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Focal motor seizure with negative myoclonus

MedGen UID:
1719948
•Concept ID:
C5397852
•
Disease or Syndrome
HPO: HP:0032846

Definition

A type of focal motor seizure characterized by a sudden interruption in normal tonic muscle activity lasting 500 ms or less, without evidence of preceding myoclonus as the initial semiological manifestation. The interruption in muscle tone is briefer than seen in a focal atonic seizure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal motor seizure with negative myoclonus

Professional guidelines

PubMed

Phenotypic and genetic spectrum of SCN8A-related disorders, treatment options, and outcomes.
Gardella E, Møller RS
Epilepsia 2019 Dec;60 Suppl 3:S77-S85. doi: 10.1111/epi.16319. PMID: 31904124
From genotype to phenotype in Dravet disease.
Gataullina S, Dulac O
Seizure 2017 Jan;44:58-64. Epub 2016 Oct 21 doi: 10.1016/j.seizure.2016.10.014. PMID: 27817982
Negative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
Rubboli G, Tassinari CA
Neurophysiol Clin 2006 Sep-Dec;36(5-6):337-43. Epub 2007 Jan 23 doi: 10.1016/j.neucli.2006.12.001. PMID: 17336779

Recent clinical studies

Etiology

Phenotypic and genetic spectrum of SCN8A-related disorders, treatment options, and outcomes.
Gardella E, Møller RS
Epilepsia 2019 Dec;60 Suppl 3:S77-S85. doi: 10.1111/epi.16319. PMID: 31904124
Encephalopathy with status epilepticus during sleep: unusual EEG patterns.
Caraballo RH, Fortini S, Flesler S, Pasteris MC, Caramuta L, Portuondo E
Seizure 2015 Feb;25:117-25. Epub 2014 Oct 13 doi: 10.1016/j.seizure.2014.10.003. PMID: 25457450
Atonic phenomena and partial seizures. A reappraisal.
So NK
Adv Neurol 1995;67:29-39. PMID: 8848976
Epileptic negative myoclonus.
Tassinari CA, Rubboli G, Parmeggiani L, Valzania F, Plasmati R, Riguzzi P, Michelucci R, Volpi L, Passarelli D, Meletti S
Adv Neurol 1995;67:181-97. PMID: 8848969
Epileptic negative myoclonus.
Guerrini R, Dravet C, Genton P, Bureau M, Roger J, Rubboli G, Tassinari CA
Neurology 1993 Jun;43(6):1078-83. doi: 10.1212/wnl.43.6.1078. PMID: 8170545

Diagnosis

From genotype to phenotype in Dravet disease.
Gataullina S, Dulac O
Seizure 2017 Jan;44:58-64. Epub 2016 Oct 21 doi: 10.1016/j.seizure.2016.10.014. PMID: 27817982
Negative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
Rubboli G, Tassinari CA
Neurophysiol Clin 2006 Sep-Dec;36(5-6):337-43. Epub 2007 Jan 23 doi: 10.1016/j.neucli.2006.12.001. PMID: 17336779
Atonic phenomena and partial seizures. A reappraisal.
So NK
Adv Neurol 1995;67:29-39. PMID: 8848976
Epileptic negative myoclonus.
Tassinari CA, Rubboli G, Parmeggiani L, Valzania F, Plasmati R, Riguzzi P, Michelucci R, Volpi L, Passarelli D, Meletti S
Adv Neurol 1995;67:181-97. PMID: 8848969
Epileptic negative myoclonus.
Guerrini R, Dravet C, Genton P, Bureau M, Roger J, Rubboli G, Tassinari CA
Neurology 1993 Jun;43(6):1078-83. doi: 10.1212/wnl.43.6.1078. PMID: 8170545

Therapy

From genotype to phenotype in Dravet disease.
Gataullina S, Dulac O
Seizure 2017 Jan;44:58-64. Epub 2016 Oct 21 doi: 10.1016/j.seizure.2016.10.014. PMID: 27817982
Magnetoencephalography localizing spike sources of atypical benign partial epilepsy.
Shiraishi H, Haginoya K, Nakagawa E, Saitoh S, Kaneko Y, Nakasato N, Chan D, Otsubo H
Brain Dev 2014 Jan;36(1):21-7. Epub 2013 Feb 4 doi: 10.1016/j.braindev.2012.12.011. PMID: 23384398
A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep.
Hughes JR
Epilepsy Behav 2011 Feb;20(2):247-53. Epub 2011 Jan 15 doi: 10.1016/j.yebeh.2010.10.015. PMID: 21242107
Negative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
Rubboli G, Tassinari CA
Neurophysiol Clin 2006 Sep-Dec;36(5-6):337-43. Epub 2007 Jan 23 doi: 10.1016/j.neucli.2006.12.001. PMID: 17336779
Epileptic encephalopathies: a brief overview.
Nabbout R, Dulac O
J Clin Neurophysiol 2003 Nov-Dec;20(6):393-7. doi: 10.1097/00004691-200311000-00002. PMID: 14734929

Prognosis

From genotype to phenotype in Dravet disease.
Gataullina S, Dulac O
Seizure 2017 Jan;44:58-64. Epub 2016 Oct 21 doi: 10.1016/j.seizure.2016.10.014. PMID: 27817982
Encephalopathy with status epilepticus during sleep: unusual EEG patterns.
Caraballo RH, Fortini S, Flesler S, Pasteris MC, Caramuta L, Portuondo E
Seizure 2015 Feb;25:117-25. Epub 2014 Oct 13 doi: 10.1016/j.seizure.2014.10.003. PMID: 25457450
Atypical benign partial epilepsy: recognition can prevent pseudocatastrophe.
Fujii A, Oguni H, Hirano Y, Osawa M
Pediatr Neurol 2010 Dec;43(6):411-9. doi: 10.1016/j.pediatrneurol.2010.06.018. PMID: 21093732
Negative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
Rubboli G, Tassinari CA
Neurophysiol Clin 2006 Sep-Dec;36(5-6):337-43. Epub 2007 Jan 23 doi: 10.1016/j.neucli.2006.12.001. PMID: 17336779
Epileptic negative myoclonus.
Guerrini R, Dravet C, Genton P, Bureau M, Roger J, Rubboli G, Tassinari CA
Neurology 1993 Jun;43(6):1078-83. doi: 10.1212/wnl.43.6.1078. PMID: 8170545

Clinical prediction guides

Encephalopathy with status epilepticus during sleep: unusual EEG patterns.
Caraballo RH, Fortini S, Flesler S, Pasteris MC, Caramuta L, Portuondo E
Seizure 2015 Feb;25:117-25. Epub 2014 Oct 13 doi: 10.1016/j.seizure.2014.10.003. PMID: 25457450
Negative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
Rubboli G, Tassinari CA
Neurophysiol Clin 2006 Sep-Dec;36(5-6):337-43. Epub 2007 Jan 23 doi: 10.1016/j.neucli.2006.12.001. PMID: 17336779
A particular type of epilepsy in children with congenital hemiparesis associated with unilateral polymicrogyria.
Caraballo R, Cersósimo R, Fejerman N
Epilepsia 1999 Jul;40(7):865-71. doi: 10.1111/j.1528-1157.1999.tb00792.x. PMID: 10403209
Apparent focal motor seizure with a jacksonian march induced by fentanyl: a case report and review of the literature.
Sprung J, Schedewie HK
J Clin Anesth 1992 Mar-Apr;4(2):139-43. doi: 10.1016/0952-8180(92)90031-u. PMID: 1562337
Asterixis: one type of negative myoclonus.
Young RR, Shahani BT
Adv Neurol 1986;43:137-56. PMID: 3946109

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