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Episodic kinesigenic dyskinesia 3(EKD3)

MedGen UID:
1840916
Concept ID:
C5830280
Disease or Syndrome
Synonyms: DYSTONIA 36; EKD3
 
Gene (location): TMEM151A (11q13.2)
 
Monarch Initiative: MONDO:0859380
OMIM®: 620245

Definition

Episodic kinesigenic dyskinesia-3 (EKD3) is an autosomal dominant form of paroxysmal kinesigenic dyskinesia (PKD), an episodic involuntary movement disorder characterized by dystonia, chorea, athetosis, and other hyperkinetic movements. The age at onset is around 9 to 12 years of age and symptoms are usually triggered by sudden movement or stress. Most patients have spontaneous resolution of episodes in their early twenties or later. Brain imaging is normal. There is a favorable response to treatment with carbamazepine (Li et al., 2021; Tian et al., 2022; Wang et al., 2022). For a general phenotypic description and a discussion of genetic heterogeneity of episodic kinesigenic dyskinesia (EKD), see EKD1 (128200). [from OMIM]

Clinical features

From HPO
Dystonic disorder
MedGen UID:
3940
Concept ID:
C0013421
Sign or Symptom
An abnormally increased muscular tone that causes fixed abnormal postures. There is a slow, intermittent twisting motion that leads to exaggerated turning and posture of the extremities and trunk.
Torticollis
MedGen UID:
11859
Concept ID:
C0040485
Sign or Symptom
Torticollis is a twisted neck as a result of shortening of sternocleidomastoid muscle. This short and fibrotic muscle pulls the head laterally and rotates the chin and face to the opposite end. Facial asymmetry may be a manifestation (summary by Engin et al., 1997).
Choreoathetosis
MedGen UID:
39313
Concept ID:
C0085583
Disease or Syndrome
Involuntary movements characterized by both athetosis (inability to sustain muscles in a fixed position) and chorea (widespread jerky arrhythmic movements).
Involuntary movements
MedGen UID:
140884
Concept ID:
C0427086
Sign or Symptom
Involuntary contractions of muscle leading to involuntary movements of extremities, neck, trunk, or face.

Professional guidelines

PubMed

Tsai JD, Chou IC, Tsai FJ, Kuo HT, Tsai CH
Acta Paediatr Taiwan 2005 May-Jun;46(3):138-42. PMID: 16231560

Recent clinical studies

Etiology

Tian WT, Huang XJ, Mao X, Liu Q, Liu XL, Zeng S, Guo XN, Shen JY, Xu YQ, Tang HD, Yin XM, Zhang M, Tang WG, Liu XR, Tang BS, Chen SD, Cao L
Mov Disord 2018 Mar;33(3):459-467. Epub 2018 Jan 22 doi: 10.1002/mds.27274. PMID: 29356177
Lowrie M, Garosi L
Vet J 2017 Feb;220:65-71. Epub 2017 Jan 3 doi: 10.1016/j.tvjl.2016.12.017. PMID: 28190498
Caraballo RH, Cersósimo RO, Espeche A, Fejerman N
Epileptic Disord 2003 Mar;5(1):45-9. PMID: 12773296
Guerrini R, Sanchez-Carpintero R, Deonna T, Santucci M, Bhatia KP, Moreno T, Parmeggiani L, Bernardina BD
Epilepsia 2002 Oct;43(10):1224-9. doi: 10.1046/j.1528-1157.2002.13802.x. PMID: 12366739
Blakeley J, Jankovic J
Mov Disord 2002 Jul;17(4):726-34. doi: 10.1002/mds.10178. PMID: 12210862

Diagnosis

Trapp SD, Noachtar S, Kaufmann E
BMJ Case Rep 2022 Mar 29;15(3) doi: 10.1136/bcr-2021-247760. PMID: 35351750Free PMC Article
Tian WT, Huang XJ, Mao X, Liu Q, Liu XL, Zeng S, Guo XN, Shen JY, Xu YQ, Tang HD, Yin XM, Zhang M, Tang WG, Liu XR, Tang BS, Chen SD, Cao L
Mov Disord 2018 Mar;33(3):459-467. Epub 2018 Jan 22 doi: 10.1002/mds.27274. PMID: 29356177
Gardiner AR, Bhatia KP, Stamelou M, Dale RC, Kurian MA, Schneider SA, Wali GM, Counihan T, Schapira AH, Spacey SD, Valente EM, Silveira-Moriyama L, Teive HA, Raskin S, Sander JW, Lees A, Warner T, Kullmann DM, Wood NW, Hanna M, Houlden H
Neurology 2012 Nov 20;79(21):2115-21. Epub 2012 Oct 17 doi: 10.1212/WNL.0b013e3182752c5a. PMID: 23077024Free PMC Article
Riant F, Roze E, Barbance C, Méneret A, Guyant-Maréchal L, Lucas C, Sabouraud P, Trébuchon A, Depienne C, Tournier-Lasserve E
Neurology 2012 Nov 20;79(21):2122-4. Epub 2012 Oct 17 doi: 10.1212/WNL.0b013e3182752cb8. PMID: 23077016
Blakeley J, Jankovic J
Mov Disord 2002 Jul;17(4):726-34. doi: 10.1002/mds.10178. PMID: 12210862

Therapy

Trapp SD, Noachtar S, Kaufmann E
BMJ Case Rep 2022 Mar 29;15(3) doi: 10.1136/bcr-2021-247760. PMID: 35351750Free PMC Article
Micheli F, Tschopp L, Cersosimo MG
Clin Neuropharmacol 2011 Nov-Dec;34(6):262-4. doi: 10.1097/WNF.0b013e3182348964. PMID: 22104638
Tsai JD, Chou IC, Tsai FJ, Kuo HT, Tsai CH
Acta Paediatr Taiwan 2005 May-Jun;46(3):138-42. PMID: 16231560
Caraballo RH, Cersósimo RO, Espeche A, Fejerman N
Epileptic Disord 2003 Mar;5(1):45-9. PMID: 12773296
Guerrini R, Sanchez-Carpintero R, Deonna T, Santucci M, Bhatia KP, Moreno T, Parmeggiani L, Bernardina BD
Epilepsia 2002 Oct;43(10):1224-9. doi: 10.1046/j.1528-1157.2002.13802.x. PMID: 12366739

Prognosis

Tsai JD, Chou IC, Tsai FJ, Kuo HT, Tsai CH
Acta Paediatr Taiwan 2005 May-Jun;46(3):138-42. PMID: 16231560
Caraballo RH, Cersósimo RO, Espeche A, Fejerman N
Epileptic Disord 2003 Mar;5(1):45-9. PMID: 12773296
Singh R, Macdonell RA, Scheffer IE, Crossland KM, Berkovic SF
Epileptic Disord 1999 Jun;1(2):93-9. PMID: 10937138

Clinical prediction guides

Valente P, Marte A, Franchi F, Sterlini B, Casagrande S, Corradi A, Baldelli P, Benfenati F
Mol Neurobiol 2023 Mar;60(3):1281-1296. Epub 2022 Nov 28 doi: 10.1007/s12035-022-03112-x. PMID: 36441479Free PMC Article
Pellegrin S, Cantalupo G, Opri R, Dalla Bernardina B, Darra F
Eur J Paediatr Neurol 2017 May;21(3):580-582. Epub 2017 Jan 17 doi: 10.1016/j.ejpn.2017.01.002. PMID: 28129950
Liu D, Zhang Y, Wang Y, Chen C, Li X, Zhou J, Song Z, Xiao B, Rasco K, Zhang F, Wen S, Li G
Sci Rep 2016 May 13;6:25790. doi: 10.1038/srep25790. PMID: 27173777Free PMC Article
Delcourt M, Riant F, Mancini J, Milh M, Navarro V, Roze E, Humbertclaude V, Korff C, Des Portes V, Szepetowski P, Doummar D, Echenne B, Quintin S, Leboucq N, Singh Amrathlal R, Rochette J, Roubertie A
J Neurol Neurosurg Psychiatry 2015 Jul;86(7):782-5. Epub 2015 Jan 16 doi: 10.1136/jnnp-2014-309025. PMID: 25595153
Singh R, Macdonell RA, Scheffer IE, Crossland KM, Berkovic SF
Epileptic Disord 1999 Jun;1(2):93-9. PMID: 10937138

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