Clinical Characteristics of Tolosa-Hunt Syndrome
Abstract & Commentary
By Joseph E. Safdieh, MD Assistant Professor of Neurology, Weill Medical College, Cornell University Dr. Safdieh reported that he received grant/research support from the American Academy of Neurology.
Synopsis: Tolosa-Hunt syndrome can be diagnosed only after elimination of all other causes for unilateral painful ophthalmoparesis.
Source: Colnaghi S, Versino M, Marchioni, et al. ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. Caphalalgia 2008;28:577-584.
Tolosa-Hunt syndrome (THS) is a rare condition that causes unilateral painful ophthalmoparesis and was initially described in 1976 by Drs. Tolosa and Hunt. The etiology of the syndrome is unknown, and is pathologically characterized by a granulomatous inflammation of the cavernous sinus. The diagnostic symptoms were most recently published in the ICHD-2.1 One of the required clinical features listed in the ICHD-2 is that "pain and paresis resolve within 72 hours if treated with adequate doses of steroids." There are no controlled trials in this rare condition, and this diagnostic criterion was based on case series and expert consensus.
In this retrospective study, the authors performed a comprehensive literature review over the years 1999 to 2007 and identified 62 cases of Tolosa-Hunt syndrome. The clinical characteristics were reviewed and compared to the published diagnostic criteria. Of note, in all but one patient, the symptom of pain, but not the diplopia, resolved within 72 hours after steroid treatment. Mean duration of steroid treatment was 2 months, with a range of several days to several years. No statistically significant difference in rate of recovery was detected in patients who received standard-dose prednisone (1 mg/kg/day) versus initiation with high-dose IV methylprednisolone, but there was a trend favoring the high-dose group. There were fewer recurrences in the high-dose group compared to the standard-dose group (44% versus 9% recurrence rate). A small number of patients had other cranial nerve involvement, specifically V and VII.
MRI demonstrated cavernous sinus abnormalities in the majority of patients, but three patients had a normal MRI and 16 patients demonstrated extension of enhancement into other intracranial structures (termed THS-plus in this study). No statistically significant differences in response to treatment were detected in patients with THS-plus compared to THS.
Based on the findings of the study, the authors suggest revising the diagnostic criteria of THS to exclude the requirement for ophthalmoparesis to resolve within 72 hours of steroid treatment.
Commentary
For the clinical neurologist, Tolosa-Hunt syndrome should remain a diagnosis of exclusion. Painful ophthalmoparesis has a number of potential underlying causes, many of which are quite serious. These include, but are not limited to, neoplasms such as lymphoma and meningioma, infections such as mucormycosis and basal meningitis, cavernous sinus thrombosis, and diabetes. Therefore, a complete evaluation, tailored to the patient, must be completed before a diagnosis of THS is entertained. MRI with gadolinium and fine coronal T2 and axial fat-saturated T1 cuts are recommended in every case. CSF examination is certainly appropriate in most cases unless another clear cause is identified. Additionally, a number of conditions listed above are steroid responsive, so steroid responsiveness is not unique to THS. THS should be suspected when there is compatible neuroimaging and no other cause is found. These patients must be followed over time to ensure that the diagnosis of THS is indeed correct and no other sinister cause surfaces.
Reference
1. Headache Classification Committee of the International Headache Society. Cephalalgia 1988;8(Suppl 7):1-96.
Tolosa-Hunt syndrome can be diagnosed only after elimination of all other causes for unilateral painful ophthalmoparesis.You have reached your article limit for the month. Subscribe now to access this article plus other member-only content.
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