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Dystonia, mitochondrial

MedGen UID:
865044
Concept ID:
C4016607
Finding
Synonym: DYSTONIA, MITOCHONDRIAL
 
OMIM®: 590020

Professional guidelines

PubMed

Wilson JL, Gregory A, Kurian MA, Bushlin I, Mochel F, Emrick L, Adang L; BPAN Guideline Contributing Author Group, Hogarth P, Hayflick SJ
Dev Med Child Neurol 2021 Dec;63(12):1402-1409. Epub 2021 Aug 4 doi: 10.1111/dmcn.14980. PMID: 34347296
Nie S, Chen G, Cao X, Zhang Y
Orphanet J Rare Dis 2014 Nov 26;9:179. doi: 10.1186/s13023-014-0179-4. PMID: 25424010Free PMC Article
Finsterer J
Eur J Paediatr Neurol 2010 Jan;14(1):29-44. Epub 2009 Aug 19 doi: 10.1016/j.ejpn.2009.07.005. PMID: 19692274

Recent clinical studies

Etiology

Deng X, Yuan L, Jankovic J, Deng H
Ageing Res Rev 2023 Aug;89:101957. Epub 2023 May 24 doi: 10.1016/j.arr.2023.101957. PMID: 37236368
Boy N, Mühlhausen C, Maier EM, Ballhausen D, Baumgartner MR, Beblo S, Burgard P, Chapman KA, Dobbelaere D, Heringer-Seifert J, Fleissner S, Grohmann-Held K, Hahn G, Harting I, Hoffmann GF, Jochum F, Karall D, Konstantopoulous V, Krawinkel MB, Lindner M, Märtner EMC, Nuoffer JM, Okun JG, Plecko B, Posset R, Sahm K, Scholl-Bürgi S, Thimm E, Walter M, Williams M, Vom Dahl S, Ziagaki A, Zschocke J, Kölker S
J Inherit Metab Dis 2023 May;46(3):482-519. Epub 2022 Nov 17 doi: 10.1002/jimd.12566. PMID: 36221165
Jinnah HA, Sun YV
Neurobiol Dis 2019 Sep;129:159-168. Epub 2019 May 18 doi: 10.1016/j.nbd.2019.05.014. PMID: 31112762
Di Meo I, Tiranti V
Eur J Paediatr Neurol 2018 Mar;22(2):272-284. Epub 2018 Jan 17 doi: 10.1016/j.ejpn.2018.01.008. PMID: 29409688
Hartig MB, Prokisch H, Meitinger T, Klopstock T
Curr Drug Targets 2012 Aug;13(9):1182-9. doi: 10.2174/138945012802002384. PMID: 22515741

Diagnosis

Kistol D, Tsygankova P, Bostanova F, Orlova M, Zakharova E
Int J Mol Sci 2024 May 8;25(10) doi: 10.3390/ijms25105127. PMID: 38791166Free PMC Article
Ng YS, McFarland R
Handb Clin Neurol 2023;195:563-585. doi: 10.1016/B978-0-323-98818-6.00025-X. PMID: 37562887
Boy N, Mühlhausen C, Maier EM, Ballhausen D, Baumgartner MR, Beblo S, Burgard P, Chapman KA, Dobbelaere D, Heringer-Seifert J, Fleissner S, Grohmann-Held K, Hahn G, Harting I, Hoffmann GF, Jochum F, Karall D, Konstantopoulous V, Krawinkel MB, Lindner M, Märtner EMC, Nuoffer JM, Okun JG, Plecko B, Posset R, Sahm K, Scholl-Bürgi S, Thimm E, Walter M, Williams M, Vom Dahl S, Ziagaki A, Zschocke J, Kölker S
J Inherit Metab Dis 2023 May;46(3):482-519. Epub 2022 Nov 17 doi: 10.1002/jimd.12566. PMID: 36221165
Amprosi M, Zech M, Steiger R, Nachbauer W, Eigentler A, Gizewski ER, Guger M, Indelicato E, Boesch S
Neurogenetics 2021 Mar;22(1):81-86. Epub 2020 Aug 24 doi: 10.1007/s10048-020-00624-3. PMID: 32830305Free PMC Article
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

Therapy

Costantini A, Trevi E, Pala MI, Fancellu R
BMJ Case Rep 2016 Jul 22;2016 doi: 10.1136/bcr-2016-216721. PMID: 27448549Free PMC Article
Berner J
J Clin Sleep Med 2013 Jan 15;9(1):71-2. doi: 10.5664/jcsm.2346. PMID: 23319908Free PMC Article
Dusek P, Schneider SA
Curr Opin Neurol 2012 Aug;25(4):499-506. doi: 10.1097/WCO.0b013e3283550cac. PMID: 22691760
Hartig MB, Prokisch H, Meitinger T, Klopstock T
Curr Drug Targets 2012 Aug;13(9):1182-9. doi: 10.2174/138945012802002384. PMID: 22515741
Finsterer J
Eur J Paediatr Neurol 2010 Jan;14(1):29-44. Epub 2009 Aug 19 doi: 10.1016/j.ejpn.2009.07.005. PMID: 19692274

Prognosis

Amprosi M, Zech M, Steiger R, Nachbauer W, Eigentler A, Gizewski ER, Guger M, Indelicato E, Boesch S
Neurogenetics 2021 Mar;22(1):81-86. Epub 2020 Aug 24 doi: 10.1007/s10048-020-00624-3. PMID: 32830305Free PMC Article
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
Finsterer J
Eur J Paediatr Neurol 2010 Jan;14(1):29-44. Epub 2009 Aug 19 doi: 10.1016/j.ejpn.2009.07.005. PMID: 19692274
Gregory A, Hayflick SJ
Folia Neuropathol 2005;43(4):286-96. PMID: 16416393Free PMC Article
Naviaux RK
Eur J Pediatr 2000 Dec;159 Suppl 3:S219-26. doi: 10.1007/pl00014407. PMID: 11216904

Clinical prediction guides

Kaiyrzhanov R, Rad A, Lin SJ, Bertoli-Avella A, Kallemeijn WW, Godwin A, Zaki MS, Huang K, Lau T, Petree C, Efthymiou S, Karimiani EG, Hempel M, Normand EA, Rudnik-Schöneborn S, Schatz UA, Baggelaar MP, Ilyas M, Sultan T, Alvi JR, Ganieva M, Fowler B, Aanicai R, Tayfun GA, Al Saman A, Alswaid A, Amiri N, Asilova N, Shotelersuk V, Yeetong P, Azam M, Babaei M, Monajemi GB, Mohammadi P, Samie S, Banu SH, Pinto Basto J, Kortüm F, Bauer M, Bauer P, Beetz C, Garshasbi M, Issa AH, Eyaid W, Ahmed H, Hashemi N, Hassanpour K, Herman I, Ibrohimov S, Abdul-Majeed BA, Imdad M, Isrofilov M, Kaiyal Q, Khan S, Kirmse B, Koster J, Lourenço CM, Mitani T, Moldovan O, Murphy D, Najafi M, Pehlivan D, Rocha ME, Salpietro V, Schmidts M, Shalata A, Mahroum M, Talbeya JK, Taylor RW, Vazquez D, Vetro A, Waterham HR, Zaman M, Schrader TA, Chung WK, Guerrini R, Lupski JR, Gleeson J, Suri M, Jamshidi Y, Bhatia KP, Vona B, Schrader M, Severino M, Guille M, Tate EW, Varshney GK, Houlden H, Maroofian R
Brain 2024 Apr 4;147(4):1436-1456. doi: 10.1093/brain/awad380. PMID: 37951597Free PMC Article
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
Ronchi D, Monfrini E, Bonato S, Mancinelli V, Cinnante C, Salani S, Bordoni A, Ciscato P, Fortunato F, Villa M, Di Fonzo A, Corti S, Bresolin N, Comi GP
Ann Clin Transl Neurol 2020 May;7(5):839-845. Epub 2020 Apr 24 doi: 10.1002/acn3.51025. PMID: 32329585Free PMC Article
Drecourt A, Babdor J, Dussiot M, Petit F, Goudin N, Garfa-Traoré M, Habarou F, Bole-Feysot C, Nitschké P, Ottolenghi C, Metodiev MD, Serre V, Desguerre I, Boddaert N, Hermine O, Munnich A, Rötig A
Am J Hum Genet 2018 Feb 1;102(2):266-277. doi: 10.1016/j.ajhg.2018.01.003. PMID: 29395073Free PMC Article
Costantini A, Trevi E, Pala MI, Fancellu R
BMJ Case Rep 2016 Jul 22;2016 doi: 10.1136/bcr-2016-216721. PMID: 27448549Free PMC Article

Recent systematic reviews

Shi Y, Chen B, Niu S, Wang X, Zhang Z
Orphanet J Rare Dis 2025 Feb 8;20(1):59. doi: 10.1186/s13023-025-03588-5. PMID: 39923090Free PMC Article
Bertini E, Gregg E, Bartlett C, Senthilnathan V, Arber M, Watkins D, Graziadio S, Tomazos I
Orphanet J Rare Dis 2023 Oct 11;18(1):320. doi: 10.1186/s13023-023-02945-6. PMID: 37821990Free PMC Article
He P, Wang Q, Hong X, Yuan H
Am J Med Genet A 2023 Jan;191(1):70-76. Epub 2022 Oct 11 doi: 10.1002/ajmg.a.62988. PMID: 36218002

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