Pediatric Tolosa–Hunt Syndrome with Ptosis and Transient Periorbital Headache

An 8-year-old girl with left ptosis and diplopia for 1 day presented with a history of transient left periorbital headache 3 days before her visit. In the left eye, elevation, depression, and adduction movements were impaired ([Fig. 1]) and diplopia was omnidirectional, indicating oculomotor and abducens nerve palsy. Contrast-enhanced magnetic resonance imaging (MRI) revealed an abnormal contrast effect within the left cavernous sinus ([Fig. 2A–D]). Blood tests and cerebrospinal fluid examination showed no abnormalities; infection, tumor markers, and autoantibodies were negative. Based on the International Classification of Headache Disorders (3rd edition, β version) diagnostic criteria for Tolosa–Hunt syndrome (THS),[1] the patient was diagnosed with THS ([Table 1]). Methylprednisolone therapy was administered 7 days after disease onset, causing rapid paralytic improvement. One year after onset, contrast-enhanced MRI revealed a reduced abnormal contrast effect ([Fig. 2E, F]), with no disease recurrence for >3 years.

Fig. 1 Neuro-ophthalmologic examination. A nine-gaze image panel showing left eye elevation and depression deficits and adduction limitations in the right gaze, indicating oculomotor nerve palsy.

Fig. 2 Brain imaging of a child with Tolosa–Hunt syndrome. (A) T1-weighted fluid attenuated inversion recovery axial and (B) T1-weighted coronal images showing a left paracavernous mass (white arrows) not clearly delineated from the cavernous sinus without contrast material. (C) Postgadolinium T1-weighted axial and (D) coronal images showing an abnormal contrast effect within the left cavernous sinus (white arrows). (E, F) Postgadolinium T1-weighted axial and coronal magnetic resonance imaging scans showing the reduction of left-sided cavernous sinus soft tissue (white arrows) 1 year after steroid therapy.

Table 1 The International Classification of Headache Disorders (3rd edition, β version) diagnostic criteria for Tolosa–Hunt syndrome[1]

Description	Unilateral orbital pain associated with paresis of one or more of the IIIrd, IVth, and/or VIth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit
Diagnostic criteria
A	Unilateral headache fulfilling criterion C
B	Both of the following
	1. Granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy
	2. Paresis of one or more of the ipsilateral IIIrd, IVth and/or VIth cranial nerves
C	Evidence of causation demonstrated by both of the following
	1. Headache has preceded paresis of the IIIrd, IVth, and/or VIth nerves by ≤2 wk, or developed with it
	2. headache is localized around the ipsilateral brow and eye
D	Not better accounted for by another ICHD-3 diagnosis

Abbreviations: ICHD-3, International Classification of Headache Disorders (3rd edition); MRI, magnetic resonance imaging.

THS is a painful ophthalmoplegia caused by nonspecific granulomatous inflammatory lesions of the cavernous sinus.[1] [2] Its estimated incidence is 1 patient per 1 million adults,[3] and it is even rarer in children.[2] [4] Immediate contrast-enhanced MRI targeting the cavernous sinus should be considered in cases with unilateral periorbital headache, even if transient and ipsilateral cranial nerve attacks.

Author Contribution

M.A., M.I., K.T., E.F., and Y.I. managed the patient. M.A., M.O., and Y.I substantially contributed to the drafting of the manuscript. All authors critically reviewed and revised the manuscript draft and approved the final version for submission.

Note

This work was performed at Showa General Hospital, Tokyo, Japan.

Publication History

Received: 05 December 2023

Accepted: 26 February 2024

Article published online:
28 March 2024

• References

• 1 Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders. 3rd ed (beta version). Cephalalgia; 2013. ; 33:629–808
• 2 Pérez CA, Evangelista M. Evaluation and management of Tolosa-Hunt syndrome in children: a clinical update. Pediatr Neurol 2016; 62: 18-26
• 3 Iaconetta G, Stella L, Esposito M, Cappabianca P. Tolosa-Hunt syndrome extending in the cerebello-pontine angle. Cephalalgia 2005; 25: 746-750
• 4 Tsirigotaki M, Ntoulios G, Lioumpas M, Voutoufianakis S, Vorgia P. Tolosa-Hunt syndrome: clinical manifestations in children. Pediatr Neurol 2019; 99: 60-63