myoclonus

Summary

Summary: Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some CENTRAL NERVOUS SYSTEM DISEASES; (e.g., EPILEPSY, MYOCLONIC). Nocturnal myoclonus is the principal feature of the NOCTURNAL MYOCLONUS SYNDROME. (From Adams et al., Principles of Neurology, 6th ed, pp102-3).

Top Publications

  1. Liao Y, Anttonen A, Liukkonen E, Gaily E, Maljevic S, Schubert S, et al. SCN2A mutation associated with neonatal epilepsy, late-onset episodic ataxia, myoclonus, and pain. Neurology. 2010;75:1454-8 pubmed publisher
    ..Mutations in SCN2A, encoding the brain sodium channel Na(V)1.2, have previously been reported to be associated with benign familial neonatal infantile seizures, febrile seizures plus, and intractable epilepsy of infancy...
  2. Gultop F, Akkaya T, Bedirli N, Gumus H. Lidocaine pretreatment reduces the frequency and severity of myoclonus induced by etomidate. J Anesth. 2010;24:300-2 pubmed publisher
    ..3 mg/kg). One minute after etomidate administration we assessed severity of myoclonus. Pretreatment with lidocaine significantly reduced both the incidence and severity of myoclonic movements...
  3. Pearce J. Palatal Myoclonus (syn. Palatal Tremor). Eur Neurol. 2008;60:312-5 pubmed publisher
    ..e. supranuclear, lesion in the striatum or rostral brainstem releasing medullary activation with denervation hypersensitivity of olivary neurones...
  4. Ritz K, Gerrits M, Foncke E, Van Ruissen F, van der Linden C, Vergouwen M, et al. Myoclonus-dystonia: clinical and genetic evaluation of a large cohort. J Neurol Neurosurg Psychiatry. 2009;80:653-8 pubmed publisher
    b>Myoclonus-dystonia (M-D) is an autosomal dominant inherited movement disorder. Various mutations within the epsilon-sarcoglycan (SGCE) gene have been associated with M-D, but mutations are detected in only about 30% of patients...
  5. Abe S, Gagnon J, Montplaisir J, Postuma R, Rompre P, Huynh N, et al. Sleep bruxism and oromandibular myoclonus in rapid eye movement sleep behavior disorder: a preliminary report. Sleep Med. 2013;14:1024-30 pubmed publisher
    We aimed to compare rhythmic masticatory muscle activity typical of sleep bruxism and oromandibular myoclonus (OMM) during rapid eye movement (REM) sleep in patients with idiopathic REM sleep behavior disorder (iRBD) and in Parkinson ..
  6. Alfa J, Bamgbade O. Acute myoclonus following spinal anaesthesia. Eur J Anaesthesiol. 2008;25:256-7 pubmed publisher
  7. La Morgia C, Achilli A, Iommarini L, Barboni P, Pala M, Olivieri A, et al. Rare mtDNA variants in Leber hereditary optic neuropathy families with recurrence of myoclonus. Neurology. 2008;70:762-70 pubmed publisher
    To investigate the mechanisms underlying myoclonus in Leber hereditary optic neuropathy (LHON).
  8. Markakis I, Alexiou E, Xifaras M, Gekas G, Rombos A. Opsoclonus-myoclonus-ataxia syndrome with autoantibodies to glutamic acid decarboxylase. Clin Neurol Neurosurg. 2008;110:619-21 pubmed publisher
    Opsoclonus-myoclonus-ataxia syndrome (OMS) is a rare neurological disorder of probably autoimmune origin...
  9. Kinugawa K, Vidailhet M, Clot F, Apartis E, Grabli D, Roze E. Myoclonus-dystonia: an update. Mov Disord. 2009;24:479-89 pubmed publisher
    Our knowledge of the clinical, neurophysiological, and genetic aspects of myoclonus-dystonia (M-D) has improved markedly in the recent years...

More Information

Publications62

  1. Tai K, Truong D. Memantine exacerbates myoclonic jerks in a rat model of posthypoxic myoclonus. Brain Res. 2010;1343:194-8 pubmed publisher
    ..inferior olive resulting in excitotoxic neuronal injury in the cerebellum is the underlying cause of posthypoxic myoclonus. To test this hypothesis, the effect of memantine, an NMDA receptor antagonist, on the intensity of myoclonic ..
  2. Liu H, Fan J, Lin S, Zhao S, Lin Z. Botox transient treatment of tinnitus due to stapedius myoclonus: case report. Clin Neurol Neurosurg. 2011;113:57-8 pubmed publisher
    To explore the feasibility of using botulinum toxin type A (BTXA) to treat tinnitus due to stapedius myoclonus.
  3. Shibasaki H. Neurophysiological classification of myoclonus. Neurophysiol Clin. 2006;36:267-9 pubmed
    b>Myoclonus can be diagnosed and classified mainly based on clinical features. Neurophysiologic studies help confirm clinical diagnosis and classification, and understand underlying physiological mechanisms...
  4. Papandrea D, Kukol W, Anderson T, Herron B, Ferland R. Analysis of flurothyl-induced myoclonus in inbred strains of mice. Epilepsy Res. 2009;87:130-6 pubmed publisher
    b>Myoclonus is often observed in epilepsy. It is characterized by sudden involuntary shock-like movements of the body (myoclonic jerks, MJs). This study examined whether epileptic myoclonus was under genetic control...
  5. Kuncel A, Turner D, Ozelius L, Greene P, Grill W, Stacy M. Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome. Clin Neurol Neurosurg. 2009;111:303-6 pubmed publisher
    We present a 74-year-old woman with inherited myoclonus-dystonia, with predominant myoclonus and a novel mutation in the epsilon-sarcoglycan gene...
  6. Mondria T, de Gier H, Boon A. New device to control combined lingual and palatal myoclonus. Mov Disord. 2007;22:573-6 pubmed
    Lingual myoclonus is a poorly understood disorder that may occur in isolation or combined with palatal myoclonus...
  7. Hutchinson M, Waters P, McHugh J, Gorman G, O Riordan S, Connolly S, et al. Progressive encephalomyelitis, rigidity, and myoclonus: a novel glycine receptor antibody. Neurology. 2008;71:1291-2 pubmed publisher
  8. Koh K, Lim B, Hwang H, Park J, Chae J, Kim K, et al. Cerebellum can be a possible generator of progressive myoclonus. J Child Neurol. 2010;25:728-31 pubmed publisher
    ..Depth electrodes were inserted deep into the mass, which recorded focal slow waves associated with the clinical myoclonus. Following the removal of the tumor, the myoclonus was completely resolved with no neurological deficit...
  9. Li J, Cunic D, Paradiso G, Gunraj C, Pal P, Lang A, et al. Electrophysiological features of myoclonus-dystonia. Mov Disord. 2008;23:2055-61 pubmed publisher
    Inherited myoclonus-dystonia (M-D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol...
  10. Becker D, Patel A, Abou Khalil B, Pina Garza J. Successful treatment of encephalopathy and myoclonus with levetiracetam in a case of subacute sclerosing panencephalitis. J Child Neurol. 2009;24:763-7 pubmed publisher
    ..treated a 12-year-old boy with subacute sclerosing panencephalitis who presented with acute encephalopathy and myoclonus. Electroencephalogram showed characteristic generalized periodic discharges...
  11. Ting S, Lee D, Maclean D, Sheerin N. Paranoid psychosis and myoclonus: flecainide toxicity in renal failure. Cardiology. 2008;111:83-6 pubmed publisher
    ..We also review the pharmacokinetics of flecainide in patients with chronic kidney disease...
  12. Foncke E, Gerrits M, Van Ruissen F, Baas F, Hedrich K, Tijssen C, et al. Distal myoclonus and late onset in a large Dutch family with myoclonus-dystonia. Neurology. 2006;67:1677-80 pubmed
    We report a large myoclonus-dystonia (M-D) pedigree with a two-base pair deletion in Exon 5 of the epsilon-sarcoglycan gene. Three individuals had onset after age 40 years...
  13. Cassim F, Houdayer E. Neurophysiology of myoclonus. Neurophysiol Clin. 2006;36:281-91 pubmed
    b>Myoclonus may be generated by any area in the central nervous system. Finding its generator is helpful in the diagnostic process...
  14. Young B, Driedger A, Wojcik J. Autoimmune thyroid disease and segmental myoclonus. Mov Disord. 2007;22:434-6 pubmed
  15. Esapa C, Waite A, Locke M, Benson M, Kraus M, McIlhinney R, et al. SGCE missense mutations that cause myoclonus-dystonia syndrome impair epsilon-sarcoglycan trafficking to the plasma membrane: modulation by ubiquitination and torsinA. Hum Mol Genet. 2007;16:327-42 pubmed
    b>Myoclonus-dystonia syndrome (MDS) is a genetically heterogeneous disorder characterized by myoclonic jerks often seen in combination with dystonia and psychiatric co-morbidities and epilepsy...
  16. Asmus F, Devlin A, Munz M, Zimprich A, Gasser T, Chinnery P. Clinical differentiation of genetically proven benign hereditary chorea and myoclonus-dystonia. Mov Disord. 2007;22:2104-9 pubmed
    Because of clinical similarities, benign hereditary chorea and myoclonus-dystonia (DYT11) might be confused...
  17. Balint B, Jarius S, Nagel S, Haberkorn U, Probst C, Blöcker I, et al. Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies. Neurology. 2014;82:1521-8 pubmed publisher
    To describe a novel and distinct variant of progressive encephalomyelitis with rigidity and myoclonus (PERM) associated with antibodies directed against dipeptidyl peptidase-like protein 6 (DPPX), a regulatory subunit of the Kv4...
  18. Mizrak A, Koruk S, Bilgi M, Kocamer B, Erkutlu I, Ganidagli S, et al. Pretreatment with dexmedetomidine or thiopental decreases myoclonus after etomidate: a randomized, double-blind controlled trial. J Surg Res. 2010;159:e11-6 pubmed publisher
    ..The aim of this study was to compare the effect of pretreatment with dexmedetomidine (0.5 microg/kg) and thiopental (1 mg/kg) on the incidence of etomidate-induced myoclonus and postoperative pain.
  19. Roze E, Bounolleau P, Ducreux D, Cochen V, Leu Semenescu S, Beaugendre Y, et al. Propriospinal myoclonus revisited: Clinical, neurophysiologic, and neuroradiologic findings. Neurology. 2009;72:1301-9 pubmed publisher
    The literature on propriospinal myoclonus (PSM) is poor and there are no systematic reviews of the subject. We sought to clarify the spectrum of PSM.
  20. Guettard E, Portnoi M, Lohmann Hedrich K, Keren B, Rossignol S, Winkler S, et al. Myoclonus-dystonia due to maternal uniparental disomy. Arch Neurol. 2008;65:1380-5 pubmed publisher
    b>Myoclonus-dystonia is a movement disorder often associated with mutations in the maternally imprinted epsilon-sarcoglycan (SGCE) gene located on chromosome 7q21...
  21. Conill Tobías N, de Paula Vernetta C, García Callejo F, Marco Algarra J. Objective tinnitus from palatal myoclonus. Use of botulinum toxin: a case report. Acta Otorrinolaringol Esp. 2012;63:391-2 pubmed publisher
    Objective tinnitus can have many different etiologies, palatal myoclonus being one of the less frequent...
  22. Lucas J, Cocchi M, Salciccioli J, Stanbridge J, Geocadin R, Herman S, et al. Neurologic recovery after therapeutic hypothermia in patients with post-cardiac arrest myoclonus. Resuscitation. 2012;83:265-9 pubmed publisher
    Early myoclonus in comatose survivors of cardiac arrest, even when it is not myoclonic status epilepticus (MSE), is considered a sign of severe global brain ischemia and has been associated with high rates of mortality and poor ..
  23. Damasio J, Leite M, Coutinho E, Waters P, Woodhall M, Santos M, et al. Progressive encephalomyelitis with rigidity and myoclonus: the first pediatric case with glycine receptor antibodies. JAMA Neurol. 2013;70:498-501 pubmed publisher
    Progressive encephalomyelitis with rigidity and myoclonus is characterized by rigidity, painful muscle spasms, hyperekplexia, and brainstem signs...
  24. Calancie B. Spinal myoclonus after spinal cord injury. J Spinal Cord Med. 2006;29:413-24 pubmed
    ..Although there are many reports of unusual muscle activation patterns associated with different forms of myoclonus, we believe that certain aspects of the patterns seen with these 6 subjects have not been previously reported...
  25. Lin C, Wei Hung C, Lee Y. Transient spinal myoclonus after spinal anaesthesia with bupivacaine in the perioperation period. Anaesthesist. 2008;57:518 pubmed publisher
  26. Blumkin L, Suls A, Deconinck T, De Jonghe P, Linder I, Kivity S, et al. Neonatal seizures associated with a severe neonatal myoclonus like dyskinesia due to a familial KCNQ2 gene mutation. Eur J Paediatr Neurol. 2012;16:356-60 pubmed publisher
    ..The son developed a severe dyskinesia clinically compatible with multifocal myoclonus in the neonatal period that only responded to carbamazepine...
  27. Tai K, Truong D. NMDA receptor-mediated excitotoxicity contributes to the cerebral hypoxic injury of a rat model of posthypoxic myoclonus. Brain Res. 2007;1133:209-15 pubmed
    ..an NMDA receptor channel blocker, on the neurodegeneration occurred in an established rat model of posthypoxic myoclonus. We found that administration of memantine for 7 days significantly reduced cerebral hypoxia-induced ..
  28. Dijk J, Tijssen M. Management of patients with myoclonus: available therapies and the need for an evidence-based approach. Lancet Neurol. 2010;9:1028-36 pubmed publisher
    b>Myoclonus is a hyperkinetic movement disorder characterised by quick and involuntary jerks...
  29. Bouwes A, van Poppelen D, Koelman J, Kuiper M, Zandstra D, Weinstein H, et al. Acute posthypoxic myoclonus after cardiopulmonary resuscitation. BMC Neurol. 2012;12:63 pubmed publisher
    Acute posthypoxic myoclonus (PHM) can occur in patients admitted after cardiopulmonary resuscitation (CPR) and is considered to have a poor prognosis...
  30. Zamidei L, Bandini M, Michelagnoli G, Campostrini R, Consales G. Propriospinal myoclonus following intrathecal bupivacaine in hip surgery: a case report. Minerva Anestesiol. 2010;76:290-3 pubmed
    Propriospinal myoclonus is a rare disorder characterized by sudden, shock-like, involuntary jerks that arise from the axial muscles and spread both rostrally and caudally to other myotomes through slow polysynaptic pathways...
  31. Foncke E, Bour L, Speelman J, Koelman J, Tijssen M. Local field potentials and oscillatory activity of the internal globus pallidus in myoclonus-dystonia. Mov Disord. 2007;22:369-76 pubmed
    The pathophysiology of myoclonus-dystonia (M-D), an autosomal dominantly inherited movement disorder characterized by myoclonic jerks and dystonic contractions, is largely unknown...
  32. Sung Y, Huang C, Fan C, Lin C, Lin S. Avermectin intoxication with coma, myoclonus, and polyneuropathy. Clin Toxicol (Phila). 2009;47:686-8 pubmed publisher
    ..Avermectin has been used in the control of parasites and insects. Severe poisoning symptoms in humans include altered mental status, hypotension, and respiratory failure...
  33. Hess C, Raymond D, Aguiar P, Frucht S, Shriberg J, Heiman G, et al. Myoclonus-dystonia, obsessive-compulsive disorder, and alcohol dependence in SGCE mutation carriers. Neurology. 2007;68:522-4 pubmed
    Although myoclonus and dystonia are the hallmarks of myoclonus-dystonia (M-D), psychiatric features, particularly obsessive-compulsive disorder and alcohol dependence, have been reported in three families linked to chromosome 7q21...
  34. Hirayama T, Fukae J, Noda K, Fujishima K, Yamamoto T, Mori K, et al. Adult-onset Alexander disease with palatal myoclonus and intraventricular tumour. Eur J Neurol. 2008;15:e16-7 pubmed publisher
  35. Williams D, Cowey M, Tuck K, Day B. Psychogenic propriospinal myoclonus. Mov Disord. 2008;23:1312-3 pubmed publisher
    We report a case of probable psychogenic propriospinal myoclonus (PSM) in a patient who developed a sudden onset of disabling axial flexor myoclonus following a cosmetic surgical procedure...
  36. English W, Giffin N, Nolan J. Myoclonus after cardiac arrest: pitfalls in diagnosis and prognosis. Anaesthesia. 2009;64:908-11 pubmed publisher
    ..It is characterised by intention myoclonus but preserved intellect...
  37. Beukers R, Booij J, Weisscher N, Zijlstra F, van Amelsvoort T, Tijssen M. Reduced striatal D2 receptor binding in myoclonus-dystonia. Eur J Nucl Med Mol Imaging. 2009;36:269-74 pubmed publisher
    To study striatal dopamine D(2) receptor availability in DYT11 mutation carriers of the autosomal dominantly inherited disorder myoclonus-dystonia (M-D).
  38. Choi J, Choi I, Jeong Y, Kim T, Hahm K. Pretreatment of rocuronium reduces the frequency and severity of etomidate-induced myoclonus. J Clin Anesth. 2008;20:601-4 pubmed publisher
    To investigate the effect of pretreatment with a low dose of rocuronium on the occurrence of etomidate-induced myoclonus.
  39. Leu Semenescu S, Roze E, Vidailhet M, Legrand A, Trocello J, Cochen V, et al. Myoclonus or tremor in orthostatism: an under-recognized cause of unsteadiness in Parkinson's disease. Mov Disord. 2007;22:2063-9 pubmed
    ..The remaining 3 patients had orthostatic myoclonus, a condition that has not previously been reported in PD. Patients with fast tremor improved on clonazepam...
  40. Ragothaman M, Kulkarni G, Ashraf V, Pal P, Chickabasavaiah Y, Shankar S, et al. Elemental mercury poisoning probably causes cortical myoclonus. Mov Disord. 2007;22:1964-8 pubmed
    ..A 23-year woman, 2 years after injecting herself with elemental mercury developed disabling generalized myoclonus and ataxia. Electrophysiological studies confirmed the myoclonus was probably of cortical origin...
  41. Hüter L, Schreiber T, Gugel M, Schwarzkopf K. Low-dose intravenous midazolam reduces etomidate-induced myoclonus: a prospective, randomized study in patients undergoing elective cardioversion. Anesth Analg. 2007;105:1298-302, table of contents pubmed
    ..Myoclonic movements are a common problem in unpremedicated patients during induction of anesthesia with etomidate...
  42. Iizuka T, Leite M, Lang B, Waters P, Urano Y, Miyakawa S, et al. Glycine receptor antibodies are detected in progressive encephalomyelitis with rigidity and myoclonus (PERM) but not in saccadic oscillations. J Neurol. 2012;259:1566-73 pubmed publisher
    ..antibodies were recently identified in a few patients with progressive encephalomyelitis with rigidity and myoclonus (PERM); none of these patients had antibodies against glutamic acid decarboxylase (GAD)...
  43. Marelli C, Canafoglia L, Zibordi F, Ciano C, Visani E, Zorzi G, et al. A neurophysiological study of myoclonus in patients with DYT11 myoclonus-dystonia syndrome. Mov Disord. 2008;23:2041-8 pubmed publisher
    Mutations in the epsilon-sarcoglycan (SGCE) gene have been associated with DYT11 myoclonus-dystonia syndrome (MDS)...
  44. Dogan E, Yuruten B. Spinal myoclonus associated with vitamin B12 deficiency. Clin Neurol Neurosurg. 2007;109:827-9 pubmed
    ..The relation of low serum vB12 level and myoclonus is speculative and very few studies have demonstrated such patients...
  45. Abrao J, Bianco M, Roma W, Krippa J, Hallak J. Spinal myoclonus after subarachnoid anesthesia with bupivacaine. Rev Bras Anestesiol. 2011;61:619-23, 339-40 pubmed publisher
    ..It is presented in this case report a very rare complication after spinal anesthesia to provide subsidies to the management and therapeutic conduct...
  46. Turner M, Irani S, Leite M, Nithi K, Vincent A, Ansorge O. Progressive encephalomyelitis with rigidity and myoclonus: glycine and NMDA receptor antibodies. Neurology. 2011;77:439-43 pubmed publisher
    The syndrome of progressive encephalopathy with limb rigidity has been historically termed progressive encephalomyelitis with rigidity and myoclonus (PERM) or stiff-person syndrome plus.
  47. Roze E, Apartis E, Clot F, Dorison N, Thobois S, Guyant Marechal L, et al. Myoclonus-dystonia: clinical and electrophysiologic pattern related to SGCE mutations. Neurology. 2008;70:1010-6 pubmed publisher
    To clarify the clinical and neurophysiologic spectrum of myoclonus-dystonia patients with mutations of the SGCE gene.
  48. Batra Y, Rajeev S, Lokesh V, Rao K. Spinal myoclonus associated with intrathecal bupivacaine and fentanyl in an infant. Can J Anaesth. 2007;54:587-8 pubmed
  49. Borg M. Symptomatic myoclonus. Neurophysiol Clin. 2006;36:309-18 pubmed
    A huge number of neurological disorders are associated with myoclonus. This paper describes these disorders whose diagnosis partly relies on the presence of myoclonus...
  50. Andrade D, Hamani C, Minassian B. Treatment options for epileptic myoclonus and epilepsy syndromes associated with myoclonus. Expert Opin Pharmacother. 2009;10:1549-60 pubmed publisher
    b>Myoclonus is a brief shock-like movement that has many different etiologies. The degree to which it disturbs quality of life is extremely variable, as is its response to treatment.
  51. Koide Y, Ikeda H, Inoue Y. [Development or worsening of myoclonus associated with gabapentin therapy]. Rinsho Shinkeigaku. 2009;49:342-7 pubmed
    To evaluate the development or worsening of myoclonus in patients receiving gabapentin (GBP).
  52. Kurtis M, San Luciano M, Yu Q, Goodman R, Ford B, Raymond D, et al. Clinical and neurophysiological improvement of SGCE myoclonus-dystonia with GPi deep brain stimulation. Clin Neurol Neurosurg. 2010;112:149-52 pubmed publisher
    b>Myoclonus-dystonia (M-D) is characterized by early onset myoclonus and dystonia. It is thought to be subcortical in origin...
  53. Misbahuddin A, Placzek M, Lennox G, Taanman J, Warner T. Myoclonus-dystonia syndrome with severe depression is caused by an exon-skipping mutation in the epsilon-sarcoglycan gene. Mov Disord. 2007;22:1173-5 pubmed
    We describe two affected individuals in a family with myoclonus-dystonia syndrome complicated with severe depression. One individual committed suicide...