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Tolosa-Hunt syndrome

MedGen UID:
21197
•Concept ID:
C0040381
•
Disease or Syndrome
Synonyms: Nonspecific inflammation of the cavernous sinus or superior orbital fissure; Ophthalmoplegia, painful
SNOMED CT: Tolosa-Hunt syndrome (95794005)
 
Monarch Initiative: MONDO:0018983
Orphanet: ORPHA64686

Definition

An ophthalmoplegic syndrome, affecting all age groups, with characteristics of acute attacks (lasting a few days to a few weeks) of periorbital pain, ipsilateral ocular motor nerve palsies, ptosis, disordered eye movements and blurred vision usually caused by a non-specific inflammatory process in the cavernous sinus and superior orbital fissure. It has an unpredictable course with spontaneous remission occurring in some and recurrence of attacks in others. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTolosa-Hunt syndrome

Professional guidelines

PubMed

Tolosa-Hunt Syndrome: Appraising the ICHD-3 beta diagnostic criteria.
Mullen E, Green M, Hersh E, Iloreta AM, Bederson J, Shrivastava R
Cephalalgia 2018 Sep;38(10):1696-1700. Epub 2017 Nov 23 doi: 10.1177/0333102417745271. PMID: 29169256
Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria.
La Mantia L, Curone M, Rapoport AM, Bussone G; International Headache Society
Cephalalgia 2006 Jul;26(7):772-81. doi: 10.1111/j.1468-2982.2006.01115.x. PMID: 16776691
The Tolosa-Hunt syndrome: a problem in differential diagnosis.
Hunt WE, Brightman RP
Acta Neurochir Suppl (Wien) 1988;42:248-52. doi: 10.1007/978-3-7091-8975-7_48. PMID: 3055832

Recent clinical studies

Etiology

Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients.
Tayebi A, Samimisedeh P, Jafari Afshar E, Mahmoudnia S, Milan N, Ayati A, Madady A, Rastad H
BMC Neurol 2023 Dec 11;23(1):437. doi: 10.1186/s12883-023-03486-y. PMID: 38082244Free PMC Article
Neuro-Ophthalmological Complications of the COVID-19 Vaccines: A Systematic Review.
Lotan I, Lydston M, Levy M
J Neuroophthalmol 2022 Jun 1;42(2):154-162. Epub 2022 Mar 25 doi: 10.1097/WNO.0000000000001537. PMID: 35427282
Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis.
Hung CH, Chang KH, Chen YL, Wu YM, Lai CL, Chang HS, Lyu RK, Wu YR, Chen CM, Huang CC, Chu CC, Chen CH, Ro LS
Headache 2015 Feb;55(2):252-64. doi: 10.1111/head.12488. PMID: 25688645
Orbital inflammatory disease.
Lutt JR, Lim LL, Phal PM, Rosenbaum JT
Semin Arthritis Rheum 2008 Feb;37(4):207-22. Epub 2007 Sep 4 doi: 10.1016/j.semarthrit.2007.06.003. PMID: 17765951
Dynamic MR imaging in Tolosa-Hunt syndrome.
Haque TL, Miki Y, Kashii S, Yamamoto A, Kanagaki M, Takahashi T, Fushimi Y, Asato R, Murase N, Shibasaki H, Konishi J
Eur J Radiol 2004 Sep;51(3):209-17. doi: 10.1016/j.ejrad.2003.09.016. PMID: 15294327

Diagnosis

Review of Tolosa-Hunt Syndrome, Recent Updates.
Kmeid M, Medrea I
Curr Pain Headache Rep 2023 Dec;27(12):843-849. Epub 2023 Nov 30 doi: 10.1007/s11916-023-01193-4. PMID: 38032539
Is Hypertrophic Pachymeningitis Really Idiopathic?
Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Cavernous sinus lesions.
Munawar K, Nayak G, Fatterpekar GM, Sen C, Zagzag D, Zan E, Hagiwara M
Clin Imaging 2020 Dec;68:71-89. Epub 2020 Jun 18 doi: 10.1016/j.clinimag.2020.06.029. PMID: 32574933
The Eye and Headache.
Friedman DI
Continuum (Minneap Minn) 2015 Aug;21(4 Headache):1109-17. doi: 10.1212/CON.0000000000000204. PMID: 26252594
Tolosa-Hunt syndrome.
Aron-Rosa D, Doyon D, Salamon G, Michotey P
Ann Ophthalmol 1978 Sep;10(9):1161-8. PMID: 736403

Therapy

Recurrence and long-term outcomes of Tolosa-Hunt syndrome.
Kim HJ, Lee SU, Lee ES, Choi JY, Kim JS
J Neurol 2024 Feb;271(2):935-943. Epub 2023 Oct 19 doi: 10.1007/s00415-023-12044-y. PMID: 37853245
Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients.
Tayebi A, Samimisedeh P, Jafari Afshar E, Mahmoudnia S, Milan N, Ayati A, Madady A, Rastad H
BMC Neurol 2023 Dec 11;23(1):437. doi: 10.1186/s12883-023-03486-y. PMID: 38082244Free PMC Article
Neuro-Ophthalmological Complications of the COVID-19 Vaccines: A Systematic Review.
Lotan I, Lydston M, Levy M
J Neuroophthalmol 2022 Jun 1;42(2):154-162. Epub 2022 Mar 25 doi: 10.1097/WNO.0000000000001537. PMID: 35427282
Dynamic MR imaging in Tolosa-Hunt syndrome.
Haque TL, Miki Y, Kashii S, Yamamoto A, Kanagaki M, Takahashi T, Fushimi Y, Asato R, Murase N, Shibasaki H, Konishi J
Eur J Radiol 2004 Sep;51(3):209-17. doi: 10.1016/j.ejrad.2003.09.016. PMID: 15294327
Recurrent Tolosa-Hunt syndrome.
Hannerz J
Cephalalgia 1992 Feb;12(1):45-51. doi: 10.1046/j.1468-2982.1992.1201045.x. PMID: 1559257

Prognosis

Recurrence and long-term outcomes of Tolosa-Hunt syndrome.
Kim HJ, Lee SU, Lee ES, Choi JY, Kim JS
J Neurol 2024 Feb;271(2):935-943. Epub 2023 Oct 19 doi: 10.1007/s00415-023-12044-y. PMID: 37853245
Is Hypertrophic Pachymeningitis Really Idiopathic?
Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis.
Hung CH, Chang KH, Chen YL, Wu YM, Lai CL, Chang HS, Lyu RK, Wu YR, Chen CM, Huang CC, Chu CC, Chen CH, Ro LS
Headache 2015 Feb;55(2):252-64. doi: 10.1111/head.12488. PMID: 25688645
Dynamic MR imaging in Tolosa-Hunt syndrome.
Haque TL, Miki Y, Kashii S, Yamamoto A, Kanagaki M, Takahashi T, Fushimi Y, Asato R, Murase N, Shibasaki H, Konishi J
Eur J Radiol 2004 Sep;51(3):209-17. doi: 10.1016/j.ejrad.2003.09.016. PMID: 15294327
Idiopathic orbital inflammatory disease.
Günalp I, Gündüz K, Yazar Z
Acta Ophthalmol Scand 1996 Apr;74(2):191-3. doi: 10.1111/j.1600-0420.1996.tb00069.x. PMID: 8739689

Clinical prediction guides

Recurrence and long-term outcomes of Tolosa-Hunt syndrome.
Kim HJ, Lee SU, Lee ES, Choi JY, Kim JS
J Neurol 2024 Feb;271(2):935-943. Epub 2023 Oct 19 doi: 10.1007/s00415-023-12044-y. PMID: 37853245
Is Hypertrophic Pachymeningitis Really Idiopathic?
Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Evaluation and Management of Tolosa-Hunt Syndrome in Children: A Clinical Update.
Pérez CA, Evangelista M
Pediatr Neurol 2016 Sep;62:18-26. Epub 2016 Jun 25 doi: 10.1016/j.pediatrneurol.2016.06.017. PMID: 27473647
Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis.
Hung CH, Chang KH, Chen YL, Wu YM, Lai CL, Chang HS, Lyu RK, Wu YR, Chen CM, Huang CC, Chu CC, Chen CH, Ro LS
Headache 2015 Feb;55(2):252-64. doi: 10.1111/head.12488. PMID: 25688645
Dynamic MR imaging in Tolosa-Hunt syndrome.
Haque TL, Miki Y, Kashii S, Yamamoto A, Kanagaki M, Takahashi T, Fushimi Y, Asato R, Murase N, Shibasaki H, Konishi J
Eur J Radiol 2004 Sep;51(3):209-17. doi: 10.1016/j.ejrad.2003.09.016. PMID: 15294327

Recent systematic reviews

Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients.
Tayebi A, Samimisedeh P, Jafari Afshar E, Mahmoudnia S, Milan N, Ayati A, Madady A, Rastad H
BMC Neurol 2023 Dec 11;23(1):437. doi: 10.1186/s12883-023-03486-y. PMID: 38082244Free PMC Article
Diagnostic controversies in recurrent painful ophthalmoplegic neuropathy: single case report with a systematic review.
Falsaperla R, Presti S, Lo Bianco M, Catanzaro S, Marino S, Ruggieri M
Ital J Pediatr 2022 Jun 3;48(1):82. doi: 10.1186/s13052-022-01274-x. PMID: 35659705Free PMC Article
Neuro-Ophthalmological Complications of the COVID-19 Vaccines: A Systematic Review.
Lotan I, Lydston M, Levy M
J Neuroophthalmol 2022 Jun 1;42(2):154-162. Epub 2022 Mar 25 doi: 10.1097/WNO.0000000000001537. PMID: 35427282
Tolosa-Hunt Syndrome in Childhood and Adolescence: A Literature Review in the Last 10 Years.
Gama BP, Silva-Néto RP
Neuropediatrics 2021 Feb;52(1):1-5. Epub 2020 Sep 6 doi: 10.1055/s-0040-1715632. PMID: 32892335
Actinomyces cavernous sinus infection: a case and systematic literature review.
Lubomski M, Dalgliesh J, Lee K, Damodaran O, McKew G, Reddel S
Pract Neurol 2018 Oct;18(5):373-377. Epub 2018 Apr 12 doi: 10.1136/practneurol-2017-001844. PMID: 29650638

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