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Spinal myoclonus

MedGen UID:
784934
•Concept ID:
C3697670
•
Disease or Syndrome
Synonym: Spinal cord myoclonus
SNOMED CT: Spinal cord myoclonus (698836007); Spinal myoclonus (698836007)
 
HPO: HP:0010531

Definition

Spinal myoclonus is generally due to a tumor, infection, injury, or degenerative process of the spinal cord, and is characterized by involuntary rhythmic muscle contractions, usually at a rate of more than one per second. Myoclonus occurs synchronously in several muscles and can be increased in severity and frequency by fatigue or stress, but is usually unaffected by sensory stimuli. Spinal myoclonus ceases during sleep or anesthesia. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSpinal myoclonus

Professional guidelines

PubMed

Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children: An International Perspective.
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
Management of patients with myoclonus: available therapies and the need for an evidence-based approach.
Dijk JM, Tijssen MA
Lancet Neurol 2010 Oct;9(10):1028-36. doi: 10.1016/S1474-4422(10)70193-9. PMID: 20864054
RLS-like symptoms: differential diagnosis by history and clinical assessment.
Ferini-Strambi L
Sleep Med 2007 Aug;8 Suppl 2:S3-6. Epub 2007 Jun 12 doi: 10.1016/j.sleep.2007.04.009. PMID: 17567532

Recent clinical studies

Etiology

Distinct movement disorders in contactin-associated-protein-like-2 antibody-associated autoimmune encephalitis.
Gövert F, Abrante L, Becktepe J, Balint B, Ganos C, Hofstadt-van Oy U, Krogias C, Varley J, Irani SR, Paneva S, Titulaer MJ, de Vries JM, Boon AJW, Schreurs MWJ, Joubert B, Honnorat J, Vogrig A, Ariño H, Sabater L, Dalmau J, Scotton S, Jacob S, Melzer N, Bien CG, Geis C, Lewerenz J, Prüss H, Wandinger KP, Deuschl G, Leypoldt F
Brain 2023 Feb 13;146(2):657-667. doi: 10.1093/brain/awac276. PMID: 35875984
Spinal myoclonus: a rare presentation of cervical myelopathy.
Christodoulides I, Giamouriadis A, Bashford J, Barkas K
BMJ Case Rep 2018 Jul 30;2018 doi: 10.1136/bcr-2018-225455. PMID: 30061134Free PMC Article
Electrophysiologic testing aids diagnosis and subtyping of myoclonus.
Zutt R, Elting JW, van Zijl JC, van der Hoeven JH, Roosendaal CM, Gelauff JM, Peall KJ, Tijssen MAJ
Neurology 2018 Feb 20;90(8):e647-e657. Epub 2018 Jan 19 doi: 10.1212/WNL.0000000000004996. PMID: 29352095Free PMC Article
Idiopathic spinal myoclonus: a clinical and neurophysiological assessment of a movement disorder of uncertain origin.
Esposito M, Edwards MJ, Bhatia KP, Brown P, Cordivari C
Mov Disord 2009 Dec 15;24(16):2344-9. doi: 10.1002/mds.22812. PMID: 19908306
A case of primary spinal myoclonus: clinical presentation and possible mechanisms involved.
Campos CR, Limongi JC, Machado FC, Brotto MW
Arq Neuropsiquiatr 2003 Mar;61(1):112-4. Epub 2003 Apr 16 doi: 10.1590/s0004-282x2003000100022. PMID: 12715032

Diagnosis

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes.
Antelmi E, Provini F
Sleep Med Rev 2015 Aug;22:54-63. Epub 2014 Nov 6 doi: 10.1016/j.smrv.2014.10.007. PMID: 25500332
Myoclonus.
Espay AJ, Chen R
Continuum (Minneap Minn) 2013 Oct;19(5 Movement Disorders):1264-86. doi: 10.1212/01.CON.0000436156.54532.1a. PMID: 24092290
Symptomatic myoclonus.
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
Therapy of myoclonus.
Obeso JA
Clin Neurosci 1995-1996;3(4):253-7. PMID: 8891399
Propriospinal myoclonus.
Chokroverty S
Clin Neurosci 1995-1996;3(4):219-22. PMID: 8891395

Therapy

Spinal Myoclonus: Is It An Anesthetic Mystery?
Silva RM, Morais IG, Almeida AV, Pereira OM, Silva AM, Sá EC
Rev Esp Anestesiol Reanim (Engl Ed) 2020 Feb;67(2):108-111. Epub 2019 Nov 20 doi: 10.1016/j.redar.2019.08.001. PMID: 31759616
Spinal myoclonus following neuraxial anesthesia: a literature review.
Shiratori T, Hotta K, Satoh M
J Anesth 2019 Feb;33(1):140-147. Epub 2019 Jan 6 doi: 10.1007/s00540-018-02607-z. PMID: 30613902
Myoclonus.
Espay AJ, Chen R
Continuum (Minneap Minn) 2013 Oct;19(5 Movement Disorders):1264-86. doi: 10.1212/01.CON.0000436156.54532.1a. PMID: 24092290
Spinal myoclonus.
Hoehn MM, Cherington M
Neurology 1977 Oct;27(10):942-6. doi: 10.1212/wnl.27.10.942. PMID: 561907
Spinal myoclonus.
Hopkins AP, Michael WF
J Neurol Neurosurg Psychiatry 1974 Oct;37(10):1112-5. doi: 10.1136/jnnp.37.10.1112. PMID: 4443806Free PMC Article

Prognosis

Electrophysiologic testing aids diagnosis and subtyping of myoclonus.
Zutt R, Elting JW, van Zijl JC, van der Hoeven JH, Roosendaal CM, Gelauff JM, Peall KJ, Tijssen MAJ
Neurology 2018 Feb 20;90(8):e647-e657. Epub 2018 Jan 19 doi: 10.1212/WNL.0000000000004996. PMID: 29352095Free PMC Article
Spinal segmental myoclonus as an unusual presentation of multiple sclerosis.
Alroughani RA, Ahmed SF, Khan RA, Al-Hashel JY
BMC Neurol 2015 Feb 27;15:15. doi: 10.1186/s12883-015-0271-y. PMID: 25879483Free PMC Article
Spinal myoclonus after spinal cord injury.
Calancie B
J Spinal Cord Med 2006;29(4):413-24. doi: 10.1080/10790268.2006.11753891. PMID: 17044393Free PMC Article
Movement disorders in 28 HIV-infected patients.
Mattos JP, Rosso AL, Correa RB, Novis SA
Arq Neuropsiquiatr 2002 Sep;60(3-A):525-30. PMID: 12244384
Spinal myoclonus resembling belly dance.
Kono I, Ueda Y, Araki K, Nakajima K, Shibasaki H
Mov Disord 1994 May;9(3):325-9. doi: 10.1002/mds.870090309. PMID: 8041373

Clinical prediction guides

Distinct movement disorders in contactin-associated-protein-like-2 antibody-associated autoimmune encephalitis.
Gövert F, Abrante L, Becktepe J, Balint B, Ganos C, Hofstadt-van Oy U, Krogias C, Varley J, Irani SR, Paneva S, Titulaer MJ, de Vries JM, Boon AJW, Schreurs MWJ, Joubert B, Honnorat J, Vogrig A, Ariño H, Sabater L, Dalmau J, Scotton S, Jacob S, Melzer N, Bien CG, Geis C, Lewerenz J, Prüss H, Wandinger KP, Deuschl G, Leypoldt F
Brain 2023 Feb 13;146(2):657-667. doi: 10.1093/brain/awac276. PMID: 35875984
Spinal Myoclonus: Is It An Anesthetic Mystery?
Silva RM, Morais IG, Almeida AV, Pereira OM, Silva AM, Sá EC
Rev Esp Anestesiol Reanim (Engl Ed) 2020 Feb;67(2):108-111. Epub 2019 Nov 20 doi: 10.1016/j.redar.2019.08.001. PMID: 31759616
Clinical neurophysiology of psychogenic movement disorders: how to diagnose psychogenic tremor and myoclonus.
Apartis E
Neurophysiol Clin 2014 Oct;44(4):417-24. Epub 2013 Sep 13 doi: 10.1016/j.neucli.2013.08.014. PMID: 25306082
Symptomatic myoclonus.
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
Segmental myoclonus. Clinical and pharmacologic study.
Jankovic J, Pardo R
Arch Neurol 1986 Oct;43(10):1025-31. doi: 10.1001/archneur.1986.00520100039012. PMID: 3753263

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