Tolosa Hunt syndrome, case report

Abstract

Introduction: Tolosa Hunt syndrome (THS) is a rare condition, incidence of 1/1.000.000 case per year, characterized by unilateral painful ophthalmoparesis caused by idiopathic inflammation in the cavernous sinus. The oculomotor nerve is most commonly involved (80%), followed by abducens nerve (70%), ophthalmic branch of trigeminal nerve (30%), trochlear nerve (29%). Case presentation: Male, 77 years old, admitted with an acute moderate-intensity orbitofrontal headache on the left, envolving with palpebral ptosis of the left eye. Neurological examination: complete palpebral ptosis on the left and ophthalmoplegia of the entire ipsilateral extrinsic ocular musculature. A complete investigation was carried out: metabolic, rheumatological, serological tests without significant alterations and study of the cerebrospinal fluid with mild hyperproteinorachia, without pleocytosis. Magnetic resonance imaging (MRI) of the skull showed thickening of the cavernous sinus on the left, with contrast enhancement; Angio-MRI of the Skull and Neck without alterations. Therefore, THS was diagnosed and treatment with Methylprednisolone 1 g for five days, with complete improvement of headache and partial improvement of ophthalmoparesis. The patient was discharged with 60 mg of prednisone orally with instructions for gradual weaning off, return to the neurology outpatient clinic. Discussion: THS diagnosis is based on the International Classification of Headache Disorders: unilateral periorbital headache; granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit on cranial MRI; paralysis of one or more of the oculomotor nerves; the headache must precede the ophthalmoparesis by up to two weeks or appear concomitantly. The exclusion of secondary causes is essential. Treatment of choice is cortico steroids, improvement of headache in the first days, and of ophthalmoparesis in 2–8 weeks. Conclusion: Unilateral headache with ipsilateral ophthalmoparesis should raise the suspicion of THS.