Advertisement

Woman with headache and retro-orbital pain

      1. Presentation

      A 54-year-old woman, presented to the Emergency Departement (ED) with a 10-days unilateral headache and progressive pain behind the left eye. The patient had normal vital parameters and no medical history. At admission in the ED, neurological examination showed a left-sided abduction deficit (consistent with a sixth nerve palsy). The rest of the clinical examination was normal. Head CT showed no evidence of infarction, hemorrhage or mass. A lumbar punction was performed with normal opening pressure and no white blood cells. The patient also underwent a cerebral MRI scan (Fig. 1).
      Fig. 1
      Fig. 1A) MRI, axial fat-suppressed T2. Ill-defined tissue formation in the left orbit with infiltration of intra- and extraconal fat, of the extraocular muscles and optic nerve. Swelling of the medial and lateral rectus muscles (white arrows). B) MRI, axial fat-suppressed and contrast-enhanced T1. Prominent post-gadolinium enhancement of the tissue formation reaching the orbital apex (white arrow).

      2. Diagnosis: Tolosa-Hunt Syndrome

      International Headache Society defines Tolosa-Hunt Syndrome (THS) as a unilateral and episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, supraorbital fissures or in the orbit [
      • Headache Classification Committee of the International Headache Society (IHS)
      The international classification of headache disorders, 3rd edition (beta version).
      ]. THS is a rare condition and its etiology remains unknown. On histopathology, there is a nonspecific inflammation (lymphocyte and plasma cell infiltration, giant cell granulomas) explaining the clinical features. Two main subtypes of THS syndrome should be differentiated: idiopathic/benign (with normal imaging) and inflammatory (i.e lesions in MRI and/or biopsy) but clinical symptoms of these two subtypes are very similar [
      • Hung C.-H.
      • Chang K.-H.
      • Wu Y.-M.
      • et al.
      A comparison of benign and inflammatory manifestations of Tolosa-Hunt syndrome.
      ]. The ipsilateral headache usually precedes the paresis, THS symptomatology remains mostly unilateral and recurrences are very rare. The duration of a THS episode lies between three days and eight months (on average 50 days). MRI scan reveals inflammation in only half of cases and should be interpreted with caution due to numbers of false postive diagnosis [
      • Mullen E.
      • Green M.
      • Hersh E.
      • et al.
      Tolosa-Hunt Syndrome: appraising the ICHD-3 beta diagnostic criteria.
      ]. Thus, differential diagnosis of THS is broad including other orbital inflammatory syndromes, lymphoma, vasculitis, diabetes mellitus and autoimmune disease (sarcoidosis). Definitive diagnosis via biopsy is only reserved for severe cases (high risk procedure). Treatment consists of high dose corticosteroids, noticeable improvements are usually seen within the first 72 h of treatment. Failure to respond to steroids suggests an alternative diagnosis. Our patient underwent a month-long oral prednisone treatment and the follow-up MRI at one month showed a complete resolution of the previously described imaging lesions and symptoms.

      Conflict of interest

      There are no relevant conflicts of interest for any of the authors listed.

      Authorship

      Both authors had access to the data and played a role in writing this manuscript. Both authors approved the final manuscript.

      Funding

      None.

      References

        • Headache Classification Committee of the International Headache Society (IHS)
        The international classification of headache disorders, 3rd edition (beta version).
        Cephalalgia. 2013; 33: 629-808
        • Hung C.-H.
        • Chang K.-H.
        • Wu Y.-M.
        • et al.
        A comparison of benign and inflammatory manifestations of Tolosa-Hunt syndrome.
        Cephalalgia. 2013; 33: 842-852
        • Mullen E.
        • Green M.
        • Hersh E.
        • et al.
        Tolosa-Hunt Syndrome: appraising the ICHD-3 beta diagnostic criteria.
        Cephalalgia. 2017; (333102417745271)https://doi.org/10.1177/0333102417745271