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Benign Rolandic epilepsy(BECTS)

MedGen UID:
432274
Concept ID:
C2363129
Disease or Syndrome
Synonyms: BECTS; BENIGN EPILEPSY OF CHILDHOOD WITH CENTROTEMPORAL SPIKES; Benign epilepsy of childhood with centrotemporal spikes (BECCT); Benign epilepsy with centro-temporal spikes (BECTS); Benign rolandic epilepsy (BRE); Benign rolandic epilepsy of childhood (BREC); CENTRALOPATHIC EPILEPSY; Centrotemporal epilepsy; TEMPORAL-CENTRAL FOCAL EPILEPSY
SNOMED CT: Centralopathic epilepsy (44145005); Temporal-central focal epilepsy (44145005); Centrotemporal epilepsy (44145005); Benign Rolandic epilepsy (44145005)
 
OMIM®: 117100

Definition

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). [from OMIM]

Clinical features

From HPO
Nocturnal seizures
MedGen UID:
581539
Concept ID:
C0393719
Disease or Syndrome
Seizures that occur while the affected individual is sleeping.
Focal-onset seizure
MedGen UID:
199670
Concept ID:
C0751495
Disease or Syndrome
A focal-onset seizure is a type of seizure originating within networks limited to one hemisphere. They may be discretely localized or more widely distributed, and may originate in subcortical structures.
Bilateral tonic-clonic seizure with focal onset
MedGen UID:
164077
Concept ID:
C0877017
Disease or Syndrome
A bilateral tonic-clonic seizure with focal onset is a focal-onset seizure which progresses into a bilateral tonic-clonic phase.
EEG with centrotemporal focal spike waves
MedGen UID:
868454
Concept ID:
C4022848
Finding
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.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

McNally MA, Kossoff EH
Epilepsy Behav 2015 Feb;43:135-8. Epub 2015 Jan 24 doi: 10.1016/j.yebeh.2014.12.007. PMID: 25623811
Peters JM, Camfield CS, Camfield PR
Neurology 2001 Aug 14;57(3):537-9. doi: 10.1212/wnl.57.3.537. PMID: 11502931
Rating D
Epileptic Disord 2000;2 Suppl 1:S69-72. PMID: 11231230

Recent clinical studies

Etiology

Curnow SR, Vogrin SJ, Barton S, Bailey CA, Harvey AS
Epilepsy Res 2020 Mar;161:106288. Epub 2020 Feb 10 doi: 10.1016/j.eplepsyres.2020.106288. PMID: 32086099
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
Park JT, Shahid AM, Jammoul A
Pediatr Ann 2015 Feb;44(2):e30-5. doi: 10.3928/00904481-20150203-09. PMID: 25658216
Guerrini R, Pellacani S
Epilepsia 2012 Sep;53 Suppl 4:9-18. doi: 10.1111/j.1528-1167.2012.03609.x. PMID: 22946717
Glauser TA
Curr Opin Pediatr 1995 Dec;7(6):640-9. doi: 10.1097/00008480-199512000-00003. PMID: 8776013

Diagnosis

Ghantasala R, Holmes GL
Epileptic Disord 2019 Dec 1;21(6):567-578. doi: 10.1684/epd.2019.1111. PMID: 31829304
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
Park JT, Shahid AM, Jammoul A
Pediatr Ann 2015 Feb;44(2):e30-5. doi: 10.3928/00904481-20150203-09. PMID: 25658216
Glauser TA
Curr Opin Pediatr 1995 Dec;7(6):640-9. doi: 10.1097/00008480-199512000-00003. PMID: 8776013
Loiseau P, Duché B
Adv Neurol 1992;57:411-7. PMID: 1543070

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
Zhao X, Wang A, Wang S, Zhang T, Chi Z, Liu Y
Pediatr Int 2017 Jul;59(7):793-797. doi: 10.1111/ped.13274. PMID: 28258599
Verrotti A, D'Adamo E, Parisi P, Chiarelli F, Curatolo P
Paediatr Drugs 2010 Jun;12(3):177-86. doi: 10.2165/11316250-000000000-00000. PMID: 20486734
Rating D
Epileptic Disord 2000;2 Suppl 1:S69-72. PMID: 11231230
Genton P
Brain Dev 2000 Mar;22(2):75-80. doi: 10.1016/s0387-7604(99)00113-8. PMID: 10722956

Prognosis

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
Park JT, Shahid AM, Jammoul A
Pediatr Ann 2015 Feb;44(2):e30-5. doi: 10.3928/00904481-20150203-09. PMID: 25658216
Guerrini R, Pellacani S
Epilepsia 2012 Sep;53 Suppl 4:9-18. doi: 10.1111/j.1528-1167.2012.03609.x. PMID: 22946717
Rating D
Epileptic Disord 2000;2 Suppl 1:S69-72. PMID: 11231230
Aicardi J
Epilepsia 1988;29 Suppl 3:S1-5. doi: 10.1111/j.1528-1157.1988.tb05803.x. PMID: 3066614

Clinical prediction guides

Curnow SR, Vogrin SJ, Barton S, Bailey CA, Harvey AS
Epilepsy Res 2020 Mar;161:106288. Epub 2020 Feb 10 doi: 10.1016/j.eplepsyres.2020.106288. PMID: 32086099
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
Verrotti A, D'Adamo E, Parisi P, Chiarelli F, Curatolo P
Paediatr Drugs 2010 Jun;12(3):177-86. doi: 10.2165/11316250-000000000-00000. PMID: 20486734
Blume WT
Curr Opin Neurol 2001 Apr;14(2):193-7. doi: 10.1097/00019052-200104000-00010. PMID: 11262735
Aicardi J
Epilepsia 1988;29 Suppl 3:S1-5. doi: 10.1111/j.1528-1157.1988.tb05803.x. PMID: 3066614

Recent systematic reviews

Wickens S, Bowden SC, D'Souza W
Epilepsia 2017 Oct;58(10):1673-1685. Epub 2017 Aug 12 doi: 10.1111/epi.13865. PMID: 28801973

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