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Trigeminal anesthesia

MedGen UID:
355635
•Concept ID:
C1866127
•
Finding
Synonym: Trigeminal anaesthesia
 
HPO: HP:0031912

Definition

Decreased or absent sensation in the distribution of the trigeminal nerve, which provides tactile, proprioceptive, and nociceptive sensation in the area of the face and mouth. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTrigeminal anesthesia

Conditions with this feature

Gomez Lopez Hernandez syndrome
MedGen UID:
163201
•Concept ID:
C0795959
•
Disease or Syndrome
Gomez-Lopez-Hernandez syndrome (GLHS), also known as cerebellotrigeminal dermal dysplasia, is a rare neurocutaneous syndrome classically characterized by the triad of rhombencephalosynapsis, trigeminal anesthesia, often giving rise to corneal opacities, and bilateral parietal or parietooccipital alopecia. However, trigeminal anesthesia is an inconsistent finding (summary by Sukhudyan et al., 2010).
See: Condition Record
Gomez Lopez Hernandez syndrome

Professional guidelines

PubMed

Gómez-López-Hernández syndrome: reappraisal of the diagnostic criteria.
Sukhudyan B, Jaladyan V, Melikyan G, Schlump JU, Boltshauser E, Poretti A
Eur J Pediatr 2010 Dec;169(12):1523-8. Epub 2010 Jul 23 doi: 10.1007/s00431-010-1259-7. PMID: 20652311
Surgical management of trigeminal schwannomas.
Ramina R, Mattei TA, Sória MG, da Silva EB Jr, Leal AG, Neto MC, Fernandes YB
Neurosurg Focus 2008;25(6):E6; discussion E6. doi: 10.3171/FOC.2008.25.12.E6. PMID: 19035703

Recent clinical studies

Etiology

Seeing through the evidence for corneal neurotization.
Jowett N, Pineda R 2nd
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):252-258. doi: 10.1097/MOO.0000000000000731. PMID: 34074877
Trigeminal nerve deficit in large and compressive acoustic neuromas and its correlation with MRI findings.
Karkas A, Lamblin E, Meyer M, Gay E, Ternier J, Schmerber S
Otolaryngol Head Neck Surg 2014 Oct;151(4):675-80. Epub 2014 Aug 1 doi: 10.1177/0194599814545440. PMID: 25085321
Surgical management of trigeminal schwannomas.
Ramina R, Mattei TA, Sória MG, da Silva EB Jr, Leal AG, Neto MC, Fernandes YB
Neurosurg Focus 2008;25(6):E6; discussion E6. doi: 10.3171/FOC.2008.25.12.E6. PMID: 19035703
Self-induced nasal ulceration.
Tollefson TT, Kriet JD, Wang TD, Cook TA
Arch Facial Plast Surg 2004 May-Jun;6(3):162-6. doi: 10.1001/archfaci.6.3.162. PMID: 15148123
Congenital trigeminal anesthesia in two siblings and their long-term follow-up.
Wong VA, Cline RA, Dubord PJ, Rees M
Am J Ophthalmol 2000 Jan;129(1):96-8. doi: 10.1016/s0002-9394(99)00290-1. PMID: 10653422

Diagnosis

Prenatal Diagnosis of Gómez-López-Hernández Syndrome.
Pomar L, Rieder W, Dubruc E, Giuliano F, Atallah I, Lebon S, Vial Y
Fetal Diagn Ther 2023;50(2):92-97. Epub 2023 Apr 14 doi: 10.1159/000530643. PMID: 37062278
Clinical and molecular evaluation of 13 Brazilian patients with Gomez-López-Hernández syndrome.
Perrone E, Perez ABA, D'Almeida V, de Mello CB, Jacobina MAA, Loureiro RM, Burlin S, Migliavacca M, do Amaral Virmond L, Graziadio C, Pedroso JL, Mendes EL, Gomy I, de Macena Sobreira NL
Am J Med Genet A 2021 Apr;185(4):1047-1058. Epub 2020 Dec 31 doi: 10.1002/ajmg.a.62059. PMID: 33381921
Genetic evaluation including exome sequencing of two patients with Gomez-Lopez-Hernandez syndrome: Case reports and review of the literature.
Lindsay F, Anderson I, Wentzensen IM, Suhrbier D, Stevens CA
Am J Med Genet A 2020 Apr;182(4):623-627. Epub 2020 Jan 31 doi: 10.1002/ajmg.a.61496. PMID: 32003537
Trigeminal Trophic Syndrome-A Unique Clinical Presentation of a Rare Condition.
Bradburn KH, Elston D, Murphey AW, Patel KG
Ear Nose Throat J 2019 Dec;98(10):606-608. Epub 2019 May 23 doi: 10.1177/0145561319850818. PMID: 31119987
Self-induced nasal ulceration.
Tollefson TT, Kriet JD, Wang TD, Cook TA
Arch Facial Plast Surg 2004 May-Jun;6(3):162-6. doi: 10.1001/archfaci.6.3.162. PMID: 15148123

Therapy

Seeing through the evidence for corneal neurotization.
Jowett N, Pineda R 2nd
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):252-258. doi: 10.1097/MOO.0000000000000731. PMID: 34074877
Gomez-López-Hernández syndrome: A case report with clinical and molecular evaluation and literature review.
Perrone E, D'Almeida V, de Macena Sobreira NL, de Mello CB, de Oliveira AC, Burlin S, Soares MFF, Cernach MCSP, Alvarez Perez AB
Am J Med Genet A 2020 Jul;182(7):1761-1766. Epub 2020 Apr 17 doi: 10.1002/ajmg.a.61594. PMID: 32302043Free PMC Article
Facial Sensory Restoration After Trigeminal Sensory Rhizotomy by Collateral Sprouting From the Occipital Nerves.
Freeman L, Wu OC, Sweet J, Cohen M, Smith GA, Miller JP
Neurosurgery 2020 May 1;86(5):E436-E441. doi: 10.1093/neuros/nyz306. PMID: 31432075
Chronic dysphagia and trigeminal anesthesia after trichloroethylene exposure.
Lawrence WH, Partyka EK
Ann Intern Med 1981 Dec;95(6):710. doi: 10.7326/0003-4819-95-6-710. PMID: 7305154

Prognosis

Adenoid Cystic Carcinoma of the Maxillary Sinus with Isolated Trigeminal Anesthesia.
Bollinger SS, DeSautel MG, Spanos WC, Tjarks BJ
S D Med 2018 Jul;71(7):294-298. PMID: 30005538
Trigeminal nerve deficit in large and compressive acoustic neuromas and its correlation with MRI findings.
Karkas A, Lamblin E, Meyer M, Gay E, Ternier J, Schmerber S
Otolaryngol Head Neck Surg 2014 Oct;151(4):675-80. Epub 2014 Aug 1 doi: 10.1177/0194599814545440. PMID: 25085321
Surgical management of trigeminal schwannomas.
Ramina R, Mattei TA, Sória MG, da Silva EB Jr, Leal AG, Neto MC, Fernandes YB
Neurosurg Focus 2008;25(6):E6; discussion E6. doi: 10.3171/FOC.2008.25.12.E6. PMID: 19035703
Gomez-Lopez-Hernandez syndrome: expansion of the phenotype.
Brocks D, Irons M, Sadeghi-Najad A, McCauley R, Wheeler P
Am J Med Genet 2000 Oct 23;94(5):405-8. doi: 10.1002/1096-8628(20001023)94:5<405::aid-ajmg12>3.0.co;2-8. PMID: 11050627
Congenital trigeminal anesthesia in two siblings and their long-term follow-up.
Wong VA, Cline RA, Dubord PJ, Rees M
Am J Ophthalmol 2000 Jan;129(1):96-8. doi: 10.1016/s0002-9394(99)00290-1. PMID: 10653422

Clinical prediction guides

Facial Sensory Restoration After Trigeminal Sensory Rhizotomy by Collateral Sprouting From the Occipital Nerves.
Freeman L, Wu OC, Sweet J, Cohen M, Smith GA, Miller JP
Neurosurgery 2020 May 1;86(5):E436-E441. doi: 10.1093/neuros/nyz306. PMID: 31432075
Gómez-López-Hernández syndrome in a Japanese patient: a case report.
Kobayashi Y, Kawashima H, Magara S, Akasaka N, Tohyama J
Brain Dev 2015 Mar;37(3):356-8. Epub 2014 May 21 doi: 10.1016/j.braindev.2014.05.002. PMID: 24856766
Gómez-López-Hernández syndrome: reappraisal of the diagnostic criteria.
Sukhudyan B, Jaladyan V, Melikyan G, Schlump JU, Boltshauser E, Poretti A
Eur J Pediatr 2010 Dec;169(12):1523-8. Epub 2010 Jul 23 doi: 10.1007/s00431-010-1259-7. PMID: 20652311
Surgical management of trigeminal schwannomas.
Ramina R, Mattei TA, Sória MG, da Silva EB Jr, Leal AG, Neto MC, Fernandes YB
Neurosurg Focus 2008;25(6):E6; discussion E6. doi: 10.3171/FOC.2008.25.12.E6. PMID: 19035703

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