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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

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
Zhang X, Zhang W, Liu R, Dong Z, Yu S
J Neurol Sci 2014 Jun 15;341(1-2):13-6. Epub 2014 Mar 21 doi: 10.1016/j.jns.2014.03.031. PMID: 24703581
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

Recent clinical studies

Etiology

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
Yang Q, Lai C, Meng C, Chang Q, Wang J
Eur Neurol 2022;85(4):265-272. Epub 2022 Mar 9 doi: 10.1159/000522187. PMID: 35263741
Kim H, Oh SY
BMC Ophthalmol 2021 May 27;21(1):237. doi: 10.1186/s12886-021-02007-0. PMID: 34044807Free PMC Article
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
Palacios E, Valvassori G, Bresler M
Ear Nose Throat J 1999 Mar;78(3):150. PMID: 10188349

Diagnosis

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
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
Friedman DI
Continuum (Minneap Minn) 2015 Aug;21(4 Headache):1109-17. doi: 10.1212/CON.0000000000000204. PMID: 26252594
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
Aron-Rosa D, Doyon D, Salamon G, Michotey P
Ann Ophthalmol 1978 Sep;10(9):1161-8. PMID: 736403

Therapy

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
Tsirigotaki M, Ntoulios G, Lioumpas M, Voutoufianakis S, Vorgia P
Pediatr Neurol 2019 Oct;99:60-63. Epub 2019 Feb 20 doi: 10.1016/j.pediatrneurol.2019.02.013. PMID: 30982655
Yeung MC, Kwong KL, Wong YC, Wong SN
J Paediatr Child Health 2004 Jul;40(7):410-3. doi: 10.1111/j.1440-1754.2004.00414.x. PMID: 15228576
Kline LB, Hoyt WF
J Neurol Neurosurg Psychiatry 2001 Nov;71(5):577-82. doi: 10.1136/jnnp.71.5.577. PMID: 11606665Free PMC Article
Hannerz J
Cephalalgia 1992 Feb;12(1):45-51. doi: 10.1046/j.1468-2982.1992.1201045.x. PMID: 1559257

Prognosis

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
Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Sim NK, Liew SY, Warren DJ, Hassan A
BMJ Case Rep 2022 Mar 1;15(3) doi: 10.1136/bcr-2021-246519. PMID: 35232735Free PMC Article
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
Palacios E, Valvassori G, Bresler M
Ear Nose Throat J 1999 Mar;78(3):150. PMID: 10188349

Clinical prediction guides

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
Ang T, Tong JY, Patel S, Khong JJ, Selva D
Int Ophthalmol 2023 Sep;43(9):3391-3401. Epub 2023 May 17 doi: 10.1007/s10792-023-02747-6. PMID: 37198501Free PMC Article
Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Lee KP, Sung PS, Lee WA
Acta Neurol Taiwan 2021 Mar;30(1):39-42. PMID: 34549400
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

Recent systematic reviews

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
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
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
Gama BP, Silva-Néto RP
Neuropediatrics 2021 Feb;52(1):1-5. Epub 2020 Sep 6 doi: 10.1055/s-0040-1715632. PMID: 32892335
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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