Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025
Rare association of Tolosa-Hunt Syndrome with Sjogren's Syndrome: case report and review of immunological links across autoimmune disorders".
СтатьяКлинический случайОбзорНазвание и аннотация: Перевод в очереди
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Elshony Hosna, Almuhanna Rakan, Muddassir Rabia
Аннотация
BACKGROUND: Tolosa-Hunt Syndrome (THS) is a rare inflammatory condition characterized by painful ophthalmoplegia, often associated with granulomatous inflammation in the cavernous sinus. While typically idiopathic, THS can sometimes be linked to underlying autoimmune diseases. This report presents the first documented case of THS as the initial manifestation of Sjögren's Syndrome (SS).
CASE PRESENTATION: A 60-year-old woman presented with symptoms of left eye pain, diplopia, and ptosis, leading to a diagnosis of THS after extensive workup and exclusion of other potential causes. MRI findings indicated inflammatory changes in the cavernous sinus, and further autoimmune testing revealed positive ANA and anti-SSA (Ro), leading to a diagnosis of Sjogren's syndrome confirmed by lip biopsy. Following corticosteroid treatment, she experienced prompt symptom relief and was subsequently placed on immunosuppressive therapy for ongoing management.
METHODS AND RESULTS: A literature review of 15 autoimmune-associated THS cases was conducted, revealing that 87% of patients presented with classic symptoms of periorbital pain, ptosis, and cranial nerve palsies, while 73% had cavernous sinus involvement on MRI. Most cases responded well to corticosteroids, with 60% requiring additional immunosuppressive treatment to manage ongoing autoimmune activity.
CONCLUSION: This case highlights the importance of considering autoimmune etiologies in THS, especially in patients with limited response to initial therapies. Pathophysiological links may include immune dysregulation, granulomatous inflammation, and vascular compromise within the cavernous sinus. Early recognition and appropriate management are crucial for optimal outcomes. Further research into the immunological mechanisms linking THS and autoimmune diseases could improve diagnostic accuracy and therapeutic approaches.
CASE PRESENTATION: A 60-year-old woman presented with symptoms of left eye pain, diplopia, and ptosis, leading to a diagnosis of THS after extensive workup and exclusion of other potential causes. MRI findings indicated inflammatory changes in the cavernous sinus, and further autoimmune testing revealed positive ANA and anti-SSA (Ro), leading to a diagnosis of Sjogren's syndrome confirmed by lip biopsy. Following corticosteroid treatment, she experienced prompt symptom relief and was subsequently placed on immunosuppressive therapy for ongoing management.
METHODS AND RESULTS: A literature review of 15 autoimmune-associated THS cases was conducted, revealing that 87% of patients presented with classic symptoms of periorbital pain, ptosis, and cranial nerve palsies, while 73% had cavernous sinus involvement on MRI. Most cases responded well to corticosteroids, with 60% requiring additional immunosuppressive treatment to manage ongoing autoimmune activity.
CONCLUSION: This case highlights the importance of considering autoimmune etiologies in THS, especially in patients with limited response to initial therapies. Pathophysiological links may include immune dysregulation, granulomatous inflammation, and vascular compromise within the cavernous sinus. Early recognition and appropriate management are crucial for optimal outcomes. Further research into the immunological mechanisms linking THS and autoimmune diseases could improve diagnostic accuracy and therapeutic approaches.