Benign rolandic epilepsy

Infobox Disease
Name = PAGENAME


Caption = Diagram showing the central sulcus of the brain.
DiseasesDB = 33998
ICD10 = ICD10|G|40|0|g|40
ICD9 =
ICDO =
OMIM = 117100
MedlinePlus =
eMedicineSubj = neuro
eMedicineTopic = 641
MeshID = 019305In 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.cite journal |author=Kramer U |title=Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review |journal=J. Child Neurol. |volume=23 |issue=7 |pages=785–90 |year=2008 |month=July |pmid=18658078 |doi=10.1177/0883073808316363 |url=http://jcn.sagepub.com/cgi/pmidlookup?view=long&pmid=18658078] 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.cite journal |author=Wirrell EC |title=Benign epilepsy of childhood with centrotemporal spikes |journal=Epilepsia |volume=39 Suppl 4 |issue= |pages=S32–41 |year=1998 |pmid=9637591 |doi= |url=] The seizures start around the central sulcus of the brain (also called the centrotemporal area, located around the Rolandic fissure, after Luigi Rolando). [http://www.epilepsy.org.uk/info/benign.html Benign rolandic epilepsy] . Retrieved August 8, 2008.]

ymptoms

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. Seizures tend to occur more often during the night. Psychic manifestations, auras and automatisms are lacking. The seizure frequency is often low. These children usually have normal intelligence and development. Atypical features however, such as developmental delay or daytime seizures, are common.

Diagnosis

The diagnosis can be confirmed when the characteristic centrotemporal spikes are seen on electroencephalography (EEG). [Blueprints Neurology, 2nd ed.] Typically, high-voltage spikes followed by slow waves are seen.cite journal |author=Stephani U |title=Typical semiology of benign childhood epilepsy with centrotemporal spikes (BCECTS) |journal=Epileptic Disord |volume=2 Suppl 1 |issue= |pages=S3–4 |year=2000 |pmid=11231216 |doi= |url=http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=2%20Suppl%201&iss=&page=S3] 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.cite journal |author=Chahine LM, Mikati MA |title=Benign pediatric localization-related epilepsies |journal=Epileptic Disord |volume=8 |issue=4 |pages=243–58 |year=2006 |month=December |pmid=17150437 |doi= |url=http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=8&iss=4&page=243] 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. 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. Bedtime dosing is advised by some.cite journal |author=McAbee GN, Wark JE |title=A practical approach to uncomplicated seizures in children |journal=Am Fam Physician |volume=62 |issue=5 |pages=1109–16 |year=2000 |month=September |pmid=10997534 |doi= |url=] Treatment can be short and drugs can almost certainly be discontinued after two years without seizures and with normal EEG findings, perhaps even earlier.

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.cite journal |author=Neubauer BA |title=The genetics of rolandic epilepsy |journal=Epileptic Disord |volume=2 Suppl 1 |issue= |pages=S67–8 |year=2000 |pmid=11231229 |doi= |url=http://www.john-libbey-eurotext.fr/medline.md?issn=1294-9361&vol=2%20Suppl%201&iss=&page=S67] cite journal |author=Bali B, Kull LL, Strug LJ, "et al" |title=Autosomal dominant inheritance of centrotemporal sharp waves in rolandic epilepsy families |journal=Epilepsia |volume=48 |issue=12 |pages=2266–72 |year=2007 |month=December |pmid=17662063 |pmc=2150739 |doi=10.1111/j.1528-1167.2007.01221.x |url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0013-9580&date=2007&volume=48&issue=12&spage=2266] Linkage studies have pointed to a possible susceptibility region on chromosome 15q14, in the vicinity of the alpha-7 subunit of the acetylcholine receptor.cite journal |author=Neubauer BA, Fiedler B, Himmelein B, "et al" |title=Centrotemporal spikes in families with rolandic epilepsy: linkage to chromosome 15q14 |journal=Neurology |volume=51 |issue=6 |pages=1608–12 |year=1998 |month=December |pmid=9855510 |doi= |url=] Most studies show a slight male predominance. Because of the benign course and age-specific occurrence, it is thought to represent a hereditary impairment of brain maturation.

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.

Footnotes

ee also

* Generalized epilepsy with febrile seizures plus
* Benign childhood epilepsy with occipital paroxysms