U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Neonatal unilateral epileptic spasm

MedGen UID:
1714410
•Concept ID:
C5397844
•
Disease or Syndrome
HPO: HP:0032838

Definition

Neonatal unilateral epileptic spasm is a sudden flexion, extension, or mixed extension-flexion of predominantly proximal and truncal muscles that occurs at one side of the body. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeonatal unilateral epileptic spasm

Recent clinical studies

Etiology

EEG before and after total corpus callosotomy for pharmacoresistant infantile spasms: Fast oscillations and slow-wave connectivity in hypsarrhythmia.
Baba S, Vakorin VA, Doesburg SM, Nagamori C, Cortez MA, Honda R, Ono T, Toda K, Nishimoto H, Ebihara T, Sakai K, Ochi A, Snead OC 3rd, Baba H, Otsubo H
Epilepsia 2019 Sep;60(9):1849-1860. Epub 2019 Aug 13 doi: 10.1111/epi.16295. PMID: 31407333
Clinical and ictal characteristics of infantile seizures: EEG correlation via long-term video EEG monitoring.
Yu HJ, Lee CG, Nam SH, Lee J, Lee M
Brain Dev 2013 Sep;35(8):771-7. Epub 2013 Mar 19 doi: 10.1016/j.braindev.2013.02.005. PMID: 23522623
Lateralizing signs during seizures in infants.
Loddenkemper T, Wyllie E, Neme S, Kotagal P, Lüders HO
J Neurol 2004 Sep;251(9):1075-9. doi: 10.1007/s00415-004-0463-7. PMID: 15372248
West syndrome: etiological and prognostic aspects.
Watanabe K
Brain Dev 1998 Jan;20(1):1-8. doi: 10.1016/s0387-7604(97)00085-5. PMID: 9533552
Etiology of convulsions in neonatal and infantile period.
Kurokawa T, Yokata K, Takashima S, Nambu Y, Hanai T
Folia Psychiatr Neurol Jpn 1976;30(3):365-78. doi: 10.1111/j.1440-1819.1976.tb02274.x. PMID: 992519

Diagnosis

Variant in the neuronal vesicular SNARE VAMP2 (synaptobrevin-2): First report in Japan.
Sunaga Y, Muramatsu K, Kosaki K, Sugai K, Mizuno T, Kouno M, Tashiro M
Brain Dev 2020 Aug;42(7):529-533. Epub 2020 Apr 23 doi: 10.1016/j.braindev.2020.04.001. PMID: 32336483
Neonatal seizures.
Plouin P, Kaminska A
Handb Clin Neurol 2013;111:467-76. doi: 10.1016/B978-0-444-52891-9.00051-8. PMID: 23622196
West syndrome due to perinatal insults.
Cusmai R, Ricci S, Pinard JM, Plouin P, Fariello G, Dulac O
Epilepsia 1993 Jul-Aug;34(4):738-42. doi: 10.1111/j.1528-1157.1993.tb00455.x. PMID: 8330586
Nonlissencephalic cortical dysplasias: correlation of imaging findings with clinical deficits.
Barkovich AJ, Kjos BO
AJNR Am J Neuroradiol 1992 Jan-Feb;13(1):95-103. PMID: 1375803Free PMC Article
Electroencephalographic aspects of hemimegalencephaly.
Paladin F, Chiron C, Dulac O, Plouin P, Ponsot G
Dev Med Child Neurol 1989 Jun;31(3):377-83. PMID: 2753242

Therapy

Surgical treatment for localization-related infantile spasms: excellent long-term outcomes.
Yum MS, Ko TS, Lee JK, Hong S, Kim DS, Kim J
Clin Neurol Neurosurg 2011 Apr;113(3):213-7. Epub 2010 Dec 13 doi: 10.1016/j.clineuro.2010.11.010. PMID: 21146918
Infantile spasms associated with proximal duplication of chromosome 15q.
Bingham PM, Spinner NB, Sovinsky L, Zackai EH, Chance PF
Pediatr Neurol 1996 Sep;15(2):163-5. doi: 10.1016/0887-8994(96)00119-1. PMID: 8888053

Prognosis

EEG before and after total corpus callosotomy for pharmacoresistant infantile spasms: Fast oscillations and slow-wave connectivity in hypsarrhythmia.
Baba S, Vakorin VA, Doesburg SM, Nagamori C, Cortez MA, Honda R, Ono T, Toda K, Nishimoto H, Ebihara T, Sakai K, Ochi A, Snead OC 3rd, Baba H, Otsubo H
Epilepsia 2019 Sep;60(9):1849-1860. Epub 2019 Aug 13 doi: 10.1111/epi.16295. PMID: 31407333
Neonatal seizures.
Plouin P, Kaminska A
Handb Clin Neurol 2013;111:467-76. doi: 10.1016/B978-0-444-52891-9.00051-8. PMID: 23622196
West syndrome: etiological and prognostic aspects.
Watanabe K
Brain Dev 1998 Jan;20(1):1-8. doi: 10.1016/s0387-7604(97)00085-5. PMID: 9533552
West syndrome due to perinatal insults.
Cusmai R, Ricci S, Pinard JM, Plouin P, Fariello G, Dulac O
Epilepsia 1993 Jul-Aug;34(4):738-42. doi: 10.1111/j.1528-1157.1993.tb00455.x. PMID: 8330586
Electroencephalographic aspects of hemimegalencephaly.
Paladin F, Chiron C, Dulac O, Plouin P, Ponsot G
Dev Med Child Neurol 1989 Jun;31(3):377-83. PMID: 2753242

Clinical prediction guides

EEG before and after total corpus callosotomy for pharmacoresistant infantile spasms: Fast oscillations and slow-wave connectivity in hypsarrhythmia.
Baba S, Vakorin VA, Doesburg SM, Nagamori C, Cortez MA, Honda R, Ono T, Toda K, Nishimoto H, Ebihara T, Sakai K, Ochi A, Snead OC 3rd, Baba H, Otsubo H
Epilepsia 2019 Sep;60(9):1849-1860. Epub 2019 Aug 13 doi: 10.1111/epi.16295. PMID: 31407333
Lateralizing signs during seizures in infants.
Loddenkemper T, Wyllie E, Neme S, Kotagal P, Lüders HO
J Neurol 2004 Sep;251(9):1075-9. doi: 10.1007/s00415-004-0463-7. PMID: 15372248
West syndrome: etiological and prognostic aspects.
Watanabe K
Brain Dev 1998 Jan;20(1):1-8. doi: 10.1016/s0387-7604(97)00085-5. PMID: 9533552
West syndrome due to perinatal insults.
Cusmai R, Ricci S, Pinard JM, Plouin P, Fariello G, Dulac O
Epilepsia 1993 Jul-Aug;34(4):738-42. doi: 10.1111/j.1528-1157.1993.tb00455.x. PMID: 8330586
Electroencephalographic aspects of hemimegalencephaly.
Paladin F, Chiron C, Dulac O, Plouin P, Ponsot G
Dev Med Child Neurol 1989 Jun;31(3):377-83. PMID: 2753242

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...