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Opsoclonus

MedGen UID:
116616
Concept ID:
C0242567
Disease or Syndrome
Synonym: Opsoclonia
SNOMED CT: Opsoclonia (194177006); Opsoclonus (194177006)
 
HPO: HP:0010543

Definition

Bursts of large-amplitude multidirectional saccades without intersaccadic interval [from HPO]

Term Hierarchy

Conditions with this feature

Spinocerebellar ataxia type 21
MedGen UID:
375311
Concept ID:
C1843891
Disease or Syndrome
Spinocerebellar ataxia-21 (SCA21) is an autosomal dominant neurologic disorder characterized by onset in the first decades of life of slowly progressive cerebellar ataxia, which is associated with cognitive impairment in most patients (summary by Delplanque et al., 2014). For a general discussion of autosomal dominant spinocerebellar ataxia, see SCA1 (164400).
Neuroblastoma, susceptibility to, 1
MedGen UID:
412713
Concept ID:
C2749485
Finding
Neurodevelopmental disorder with eye movement abnormalities and ataxia
MedGen UID:
1824014
Concept ID:
C5774241
Disease or Syndrome
Neurodevelopmental disorder with eye movement abnormalities and ataxia (NEDEMA) is characterized by global developmental delay apparent from infancy. Affected individuals show delayed walking with an unsteady gait, variably impaired intellectual development, learning disabilities, and speech difficulties. Abnormal eye movements, which are often noted in early childhood, include opsoclonus, nystagmus, and strabismus. Some patients have seizures, which may be refractory (Lu et al., 2022).

Professional guidelines

PubMed

Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free PMC Article
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article

Recent clinical studies

Etiology

Abbatemarco JR, Vedeler CA, Greenlee JE
Handb Clin Neurol 2024;200:173-191. doi: 10.1016/B978-0-12-823912-4.00030-X. PMID: 38494276
Meira AT, de Moraes MPM, Ferreira MG, Franklin GL, Rezende Filho FM, Teive HAG, Barsottini OGP, Pedroso JL
Parkinsonism Relat Disord 2023 Dec;117:105861. Epub 2023 Sep 20 doi: 10.1016/j.parkreldis.2023.105861. PMID: 37748994
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free PMC Article
Joubert B, Rostásy K, Honnorat J
Handb Clin Neurol 2018;155:313-332. doi: 10.1016/B978-0-444-64189-2.00021-4. PMID: 29891069

Diagnosis

Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Bhatia P, Heim J, Cornejo P, Kane L, Santiago J, Kruer MC
J Neurol 2022 Feb;269(2):750-757. Epub 2021 Mar 29 doi: 10.1007/s00415-021-10536-3. PMID: 33779841
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free PMC Article
Oh SY, Kim JS, Dieterich M
J Neurol 2019 Jun;266(6):1541-1548. Epub 2018 Nov 27 doi: 10.1007/s00415-018-9138-7. PMID: 30483882
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article

Therapy

Basu S, Chakravarty A
Curr Neurol Neurosci Rep 2022 Aug;22(8):491-498. Epub 2022 Jun 21 doi: 10.1007/s11910-022-01215-5. PMID: 35727462
Pranzatelli MR, Tate ED
Brain Dev 2016 May;38(5):439-48. Epub 2016 Jan 16 doi: 10.1016/j.braindev.2015.11.007. PMID: 26786246
Pike M
Handb Clin Neurol 2013;112:1209-11. doi: 10.1016/B978-0-444-52910-7.00042-8. PMID: 23622330
Borker A, Choudhary N
Indian Pediatr 2011 Aug;48(8):627-32. doi: 10.1007/s13312-011-0098-6. PMID: 21918268
Wong A
Curr Opin Neurol 2007 Feb;20(1):25-31. doi: 10.1097/WCO.0b013e3280126b51. PMID: 17215685

Prognosis

Parrado-Carrillo A, Alcubierre R, Camós-Carreras A, Sanchez-Dalmau BF
Arch Soc Esp Oftalmol (Engl Ed) 2022 Jun;97(6):350-357. Epub 2022 Mar 12 doi: 10.1016/j.oftale.2022.03.006. PMID: 35292226
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article
Pike M
Handb Clin Neurol 2013;112:1209-11. doi: 10.1016/B978-0-444-52910-7.00042-8. PMID: 23622330
López-Ibor B, Schwartz AD
Pediatr Clin North Am 1985 Jun;32(3):755-78. doi: 10.1016/s0031-3955(16)34835-0. PMID: 3889804

Clinical prediction guides

Rosenberg MI, Greenstein E, Buchkovich M, Peres A, Santoni-Rugiu E, Yang L, Mikl M, Vaksman Z, Gibbs DL, Reshef D, Salovin A, Irwin MS, Naranjo A, Ulitsky I, de Alarcon PA, Matthay KK, Weigman V, Yaari G, Panzer JA, Friedman N, Maris JM
Cell Rep 2023 Aug 29;42(8):112879. Epub 2023 Aug 2 doi: 10.1016/j.celrep.2023.112879. PMID: 37537844Free PMC Article
Dinkin M, Sathi S
Curr Opin Ophthalmol 2022 Nov 1;33(6):471-484. Epub 2022 Sep 13 doi: 10.1097/ICU.0000000000000904. PMID: 36165417
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free PMC Article
Blaes F, Tschernatsch M
Expert Rev Neurother 2010 Oct;10(10):1559-68. doi: 10.1586/ern.10.134. PMID: 20925471
Borg M
Neurophysiol Clin 2006 Sep-Dec;36(5-6):309-18. Epub 2007 Jan 17 doi: 10.1016/j.neucli.2006.12.006. PMID: 17336775

Recent systematic reviews

Amod F, Holla VV, Ojha R, Pandey S, Yadav R, Pal PK
Parkinsonism Relat Disord 2023 Sep;114:105774. Epub 2023 Jul 28 doi: 10.1016/j.parkreldis.2023.105774. PMID: 37532621
Allahyari F, Molaee H, Hosseini Nejad J
Z Naturforsch C J Biosci 2023 Jan 27;78(1-2):1-8. Epub 2022 Sep 12 doi: 10.1515/znc-2022-0092. PMID: 36087300
Sturchio A, Dwivedi AK, Gastaldi M, Grimberg MB, Businaro P, Duque KR, Vizcarra JA, Abdelghany E, Balint B, Marsili L, Espay AJ
J Neurol 2022 Jul;269(7):3511-3521. Epub 2022 Jan 13 doi: 10.1007/s00415-021-10934-7. PMID: 35024921Free PMC Article
Gadian J, Kirk E, Holliday K, Lim M, Absoud M
Dev Med Child Neurol 2017 Feb;59(2):136-144. Epub 2016 Nov 30 doi: 10.1111/dmcn.13349. PMID: 27900773
Ben-Pazi H, Jaworowski S, Shalev RS
Dev Med Child Neurol 2011 Dec;53(12):1077-84. Epub 2011 Sep 27 doi: 10.1111/j.1469-8749.2011.04134.x. PMID: 21950517

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