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EEG with polyspike wave complexes

MedGen UID:
867392
Concept ID:
C4021757
Finding
Synonym: EEG: spike and multispike waves, 3-4 hz
 
HPO: HP:0002392

Definition

The presence of complexes of repetitive spikes and waves in EEG. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEEG with polyspike wave complexes

Conditions with this feature

Unverricht-Lundborg syndrome
MedGen UID:
155923
Concept ID:
C0751785
Disease or Syndrome
Progressive myoclonic epilepsy type 1(EPM1) is a neurodegenerative disorder characterized by onset from age six to 15 years, stimulus-sensitive myoclonus, and tonic-clonic epileptic seizures. Some years after the onset, ataxia, incoordination, intentional tremor, and dysarthria develop. Individuals with EPM1 are cognitively mostly within the normal range, but show emotional lability and depression. The epileptic seizures are usually well controlled by anti-seizure medication, but the myoclonic jerks are progressive, action activated, and treatment resistant, and can be severely disabling.
Epilepsy, childhood absence, susceptibility to, 1
MedGen UID:
325057
Concept ID:
C1838604
Finding
Childhood absence epilepsy (CAE, ECA), a subtype of idiopathic generalized epilepsy (EIG; 600669), is characterized by a sudden and brief impairment of consciousness that is accompanied by a generalized, synchronous, bilateral, 2.5- to 4-Hz spike and slow-wave discharge (SWD) on EEG. Seizure onset occurs between 3 and 8 years of age and seizures generally occur multiple times per day. About 70% of patients experience spontaneous remission of seizures, often around adolescence. There are no structural neuropathologic findings in patients with ECA (Crunelli and Leresche, 2002). Genetic Heterogeneity of Susceptibility to Childhood Absence Epilepsy The ECA1 locus has been mapped to chromosome 8q24; see also EIG1 (see 600669), which also maps to 8q24. Susceptibility to the development of childhood absence epilepsy may be conferred by variation in several genes: ECA2 (see 607681), conferred by variation in the GABRG2 gene (137164) on chromosome 5q31.1; ECA4 (611136), conferred by variation in the GABRA1 gene (137160) on chromosome 5q34; ECA5 (612269), conferred by variation in the GABRB3 gene (137192) on chromosome 15q12; and ECA6 (see 611942), conferred by variation in the CACNA1H gene (607904) on chromosome 16p13. See EIG11 (607628) for discussion of a locus previously designated ECA3 on chromosome 3q26.
Myoclonic epilepsy, juvenile, susceptibility to, 4
MedGen UID:
370067
Concept ID:
C1969656
Disease or Syndrome
Febrile seizures, familial, 8
MedGen UID:
370755
Concept ID:
C1969810
Disease or Syndrome
Mutations in the GABRG2 gene cause a spectrum of seizure disorders, ranging from early-onset isolated febrile seizures (FS) to childhood absence epilepsy (CAE) to generalized epilepsy with febrile seizures plus, type 3 (GEFS+3), which tends to represent a more severe phenotype. Patients with isolated febrile seizures usually have onset in the first year of life and show spontaneous remission by age 6 years. Many of these patients may later develop absence seizures, which may also spontaneously remit, whereas a few may continue to have various types of febrile and afebrile seizures that persist beyond childhood, consistent with GEFS+. There is phenotypic variability in the seizure type, even within a family carrying the same mutation, suggesting that other loci may be involved (summary by Singh et al., 1999 and Marini et al., 2003). For a phenotypic description and a discussion of genetic heterogeneity of familial febrile seizures, see 121210. For a general phenotypic description and a discussion of genetic heterogeneity of GEFS+, see 604233. For a phenotypic description and discussion of genetic heterogeneity of childhood absence epilepsy, see 600131.
Epilepsy, idiopathic generalized, susceptibility to, 9
MedGen UID:
413424
Concept ID:
C2750887
Finding
For a general phenotypic description and a discussion of genetic heterogeneity of idiopathic generalized epilepsy, see 600669. Juvenile myoclonic epilepsy is a subtype of idiopathic generalized epilepsy; see 254770 for a general phenotypic description and a discussion of genetic heterogeneity of JME.
Epilepsy, familial adult myoclonic, 4
MedGen UID:
767474
Concept ID:
C3554560
Disease or Syndrome
Intellectual developmental disorder, autosomal recessive 74
MedGen UID:
934651
Concept ID:
C4310684
Disease or Syndrome
MRT74 is characterized by intellectual impairment, macrocephaly, and dysmorphic features. Epilepsy with eyelid myoclonus has also been reported (Almuriekhi et al., 2015; Mastrangelo et al., 2020).
Developmental and epileptic encephalopathy, 56
MedGen UID:
1621755
Concept ID:
C4540034
Disease or Syndrome
Developmental and epileptic encephalopathy-56 (DEE56) is a neurodevelopmental disorder characterized by early-onset seizures in most patients, followed by impaired intellectual development, variable behavioral abnormalities, and sometimes additional neurologic features, such as ataxia (summary by Guella et al., 2017). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.
Intellectual developmental disorder 60 with seizures
MedGen UID:
1684702
Concept ID:
C5231497
Disease or Syndrome
Autosomal dominant intellectual developmental disorder-60 with seizures is characterized by global developmental delay apparent in infancy, followed by onset of seizures in the first years of life. Patients have delayed walking, an ataxic gait, and moderately to severely impaired intellectual development with poor speech (summary by Helbig et al., 2019).
Intellectual developmental disorder with seizures and language delay
MedGen UID:
1740295
Concept ID:
C5436574
Disease or Syndrome
SETD1B-related neurodevelopmental disorder (SETD1B-NDD) is characterized by developmental delay (mainly affecting speech and language), intellectual disability, seizures, autism spectrum disorder or autism-like behaviors, and additional behavioral concerns. Speech delay and/or language disorder has been reported in most affected individuals. Delay in gross motor skills and mild-to-moderate intellectual disability are common. Most affected individuals have seizures with variable onset and seizure type. Behavioral issues including hyperactivity, aggression, anxiety, and sleep disorders have been reported in approximately half of individuals. Less common features include ophthalmologic manifestations and feeding issues.
Developmental and epileptic encephalopathy 103
MedGen UID:
1809962
Concept ID:
C5677002
Disease or Syndrome
Developmental and epileptic encephalopathy-103 (DEE103) is characterized by onset of various types of seizures in the first year of life, most of which are refractory to treatment. Affected individuals show global developmental delay with impaired intellectual development ranging from mild to severe. Additional features may include hypotonia, ataxia, and behavioral abnormalities, including autism and hyperactivity (Schwarz et al., 2022). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.

Professional guidelines

PubMed

Ma X, Zhang Y, Yang Z, Liu X, Sun H, Qin J, Wu X, Liang J
Brain Dev 2011 Feb;33(2):114-9. Epub 2010 Apr 7 doi: 10.1016/j.braindev.2010.02.004. PMID: 20378290

Recent clinical studies

Etiology

Jiang Y, Zhou X
Medicine (Baltimore) 2022 Sep 23;101(38):e30512. doi: 10.1097/MD.0000000000030512. PMID: 36197249Free PMC Article
Gaballa A, Woermann FG, Cloppenborg T, Kalbhenn T, Blümcke I, Bien CG, Fauser S
Epilepsia 2021 Dec;62(12):2920-2931. Epub 2021 Oct 11 doi: 10.1111/epi.17084. PMID: 34636039
Camacho A, García-Navarro M, Martínez B, Villarejo A, Pomares E
Clin Neuropharmacol 2005 May-Jun;28(3):145-7. doi: 10.1097/01.wnf.0000165351.10841.fa. PMID: 15965316
Oguni H, Fukuyama Y, Tanaka T, Hayashi K, Funatsuka M, Sakauchi M, Shirakawa S, Osawa M
Brain Dev 2001 Nov;23(7):757-64. doi: 10.1016/s0387-7604(01)00281-9. PMID: 11701290
Talwar D, Arora MS, Sher PK
Epilepsia 1994 Nov-Dec;35(6):1154-9. doi: 10.1111/j.1528-1157.1994.tb01782.x. PMID: 7988504

Diagnosis

Jiang Y, Zhou X
Medicine (Baltimore) 2022 Sep 23;101(38):e30512. doi: 10.1097/MD.0000000000030512. PMID: 36197249Free PMC Article
Gaballa A, Woermann FG, Cloppenborg T, Kalbhenn T, Blümcke I, Bien CG, Fauser S
Epilepsia 2021 Dec;62(12):2920-2931. Epub 2021 Oct 11 doi: 10.1111/epi.17084. PMID: 34636039
Scheffer IE
Eur J Paediatr Neurol 2012 Sep;16 Suppl 1:S5-8. Epub 2012 Jun 16 doi: 10.1016/j.ejpn.2012.04.007. PMID: 22704920
Seneviratne U, Cook M, D'Souza W
Epilepsia 2012 Feb;53(2):234-48. Epub 2011 Dec 9 doi: 10.1111/j.1528-1167.2011.03344.x. PMID: 22150583
Shian WJ, Chi CS
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Jun;53(6):375-8. PMID: 8087714

Therapy

van Gemert LA, van Alfen N, van Gaal L, Wortmann S, Willemsen MA
Neuropediatrics 2023 Dec;54(6):365-370. Epub 2023 Jul 21 doi: 10.1055/a-2134-8766. PMID: 37478891Free PMC Article
Jiang Y, Zhou X
Medicine (Baltimore) 2022 Sep 23;101(38):e30512. doi: 10.1097/MD.0000000000030512. PMID: 36197249Free PMC Article
Hogan RE, Trammel ER, Farber NB, Avidan MS, Palanca BJA
J ECT 2019 Dec;35(4):e39-e45. doi: 10.1097/YCT.0000000000000597. PMID: 31764454Free PMC Article
Camacho A, García-Navarro M, Martínez B, Villarejo A, Pomares E
Clin Neuropharmacol 2005 May-Jun;28(3):145-7. doi: 10.1097/01.wnf.0000165351.10841.fa. PMID: 15965316
Shian WJ, Chi CS
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Jun;53(6):375-8. PMID: 8087714

Prognosis

Gaballa A, Woermann FG, Cloppenborg T, Kalbhenn T, Blümcke I, Bien CG, Fauser S
Epilepsia 2021 Dec;62(12):2920-2931. Epub 2021 Oct 11 doi: 10.1111/epi.17084. PMID: 34636039
Hogan RE, Trammel ER, Farber NB, Avidan MS, Palanca BJA
J ECT 2019 Dec;35(4):e39-e45. doi: 10.1097/YCT.0000000000000597. PMID: 31764454Free PMC Article
Scheffer IE
Eur J Paediatr Neurol 2012 Sep;16 Suppl 1:S5-8. Epub 2012 Jun 16 doi: 10.1016/j.ejpn.2012.04.007. PMID: 22704920
Oguni H, Fukuyama Y, Tanaka T, Hayashi K, Funatsuka M, Sakauchi M, Shirakawa S, Osawa M
Brain Dev 2001 Nov;23(7):757-64. doi: 10.1016/s0387-7604(01)00281-9. PMID: 11701290
Talwar D, Arora MS, Sher PK
Epilepsia 1994 Nov-Dec;35(6):1154-9. doi: 10.1111/j.1528-1157.1994.tb01782.x. PMID: 7988504

Clinical prediction guides

Gaballa A, Woermann FG, Cloppenborg T, Kalbhenn T, Blümcke I, Bien CG, Fauser S
Epilepsia 2021 Dec;62(12):2920-2931. Epub 2021 Oct 11 doi: 10.1111/epi.17084. PMID: 34636039
Khachidze I, Gugushvili M, Makashvili M, Maloletnev V
Int J Neurosci 2016 Oct;126(10):912-21. Epub 2015 Aug 17 doi: 10.3109/00207454.2015.1083991. PMID: 26290045
Marten F, Rodrigues S, Benjamin O, Richardson MP, Terry JR
Philos Trans A Math Phys Eng Sci 2009 Mar 28;367(1891):1145-61. doi: 10.1098/rsta.2008.0255. PMID: 19218156
Santiago-Rodríguez E, Harmony T, Fernández-Bouzas A, Hernández-Balderas A, Martínez-López M, Graef A, Carlos García J, Fernández T
Seizure 2002 Jul;11(5):320-4. doi: 10.1053/seiz.2002.0676. PMID: 12076104
Serratosa JM, Delgado-Escueta AV, Medina MT, Zhang Q, Iranmanesh R, Sparkes RS
Ann Neurol 1996 Feb;39(2):187-95. doi: 10.1002/ana.410390208. PMID: 8967750

Recent systematic reviews

Tashkandi M, Baarma D, Tricco AC, Boelman C, Alkhater R, Minassian BA
Epileptic Disord 2019 Feb 1;21(1):30-41. doi: 10.1684/epd.2019.1024. PMID: 30767897

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