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EEG with centrotemporal focal spike waves

MedGen UID:
868454
Concept ID:
C4022848
Finding
HPO: HP:0012557

Definition

EEG with focal sharp transient waves in the centrotemporal region of the brain (also known as the central sulcus), i.e., focal sharp waves of a duration less than 80 msec followed by a slow wave. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEEG with centrotemporal focal spike waves

Conditions with this feature

Landau-Kleffner syndrome
MedGen UID:
79465
Concept ID:
C0282512
Disease or Syndrome
GRIN2A-related speech disorders and epilepsy are characterized by speech disorders in all affected individuals and a range of epilepsy syndromes present in about 90%. Severe speech disorders observed can include dysarthria and speech dyspraxia, and both receptive and expressive language delay/regression; more mildly affected individuals may display subtly impaired intelligibility of conversational speech. Epilepsy features include seizure onset usually between ages three and six years, focal epilepsy with language and/or global developmental regression, and electroencephalogram (EEG) showing continuous spike-and-wave discharges in sleep or very active centrotemporal discharges. Seizure types include seizures associated with aura of perioral paresthesia, focal or focal motor seizures (often evolving to generalized tonic-clonic), and atypical absence seizures. Epilepsy syndromes can include: Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (ECSWS), childhood epilepsy with centrotemporal spikes (CECTS), atypical childhood epilepsy with centrotemporal spikes (ACECTS), autosomal dominant rolandic epilepsy with speech dyspraxia (ADRESD), and infantile-onset epileptic encephalopathy.
Benign Rolandic epilepsy
MedGen UID:
432274
Concept ID:
C2363129
Disease or Syndrome
Benign epilepsy of childhood with centrotemporal spikes (BECTS) or sharp waves, also known as rolandic epilepsy, is the most common idiopathic childhood epilepsy syndrome (Neubauer et al., 1998). It is termed 'rolandic' epilepsy because of the characteristic features of partial seizures involving the region around the lower portion of the central gyrus of Rolando. This results in classic focal seizures that affect the vocal tract, beginning with guttural sounds at the larynx and sensorimotor symptoms that progress to the tongue, mouth, and face, resulting in hypersalivation and speech arrest. Seizures most often occur in sleep shortly before awakening. The disorder occurs more often in boys than in girls (3:2). Rolandic epilepsy is considered a neurodevelopmental disorder, affecting 0.2% of the population. Affected individuals may have learning disabilities or behavioral problems; however, the seizures and accompanying problems usually remit during adolescence (summary by Strug et al., 2009). See also focal epilepsy and speech disorder (FESD; 245570), which is caused by mutation in the GRIN2A gene (138253) on chromosome 16p13. Some patients with GRIN2A mutations show features consistent with a clinical diagnosis of BECTS. Some patients with DEPDC5 (614191) mutations may show features consistent with rolandic epilepsy (see FFEVF, 604364).

Recent clinical studies

Etiology

Chen LC, Li X, Shen L
Acta Neurol Scand 2021 Feb;143(2):188-194. Epub 2020 Oct 13 doi: 10.1111/ane.13350. PMID: 32975833
Pal DK, Ferrie C, Addis L, Akiyama T, Capovilla G, Caraballo R, de Saint-Martin A, Fejerman N, Guerrini R, Hamandi K, Helbig I, Ioannides AA, Kobayashi K, Lal D, Lesca G, Muhle H, Neubauer BA, Pisano T, Rudolf G, Seegmuller C, Shibata T, Smith A, Striano P, Strug LJ, Szepetowski P, Valeta T, Yoshinaga H, Koutroumanidis M
Epileptic Disord 2016 Sep 1;18(3):252-88. doi: 10.1684/epd.2016.0839. PMID: 27435520
van Klink NE, van 't Klooster MA, Leijten FS, Jacobs J, Braun KP, Zijlmans M
Epilepsia 2016 Jul;57(7):1179-89. Epub 2016 Jun 8 doi: 10.1111/epi.13423. PMID: 27270830
Vigevano F, Specchio N, Fejerman N
Handb Clin Neurol 2013;111:591-604. doi: 10.1016/B978-0-444-52891-9.00061-0. PMID: 23622206
Sánchez Fernández I, Loddenkemper T
J Clin Neurophysiol 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943. PMID: 23027100

Diagnosis

Moeller F, Stephani U, Siniatchkin M
Epilepsia 2013 Jun;54(6):971-82. Epub 2013 May 3 doi: 10.1111/epi.12197. PMID: 23647021
Vigevano F, Specchio N, Fejerman N
Handb Clin Neurol 2013;111:591-604. doi: 10.1016/B978-0-444-52891-9.00061-0. PMID: 23622206
Sánchez Fernández I, Loddenkemper T
J Clin Neurophysiol 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943. PMID: 23027100
Westmoreland BF
Epilepsia 1998;39 Suppl 4:S1-8. doi: 10.1111/j.1528-1157.1998.tb05121.x. PMID: 9637588
Westmoreland BF
Mayo Clin Proc 1996 May;71(5):501-11. doi: 10.4065/71.5.501. PMID: 8628033

Therapy

Vargas R, Beltrán L, Lizama R, Valenzuela GR, Caraballo R
Seizure 2018 Apr;57:27-31. Epub 2018 Mar 9 doi: 10.1016/j.seizure.2018.03.004. PMID: 29547828
Pal DK, Ferrie C, Addis L, Akiyama T, Capovilla G, Caraballo R, de Saint-Martin A, Fejerman N, Guerrini R, Hamandi K, Helbig I, Ioannides AA, Kobayashi K, Lal D, Lesca G, Muhle H, Neubauer BA, Pisano T, Rudolf G, Seegmuller C, Shibata T, Smith A, Striano P, Strug LJ, Szepetowski P, Valeta T, Yoshinaga H, Koutroumanidis M
Epileptic Disord 2016 Sep 1;18(3):252-88. doi: 10.1684/epd.2016.0839. PMID: 27435520
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
Sánchez Fernández I, Loddenkemper T
J Clin Neurophysiol 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943. PMID: 23027100
Gelisse P, Genton P, Raybaud C, Thiry A, Pincemaille O
Epilepsia 1999 Sep;40(9):1312-5. doi: 10.1111/j.1528-1157.1999.tb00864.x. PMID: 10487198

Prognosis

Alving J, Fabricius M, Rosenzweig I, Beniczky S
Seizure 2017 Nov;52:7-10. Epub 2017 Sep 14 doi: 10.1016/j.seizure.2017.09.006. PMID: 28926747Free PMC Article
Pal DK, Ferrie C, Addis L, Akiyama T, Capovilla G, Caraballo R, de Saint-Martin A, Fejerman N, Guerrini R, Hamandi K, Helbig I, Ioannides AA, Kobayashi K, Lal D, Lesca G, Muhle H, Neubauer BA, Pisano T, Rudolf G, Seegmuller C, Shibata T, Smith A, Striano P, Strug LJ, Szepetowski P, Valeta T, Yoshinaga H, Koutroumanidis M
Epileptic Disord 2016 Sep 1;18(3):252-88. doi: 10.1684/epd.2016.0839. PMID: 27435520
Vigevano F, Specchio N, Fejerman N
Handb Clin Neurol 2013;111:591-604. doi: 10.1016/B978-0-444-52891-9.00061-0. PMID: 23622206
Sánchez Fernández I, Loddenkemper T
J Clin Neurophysiol 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943. PMID: 23027100
Specchio N, Vigevano F
Epilepsy Res 2006 Aug;70 Suppl 1:S156-67. Epub 2006 Jul 11 doi: 10.1016/j.eplepsyres.2006.01.018. PMID: 16837167

Clinical prediction guides

Chen LC, Li X, Shen L
Acta Neurol Scand 2021 Feb;143(2):188-194. Epub 2020 Oct 13 doi: 10.1111/ane.13350. PMID: 32975833
Vargas R, Beltrán L, Lizama R, Valenzuela GR, Caraballo R
Seizure 2018 Apr;57:27-31. Epub 2018 Mar 9 doi: 10.1016/j.seizure.2018.03.004. PMID: 29547828
Pal DK, Ferrie C, Addis L, Akiyama T, Capovilla G, Caraballo R, de Saint-Martin A, Fejerman N, Guerrini R, Hamandi K, Helbig I, Ioannides AA, Kobayashi K, Lal D, Lesca G, Muhle H, Neubauer BA, Pisano T, Rudolf G, Seegmuller C, Shibata T, Smith A, Striano P, Strug LJ, Szepetowski P, Valeta T, Yoshinaga H, Koutroumanidis M
Epileptic Disord 2016 Sep 1;18(3):252-88. doi: 10.1684/epd.2016.0839. PMID: 27435520
van Klink NE, van 't Klooster MA, Leijten FS, Jacobs J, Braun KP, Zijlmans M
Epilepsia 2016 Jul;57(7):1179-89. Epub 2016 Jun 8 doi: 10.1111/epi.13423. PMID: 27270830
Sánchez Fernández I, Loddenkemper T
J Clin Neurophysiol 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943. PMID: 23027100

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