Tolosa-Hunt Syndrome: Appraising the ICHD-3 beta diagnostic criteria

Abstract

Introduction The term Tolosa-Hunt Syndrome was first used more than half a century ago to describe painful ophthalmoplegia accompanied by cranial nerve palsies. In the decades since, its diagnostic criteria have evolved considerably. The beta version of the 3rd Edition of the International Classification of Headache Disorders narrows these criteria to require the demonstration of granulomatous inflammation on MRI or biopsy. We believe this may introduce challenges to accurate diagnosis. Discussion Requiring the demonstration of granulomatous inflammation for a diagnosis of Tolosa-Hunt Syndrome may introduce the potential for false negative and false positive diagnoses. Although the disorder presents secondary to granulomatous inflammation, MRI technology may not be able to identify it reliably, and biopsy is not always indicated for its symptomatology. Additionally, several cases have been reported of Tolosa-Hunt Syndrome diagnosed with MRI-confirmed granulomatous inflammation that later prove to be attributable to other pathologies. The emphasis on neuroimaging may therefore exclude some true Tolosa-Hunt Syndrome cases and include others resulting from other latent pathologies that are not visible on MRI. Conclusion We wish to offer several potential modifications to the International Classification of Headache Disorders guidelines for Tolosa-Hunt Syndrome, including making the demonstration of granulomatous inflammation on MRI or biopsy non-mandatory and lengthening patient follow-up to two years for cases in which MRI is unrevealing.

Orbital Pain, Ophthalmoplegia, and Oligoclonal Bands in the Cerebrospinal Fluid: A Case Report of Tolosa‐Hunt Syndrome

“…In contrast, our patient fulfilled the diagnostic criteria, had a typical history, radiological findings, and treatment response . The pain recurred initially, but resolved quickly when given adequately dosage and duration of steroid treatment.…”

“…A previous report described a 63‐year‐old man with a clinically THS with MRI granulomatous findings and oligoclonal bands in CSF. However, after years with recurrent pain and investigations this patient was rediagnosed with cryopyrin‐associated periodic fever syndrome …”

“…No symptoms were reported at the last telephone follow‐up more than 3 years after the first hospitalization. THS is a diagnosis of exclusion and other causes of a painful ophthalmoplegia such as tumors, lymphoma, vasculitis, (pachy)meningitis, Ig4‐related diseases, carotid artery dissection, sarcoidosis, Eales disease, cryopyrin‐associated periodic fever syndrome and diabetes mellitus induced mononeuritis have been described in the literature . Thus, all patients with suspected THS should have a broad evaluation including contrast‐enhanced MRI.…”

“…THS is a diagnosis of exclusion and other causes of a painful ophthalmoplegia such as tumors, lymphoma, vasculitis, (pachy)meningitis, Ig4-related diseases, carotid artery dissection, sarcoidosis, Eales disease, cryopyrinassociated periodic fever syndrome and diabetes mellitus induced mononeuritis have been described in the literature. [1][2][3][4] Thus, all patients with suspected THS should have a broad evaluation including contrast-enhanced MRI. However, the introduction of the mandatory positive MRI finding as a diagnostic criteria is disputed since case series indicate that 20-50% of all THS have a negative MRI, and it has been suggested that granulomatous inflammation may be present even in the absence of a positive MRI.…”

“…2,4 However, how the granulomatous inflammation occurs is unknown. 1,2,4 Moreover, as THS is a rare condition we have to rely on case reports/series to fill the knowledge gap. CSF analyses have usually not been conducted in the diagnostic workup of THS, and previous reviews have concluded that the expected findings in THS are normal and if not, other alternative diagnoses are more likely.…”