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Dystonia 31(DYT31)

MedGen UID:
1794211
Concept ID:
C5562001
Disease or Syndrome
Synonym: ZECH-BOESCH SYNDROME
 
Gene (location): AOPEP (9q22.32)
 
Monarch Initiative: MONDO:0030455
OMIM®: 619565

Definition

Dystonia-31 (DYT31) is an autosomal recessive progressive neurologic disorder characterized by involuntary muscle twisting movements and postural abnormalities affecting the upper and lower limbs, neck, face, and trunk. Some patients may have orofacial dyskinesia resulting in articulation and swallowing difficulties. The age at onset ranges from childhood to young adulthood. There are usually no additional neurologic symptoms, although late-onset parkinsonism was reported in 1 family (summary by Zech et al., 2022). [from OMIM]

Clinical features

From HPO
Dysphagia
MedGen UID:
41440
Concept ID:
C0011168
Disease or Syndrome
Difficulty in swallowing.
Depression
MedGen UID:
4229
Concept ID:
C0011581
Mental or Behavioral Dysfunction
Frequently experiencing feelings of being down, miserable, and/or hopeless; struggling to recover from these moods; having a pessimistic outlook on the future; feeling a pervasive sense of shame; having a low self-worth; experiencing thoughts of suicide and engaging in suicidal behavior.
Writer cramp
MedGen UID:
57821
Concept ID:
C0154676
Disease or Syndrome
A focal dystonia of the fingers, hand, and/or forearm that appears when the affected person attempts to do a task that requires fine motor movements such as writing or playing a musical instrument.
Abnormal posturing
MedGen UID:
66660
Concept ID:
C0231471
Finding
Involuntary flexion or extension of the arms and legs.
Parkinsonian disorder
MedGen UID:
66079
Concept ID:
C0242422
Disease or Syndrome
Characteristic neurologic anomaly resulting from degeneration of dopamine-generating cells in the substantia nigra, a region of the midbrain, characterized clinically by shaking, rigidity, slowness of movement and difficulty with walking and gait.
Gait disturbance
MedGen UID:
107895
Concept ID:
C0575081
Finding
The term gait disturbance can refer to any disruption of the ability to walk.
Generalized dystonia
MedGen UID:
341342
Concept ID:
C1848954
Finding
A type of dystonia that affects all or most of the body.
Craniofacial dystonia
MedGen UID:
868612
Concept ID:
C4023011
Disease or Syndrome
A form of focal dystonia affecting the face and especially the jaw that is induced by the act of speaking. It is an involuntary contraction of the masticatory muscles, resulting in dysarthria or dysphagia.
Leg dystonia
MedGen UID:
1671070
Concept ID:
C4732775
Sign or Symptom
A type of dystonia (abnormally increased muscular tone causing fixed abnormal postures) that affects muscles of the legs.
Arm dystonia
MedGen UID:
1671069
Concept ID:
C4732776
Finding
A type of dystonia (abnormally increased muscular tone causing fixed abnormal postures) that affects muscles of the arms.
Muscle stiffness
MedGen UID:
113151
Concept ID:
C0221170
Sign or Symptom
A condition in which muscles cannot be moved quickly without accompanying pain or spasm.
Weak voice
MedGen UID:
66033
Concept ID:
C0241700
Finding
Reduced intensity (volume) of speech.

Professional guidelines

PubMed

Szejko N, Robinson S, Hartmann A, Ganos C, Debes NM, Skov L, Haas M, Rizzo R, Stern J, Münchau A, Czernecki V, Dietrich A, Murphy TL, Martino D, Tarnok Z, Hedderly T, Müller-Vahl KR, Cath DC
Eur Child Adolesc Psychiatry 2022 Mar;31(3):383-402. Epub 2021 Oct 18 doi: 10.1007/s00787-021-01842-2. PMID: 34661764Free PMC Article
Dressler D, Altavista MC, Altenmueller E, Bhidayasiri R, Bohlega S, Chana P, Chung TM, Colosimo C, Fheodoroff K, Garcia-Ruiz PJ, Jeon B, Jin L, Kanovsky P, Milanov I, Micheli F, Orlova O, Pandey S, Pirtosek Z, Relja M, Rosales R, Sagástegui-Rodríguez JA, Shahidi GA, Timerbaeva S, Wan X, Walter U, Saberi FA
J Neural Transm (Vienna) 2021 Mar;128(3):321-335. Epub 2021 Feb 26 doi: 10.1007/s00702-021-02312-4. PMID: 33635442Free PMC Article
Gerrits MC, Foncke EM, de Haan R, Hedrich K, van de Leemput YL, Baas F, Ozelius LJ, Speelman JD, Klein C, Tijssen MA
Neurology 2006 Mar 14;66(5):759-61. doi: 10.1212/01.wnl.0000201192.66467.a3. PMID: 16534121

Recent clinical studies

Etiology

Bilbao A, Spanagel R
BMC Med 2022 Aug 19;20(1):259. doi: 10.1186/s12916-022-02459-1. PMID: 35982439Free PMC Article
Ruberto AJ, Sivilotti MLA, Forrester S, Hall AK, Crawford FM, Day AG
Ann Emerg Med 2021 Jun;77(6):613-619. Epub 2020 Nov 5 doi: 10.1016/j.annemergmed.2020.08.021. PMID: 33160719
Keklicek H, Uygur F
J Back Musculoskelet Rehabil 2018;31(2):315-321. doi: 10.3233/BMR-169746. PMID: 28946532
Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT; Consensus Working Group
J Neurotrauma 2014 Sep 1;31(17):1515-20. Epub 2014 Jul 28 doi: 10.1089/neu.2013.3301. PMID: 24731076
Jinnah HA, Ceballos-Picot I, Torres RJ, Visser JE, Schretlen DJ, Verdu A, Laróvere LE, Chen CJ, Cossu A, Wu CH, Sampat R, Chang SJ, de Kremer RD, Nyhan W, Harris JC, Reich SG, Puig JG; Lesch-Nyhan Disease International Study Group
Brain 2010 Mar;133(Pt 3):671-89. Epub 2010 Feb 22 doi: 10.1093/brain/awq013. PMID: 20176575Free PMC Article

Diagnosis

Gardella E, Michelucci R, Christensen HM, Fenger CD, Reale C, Riguzzi P, Pasini E, Albini-Riccioli L, Papa V, Foschini MP, Cenacchi G, Furia F, Marjanovic D, Hammer TB, Møller RS, Rubboli G
Epilepsia 2023 Aug;64(8):e170-e176. Epub 2023 Jun 8 doi: 10.1111/epi.17634. PMID: 37114479
Ruberto AJ, Sivilotti MLA, Forrester S, Hall AK, Crawford FM, Day AG
Ann Emerg Med 2021 Jun;77(6):613-619. Epub 2020 Nov 5 doi: 10.1016/j.annemergmed.2020.08.021. PMID: 33160719
Moore DM, Rizzolo D
JAAPA 2018 Apr;31(4):18-22. doi: 10.1097/01.JAA.0000531044.72598.26. PMID: 29517619
Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT; Consensus Working Group
J Neurotrauma 2014 Sep 1;31(17):1515-20. Epub 2014 Jul 28 doi: 10.1089/neu.2013.3301. PMID: 24731076
Jinnah HA, Ceballos-Picot I, Torres RJ, Visser JE, Schretlen DJ, Verdu A, Laróvere LE, Chen CJ, Cossu A, Wu CH, Sampat R, Chang SJ, de Kremer RD, Nyhan W, Harris JC, Reich SG, Puig JG; Lesch-Nyhan Disease International Study Group
Brain 2010 Mar;133(Pt 3):671-89. Epub 2010 Feb 22 doi: 10.1093/brain/awq013. PMID: 20176575Free PMC Article

Therapy

Ruberto AJ, Sivilotti MLA, Forrester S, Hall AK, Crawford FM, Day AG
Ann Emerg Med 2021 Jun;77(6):613-619. Epub 2020 Nov 5 doi: 10.1016/j.annemergmed.2020.08.021. PMID: 33160719
Thomas SP, Addison AP, Curry DJ
Phys Med Rehabil Clin N Am 2020 Feb;31(1):91-105. Epub 2019 Nov 8 doi: 10.1016/j.pmr.2019.09.008. PMID: 31760996
Moore DM, Rizzolo D
JAAPA 2018 Apr;31(4):18-22. doi: 10.1097/01.JAA.0000531044.72598.26. PMID: 29517619
Keklicek H, Uygur F
J Back Musculoskelet Rehabil 2018;31(2):315-321. doi: 10.3233/BMR-169746. PMID: 28946532
Scaglione F
Toxins (Basel) 2016 Mar 4;8(3) doi: 10.3390/toxins8030065. PMID: 26959061Free PMC Article

Prognosis

Rafee S, Hutchinson M, Reilly R
Adv Neurobiol 2023;31:195-210. doi: 10.1007/978-3-031-26220-3_11. PMID: 37338703
Aladeen TS, Mattle AG, Zelen K, Mesha M, Rainka MM, Geist T, Myers B, Mechtler L
Clin Neuropharmacol 2023 May-Jun 01;46(3):98-104. Epub 2023 Apr 19 doi: 10.1097/WNF.0000000000000550. PMID: 37191563
Zech M, Jech R, Boesch S, Škorvánek M, Weber S, Wagner M, Zhao C, Jochim A, Necpál J, Dincer Y, Vill K, Distelmaier F, Stoklosa M, Krenn M, Grunwald S, Bock-Bierbaum T, Fečíková A, Havránková P, Roth J, Příhodová I, Adamovičová M, Ulmanová O, Bechyně K, Danhofer P, Veselý B, Haň V, Pavelekova P, Gdovinová Z, Mantel T, Meindl T, Sitzberger A, Schröder S, Blaschek A, Roser T, Bonfert MV, Haberlandt E, Plecko B, Leineweber B, Berweck S, Herberhold T, Langguth B, Švantnerová J, Minár M, Ramos-Rivera GA, Wojcik MH, Pajusalu S, Õunap K, Schatz UA, Pölsler L, Milenkovic I, Laccone F, Pilshofer V, Colombo R, Patzer S, Iuso A, Vera J, Troncoso M, Fang F, Prokisch H, Wilbert F, Eckenweiler M, Graf E, Westphal DS, Riedhammer KM, Brunet T, Alhaddad B, Berutti R, Strom TM, Hecht M, Baumann M, Wolf M, Telegrafi A, Person RE, Zamora FM, Henderson LB, Weise D, Musacchio T, Volkmann J, Szuto A, Becker J, Cremer K, Sycha T, Zimprich F, Kraus V, Makowski C, Gonzalez-Alegre P, Bardakjian TM, Ozelius LJ, Vetro A, Guerrini R, Maier E, Borggraefe I, Kuster A, Wortmann SB, Hackenberg A, Steinfeld R, Assmann B, Staufner C, Opladen T, Růžička E, Cohn RD, Dyment D, Chung WK, Engels H, Ceballos-Baumann A, Ploski R, Daumke O, Haslinger B, Mall V, Oexle K, Winkelmann J
Lancet Neurol 2020 Nov;19(11):908-918. doi: 10.1016/S1474-4422(20)30312-4. PMID: 33098801Free PMC Article
Bohlega S, Abusrair AH, Al-Ajlan FS, Alharbi N, Al-Semari A, Bohlega B, Abualsaud D, Alkuraya F
Parkinsonism Relat Disord 2019 Dec;69:99-103. Epub 2019 Oct 13 doi: 10.1016/j.parkreldis.2019.10.007. PMID: 31726291
Mali YP, Simon JW, Chaudhri I, Zobal-Ratner J, Barry GP
J Pediatr Ophthalmol Strabismus 2016 Jan-Feb;53(1):22-4. doi: 10.3928/01913913-20150929-18. PMID: 26835998

Clinical prediction guides

Oexle K, Zech M, Stühn LG, Siegert S, Brunet T, Schmidt WM, Wagner M, Schmidt A, Engels H, Tilch E, Monestier O, Destrėe A, Hanker B, Boesch S, Jech R, Berutti R, Kaiser F, Haslinger B, Haack TB, Garavaglia B, Krawitz P, Winkelmann J, Mirza-Schreiber N
Eur J Hum Genet 2023 Sep;31(9):1032-1039. Epub 2023 Jun 26 doi: 10.1038/s41431-023-01406-9. PMID: 37365401Free PMC Article
Rafee S, Hutchinson M, Reilly R
Adv Neurobiol 2023;31:195-210. doi: 10.1007/978-3-031-26220-3_11. PMID: 37338703
Zech M, Jech R, Boesch S, Škorvánek M, Weber S, Wagner M, Zhao C, Jochim A, Necpál J, Dincer Y, Vill K, Distelmaier F, Stoklosa M, Krenn M, Grunwald S, Bock-Bierbaum T, Fečíková A, Havránková P, Roth J, Příhodová I, Adamovičová M, Ulmanová O, Bechyně K, Danhofer P, Veselý B, Haň V, Pavelekova P, Gdovinová Z, Mantel T, Meindl T, Sitzberger A, Schröder S, Blaschek A, Roser T, Bonfert MV, Haberlandt E, Plecko B, Leineweber B, Berweck S, Herberhold T, Langguth B, Švantnerová J, Minár M, Ramos-Rivera GA, Wojcik MH, Pajusalu S, Õunap K, Schatz UA, Pölsler L, Milenkovic I, Laccone F, Pilshofer V, Colombo R, Patzer S, Iuso A, Vera J, Troncoso M, Fang F, Prokisch H, Wilbert F, Eckenweiler M, Graf E, Westphal DS, Riedhammer KM, Brunet T, Alhaddad B, Berutti R, Strom TM, Hecht M, Baumann M, Wolf M, Telegrafi A, Person RE, Zamora FM, Henderson LB, Weise D, Musacchio T, Volkmann J, Szuto A, Becker J, Cremer K, Sycha T, Zimprich F, Kraus V, Makowski C, Gonzalez-Alegre P, Bardakjian TM, Ozelius LJ, Vetro A, Guerrini R, Maier E, Borggraefe I, Kuster A, Wortmann SB, Hackenberg A, Steinfeld R, Assmann B, Staufner C, Opladen T, Růžička E, Cohn RD, Dyment D, Chung WK, Engels H, Ceballos-Baumann A, Ploski R, Daumke O, Haslinger B, Mall V, Oexle K, Winkelmann J
Lancet Neurol 2020 Nov;19(11):908-918. doi: 10.1016/S1474-4422(20)30312-4. PMID: 33098801Free PMC Article
Teemul TA, Patel R, Kanatas A, Carter LM
Br J Oral Maxillofac Surg 2016 Dec;54(10):1080-1084. Epub 2016 Oct 21 doi: 10.1016/j.bjoms.2016.06.028. PMID: 27776922
St Germaine-Smith C, Metcalfe A, Pringsheim T, Roberts JI, Beck CA, Hemmelgarn BR, McChesney J, Quan H, Jette N
Neurology 2012 Sep 4;79(10):1049-55. Epub 2012 Aug 22 doi: 10.1212/WNL.0b013e3182684707. PMID: 22914826Free PMC Article

Recent systematic reviews

Bilbao A, Spanagel R
BMC Med 2022 Aug 19;20(1):259. doi: 10.1186/s12916-022-02459-1. PMID: 35982439Free PMC Article
Qiu X, Cui Z, Tang G, Deng H, Xiong Z, Han S, Tang S
J Craniofac Surg 2020 Nov/Dec;31(8):2160-2166. doi: 10.1097/SCS.0000000000006652. PMID: 33136847
Ellwood J, Draper-Rodi J, Carnes D
Chiropr Man Therap 2020 Jun 11;28(1):31. doi: 10.1186/s12998-020-00321-w. PMID: 32522230Free PMC Article
Boelig RC, Barton SJ, Saccone G, Kelly AJ, Edwards SJ, Berghella V
J Matern Fetal Neonatal Med 2018 Sep;31(18):2492-2505. Epub 2017 Jul 11 doi: 10.1080/14767058.2017.1342805. PMID: 28614956
St Germaine-Smith C, Metcalfe A, Pringsheim T, Roberts JI, Beck CA, Hemmelgarn BR, McChesney J, Quan H, Jette N
Neurology 2012 Sep 4;79(10):1049-55. Epub 2012 Aug 22 doi: 10.1212/WNL.0b013e3182684707. PMID: 22914826Free PMC Article

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