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Rolandic epilepsy
Classification and external resources

Diagram showing the central sulcus of the brain.
ICD-10 G40.0
OMIM 117100
DiseasesDB 33998
eMedicine neuro/641
MeSH D019305

In neurology and pediatrics, benign rolandic epilepsy or benign (childhood) epilepsy with centrotemporal (EEG) spikes (also known as sylvian seizures) is the most common epilepsy syndrome in childhood.[1] Most children will outgrow the syndrome (it starts around the age of 3-13 with a peak around 8–9 years and stops around age 14-18), hence the label benign.[2][3] The seizures start around the central sulcus of the brain (also called the centrotemporal area, located around the Rolandic fissure, after Luigi Rolando).[4]

Contents

Symptoms

Benign rolandic epilepsy is characterized by either simple partial seizures involving the mouth and face or generalized tonic-clonic seizures. There can be one-sided somatosensory manifestations such as tingling (paresthesia) of one side of the tongue, speech arrest (anarthria), gurgling or grunting noises or drooling.[2][3] Seizures tend to occur more often during the night.[2] Psychic manifestations, auras and automatisms are lacking. The seizure frequency is often low. These children usually have normal intelligence and development.[2] Atypical features however, such as developmental delay or daytime seizures, are common.[3] There is increasing data that a subset of children with atypical EEG or clinical features appear to be at risk for cognitive and behavioral problems, thus in some children the diagnosis may not be as "benign" as previously thought. Most children however appear to do well, typically outgrowing their seizures by adolescence.

Diagnosis

The diagnosis can be confirmed when the characteristic centrotemporal spikes are seen on electroencephalography (EEG).[5] Typically, high-voltage spikes followed by slow waves are seen.[6] Given the nocturnal activity, a sleep EEG can often be helpful. Technically, the label "benign" can only be confirmed if the child's development continues to be normal during follow-up.[3] Neuroimaging, usually with an MRI scan, is only advised for cases with atypical presentation or atypical findings on clinical examination or EEG.

Treatment

Given the benign nature of the condition and the low seizure frequency, treatment is often unnecessary. If treatment is warranted or preferred by the child and its family, antiepileptic drugs can usually control the seizures easily.[2] Carbamazepine is the most frequently used first-line drug, but many other antiepileptic drugs, including valproate, phenytoin, gabapentin, levetiracetam and sultiame have been found effective as well.[3] Bedtime dosing is advised by some.[7] Treatment can be short and drugs can almost certainly be discontinued after two years without seizures and with normal EEG findings, perhaps even earlier.[3]

Genetics and disease mechanism

Benign epilepsy with centrotemporal spikes is thought to be a genetic disorder. An autosomal dominant inheritance with age dependency and variable penetrance has been reported, although not all studies support this theory.[3][8][9] Linkage studies have pointed to a possible susceptibility region on chromosome 15q14, in the vicinity of the alpha-7 subunit of the acetylcholine receptor.[10] Most studies show a slight male predominance.[3] Because of the benign course and age-specific occurrence, it is thought to represent a hereditary impairment of brain maturation.[3]

An association with ELP4 has been identified.[11]

Differential diagnosis

The disorder should be differentiated from several other conditions, especially centrotemporal spikes without seizures, centrotemporal spikes with local brain pathology, central spikes in Rett syndrome and fragile X syndrome, malignant rolandic epilepsy, temporal lobe epilepsy and Landau-Kleffner syndrome.

References

  1. ^ Kramer U (July 2008). "Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review". J. Child Neurol. 23 (7): 785–90. doi:10.1177/0883073808316363. PMID 18658078. http://jcn.sagepub.com/cgi/pmidlookup?view=long&pmid=18658078.  
  2. ^ a b c d e Wirrell EC (1998). "Benign epilepsy of childhood with centrotemporal spikes". Epilepsia 39 Suppl 4: S32–41. PMID 9637591.  
  3. ^ a b c d e f g h i Chahine LM, Mikati MA (December 2006). "Benign pediatric localization-related epilepsies". Epileptic Disord 8 (4): 243–58. PMID 17150437. http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=8&iss=4&page=243.  
  4. ^ Benign rolandic epilepsy. Retrieved August 8, 2008.
  5. ^ Blueprints Neurology, 2nd ed.
  6. ^ Stephani U (2000). "Typical semiology of benign childhood epilepsy with centrotemporal spikes (BCECTS)". Epileptic Disord 2 Suppl 1: S3–4. PMID 11231216. http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=2%20Suppl%201&iss=&page=S3.  
  7. ^ McAbee GN, Wark JE (September 2000). "A practical approach to uncomplicated seizures in children". Am Fam Physician 62 (5): 1109–16. PMID 10997534.  
  8. ^ Neubauer BA (2000). "The genetics of rolandic epilepsy". Epileptic Disord 2 Suppl 1: S67–8. PMID 11231229. http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=2%20Suppl%201&iss=&page=S67.  
  9. ^ Bali B, Kull LL, Strug LJ, et al. (December 2007). "Autosomal dominant inheritance of centrotemporal sharp waves in rolandic epilepsy families". Epilepsia 48 (12): 2266–72. doi:10.1111/j.1528-1167.2007.01221.x. PMID 17662063. PMC 2150739. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0013-9580&date=2007&volume=48&issue=12&spage=2266.  
  10. ^ Neubauer BA, Fiedler B, Himmelein B, et al. (December 1998). "Centrotemporal spikes in families with rolandic epilepsy: linkage to chromosome 15q14". Neurology 51 (6): 1608–12. PMID 9855510.  
  11. ^ Strug LJ, Clarke T, Chiang T, et al. (January 2009). "Centrotemporal sharp wave EEG trait in rolandic epilepsy maps to Elongator Protein Complex 4 (ELP4)". Eur. J. Hum. Genet.. doi:10.1038/ejhg.2008.267. PMID 19172991.  

See also

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