Painful Opthalmoplegia

1. Ade Wijaya, MD – June 2019
Tolosa Hunt Syndrome

2. Introduction
 A rare idiopathic disease
 First described by Tolosa (1954) and Hunt (1961)
Gameiro Filho, A. R., Faccenda, P. G., Estacia, C. T., Correa, B. S., & Curi, I. (2018). Tolosa-Hunt Syndrome. Revista Brasileira de Oftalmologia, 77(5), 289-291.
Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry. 1954;17(4):300-2.
Hunt WE, Meagher JN, Lefever HE, Zeman W. Painful opthalmoplegia. Its relation to indolent inflammation of the carvernous sinus. Neurology. 1961;11(1):56-62.

3. Diagnosis
 One or more episodes of unilateral orbital pain
persisting for weeks if untreated;
 Paresis of one or more of the third, fourth, and/or
sixth cranial nerves and/or demonstration of
granuloma in MRI or biopsy;
 Paresis coincides with the onset of pain or follows
it within 2 weeks;
 Pain and paresis resolve within 72 hours when
treat adequately with steroids;
 Other causes have been excluded with
appropriate evaluation.
La Mantia L, Curone M, Rapoport AM, Bussone G; International Headache Society. Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria. Cephalalgia.
2006;26(7):772-81

4. Differential Diagnosis
Trauma Vascular
Neoplasm Others
Gameiro Filho, A. R., Faccenda, P. G., Estacia, C. T., Correa, B. S., & Curi, I. (2018). Tolosa-Hunt Syndrome. Revista Brasileira de Oftalmologia, 77(5), 289-291.

5. Differential Diagnosis
VASCULAR
 Intracavernous carotid artery aneurysm
 Posterior cerebral artery aneurysm
 Carotid-cavernous fystula
 Carotid-cavernous trombosis
Gameiro Filho, A. R., Faccenda, P. G., Estacia, C. T., Correa, B. S., & Curi, I. (2018). Tolosa-Hunt Syndrome. Revista Brasileira de Oftalmologia, 77(5), 289-291.

6. Differential Diagnosis
NEOPLASM
• Primary intracranial tumor;
• Pituitary adenoma;
• Meningioma;
• Craniopharyngioma;
• Sarcoma;
• Neurofibroma;
• Gasserian ganglion neuroma;
• Neurofibroma
• Lymphoma
• Leukemia
• Primary cranial tumor: chordoma, chondroma, giant cell tumor
• Metastasis: Nasopharyngeal tumor Cylindroma Adamantinoma
Squamous cell carcinoma, myeloma
Gameiro Filho, A. R., Faccenda, P. G., Estacia, C. T., Correa, B. S., & Curi, I. (2018). Tolosa-Hunt Syndrome. Revista Brasileira de Oftalmologia, 77(5), 289-291.

7. Differential Diagnosis
OTHERS:
Bacterial Sinusitis, mucocele, periostitis
Viral: Herpes zoster
Fungal: Mucormycosis
Spirochetal: syphilis
Mycobacterial: tuberculosis
Sarcoidosis
Wegener's granulomatosis
Eosinophilic granuloma
Diabetic ophthalmoplegia
Giant cell arteritis
Ophthalmoplegic migraine
Gameiro Filho, A. R., Faccenda, P. G., Estacia, C. T., Correa, B. S., & Curi, I. (2018). Tolosa-Hunt Syndrome. Revista Brasileira de Oftalmologia, 77(5), 289-291.

8. Treatment
 Oral Prednisone 1 mg/kg/day
 Azathioprine and Methotrexate can be beneficial
if no response to steroid
Monzillo PH, Saab VM, Protti GG, Costa AR, Sanvito WL. Síndrome de Tolosa-Hunt: análise de seis casos. Arq Neuropsiquiatr. 2005;63(3a):648-51

9. Summary
 A rare Idiopathic disease
 Orbital pain
 Unilateral ophtlmoplegia
 Granulomatous inflammatory process of
cavernous sinus
 Dramatic response to steroids

10. THANK YOU