mendonça, Marcelo; Guedes, Marta; Matias, Gonçalo; Costa, João; Viana-Baptista, Miguel

doi:10.1177/0333102416631282

Cited by 5 publications

(5 citation statements)

References 12 publications

(20 reference statements)

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“…neoplasms) [2]. Mendonca et al reported a case in which an initial diagnosis of THS was later revealed to be Eales disease during the follow-up [14]. Similarly, Brandy-Garcia et al reported a case of a 55-year-old woman presenting THS symptoms, who was diagnosed with sarcoidosis after additional testing [15].…”

Section: Discussionmentioning

confidence: 99%

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Tolosa-Hunt syndrome: a review of diagnostic criteria based on a case series

Chądzyński,

Stopińska,

Domitrz

2024

ppn

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Purpose: Tolosa-Hunt syndrome (THS) is a rare cause of painful ophtalmoplegia with different clinical manifestations. It is described as a unilateral periorbital headache with concomitant dysfunction of at least one out of the III rd , IV th and VI th cranial nerves due to the granulomatous inflammation of periorbital structures, but no underlying cause has been established. Case description:We present six patients referred to the Neurology Department due to a unilateral headache with ipsilateral paresis of at least one cranial nerve responsible for eye movements. The THS diagnostic criteria of the International Headache Disorders Classification (ICHD-3) were applied and analysed. Few patients had atypical clinical manifestations according to these criteria. Comment: Diagnosing THS may prove very challenging. There is a lack of specific markers for the disorder, whereas diagnostic criteria leave a wide area for misdiagnosis. The diagnostic approach should be focused on the exclusion of other pathologies because typical steroid therapy may prove fatal in otherwise benign cases.

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Section: Discussionmentioning

confidence: 99%

“…Mendonca et al . reported a case in which an initial diagnosis of THS was later revealed to be Eales disease during the follow-up [ 14 ]. Similarly, Brandy-Garcia et al .…”

Section: Discussionmentioning

confidence: 99%

Tolosa-Hunt syndrome: a review of diagnostic criteria based on a case series

Chądzyński,

Stopińska,

Domitrz

2024

ppn

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“…A detailed ophthalmological examination should not complemented without fluorescein angiography to exclude Eales' disease which is steroid responsive vasculopathy PO mimicking THS, specifically retinal or optic disc neovascularization, peripheral venous leakage, oedematous macular area, perivasculitis of the retina and non-perfused areas should be examined [57]. Slit-lamp examination for the inspection should be done to preclude the possibilities of sclerokeratitis bilaterally and optic atrophy as manifestation of Takayasu's arteritis (TA) which may mimick THS by 6th and 7th CNs palsies and corticosteroid responsiveness, hence confirmatory CT aortic angiography should be ordered [58].…”

Section: Physical Examintion/signsmentioning

confidence: 99%

Tolosa Hunt Syndrome: Current Diagnostic Challenges and Treatment

Msigwa¹,

Li²,

Cheng³

2020

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Tolosa-Hunt syndrome (THS) is an uncommon diagnosis with an incidence of nearly 1 to 2 cases per million hallmarked by the presence of painful ophthalmoplegia (PO) due to a granulomatous inflammation (GI). Diagnostically, the major THS challenges encountered are owing to the exclusion of other GI presenting conditions necessitating multi-specialization consultations. This article presents uniquely advances in diagnosis and challenges encountered attempting to exclude THS mimics, details on physical examination and laboratory investigations have been incorporated. Tolosa Hunt MRI protocol (contrast-enhanced MRI), restricted diffusion and CISS MRI have lately proved to be precise investigations for THS diagnosis and follow up, on the contrary, number of false-negative/positive MRI diagnoses appears to be rising, hence proposed that MRI or biopsy shouldn't be mandatory criteria for diagnosis as opposed to IHS 2018 guidelines. Despite corticosteroids being the cornerstone therapy for THS, there are controversies concerning the better administration route, optimal dosage, and therapy longevity, through case reports, high dose IV mPSL of 500 mg BID for 3 days, followed with maintenance dose of prednisone 60 mg resulted in the earliest recovery, hereafter standardized guidelines are required. Alternatively, infliximab (300 mg infusion), azathioprine, methotrexate, and acupuncture can be used, farther studies are required to appraise the effectiveness and their safety. On the other side, microsurgery can be utilized for GI evacuation however associated risks of permanent CN VI palsy have been stated, nonetheless, GKRS can be employed when contraindication or corticosteroid intolerability exists though the hazard of late malignancy is a drawback.

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“…Therefore, it has been suggested that Tolosa-Hunt syndrome should be made a diagnosis of exclusion, since the MRI can be normal. Another aspect of the differential diagnosis of Tolosa-Hunt syndrome is that other entities or syndromes exist that might also mimic Tolosa-Hunt syndrome, such as Eales disease (23) and Gradenigo syndrome (24).…”

Section: Diagnostic Considerationsmentioning

confidence: 99%

Facial pain: Overlapping syndromes

Evers

2017

Cephalalgia

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Premise This review summarises the pain syndromes that overlap between headache and facial pain and overlap between pain and cranial nerve lesion. Problem These syndromes share two features in common. First, they show both cranial nerve impairment (e.g. palsy, autonomic dysfunction) and pain; second, they have inflammatory (and/or small vessel) processes as the underlying mechanism. A typical representative of these syndromes is recurrent painful ophthalmoplegic neuropathy, which was previously called ophthalmoplegic migraine and was regarded as a migraine subtype. It is now supposed that this syndrome is caused by an inflammation of one of the ocular motor nerves. Other syndromes discussed in this review are optic neuritis, Tolosa-Hunt syndrome, ischaemic ocular motor nerve palsy, and the very rare Raeder's syndrome. Treatment of all these syndromes is mainly based on steroids. Potential solution Management of possibly underlying disorders such as multiple sclerosis or atherosclerosis should also be considered.

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Steroid-responsive painful ophthalmoplegia: Tolosa–Hunt syndrome, Eales disease, or both?

Cited by 5 publications

References 12 publications

Tolosa-Hunt syndrome: a review of diagnostic criteria based on a case series

Tolosa-Hunt syndrome: a review of diagnostic criteria based on a case series

Tolosa Hunt Syndrome: Current Diagnostic Challenges and Treatment

Facial pain: Overlapping syndromes

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