Year: 2026
Type: article
Study design: Case Report
Abstract: Tolosa-Hunt syndrome is a clinical quadrad consisting of unilateral headache, ipsilateral cranial nerve palsy, granulomatous inflammation on imaging or biopsy and no other headache syndrome to explain the diagnosis. It may be secondary to systemic inflammatory disease, and clinicians should aggressively search for underlying causative pathology. A 73-year-old patient presented with this quadrad, in whom workup identified a systemic large-vessel vasculitis secondary to Sjögren's disease. The patient was treated with rituximab and steroids, with considerable improvements in both her orbital pathology and vasculitis. The quadrad of Tolosa-Hunt syndrome should prompt investigation for systemic autoimmune and inflammatory disease rather than being assumed to be idiopathic. LEARNING POINTS: 'Tolosa-Hunt syndrome' is a descriptive term for an idiopathic presentation; it does not establish aetiology. Secondary causes should be considered and thoroughly worked up.The workup should extend beyond local orbital pathology to systemic autoimmune and inflammatory disorders, including potentially serious vasculitis.Sjögren's-associated vasculitis may produce neurological manifestations resembling polyarteritis nodosa; rheumatology input and treatment of the underlying vasculitis may improve the neurological syndrome.
DOI: 10.12890/2026_007497
Institution: Mater Dei Hospital
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $323
OpenAlex ID: W7222394896