Reappraising the Tolosa‐Hunt Syndrome Diagnostic Criteria: A Case Series

Abstract

IntroductionTolosa‐Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses.MethodsWe extracted all patient charts from our institution’s electronic medical record associated with ICD‐9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD‐3 criteria for THS and compared our findings with their final diagnoses.ResultsOf 62 patients associated with ICD‐9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS‐like symptoms was 58 years. Five of the 10 (50%) met ICHD‐3 criteria for THS. Two of the 5 (40%) meeting ICHD‐3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD‐3 criteria for THS nevertheless received a final diagnosis of THS.DiscussionOur false‐negative rate of 40% is consistent with previous case series found in the literature. Our false‐positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false‐positive. The false‐positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment.ConclusionOur case series suggests that ICHD‐3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow‐up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD‐3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.

Tolosa–Hunt syndrome: A review of diagnostic criteria and unresolved issues

“… Presence of additional systemic features 26 44 45 46 47 48 49 50 51 Other neurological deficits 52 53 54 Evidence of immune suppression or previous malignancy 55 56 57 58 59 60 61 62 63 Atypical features on MRI including nonenhancement on contrast 45 51 53 63 64 65 Involvement of mandibular or maxillary division of trigeminal (THS is primarily an inflammation of anterior CS) 28 44 Failure of resolution clinically/on MRI 26 28 46 53 66 Severe vision loss. 26 …”

The Value of Enhanced Three-dimensional Brain volume magnetic Resonance Imaging in tolosa-hunt Syndrome: A Clinical Retrospective Study

“…They reported 10 patients who were concerning as THS, but with high false-negative (40-57%) due to unremarkable nonenhanced MRI. [11] In our study, nearly 86% (19/21) were negative on their non-enhanced MRI. It seemed insu cient to show granulomatous in ammation on non-enhanced MRI.…”

“…The application of the criteria-3 produced more often false-positive and false-negative diagnoses. [11] Conclusion:…”

Cavernous sinus thrombosis: An insidious and dangerous “do‐not‐miss” diagnosis

“…However, systemic symptoms, such as fever and altered consciousness, and CSF abnormalities did not support a diagnosis of THS. Furthermore, THS could not be considered as the primary cause responsible for triggering the infection and for the bilateral ophthalmoplegia lesions in this case 13 …”