SIR-Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the paediatric population, characterized by unilateral painful ophthalmoplegia of, typically, a relapsing-remitting course and a substantial response to corticosteroids. The symptoms of THS are not specific and clinical criteria themselves do not guarantee a firm diagnosis. Neuroimaging or biopsy confirming granulomatous inflammation is essential to a diagnosis of THS.Although diagnostic criteria were established by the International Headache Society in 1988, then revised in 2004 (Table I), 1 THS remains a diagnosis of exclusion of other causes of painful ophthalmoplegia, (e.g. neoplasms, aneurysms, cranial neuropathy [diabetes mellitus], opthalmoplegic migraine, intracranial vasculitis, pseudotumor of the orbit, basal meningitis, or cavernous sinus thrombosis).
2Scarcity of data on paediatric THS means that steroid doses as well as the duration of steroid therapy are not clearly specified. In the patient we report, lack of precise therapeutic guidelines resulted in prolonged steroid administration and subsequent severe side effects of the treatment.A 14-year-old female with no previous history of recurrent headaches or any chronic disorder was admitted to the Developmental Neurology Department of the Medical University of Gdansk with left-sided frontal headache and retro-orbital pain of subacute onset that was present for about a month before admission. The symptoms were not preceded by injury, fever, or infection. The symptoms were constant (present also at night), of mild intensity, and unresponsive to intermittent analgesics. As time went by, diplopia appeared and the pain became more intense.On admission, neurological examination revealed paresis of the left VI cranial nerve. The laboratory tests (e.g. blood morphology, C-reactive protein, erythrocyte sedimentation rate, coagulation studies) were normal. Magnetic resonance imaging (MRI) with magnetic resonance angiography (MRA) did not show any abnormalities. Steroid therapy was started with low doses of dexamethasone (about 0.2mg ⁄ kg ⁄ day). The pain diminished within 24 hours and disappeared completely after 3 days. However, it was only after almost a week that diplopia disappeared. The steroids were gradually reduced and totally withdrawn within 2 weeks. After 24 hours of steroid discontinuation the female reported recurrence of severe pain in the same location. Exophthalmos and paresis of the left IV and VI cranial nerves were observed. Repeat MRI and MRA showed narrowing of the carotid artery within the cavernous sinus and post-gadolin enhancement of fatty tissue within the left orbit (Fig. 1a,b), most probably of inflammatory origin. Steroid therapy was restarted (again about 0.2mg ⁄ kg ⁄ day). Pain resolved within 24 hours, other symptoms within 5 days. The patient was discharged with the recommendation of gradual, slow reduction of dexamethasone. After 4 days she was admitted again owing to severe pain in the same location. Exophthalmos and slight paresis of the left III and VI crania...