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Trismus

MedGen UID:
21671
Concept ID:
C0041105
Disease or Syndrome
Synonyms: Decrease in jaw mobility; Decrease in jaw movement; Decrease in jaw opening; Decrease in mandibular mobility; Decrease in mandibular movement; Decrease in mandibular opening; Limited jaw mobility; Limited jaw movement; Limited jaw opening; Limited mandibular mobility; Limited mandibular opening; Limited mouth opening; Lockjaw; Pain of muscles of mastication
SNOMED CT: Trismus (87866006)
 
HPO: HP:0000211

Definition

Limitation in the ability to open the mouth. [from HPO]

Conditions with this feature

Arthrogryposis, distal, type 1A
MedGen UID:
113099
Concept ID:
C0220662
Congenital Abnormality
Distal arthrogryposis type 1 is a disorder characterized by joint deformities (contractures) that restrict movement in the hands and feet. The term "arthrogryposis" comes from the Greek words for joint (arthro-) and crooked or hooked (gryposis). The characteristic features of this condition include permanently bent fingers and toes (camptodactyly), overlapping fingers, and a hand deformity in which all of the fingers are angled outward toward the fifth finger (ulnar deviation). Clubfoot, which is an inward- and upward-turning foot, is also commonly seen with distal arthrogryposis type 1. The specific hand and foot abnormalities vary among affected individuals. However, this condition typically does not cause any signs and symptoms affecting other parts of the body.
Hecht syndrome
MedGen UID:
78540
Concept ID:
C0265226
Disease or Syndrome
The trismus-pseudocamptodactyly syndrome is a distal arthrogryposis characterized by an inability to open the mouth fully (trismus) and pseudocamptodactyly in which wrist dorsiflexion, but not volar flexion, produces involuntary flexion contracture of distal and proximal interphalangeal joints. In these patients, trismus complicates dental care, feeding during infancy, and intubation for anesthesia, and the pseudocamptodactyly impairs manual dexterity, with consequent occupational and social disability (summary by Veugelers et al., 2004).
Nager syndrome
MedGen UID:
120519
Concept ID:
C0265245
Disease or Syndrome
Nager syndrome is the prototype for a group of disorders collectively referred to as the acrofacial dysostoses (AFDs), which are characterized by malformation of the craniofacial skeleton and the limbs. The major facial features of Nager syndrome include downslanted palpebral fissures, midface retrusion, and micrognathia, the latter of which often requires the placement of a tracheostomy in early childhood. Limb defects typically involve the anterior (radial) elements of the upper limbs and manifest as small or absent thumbs, triphalangeal thumbs, radial hypoplasia or aplasia, and radioulnar synostosis. Phocomelia of the upper limbs and, occasionally, lower-limb defects have also been reported. The presence of anterior upper-limb defects and the typical lack of lower-limb involvement distinguishes Nager syndrome from Miller syndrome (263750), another rare AFD; however, distinguishing Nager syndrome from other AFDs, including Miller syndrome, can be challenging (summary by Bernier et al., 2012).
Gaucher disease type II
MedGen UID:
78652
Concept ID:
C0268250
Disease or Syndrome
Gaucher disease (GD) encompasses a continuum of clinical findings from a perinatal lethal disorder to an asymptomatic type. The identification of three major clinical types (1, 2, and 3) and two other subtypes (perinatal-lethal and cardiovascular) is useful in determining prognosis and management. GD type 1 is characterized by the presence of clinical or radiographic evidence of bone disease (osteopenia, focal lytic or sclerotic lesions, and osteonecrosis), hepatosplenomegaly, anemia and thrombocytopenia, lung disease, and the absence of primary central nervous system disease. GD types 2 and 3 are characterized by the presence of primary neurologic disease; in the past, they were distinguished by age of onset and rate of disease progression, but these distinctions are not absolute. Disease with onset before age two years, limited psychomotor development, and a rapidly progressive course with death by age two to four years is classified as GD type 2. Individuals with GD type 3 may have onset before age two years, but often have a more slowly progressive course, with survival into the third or fourth decade. The perinatal-lethal form is associated with ichthyosiform or collodion skin abnormalities or with nonimmune hydrops fetalis. The cardiovascular form is characterized by calcification of the aortic and mitral valves, mild splenomegaly, corneal opacities, and supranuclear ophthalmoplegia. Cardiopulmonary complications have been described with all the clinical subtypes, although varying in frequency and severity.
Branchiogenic deafness syndrome
MedGen UID:
322970
Concept ID:
C1836673
Disease or Syndrome
A multiple congenital anomalies syndrome, described in one family to date, with characteristics of branchial cysts or fistula, ear malformations, congenital hearing loss (conductive, sensorineural, and mixed), internal auditory canal hypoplasia, strabismus, trismus, abnormal fifth fingers, vitiliginous lesions, short stature and mild learning disability. Renal and urethral abnormalities are absent.
Carney complex - trismus - pseudocamptodactyly syndrome
MedGen UID:
332400
Concept ID:
C1837245
Disease or Syndrome
Carney complex-trismus-pseudocamptodactyly syndrome is a rare genetic heart-hand syndrome characterized by typical manifestations of the Carney complex (spotty pigmentation of the skin, familial cardiac and cutaneous myxomas and endocrinopathy) associated with trismus and distal arthrogryposis (presenting as involuntary contraction of distal and proximal interphalangeal joints of hands evident only on dorsiflexion of wrist and similar lower-limb contractures producing foot deformities).
Whistling face syndrome, recessive form
MedGen UID:
376364
Concept ID:
C1848470
Disease or Syndrome
Whistling face syndrome is characterized by an atypical facial appearance with anomalies of the hands and feet. Most cases show autosomal dominant inheritance: see distal arthrogryposis 2A (DA2A; 193700). There are rare reports of presumably autosomal recessive inheritance (summary by Altunhan et al., 2010).
Cold-induced sweating syndrome 1
MedGen UID:
338577
Concept ID:
C1848947
Disease or Syndrome
Cold-induced sweating syndrome (CISS) and its infantile presentation, Crisponi syndrome(CS) is characterized by dysmorphic features (distinctive facies, lower facial weakness, flexion deformity at the elbows, camptodactyly with fisted hands, misshapen feet, and overriding toes); intermittent contracture of facial and oropharyngeal muscles when crying or being handled with puckering of lips and drooling of foamy saliva often associated with laryngospasm and respiratory distress; excessive startling and opisthotonus-like posturing with unexpected tactile or auditory stimuli; poor suck reflex and severely impaired swallowing; and a scaly erythematous rash. During the first decade of life, children with CISS/CS develop profuse sweating of the face, arms, and chest with ambient temperatures below 18º to 22º C, and with other stimuli including nervousness or ingestion of sweets. Affected individuals sweat very little in hot environments and may feel overheated. Progressive thoracolumbar kyphoscoliosis occurs, requiring intervention in the second decade.
Arthrogryposis, distal, type 2E
MedGen UID:
343844
Concept ID:
C1852597
Disease or Syndrome
Autosomal recessive faciodigitogenital syndrome
MedGen UID:
341637
Concept ID:
C1856871
Disease or Syndrome
A very rare syndrome including short stature, facial dysmorphism, hand abnormalities and shawl scrotum. It has been observed in 16 subjects from five distantly related sibships of a large Kuwaiti Bedouin tribe. The affected patients had no intellectual deficit. Transmitted as an autosomal recessive trait.
Spondyloepiphyseal dysplasia, Stanescu type
MedGen UID:
905084
Concept ID:
C4225273
Disease or Syndrome
Spondyloepiphyseal dysplasia with accumulation of glycoprotein in chondrocytes has been designated the 'Stanescu type.' Clinical hallmarks include progressive joint contracture with premature degenerative joint disease, particularly in the knee, hip, and finger joints. Interphalangeal joints of the hands are swollen due to osseous distention of the metaphyseal ends of the phalanges. Affected individuals may be relatively tall despite the presence of a short trunk. Radiologically, there is generalized platyspondyly with mild modification of the endplates, hypoplastic pelvis, epiphyseal flattening with metaphyseal splaying of the long bones, and enlarged phalangeal epimetaphyses of the hands. In addition, the proximal femora are characteristically broad and elongated with striking coxa valga (summary by Nishimura et al., 1998).
Mandibulofacial dysostosis with alopecia
MedGen UID:
898794
Concept ID:
C4225349
Disease or Syndrome
A rare mandibulofacial dysostosis with the association with scalp alopecia and sparse eyebrows and eyelashes. Craniofacial dysmorphic features include zygomatic and mandibular dysplasia or hypoplasia, cleft palate, micrognathia, dental anomalies, auricular dysmorphism and eyelid anomalies among others. Patients may experience limited jaw mobility, glossoptosis, upper airway obstruction and conductive hearing loss.
Carey-Fineman-Ziter syndrome 1
MedGen UID:
1804638
Concept ID:
C5676876
Disease or Syndrome
Carey-Fineman-Ziter syndrome-1 (CFZS1) is a multisystem congenital disorder characterized by hypotonia, Moebius sequence (bilateral congenital facial palsy with impairment of ocular abduction), Pierre Robin complex (micrognathia, glossoptosis, and high-arched or cleft palate), delayed motor milestones, and failure to thrive. More variable features include dysmorphic facial features, brain abnormalities, and intellectual disability. It has been postulated that many clinical features in CFZS1 may be secondary effects of muscle weakness during development or brainstem anomalies (summary by Pasetti et al., 2016). Di Gioia et al. (2017) determined that CFZS1 represents a slowly progressive congenital myopathy resulting from a defect in myoblast fusion. Genetic Heterogeneity of Carey-Fineman-Ziter Syndrome Carey-Fineman-Ziter syndrome-2 (CFZS2) is caused by mutation in the MYMX gene (619912) on chromosome 6p21.
3-methylglutaconic aciduria, type VIIB
MedGen UID:
1810214
Concept ID:
C5676893
Disease or Syndrome
CLPB (caseinolytic peptidase B) deficiency is characterized by neurologic involvement and neutropenia, which can range from severe to mild. In severe CLPB deficiency, death usually occurs at a few months of age due to significant neonatal neurologic involvement (hyperekplexia or absence of voluntary movements, hypotonia or hypertonia, swallowing problems, respiratory insufficiency, and epilepsy) and severe neutropenia associated with life-threatening infections. Individuals with moderate CLPB deficiency present with neurologic abnormalities in infancy including hypotonia and feeding problems, and develop spasticity, a progressive movement disorder (ataxia, dystonia, and/or dyskinesia), epilepsy, and intellectual disability. Neutropenia is variable, but not life threatening. In those with mild CLPB deficiency there is no neurologic involvement, intellect is normal, neutropenia is mild and intermittent, and life expectancy is normal.
Developmental and epileptic encephalopathy 103
MedGen UID:
1809962
Concept ID:
C5677002
Disease or Syndrome
Developmental and epileptic encephalopathy-103 (DEE103) is characterized by onset of various types of seizures in the first year of life, most of which are refractory to treatment. Affected individuals show global developmental delay with impaired intellectual development ranging from mild to severe. Additional features may include hypotonia, ataxia, and behavioral abnormalities, including autism and hyperactivity (Schwarz et al., 2022). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.

Professional guidelines

PubMed

Bridwell R, Gottlieb M, Koyfman A, Long B
Am J Emerg Med 2021 Mar;41:1-5. Epub 2020 Dec 23 doi: 10.1016/j.ajem.2020.12.030. PMID: 33383265
Levi LE, Lalla RV
Dent Clin North Am 2018 Jan;62(1):121-130. Epub 2017 Oct 7 doi: 10.1016/j.cden.2017.08.009. PMID: 29126489
Strojan P, Hutcheson KA, Eisbruch A, Beitler JJ, Langendijk JA, Lee AWM, Corry J, Mendenhall WM, Smee R, Rinaldo A, Ferlito A
Cancer Treat Rev 2017 Sep;59:79-92. Epub 2017 Jul 18 doi: 10.1016/j.ctrv.2017.07.003. PMID: 28759822Free PMC Article

Recent clinical studies

Etiology

Herrera-Barraza V, Arroyo-Larrondo S, Fernández-Córdova M, Catricura-Cerna D, Garrido-Urrutia C, Ferrer-Valdivia N
Dent Med Probl 2022 Oct-Dec;59(4):593-601. doi: 10.17219/dmp/144596. PMID: 36516334
E C, M D, K C, V A, P M, C F, Jr D, Jr C
Oral Oncol 2022 Mar;126:105728. Epub 2022 Jan 29 doi: 10.1016/j.oraloncology.2022.105728. PMID: 35104753
Strojan P, Hutcheson KA, Eisbruch A, Beitler JJ, Langendijk JA, Lee AWM, Corry J, Mendenhall WM, Smee R, Rinaldo A, Ferlito A
Cancer Treat Rev 2017 Sep;59:79-92. Epub 2017 Jul 18 doi: 10.1016/j.ctrv.2017.07.003. PMID: 28759822Free PMC Article
Ngeow WC, Lim D
Adv Ther 2016 Jul;33(7):1105-39. Epub 2016 Jun 10 doi: 10.1007/s12325-016-0357-y. PMID: 27287853Free PMC Article
Shires PM, Chow G
Dev Med Child Neurol 2015 Apr;57(4):339-43. Epub 2014 Nov 19 doi: 10.1111/dmcn.12636. PMID: 25407568

Diagnosis

Chaabouni H, Bechraoui R, Kriaa M, Zainine R, Besbes G
Tunis Med 2023 Aug-Sep;101(8-9):718-720. PMID: 38445409
Martins CA, Goldenberg DC, Narikawa R, Kowalski LP
Braz J Otorhinolaryngol 2020 Sep-Oct;86(5):552-557. Epub 2019 Mar 18 doi: 10.1016/j.bjorl.2019.02.004. PMID: 30962143Free PMC Article
Rapidis AD, Dijkstra PU, Roodenburg JL, Rodrigo JP, Rinaldo A, Strojan P, Takes RP, Ferlito A
Clin Otolaryngol 2015 Dec;40(6):516-26. doi: 10.1111/coa.12488. PMID: 26098612
Baldassari C, Shah RK
Infect Disord Drug Targets 2012 Aug;12(4):277-80. doi: 10.2174/187152612801319258. PMID: 22338588
Galioto NJ
Am Fam Physician 2008 Jan 15;77(2):199-202. PMID: 18246890

Therapy

Petronis Ž, Zigmantavičius J, Gervickas A
Stomatologija 2020;22(4):107-115. PMID: 33847282
Bailey E, Kashbour W, Shah N, Worthington HV, Renton TF, Coulthard P
Cochrane Database Syst Rev 2020 Jul 26;7(7):CD004345. doi: 10.1002/14651858.CD004345.pub3. PMID: 32712962Free PMC Article
Nichols AC, Theurer J, Prisman E, Read N, Berthelet E, Tran E, Fung K, de Almeida JR, Bayley A, Goldstein DP, Hier M, Sultanem K, Richardson K, Mlynarek A, Krishnan S, Le H, Yoo J, MacNeil SD, Winquist E, Hammond JA, Venkatesan V, Kuruvilla S, Warner A, Mitchell S, Chen J, Corsten M, Johnson-Obaseki S, Eapen L, Odell M, Parker C, Wehrli B, Kwan K, Palma DA
Lancet Oncol 2019 Oct;20(10):1349-1359. Epub 2019 Aug 12 doi: 10.1016/S1470-2045(19)30410-3. PMID: 31416685
Kamstra JI, van Leeuwen M, Roodenburg JL, Dijkstra PU
Head Neck 2017 Jan;39(1):160-169. Epub 2016 Feb 15 doi: 10.1002/hed.24366. PMID: 26876238
THOMPSON PW
Ann R Coll Surg Engl 1964 Dec;35(6):362-78. PMID: 14248322Free PMC Article

Prognosis

van der Geer SJ, van Rijn PV, Kamstra JI, Langendijk JA, van der Laan BFAM, Roodenburg JLN, Dijkstra PU
Head Neck 2019 Jan;41(1):64-71. Epub 2018 Dec 18 doi: 10.1002/hed.25369. PMID: 30561067Free PMC Article
Singh K, Kumar S, Singh S, Mishra V, Sharma PK, Singh D
Indian J Dent Res 2018 Sep-Oct;29(5):605-610. doi: 10.4103/ijdr.IJDR_549_16. PMID: 30409940
Strojan P, Hutcheson KA, Eisbruch A, Beitler JJ, Langendijk JA, Lee AWM, Corry J, Mendenhall WM, Smee R, Rinaldo A, Ferlito A
Cancer Treat Rev 2017 Sep;59:79-92. Epub 2017 Jul 18 doi: 10.1016/j.ctrv.2017.07.003. PMID: 28759822Free PMC Article
Brennan B
Orphanet J Rare Dis 2006 Jun 26;1:23. doi: 10.1186/1750-1172-1-23. PMID: 16800883Free PMC Article
Von Mühlendahl KE, Krienke EG
Clin Toxicol 1979;14(1):97-106. doi: 10.3109/15563657909030119. PMID: 436387

Clinical prediction guides

E C, M D, K C, V A, P M, C F, Jr D, Jr C
Oral Oncol 2022 Mar;126:105728. Epub 2022 Jan 29 doi: 10.1016/j.oraloncology.2022.105728. PMID: 35104753
Kiencało A, Jamka-Kasprzyk M, Panaś M, Wyszyńska-Pawelec G
Dent Med Probl 2021 Jan-Mar;58(1):75-80. doi: 10.17219/dmp/127028. PMID: 33789003
Bridwell R, Gottlieb M, Koyfman A, Long B
Am J Emerg Med 2021 Mar;41:1-5. Epub 2020 Dec 23 doi: 10.1016/j.ajem.2020.12.030. PMID: 33383265
Martins CA, Goldenberg DC, Narikawa R, Kowalski LP
Braz J Otorhinolaryngol 2020 Sep-Oct;86(5):552-557. Epub 2019 Mar 18 doi: 10.1016/j.bjorl.2019.02.004. PMID: 30962143Free PMC Article
Nichols AC, Theurer J, Prisman E, Read N, Berthelet E, Tran E, Fung K, de Almeida JR, Bayley A, Goldstein DP, Hier M, Sultanem K, Richardson K, Mlynarek A, Krishnan S, Le H, Yoo J, MacNeil SD, Winquist E, Hammond JA, Venkatesan V, Kuruvilla S, Warner A, Mitchell S, Chen J, Corsten M, Johnson-Obaseki S, Eapen L, Odell M, Parker C, Wehrli B, Kwan K, Palma DA
Lancet Oncol 2019 Oct;20(10):1349-1359. Epub 2019 Aug 12 doi: 10.1016/S1470-2045(19)30410-3. PMID: 31416685

Recent systematic reviews

Herrera-Barraza V, Arroyo-Larrondo S, Fernández-Córdova M, Catricura-Cerna D, Garrido-Urrutia C, Ferrer-Valdivia N
Dent Med Probl 2022 Oct-Dec;59(4):593-601. doi: 10.17219/dmp/144596. PMID: 36516334
E C, M D, K C, V A, P M, C F, Jr D, Jr C
Oral Oncol 2022 Mar;126:105728. Epub 2022 Jan 29 doi: 10.1016/j.oraloncology.2022.105728. PMID: 35104753
Bailey E, Kashbour W, Shah N, Worthington HV, Renton TF, Coulthard P
Cochrane Database Syst Rev 2020 Jul 26;7(7):CD004345. doi: 10.1002/14651858.CD004345.pub3. PMID: 32712962Free PMC Article
Cohen EE, LaMonte SJ, Erb NL, Beckman KL, Sadeghi N, Hutcheson KA, Stubblefield MD, Abbott DM, Fisher PS, Stein KD, Lyman GH, Pratt-Chapman ML
CA Cancer J Clin 2016 May;66(3):203-39. Epub 2016 Mar 22 doi: 10.3322/caac.21343. PMID: 27002678
Bouman MA, Dijkstra PU, Reintsema H, Roodenburg JL, Werker PM
Br J Oral Maxillofac Surg 2016 Apr;54(3):253-9. Epub 2015 Oct 9 doi: 10.1016/j.bjoms.2015.08.269. PMID: 26455659

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