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Focal hemiclonic seizure

MedGen UID:
335477
Concept ID:
C1846620
Disease or Syndrome; Finding
Synonyms: Hemiclonic seizures; Unilateral clonic seizures
 
HPO: HP:0006813

Definition

A type of focal clonic seizure characterized by sustained rhythmic jerking rapidly involves one side of the body at seizure onset. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal hemiclonic seizure

Conditions with this feature

Severe myoclonic epilepsy in infancy
MedGen UID:
148243
Concept ID:
C0751122
Disease or Syndrome
SCN1A seizure disorders encompass a spectrum that ranges from simple febrile seizures and generalized epilepsy with febrile seizures plus (GEFS+) at the mild end to Dravet syndrome and intractable childhood epilepsy with generalized tonic-clonic seizures (ICE-GTC) at the severe end. Phenotypes with intractable seizures including Dravet syndrome are often associated with cognitive decline. Less commonly observed phenotypes include myoclonic astatic epilepsy (MAE), Lennox-Gastaut syndrome, infantile spasms, epilepsy with focal seizures, and vaccine-related encephalopathy and seizures. The phenotype of SCN1A seizure disorders can vary even within the same family.
Developmental and epileptic encephalopathy, 9
MedGen UID:
338393
Concept ID:
C1848137
Disease or Syndrome
Developmental and epileptic encephalopathy-9 (DEE9) is an X-linked disorder characterized by seizure onset in infancy and mild to severe intellectual impairment. Autistic and psychiatric features have been reported in some individuals. The disorder affects heterozygous females only; transmitting males are unaffected (summary by Jamal et al., 2010). For a general phenotypic description and a discussion of genetic heterogeneity of developmental and epileptic encephalopathy, see 308350.
Generalized epilepsy with febrile seizures plus, type 2
MedGen UID:
388117
Concept ID:
C1858673
Disease or Syndrome
SCN1A seizure disorders encompass a spectrum that ranges from simple febrile seizures and generalized epilepsy with febrile seizures plus (GEFS+) at the mild end to Dravet syndrome and intractable childhood epilepsy with generalized tonic-clonic seizures (ICE-GTC) at the severe end. Phenotypes with intractable seizures including Dravet syndrome are often associated with cognitive decline. Less commonly observed phenotypes include myoclonic astatic epilepsy (MAE), Lennox-Gastaut syndrome, infantile spasms, epilepsy with focal seizures, and vaccine-related encephalopathy and seizures. The phenotype of SCN1A seizure disorders can vary even within the same family.
Developmental and epileptic encephalopathy, 13
MedGen UID:
482821
Concept ID:
C3281191
Disease or Syndrome
SCN8A-related epilepsy with encephalopathy is characterized by developmental delay, seizure onset in the first 18 months of life (mean 4 months), and intractable epilepsy characterized by multiple seizure types (generalized tonic-clonic seizures, infantile spasms, and absence and focal seizures). Epilepsy syndromes can include Lennox-Gastaut syndrome, West syndrome, and epileptic encephalopathies (e.g., Dravet syndrome). Hypotonia and movement disorders including dystonia, ataxia, and choreoathetosis are common. Psychomotor development varies from normal prior to seizure onset (with subsequent slowing or regression after seizure onset) to abnormal from birth. Intellectual disability, present in all, ranges from mild to severe (in ~50% of affected individuals). Autistic features are noted in some. Sudden unexpected death in epilepsy (SUDEP) of unknown cause has been reported in approximately 10% of published cases. To date SCN8A-related epilepsy with encephalopathy has been reported in the literature in about 50 individuals.
Developmental and epileptic encephalopathy, 19
MedGen UID:
816730
Concept ID:
C3810400
Disease or Syndrome
Developmental and epileptic encephalopathy-19 (DEE19) is a neurologic disorder characterized by the onset of various types of seizures in the first year of life, usually between 8 and 12 months of age. Seizures are often triggered by fever, and status epilepticus may occur. Affected individuals subsequently show mildly to moderately impaired intellectual development. Brain imaging is typically normal. The clinical phenotype is similar to that of Dravet syndrome (DRVT; 607208) (summary by Carvill et al., 2014). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.
Developmental and epileptic encephalopathy, 34
MedGen UID:
899149
Concept ID:
C4225257
Disease or Syndrome
SLC12A5-related epilepsy of infancy with migrating focal seizures (SLC12A5-EIMFS), reported to date in nine children, is characterized by onset of seizures before age six months and either developmental delay or developmental regression with seizure onset. Of these nine children, six had severe developmental delay with no progress of abilities and three made notable neurodevelopmental progress. Eight had postnatal microcephaly and hypotonia. In most children epilepsy begins as focal motor seizures (typically involving head and eye deviation) that become multifocal and intractable to conventional anti-seizure medication (ASM).
Developmental and epileptic encephalopathy, 37
MedGen UID:
934737
Concept ID:
C4310770
Disease or Syndrome
Developmental and epileptic encephalopathy-37 (DEE37) is an autosomal recessive epileptic-dyskinetic neurologic disorder characterized by the onset of intractable seizures or abnormal movements in the first months or years of life. Patients typically have normal or only mildly delayed development in early infancy, but then show developmental regression and stagnation after the onset of seizures, which can occur between about 6 months to 2 years of age. In addition to epileptic encephalopathy, affected individuals also manifest a hyperkinetic movement disorder with choreoathetosis, spasticity, and rigidity. There is severely impaired intellectual development and function, loss of verbal skills with absent speech, and impaired volitional movements (summary by Madeo et al., 2016). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.
Intellectual disability, autosomal dominant 42
MedGen UID:
934741
Concept ID:
C4310774
Mental or Behavioral Dysfunction
GNB1 encephalopathy (GNB1-E) is characterized by moderate-to-severe developmental delay / intellectual disability, structural brain abnormalities, and often infantile hypotonia and seizures. Other less common findings include dystonia, reduced vision, behavior issues, growth delay, gastrointestinal (GI) problems, genitourinary (GU) abnormalities in males, and cutaneous mastocytosis.
Developmental and epileptic encephalopathy, 52
MedGen UID:
1376462
Concept ID:
C4479236
Disease or Syndrome
Developmental and epileptic encephalopathy-52 (DEE52) is a severe autosomal recessive seizure disorder characterized by infantile onset of refractory seizures with resultant delayed global neurologic development. Affected individuals have impaired intellectual development and may have other persistent neurologic abnormalities, including axial hypotonia and spasticity; death in childhood may occur (summary by Patino et al., 2009 and Ramadan et al., 2017). Some patients with DEE52 may have a clinical diagnosis of Dravet syndrome (607208), which is characterized by the onset of seizures in the first year or 2 of life after normal early development. Developmental delay, impaired intellectual development, and behavioral abnormalities usually become apparent later between 1 and 4 years of age. Dravet syndrome may also include 'severe myoclonic epilepsy in infancy' (SMEI) (summary by Patino et al., 2009). For a discussion of genetic heterogeneity of DEE, see 308350.
Developmental and epileptic encephalopathy, 67
MedGen UID:
1648285
Concept ID:
C4748341
Disease or Syndrome
Developmental and epileptic encephalopathy-67 (DEE67) is characterized by the onset of various types of seizures in the first months of life, although later onset may occur in milder cases. The seizures tend to be resistant to treatment. Affected individuals have global developmental delay with impaired motor and intellectual development, poor or absent speech, movement disorders, and stereotypic or autistic behavior (summary by Chatron et al., 2018). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.
Developmental and epileptic encephalopathy 6B
MedGen UID:
1779648
Concept ID:
C5543353
Disease or Syndrome
SCN1A seizure disorders encompass a spectrum that ranges from simple febrile seizures and generalized epilepsy with febrile seizures plus (GEFS+) at the mild end to Dravet syndrome and intractable childhood epilepsy with generalized tonic-clonic seizures (ICE-GTC) at the severe end. Phenotypes with intractable seizures including Dravet syndrome are often associated with cognitive decline. Less commonly observed phenotypes include myoclonic astatic epilepsy (MAE), Lennox-Gastaut syndrome, infantile spasms, epilepsy with focal seizures, and vaccine-related encephalopathy and seizures. The phenotype of SCN1A seizure disorders can vary even within the same family.
Developmental and epileptic encephalopathy 99
MedGen UID:
1794228
Concept ID:
C5562018
Disease or Syndrome
Developmental and epileptic encephalopathy-99 (DEE99) is characterized by onset of seizures in early childhood associated with global developmental delay and severely impaired intellectual development. Other features may include hypotonia, quadriparesis, nystagmus, and apnea. Brain imaging may be normal or show nonspecific and variable abnormalities, including cerebral atrophy and polymicrogyria. The severity is variable; some patients die of refractory status epilepticus (summary by Vetro et al., 2021). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.
Developmental and epileptic encephalopathy 109
MedGen UID:
1824036
Concept ID:
C5774263
Disease or Syndrome
Developmental and epileptic encephalopathy-109 (DEE109) is characterized by the onset of various types of seizures in the first months or years of life. Affected individuals show developmental delay before and concurrent with the onset of seizures. Features include impaired intellectual development with poor speech, ataxic gait, coordination problems, and behavioral abnormalities (Manivannan et al., 2022). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.

Professional guidelines

PubMed

He Z, Li Y, Zhao X, Li B
Epilepsy Res 2022 Dec;188:107041. Epub 2022 Oct 29 doi: 10.1016/j.eplepsyres.2022.107041. PMID: 36368227
Verrotti A, Soldani C, Laino D, d'Alonzo R, Grosso S
World J Pediatr 2014 May;10(2):108-13. Epub 2014 May 7 doi: 10.1007/s12519-014-0478-9. PMID: 24801229

Recent clinical studies

Etiology

Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Gardella E, Marini C, Trivisano M, Fitzgerald MP, Alber M, Howell KB, Darra F, Siliquini S, Bölsterli BK, Masnada S, Pichiecchio A, Johannesen KM, Jepsen B, Fontana E, Anibaldi G, Russo S, Cogliati F, Montomoli M, Specchio N, Rubboli G, Veggiotti P, Beniczky S, Wolff M, Helbig I, Vigevano F, Scheffer IE, Guerrini R, Møller RS
Neurology 2018 Sep 18;91(12):e1112-e1124. Epub 2018 Aug 31 doi: 10.1212/WNL.0000000000006199. PMID: 30171078
Demarest ST, Whitehead MT, Turnacioglu S, Pearl PL, Gropman AL
J Child Neurol 2014 Sep;29(9):1249-56. Epub 2014 Jul 17 doi: 10.1177/0883073814538511. PMID: 25038129
Specchio N, Pontrelli G, Serino D, Trivisano M, Cappelletti S, Terracciano A, Vigevano F, Fusco L
Seizure 2014 Apr;23(4):309-13. Epub 2014 Jan 5 doi: 10.1016/j.seizure.2013.12.009. PMID: 24472396
Xu X, Zhang Y, Sun H, Liu X, Yang X, Xiong H, Jiang Y, Bao X, Wang S, Yang Z, Wu Y, Qin J, Lin Q, Wu X
Brain Dev 2014 Sep;36(8):676-81. Epub 2013 Oct 26 doi: 10.1016/j.braindev.2013.10.004. PMID: 24168886

Diagnosis

Caraballo R, Guzman A, Beltrán L, Espeche A
Epileptic Disord 2024 Feb;26(1):121-125. Epub 2023 Nov 2 doi: 10.1002/epd2.20170. PMID: 37815756
Connolly MB
Can J Neurol Sci 2016 Jun;43 Suppl 3:S3-8. doi: 10.1017/cjn.2016.243. PMID: 27264139
Verrotti A, Soldani C, Laino D, d'Alonzo R, Grosso S
World J Pediatr 2014 May;10(2):108-13. Epub 2014 May 7 doi: 10.1007/s12519-014-0478-9. PMID: 24801229
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
Sakuma H
Brain Dev 2009 Aug;31(7):510-4. Epub 2009 Mar 26 doi: 10.1016/j.braindev.2009.02.010. PMID: 19327924

Therapy

He Z, Li Y, Zhao X, Li B
Epilepsy Res 2022 Dec;188:107041. Epub 2022 Oct 29 doi: 10.1016/j.eplepsyres.2022.107041. PMID: 36368227
Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Verrotti A, Soldani C, Laino D, d'Alonzo R, Grosso S
World J Pediatr 2014 May;10(2):108-13. Epub 2014 May 7 doi: 10.1007/s12519-014-0478-9. PMID: 24801229
Sakuma H
Brain Dev 2009 Aug;31(7):510-4. Epub 2009 Mar 26 doi: 10.1016/j.braindev.2009.02.010. PMID: 19327924
Yalçin AD, Kaymaz A, Forta H
Brain Dev 1997 Sep;19(6):408-13. doi: 10.1016/s0387-7604(97)00054-5. PMID: 9339869

Prognosis

He Z, Li Y, Zhao X, Li B
Epilepsy Res 2022 Dec;188:107041. Epub 2022 Oct 29 doi: 10.1016/j.eplepsyres.2022.107041. PMID: 36368227
Connolly MB
Can J Neurol Sci 2016 Jun;43 Suppl 3:S3-8. doi: 10.1017/cjn.2016.243. PMID: 27264139
Verrotti A, Soldani C, Laino D, d'Alonzo R, Grosso S
World J Pediatr 2014 May;10(2):108-13. Epub 2014 May 7 doi: 10.1007/s12519-014-0478-9. PMID: 24801229
Xu X, Zhang Y, Sun H, Liu X, Yang X, Xiong H, Jiang Y, Bao X, Wang S, Yang Z, Wu Y, Qin J, Lin Q, Wu X
Brain Dev 2014 Sep;36(8):676-81. Epub 2013 Oct 26 doi: 10.1016/j.braindev.2013.10.004. PMID: 24168886
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

Clinical prediction guides

Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Xu X, Zhang Y, Sun H, Liu X, Yang X, Xiong H, Jiang Y, Bao X, Wang S, Yang Z, Wu Y, Qin J, Lin Q, Wu X
Brain Dev 2014 Sep;36(8):676-81. Epub 2013 Oct 26 doi: 10.1016/j.braindev.2013.10.004. PMID: 24168886

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