Paediatric Tolosa‐Hunt syndrome: the need for treatment guidelines and renewed criteria

Abstract

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 The steroids were gradually reduced and totally withdrawn within 2 weeks.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...

Evaluation and Management of Tolosa–Hunt Syndrome in Children: A Clinical Update

“…Of all identified cases, only 15 qualified for a diagnosis of THS according to ICHD-3 beta (Table 1) and were included in the study [1,8,[19][20][21][22][23][24][25][26][27][28][29][30]. year-old patient, who also met these criteria, was included in the final analysis.…”

“…Smith and Taxdal later coined the term "Tolosa-Hunt syndrome" in 1966 [16]. With an estimated yearly incidence of one case per million in the United States [6,17] and a mean age of onset of 38-41 ± 14-16 years [3,13], THS is extremely rare in children [8,18,19]. The rarity of this syndrome contributes to the controversy that surrounds diagnostic approach and treatment strategies.…”

Recurrent Tolosa-Hunt syndrome in a child : A Case Report

“…THS is a rare disease, especially in the pediatric population [5][6][7] . A few case reports of pediatric THS in Korea were made after the introduction of the International Headache Society (IHS) criteria in 2004 [8][9][10][11] .…”

“…There is also a recent report which showed the recurrence of adult THS to be higher in younger patients 13) . However, there are only a few reports of the recurrence of pediatric THS 7,14) . This case illustrated a relatively rare recurrent case in a pediatric patient, which recurred within a year and showed good response to oral steroid treatment during both episodes.…”

Utility of Thallium-201 Scintigraphy in Tolosa-Hunt Syndrome

“…In this patient, the conventional diagnostic criteria did not fit in terms of a longer period from starting prednisolone treatment to remission of the ophthalmoplegia (2 weeks), compared to the diagnostic criteria (within 72 hours). This might reflect the differences in the pathophysiological profile of THS in pediatric and adult cases, and the profile of the former remains to be clarified (Pienczk-Reclawowicz et al 2010). It might also be due to the undefined optimal dose of prednisolone, which was described as "adequate steroid therapy" in the diagnostic criteria (PienczkReclawowicz et al 2010).…”