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Posthypoxic Myoclonus and Its Management

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

Posthypoxic myoclonus (PHM) is a possible sequela of acute hypoxic events. An acute form and a chronic form (the latter also referred to as Lance-Adams syndrome, LAS) have been described. Acute PHM is usually associated with a poor prognosis. LAS, instead, is characterized by myoclonus presenting or persisting as patients regain consciousness. It can improve over time, and when additional neurological symptoms are present those are usually mild. Differentiating these two phenotypes based on clinical and neurophysiological assessments (such as electroencephalogram recordings and somatosensory evoked potentials) is not always straightforward, although very important because of the different prognostic implications. There are differences in therapeutic approaches for acute and chronic PHM because of the different nature of the myoclonus (cortical vs. subcortical or a combination of the two) and concurrent events. Multidrug approaches are usually required in both settings, mostly leveraging antiepileptic medications. In patients with persistent and debilitating LAS, multistep and even surgical approaches, such as deep brain stimulation, can be attempted to maximize functional recovery.

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References

  1. Snyder BD, Hauser WA, Loewenson RB, Leppik IE, Ramirez-Lassepas M, Gumnit RJ. Neurologic prognosis after cardiopulmonary arrest: III. Seizure activity. Neurology. 1980;30:1292–7.

    Article  CAS  PubMed  Google Scholar 

  2. Wijdicks EFM, Parisi JE, Sharbrough FW. Prognostic value of myoclonus status in comatose survivors of cardiac arrest. Ann Neurol. 1994;35:239–43.

    Article  CAS  PubMed  Google Scholar 

  3. Bouwes A, van Poppelen D, Koelman JH, Kuiper MA, Zandstra DF, Weinstein HC, et al. Acute posthypoxic myoclonus after cardiopulmonary resuscitation. BMC Neurol. 2012;12:63.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Oddo M, Rossetti AO. Early multimodal outcome prediction after cardiac arrest in patients treated with hypothermia. Crit Care Med. 2014;42:1340–7.

    Article  PubMed  Google Scholar 

  5. Reynolds AS, Rohaut B, Holmes MG, Robinson D, Roth W, Velazquez A, et al. Early myoclonus following anoxic brain injury. Neurol Clin Pract. 2018;8(3):249–56.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Freund B, Sutter R, Kaplan PW. Lance-adams syndrome in the pretargeted temperature management era. Clin EEG Neurosci. 2017;48(2):130–8.

    Article  PubMed  Google Scholar 

  7. Wijdicks EFM, Hijdra A, Young GB, Bassetti CL, Wiebe S. Practice parameter: prediction of outcome in comatose survivors after cardiopulmonary resuscitation (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2006;67:203–10.

    Article  CAS  PubMed  Google Scholar 

  8. Werhahn KJ, Brown P, Thompson PD, Marsden CD. The clinical features and prognosis of chronic posthypoxic myoclonus. Mov Disord. 1997;12:216–20.

    Article  CAS  PubMed  Google Scholar 

  9. Frucht S, Fahn S. The clinical spectrum of posthypoxic myoclonus. Mov Disord. 2000;15:2–7.

    Article  PubMed  Google Scholar 

  10. Sugama S, Kusano K. A case of dyskinetic cerebral palsy resembling post-anoxic action myoclonus. Brain and Development. 1995;17:210–2.

    Article  CAS  PubMed  Google Scholar 

  11. Obeso JA, Lang AE, Rothwell JC, Marsden CD. Postanoxic symptomatic oscillatory myoclonus. Neurology. 1983;33:240–3.

    Article  CAS  PubMed  Google Scholar 

  12. Kakisaka Y, Haginoya K, Togashi N, Kitamiura T, Uematsu M, Hino-Fukuyo N, et al. Neonatal-onset brainstem reticular reflex myoclonus following a prenatal brain insult: generalized myoclonic jerk and a brainstem lesion. Tohoku J Exp Med. 2007;211:303–8.

    Article  PubMed  Google Scholar 

  13. Ong MT, Sarrigiannis PG, Baxter PS. Post-anoxic reticular reflex myoclonus in a child and proposed classification of post-anoxic myoclonus. Pediatr Neurol. 2020;68:68–72.

    Article  Google Scholar 

  14. Hallett M. Physiology of human posthypoxic myoclonus. Mov Disord. 2000;15:8–13.

    Article  PubMed  Google Scholar 

  15. Lance JW, Adams RD. The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy. Brain. 1963;86:111–36.

    Article  CAS  PubMed  Google Scholar 

  16. Mikhaeil-Demo Y, Gavvala JR, Bellinski II, Macken MP, Narechania A, Templer JW, et al. Clinical classification of post anoxic myoclonic status. Resuscitation. 2017;119:76–80.

    Article  PubMed  Google Scholar 

  17. Hallett M, Chadwick D, Adam J, Marsden CD. Reticular reflex myoclonus: a physiological type of human post hypoxic myoclonus. J Neurol Neurosurg Psychiatry. 1977;40:253–64.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Brown P, Thompson PD, Rothwell JC, Day BL, Marsden CD. A case of postanoxic encephalopathy with cortical action and brainstem reticular reflex myoclonus. Mov Disord. 1991;6:139–44.

    Article  CAS  PubMed  Google Scholar 

  19. Sandroni C, Cariou A, Cavallaro F, Cronberg T, Friberg H, Hoedemaekers C, et al. Prognostication in comatose survivors of cardiac arrest: an advisory statement from the European Resuscitation Council and the European Society of Intensive Care Medicine. Resuscitation. 2014;85:1779–89.

    Article  PubMed  Google Scholar 

  20. Fernandez-Torre JL, González-Rato J, Martínez-Martínez M. Acute stimulus-sensitive postanoxic myoclonus: description of a case. Clin EEG Neurosci. 2005;36:199–201.

    Article  CAS  PubMed  Google Scholar 

  21. Fernández-Torre JL, Hernández-Hernández MA. Acute stimulus-sensitive postanoxic myoclonus: the importance of sensory stimulation in comatose patients. Clin EEG Neurosci. 2012;43:312–4.

    Article  PubMed  Google Scholar 

  22. Niedermeyer E, Bauer G, Burnite R, Reichenbach D. Selective stimulus-sensitive myoclonus in acute cerebral anoxia: a case report. Arch Neurol. 1977;34:365–8.

    Article  CAS  PubMed  Google Scholar 

  23. Zivkovic SA, Brenner RP. A case of area-specific stimulus-sensitive postanoxic myoclonus. J Clin Neurophysiol. 2003;20:111–6.

    Article  PubMed  Google Scholar 

  24. Bruno MK, Lee HY, Auburger GWJ, Friedman A, Nielsen JE, Lang AE, et al. Genotype-phenotype correlation of paroxysmal nonkinesigenic dyskinesia. Neurology. 2007;68:1782–9.

    Article  CAS  PubMed  Google Scholar 

  25. Cott AC, Blatt I, Brenner RP. Stimulus-sensitive seizures in postanoxic coma. Epilepsia. 1996;37:868–74.

    Article  PubMed  Google Scholar 

  26. Harper SJ, Wilkes RG. Posthypoxic myoclonus (the Lance-Adams syndrome) in the intensive care unit. Anaesthesia. 1991;46:199–201.

    Article  CAS  PubMed  Google Scholar 

  27. Gupta HV, Caviness JN. Post-hypoxic myoclonus: current concepts, neurophysiology, and treatment. Tremor Other Hyperkinet Mov (N Y). 2016;6:409.

    Article  Google Scholar 

  28. Fahn S. Posthypoxic action myoclonus: literature review update. Adv Neurol. 1986;43:157–69.

    CAS  PubMed  Google Scholar 

  29. Aicua Rapun I, Novy J, Solari D, Oddo M, Rossetti AO. Early Lance–Adams syndrome after cardiac arrest: prevalence, time to return to awareness, and outcome in a large cohort. Resuscitation. 2017;115:169–72.

    Article  PubMed  Google Scholar 

  30. Young GB, Gilbert JJ, Zochodne DW. The significance of myoclonic status epilepticus in postanoxic coma. Neurology. 1990;40:1843–8.

    Article  CAS  PubMed  Google Scholar 

  31. Budhram A, Lipson D, Nesathurai S, Harvey D, Rathbone MP. Postanoxic myoclonus: two case presentations and review of medical management. Arch Phys Med Rehabil. 2014;95:588–90.

    Article  PubMed  Google Scholar 

  32. Dekker MCJ, Kilonzo KG, Howlett WP, Guttman M, Cubo E. Inspiratory myoclonus. Tremor Other Hyperkinet Mov (N Y). 2019;9:625.

    Article  Google Scholar 

  33. Gasca-Salas C, Lang AE. Focal predominant forms of posthypoxic action myoclonus. Mov Disord Clin Pract. 2016;3:417–20.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Chee YC, Poh SC. Myoclonus following severe asthma: clonazepam relieves. Aust NZ J Med. 1983;13:285–6.

    Article  CAS  Google Scholar 

  35. Magnussen I, Dupont E, Engbaek F, De Fine Olivarius B. Post-hypoxic intention myoclonus treated with 5-hydroxytryptophan and an extracerebral decarboxylase inhibitor. Acta Neurol Scand. 1978;57:289–94.

    Article  CAS  PubMed  Google Scholar 

  36. Magnussen I, Mondrup K, Engbék F, Lademann A, de Fine Olivarius B. Treatment of myoclonic syndromes with paroxetine alone or combined with 5-HTP. Acta Neurol Scand. 1982;66:276–82.

    Article  CAS  PubMed  Google Scholar 

  37. Huang HC, Chen JC, Lu MK, Chen JM, Tsai CH. Post-hypoxic cortical myoclonus mimicking spinal myoclonus – electrophysiological and functional MRI manifestations. Eur J Neurol. 2011;18(1):e4–5.

    Article  PubMed  Google Scholar 

  38. Yamada K, Sakurama T, Soyama N, Kuratsu JI. GPi Pallidal stimulation for Lance-Adams syndrome. Neurology. 2011;76:1270–2.

    Article  PubMed  Google Scholar 

  39. Legriel S, Le Stang MB, Merceron S, Cronier P, Troche G. Ongoing abdominal status myoclonus in postanoxic coma. Neurocrit Care. 2012;16:136–8.

    Article  PubMed  Google Scholar 

  40. Cho AR, Kwon JY, Kim JY, Kim ES, Kim HY. Acute onset Lance-Adams syndrome following brief exposure to severe hypoxia without cardiac arrest – a case report. Korean J Anesthesiol. 2013;65:341–4.

    Article  PubMed  PubMed Central  Google Scholar 

  41. van Zijl JC, Beudel M, Elting JJ, de Jong BM, van der Naalt J, van den Bergh WM, et al. The inter-rater variability of clinical assessment in post-anoxic myoclonus. Tremor Other Hyperkinet Mov (N Y). 2017;7:470.

    Article  Google Scholar 

  42. Kanemoto K, Ozawa K. A case of post-anoxic encephalopathy with initial massive myoclonic status followed by alternating Jacksonian seizures. Seizure. 2000;9:352–5.

    Article  CAS  PubMed  Google Scholar 

  43. Elmer J, Rittenberger JC, Faro J, Molyneaux BJ, Popescu A, Callaway CW, et al. Clinically distinct electroencephalographic phenotypes of early myoclonus after cardiac arrest. Ann Neurol. 2016;80:175–84.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Sandroni C, Cavallaro F, Callaway CW, Sanna T, D’Arrigo S, Kuiper M, et al. Predictors of poor neurological outcome in adult comatose survivors of cardiac arrest: a systematic review and meta-analysis. Part 1: patients not treated with therapeutic hypothermia. Resuscitation. 2013;84(10):1310–23.

    Article  PubMed  Google Scholar 

  45. Sandroni C, Cavallaro F, Callaway CW, D’Arrigo S, Sanna T, Kuiper MA, et al. Predictors of poor neurological outcome in adult comatose survivors of cardiac arrest: a systematic review and meta-analysis. Part 2: patients treated with therapeutic hypothermia. Resuscitation. 2013;84(10):1324–38.

    Article  PubMed  Google Scholar 

  46. Rossetti AO, Oddo M, Logroscino G, Kaplan PW. Prognostication after cardiac arrest and hypothermia: a prospective study. Ann Neurol. 2010;67:301–7.

    PubMed  Google Scholar 

  47. Tassinari CA, Coccagna G, Mantovani M, Dalla Bernardina B, Roger J. Polygraphic study of dyssynergia cerebellaris myoclonica (Ramsay-Hunt syndrome) and of the intention myoclonus (lance-adams syndrome) during sleep. Eur Neurol. 1973;9:105–20.

    Article  CAS  PubMed  Google Scholar 

  48. Witte OW, Niedermeyer E, Arendt G, Freund HJ. Post-hypoxic action (intention) myoclonus: a clinico-electroencephalographic study. J Neurol. 1988;235:214–8.

    Article  CAS  PubMed  Google Scholar 

  49. Waddell A, Dirweesh A, Ordonez F, Kososky C, Reddy Peddareddygari L, Grewal RP. Lance–Adams syndrome associated with cerebellar pathology. J Community Hosp Intern Med Perspect. 2017;7:182–4.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Ferlazzo E, Gasparini S, Cianci V, Cherubini A, Aguglia U. Serial MRI findings in brain anoxia leading to Lance-Adams syndrome: a case report. Neurol Sci. 2013;34:2047–50.

    Article  PubMed  Google Scholar 

  51. Frucht SJ, Trošt M, Ma Y, Eidelberg D. The metabolic topography of posthypoxic myoclonus. Neurology. 2004;62:1879–81.

    Article  PubMed  Google Scholar 

  52. Zhang YX, Liu JR, Jiang B, Liu HQ, Ding MP, Song SJ, et al. Lance-Adams syndrome: a report of two cases. J Zhejiang Univ Sci B. 2007;8(10):715–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Park KM, Han YH, Kim TH, Mun CW, Shin KJ, Ha SY, et al. Increased functional connectivity between motor and sensory cortex in a patient with Lance-Adams syndrome. Clin Neurol Neurosurg. 2015;139:241–3.

    Article  PubMed  Google Scholar 

  54. Wolf P. Periodic synchronous and stereotyped myoclonus with postanoxic coma. J Neurol. 1977;215:39–47.

    Article  CAS  PubMed  Google Scholar 

  55. De Léan J, Richardson JC, Rewcastle NB. Pathological findings in a case of hypoxic myoclonus treated with 5-hydroxytryptophan and a decarboxylase inhibitor. Adv Neurol. 1986;43:215–23.

    PubMed  Google Scholar 

  56. Hauw JJ, Escourolle R, Baulac M, Morel-Maroger A, Goulon M, Castaigne P. Postmortem studies on posthypoxic and post-methyl bromide intoxication: case reports. Adv Neurol. 1986;43:201–14.

    CAS  PubMed  Google Scholar 

  57. Lhermitte F, Talairach J, Buser P, Gautier JC, Bancaud J, Gras R, et al. Myoclonies d’intention et d’acton post-anoxiques. Etude stéréotaxique et destruction du noyau ventral latéral du thalamus [Postanoxic-action and intention myoclonus. Stereotaxic study and destruction of the lateral ventral nucleus of the thalamus]. Rev Neurol (Paris). 1971;124(1):5–20. French

    CAS  Google Scholar 

  58. Quillinan N, Grewal H, Deng G, Shimizu K, Yonchek JC, Strnad F, et al. Region-specific role for GluN2B-containing NMDA receptors in injury to Purkinje cells and CA1 neurons following global cerebral ischemia. Neuroscience. 2015;284:555–65.

    Article  CAS  PubMed  Google Scholar 

  59. Geocadin RG, Muthuswamy J, Sherman DL, Thakor NV, Hanley DF. Early electrophysiological and histologic changes after global cerebral ischemia in rats. Mov Disord. 2000;15:14–21.

    Article  PubMed  Google Scholar 

  60. Tai KK, Bhidayasiri R, Truong DD. Post-hypoxic animal model of myoclonus. Parkinsonism Relat Disord. 2007;13(7):377–81.

    Article  PubMed  Google Scholar 

  61. Paine MG, Che D, Li L, Neumar RW. Cerebellar Purkinje cell neurodegeneration after cardiac arrest: effect of therapeutic hypothermia. Resuscitation. 2012;83:1511–6.

    Article  PubMed  Google Scholar 

  62. Guilleminault C, Tharp BR, Cousin D. HVA and 5HIAA CSF measurements and 5HTP trials in some patients with involuntary movements. J Neurol Sci. 1973;18:435–41.

    Article  CAS  PubMed  Google Scholar 

  63. Matsumoto RR, Truong DD, Nguyen KD, Dang AT, Hoang TT, Vo PQ, et al. Involvement of GABAA receptors in myoclonus. Mov Disord. 2000;15:47–52.

    Article  PubMed  Google Scholar 

  64. Tarsy D, Pycock CJ, Meldrum BS, Marsden CD. Focal contralateral myoclonus produced by inhibition of GABA action in the caudate nucleus of rats. Brain. 1978;101(1):143–62.

    Article  CAS  PubMed  Google Scholar 

  65. Jaw SP, Nguyen B, Vuong QTV, Trinh TA, Nguyen M, Truong DD. Effects of GABA uptake inhibitors on posthypoxic myoclonus in rats. Brain Res Bull. 1996;39:189–92.

    Article  CAS  PubMed  Google Scholar 

  66. Pappert EJ, Goetz CG, Vu TQ, Ling ZD, Leurgans S, Raman R, et al. Animal model of posthypoxic myoclonus: effects of serotonergic antagonists. Neurology. 1999;52:16–21.

    Article  CAS  PubMed  Google Scholar 

  67. Carroll WM, Walsh PJ. Functional independence in post-anoxic myoclonus: contribution of L-5-HTP sodium valproate and clonazepam. Br Med J. 1978;2(6152):1612.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Van Woert and myoclonus – Search Results – PubMed [Internet]. [cited 2020 May 31]. Available from: https://pubmed.ncbi.nlm.nih.gov/?term=VanWoert and myoclonus&sort=date

  69. Welsh JP, Placantonakis DG, Warsetsky SI, Marquez RG, Bernstein L, Aicher SA. The serotonin hypothesis of myoclonus from the perspective of neuronal rhythmicity. Adv Neurol. 2002;89:307–29.

    PubMed  Google Scholar 

  70. Tai KK, Truong DD. Post-hypoxic myoclonus induces Fos expression in the reticular thalamic nucleus and neurons in the brainstem. Brain Res. 2005;1059:122–8.

    Article  CAS  PubMed  Google Scholar 

  71. English WA, Giffin NJ, Nolan JP. Myoclonus after cardiac arrest: pitfalls in diagnosis and prognosis. Anaesthesia. 2009;64(8):908–11.

    Article  CAS  PubMed  Google Scholar 

  72. Simon RP. Hypoxia versus ischemia. Neurology. 1999;52(1):7–8.

    Article  CAS  PubMed  Google Scholar 

  73. Obrenovitch TP. Molecular physiology of preconditioning-induced brain tolerance to ischemia. Physiol Rev. 2008;88(1):211–47.

    Article  CAS  PubMed  Google Scholar 

  74. Geocadin RG, Wijdicks E, Armstrong MJ, Damian M, Mayer SA, Ornato JP, et al. Practice guideline summary: reducing brain injury following cardiopulmonary resuscitation: report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017;88:2141–9.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Thömke F, Weilemann SL. Poor prognosis despite successful treatment of postanoxic generalized myoclonus. Neurology. 2010;74(17):1392–4.

    Article  PubMed  Google Scholar 

  76. Thömke F, Marx JJ, Sauer O, Hundsberger T, Hägele S, Wiechelt J, et al. Observations on comatose survivors of cardiopulmonary resuscitation with generalized myoclonus. BMC Neurol. 2005;5:14.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Wijdicks EFM. Propofol in myoclonus status epilepticus in comatose patients following cardiac resuscitation. J Neurol Neurosurg Psychiatry. 2002;73(1):94–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  78. Rayadurg V, Muthuchellappan R, Rao U. Volatile anesthetic for the control of posthypoxic refractory myoclonic status. Indian J Crit Care Med. 2016;20:485–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  79. Samaniego EA, Mlynash M, Caulfield AF, Eyngorn I, Wijman CAC. Sedation confounds outcome prediction in cardiac arrest survivors treated with hypothermia. Neurocrit Care. 2011;15:113–9.

    Article  PubMed  PubMed Central  Google Scholar 

  80. Al Thenayan E, Savard M, Sharpe M, Norton L, Young B. Predictors of poor neurologic outcome after induced mild hypothermia following cardiac arrest. Neurology. 2008;71:1535–7.

    Article  PubMed  Google Scholar 

  81. Kim YM, Youn CS, Kim SH, Lee BK, Cho IS, Cho GC, et al. Adverse events associated with poor neurological outcome during targeted temperature management and advanced critical care after out-of-hospital cardiac arrest. Crit Care. 2015;19(1):283.

    Article  PubMed  PubMed Central  Google Scholar 

  82. Fugate JE, Wijdicks EFM, Mandrekar J, Claassen DO, Manno EM, White RD, et al. Predictors of neurologic outcome in hypothermia after cardiac arrest. Ann Neurol. 2010;68:907–14.

    Article  PubMed  Google Scholar 

  83. Lucas JM, Cocchi MN, Salciccioli J, Stanbridge JA, Geocadin RG, Herman ST, et al. Neurologic recovery after therapeutic hypothermia in patients with post-cardiac arrest myoclonus. Resuscitation. 2012;83:265–9.

    Article  PubMed  Google Scholar 

  84. Rossetti AO, Oddo M, Liaudet L, Kaplan PW. Predictors of awakening from postanoxic status epilepticus after therapeutic hypothermia. Neurology. 2009;72:744–9.

    Article  PubMed  Google Scholar 

  85. Rollinson RD, Gilligan BS. Postanoxic action myoclonus (Lance-Adams syndrome) responding to valproate. Arch Neurol. 1979;36(1):44–5.

    Article  CAS  PubMed  Google Scholar 

  86. Bruni J, Willmore LJ, Wilder BJ. Treatment of postanoxic intention myoclonus with valproic acid. Can J Neurol Sci. 1979;6:39–42.

    Article  CAS  PubMed  Google Scholar 

  87. Fahn S. Post-anoxic action myoclonus: improvement with valproic acid. N Engl J Med. 1978;299(6):313–4.

    CAS  PubMed  Google Scholar 

  88. Frucht SJ, Louis ED, Chuang C, Fahn S. A pilot tolerability and efficacy study of Levetiracetam in patients with chronic myoclonus. Neurology. 2001;57:1112–4.

    Article  CAS  PubMed  Google Scholar 

  89. Krauss GL, Bergin A, Kramer RE, Cho YW, Reich SG. Suppression of posthypoxic and postencephalitic myoclonus with levetiracetam. Neurology. 2001;56:411–2.

    Article  CAS  PubMed  Google Scholar 

  90. Genton P, Gelisse P. Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam. Neurology. 2001;57(6):1144–5.

    Article  CAS  PubMed  Google Scholar 

  91. Venot M, Weiss N, Espinoza S, Imbert A, Tadie JM, Fagon JY, et al. Improvement of early diagnosed post-anoxic myoclonus with levetiracetam. Intensive Care Med. 2011;37(1):177–9.

    Article  PubMed  Google Scholar 

  92. Tai KK, Truong DD. Brivaracetam is superior to levetiracetam in a rat model of post-hypoxic myoclonus. J Neural Transm (Vienna). 2007;114(12):1547–51. [cited 2020 May 29]. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17690949

    Article  CAS  Google Scholar 

  93. Chadwick D, Hallett M, Harris R, Jenner P, Reynolds EH, Marsden CD. Clinical, biochemical, and physiological features distinguishing myoclonus responsive to 5-hydroxytryptophan, tryptophan with a monoamine oxidase inhibitor, and clonazepam. Brain. 1977;100(3):455–87.

    Article  CAS  PubMed  Google Scholar 

  94. Lim S-Y, Jasti DB, Tan AH. Improvement of “Bouncy Gait” in Lance-Adams syndrome with perampanel. Cureus. 2020;12(1):e6773.

    PubMed  PubMed Central  Google Scholar 

  95. Steinhoff BJ, Bacher M, Kurth C, Staack AM, Kornmeier R. Add-on perampanel in Lance-Adams syndrome. Epilepsy Behav Case Rep. 2016;6:28–9.

    Article  PubMed  PubMed Central  Google Scholar 

  96. Oi K, Neshige S, Hitomi T, Kobayashi K, Tojima M, Matsuhashi M, et al. Low-dose perampanel improves refractory cortical myoclonus by the dispersed and suppressed paroxysmal depolarization shifts in the sensorimotor cortex. Clin Neurophysiol. 2019;130:1804–12.

    Article  PubMed  Google Scholar 

  97. Gil-López FJ, Montoya J, Falip M, Aparicio J, López-González FJ, Toledano R, et al. Retrospective study of perampanel efficacy and tolerability in myoclonic seizures. Acta Neurol Scand. 2018;138:122–9.

    Article  PubMed  CAS  Google Scholar 

  98. Kamel WA, Al-Hashel JY, Abdulsalam AJ, Arabi M. Perampanel in refractory post-hypoxic myoclonus: see the difference! Acta Neurol Belg. 2020;120(3):741–2.

    Article  PubMed  Google Scholar 

  99. Santamarina E, Sueiras M, Lidón RM, Guzmán L, Bañeras J, González M, et al. Use of perampanel in one case of super-refractory hypoxic myoclonic status: case report. Epilepsy Behav Case Rep. 2015;4:56–9.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Birthi P, Walters C, Vargas OO, Karandikar N. The use of intrathecal baclofen therapy for myoclonus in a patient with Lance Adams syndrome. PM R. 2011;3:671–3.

    Article  PubMed  Google Scholar 

  101. Wicklein EM, Schwendemann G. Use of clonazepam and valproate in patients with Lance Adams syndrome. J R Soc Med. 1993;86(10):618.

    CAS  PubMed  PubMed Central  Google Scholar 

  102. Galldiks N, Timmermann L, Fink GR, Burghaus L. Posthypoxic myoclonus (Lance-Adams Syndrome) treated with lacosamide. Clin Neuropharmacol. 2010;33(4):216–7.

    Article  PubMed  Google Scholar 

  103. De La Aleja JG, Saiz-Díaz RA, De La Peña P. Relief of intractable posthypoxic myoclonus after administration of agomelatine. Clin Neuropharmacol. 2012;35:258–9.

    Article  Google Scholar 

  104. Polesin A, Stern M. Post-anoxic myoclonus: a case presentation and review of management in the rehabilitation setting. Brain Inj. 2006;20(2):213–7.

    Article  PubMed  Google Scholar 

  105. Zöllner JP, Noda AH, Rosenow F, Strzelczyk A. Improving post-hypoxic myoclonus using cannabidiol. Seizure. 2019;67:38–9.

    Article  PubMed  Google Scholar 

  106. Coletti A, Mandelli A, Minoli G, Tredici G. Post-anoxic action myoclonus (Lance-Adams syndrome) treated with levodopa and gabaergic drugs. J Neurol. 1980;223:67–70.

    Article  CAS  PubMed  Google Scholar 

  107. Whitlock JA Jr, Dumigan RW. Treatment of postanoxic action myoclonus with intrathecal baclofen: a case report. PM R. 2018;10(8):870–2.

    Article  PubMed  Google Scholar 

  108. Genton P, Guerrini R. Effect of alcohol on action myoclonus in Lance-Adams syndrome and progressive myoclonus epilepsy. Mov Disord. 1992;7(1):92.

    Article  CAS  PubMed  Google Scholar 

  109. Jain S, Jain M. Action myoclonus (Lance-Adam syndrome) secondary to strangulation with dramatic response to alcohol. Mov Disord. 1991;6(2):183.

    Article  CAS  PubMed  Google Scholar 

  110. Riboldi GM, Frucht SJ. Increasing evidence for the use of sodium oxybate in multi-drug-resistant Lance–Adams syndrome. Tremor Other Hyperkinet Mov (N Y). 2019;9 https://doi.org/10.7916/d8-rnsh-c024.

  111. Frucht SJ, Bordelon Y, Houghton WH, Reardan D. A pilot tolerability and efficacy trial of sodium oxybate in ethanol-responsive movement disorders. Mov Disord. 2005;20(10):1330–7.

    Article  PubMed  Google Scholar 

  112. Frucht SJ, Bordelon Y, Houghton WH. Marked amelioration of alcohol-responsive posthypoxic myoclonus by gamma-hydroxybutyric acid (Xyrem). Mov Disord. 2005;20(6):745–51.

    Article  PubMed  Google Scholar 

  113. Frucht SJ, Houghton WC, Bordelon Y, Greene PE, Louis ED. A single-blind, open-label trial of sodium oxybate for myoclonus and essential tremor. Neurology. 2005;65(12):1967–9.

    Article  CAS  PubMed  Google Scholar 

  114. Arpesella R, Dallocchio C, Arbasino C, Imberti R, Martinotti R, Frucht SJ. A patient with intractable posthypoxic myoclonus (Lance-Adams syndrome) treated with sodium oxybate. Anaesth Intensive Care. 2009;37:314–8.

    Article  CAS  PubMed  Google Scholar 

  115. Asahi T, Kashiwazaki D, Dougu N, Oyama G, Takashima S, Tanaka K, et al. Alleviation of myoclonus after bilateral pallidal deep brain stimulation for Lance–Adams syndrome. J Neurol. 2015;262(6):1581–3.

    Article  PubMed  Google Scholar 

  116. Kobayashi K, Katayama Y, Otaka T, Obuchi T, Kano T, Nagaoka T, et al. Thalamic deep brain stimulation for the treatment of action myoclonus caused by perinatal anoxia. Stereotact Funct Neurosurg. 2010;88:259–63.

    Article  PubMed  Google Scholar 

  117. Ramdhani RA, Frucht SJ, Kopell BH. Improvement of post-hypoxic myoclonus with bilateral pallidal deep brain stimulation: a case report and review of the literature. Tremor Other Hyperkinet Mov (N Y). 2017;7:461.

    Article  Google Scholar 

  118. Seder DB, Sunde K, Rubertsson S, Mooney M, Stammet P, Riker RR, et al. Neurologic outcomes and postresuscitation care of patients with myoclonus following cardiac arrest. Crit Care Med. 2015;43:965–72.

    Article  PubMed  Google Scholar 

  119. Hui ACF, Cheng C, Lam A, Mok V, Joynt GM. Prognosis following postanoxic myoclonus status epilepticus. Eur Neurol. 2005;54:10–3.

    Article  PubMed  Google Scholar 

  120. Lagrand T, Winogrodzka A. Late relapse myoclonus in a case of Lance-Adams syndrome. BMJ Case Rep. 2013;2013:bcr2013201543. https://doi.org/10.1136/bcr-2013-201543.

    Article  PubMed  PubMed Central  Google Scholar 

  121. van Zijl JC, Beudel M, de Jong BM, van der Naalt J, Zutt R, Lange F, et al. The interrelation between clinical presentation and neurophysiology of posthypoxic myoclonus. Ann Clin Transl Neurol. 2018;5:386–96.

    Article  PubMed  PubMed Central  Google Scholar 

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  1. Department of Neurology, NYU Langone Health, The Marlene and Paolo Fresco Institute for Parkinson’s and Movement Disorders, New York, NY, USA

    Giulietta Maria Riboldi

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  1. Department of Neurology, NYU Langone Health, New York, NY, USA

    Steven J. Frucht

Electronic Supplementary Material

Seven patients with Lance-Adams syndrome (LAS, chronic posthypoxic myoclonus) are seen in this video segment. The first young woman survived an asthmatic arrest and is shown after discharge from the intensive care unit on her initial hospitalization. Myoclonic jerks of both outstretched hands are seen, with action myoclonus affecting attempt to write. The next patient developed LAS after surviving a respiratory arrest following a complication of thyroid surgery. Severe action and intention myoclonus are seen when she attempts to point to a letter board. The following young man developed LAS after a respiratory arrest. Despite treatment with multiple anti-myoclonic medications, he experienced episodes of exhausting continuous myoclonic jerking (myoclonic status) that would last up to an hour. Myoclonus was alcohol-responsive, and his family would administer wine to help terminate an episode. In between episodes, severe action myoclonus on finger to nose and manipulating a cup is present. Axial negative postural lapses triggered by standing prevented him from walking. The next patient developed LAS 25 years prior to this visit after an asthmatic respiratory arrest. Pendular rotatory nystagmus with oscillopsia accompanied his myoclonus. Myoclonus is present in both hands held forward, a bit worse with action, and accompanied by mild past-pointing on the left. Axial negative myoclonic jerks interfere with his walking, while providing sensory input with a guiding hand helps eliminate axial myoclonus. Touching a guard rail provides a similar proprioceptive input. The next patient, a 27-year-old man, developed severe LAS after surviving an arrest provoked by a pulmonary embolism. Severe action myoclonus on holding a cup or spoon prevented him from performing basic activities of living. Profound negative axial myoclonic jerks prevented him from standing even with two-person assist. The next patient, a young woman who developed LAS after an unrecognized esophageal intubation during a gynecologic surgery, was incapacitated by medication-refractory myoclonus. She is shown before and 20 min after ingestion of two glasses of wine, with profound improvement in myoclonus and functional performance. The final patient developed LAS after surviving a cardiopulmonary arrest from spontaneous bilateral pneumothorax. Vocal myoclonus prevented him from communicating, and myoclonus at rest and with action left him completely functionally disabled. He is shown in hospital before and 1 h after ingestion of two grams of sodium oxybate, with profound improvement in myoclonus (MP4 745754 kb)

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Riboldi, G.M. (2022). Posthypoxic Myoclonus and Its Management. In: Frucht, S.J. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana, Cham. https://doi.org/10.1007/978-3-030-75898-1_11

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  • DOI: https://doi.org/10.1007/978-3-030-75898-1_11

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