Tolosa-Hunt Syndrome Associated with Hypophysitis and Transient Hypopituitarism: A Case Report

Abstract

Introduction: Tolosa-Hunt syndrome is a rare disease related to autoimmune granulomatous inflammation of the cavernous sinuses, which is unusually associated with a pituitary involvement. Clinical Case: Herein we describe a 36-year-old man, who presented with left painful ophthalmoplegia and eyelid ptosis. Magnetic resonance imaging (MRI) demonstrated infiltration of both cavernous sinuses and enlargement of the pituitary gland without changes in the intraorbital structures. Laboratory tests established central hypothyroidism and secondary hypogonadism, together with negative thyroid antibodies. High-dose intravenous methylprednisolone (MPS) pulse therapy (500 mg daily for 3 consecutive days) was initiated, followed by oral MPS, reaching a cumulative dose of 5.5 g over 2 months. This treatment resulted in significant amelioration of the ocular signs and symptoms. The follow-up MRI showed normalization of the pituitary size concomitantly with a resolvement of hypopituitarism. Nevertheless, the infiltration of the left cavernous sinus and orbital pain persisted, requiring continued corticosteroid therapy with intramuscular MPS (30 mg/24h) for six additional months. Adverse effects subsequently occurred, including steroid-induced diabetes, and weight gain more than 10 kg. For these reasons, a single infusion of Infliximab 300 mg (4 mg/kg) was administered, which resulted in successful control of all symptoms, alongside a dramatic disappearance of pain and diplopia without any side effects. This allowed for the discontinuation of corticosteroid therapy followed by beneficial effects on glucose metabolism and body weight.  Conclusion: The successful management of this case with Infliximab, a TNF-alpha antibody that targets proinflammatory cytokines, supports the concept of the autoimmunne pathogenesis of Tolosa-Hunt syndrome, which presented in combination with auto-immune hypophysitis in the described case.