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Rolandic epilepsy
Classification and external resources
Template:Px
Diagram showing the central sulcus of the brain.
ICD-10 G400
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]

Epidemiology Edit

The age of onset ranges from 1 to 14 years with 75% starting between 7–10 years. There is a 1.5 male predominance, prevalence is around 15% in children aged 1–15 years with non-febrile seizures and incidence is 10–20/100,000 of children aged 0–15 years [5][6][7][8][9]

Clinical manifestationsEdit

The cardinal features of rolandic epilesy are infrequent, often single, focal seizures consisting of:

  • a. unilateral facial sensorimotor symptoms (30% of patients),
  • b. oropharyngolaryngeal manifestations (53% of patients),
  • c. speech arrest (40% of patients) and
  • d. hypersalivation (30% of patients) [10][11][12][13][14][15]
  • Hemifacial sensorimotor seizures are often entirely localised in the lower lip or spread to the ipsilateral hand. Motor manifestations are sudden, continuous or bursts of clonic contractions, usually lasting from a few seconds to a minute. Ipsilateral tonic deviation of the mouth is also common. Hemifacial sensory symptoms consist of unilateral numbness mainly in the corner of the mouth.

Hemifacial seizures are often associated with an inability to speak and hypersalivation:

  • The left side of my mouth felt numb and started jerking and pulling to the left, and I could not speak to say what was happening to me.

Negative myoclonus can be observed in some cases, as an interruption of tonic muscular activity

  • Oropharyngolaryngeal ictal manifestations are unilateral sensorimotor symptoms inside the mouth. Numbness, and more commonly paraesthesias (tingling, prickling, freezing), are usually diffuse on one side or, exceptionally, may be highly localised even to one tooth. Motor oropharyngolaryngeal symptoms produce strange sounds, such as death rattle, gargling, grunting and guttural sounds, and combinations:

In his sleep, he was making guttural noises, with his mouth pulled to the right, ‘as if he was chewing his tongue’. We heard her making strange noises ‘like roaring’ and found her unresponsive, head raised from the pillow, eyes wide open, rivers of saliva coming out of her mouth, rigid.

  • Arrest of speech is a form of anarthria. The child is unable to utter a single intelligible word and attempts to communicate with gestures.

My mouth opened and I could not speak. I wanted to say I cannot speak. At the same time, it was as if somebody was strangling me.

  • Hypersalivation, a prominent autonomic manifestation, is often associated with hemifacial seizures, oro-pharyngo-laryngeal symptoms and speech arrest. Hypersalivation is not just frothing:

Suddenly my mouth is full of saliva, it runs out like a river and I cannot speak.

She lies there, unconscious with no movements, no convulsions, like a wax work, no life.

  • Consciousness and recollection are fully retained in more than half (58%) of rolandic seizures.

I felt that air was forced into my mouth, I could not speak and I could not close my mouth. I could understand well everything said to me. Other times I feel that there is food in my mouth and there is also a lot of salivation. I cannot speak. In the remainder (42%), consciousness becomes impaired during the ictal progress and in one third there is no recollection of ictal events.

  • Progression to hemiconvulsions or generalised tonic–clonic seizures occurs in around half of children and hemiconvulsions may be followed by postictal Todd’s hemiparesis .
  • Duration and circadian distribution: Rolandic seizures are usually brief, lasting for 1–3 min. Three ­quarters of seizures occur during non­rapid eye movement sleep, mainly at sleep onset or just before awakening.
  • Status epilepticus: Although rare, focal motor status or hemiconvulsive status epilepticus is more likely to occur than secondarily generalised convulsive status epilepticus, which is exceptional.[16][17] Opercular status epilepticus usually occurs in children with atypical evolution or may be induced by carbamazepine or lamotrigine. This state lasts for hours to months and consists of ongoing unilateral or bilateral contractions of the mouth, tongue or eyelids, positive or negative subtle perioral or other myoclonus, dysarthria, speech arrest, difficulties in swallowing, buccofacial apraxia and hypersalivation. These are often associated with continuous spikes and waves on an EEG during NREM sleep.
  • Other seizure types: Despite prominent hypersalivation, focal seizures with primarily autonomic manifestations autonomic seizures are not considered part of the core clinical syndrome of rolandic epilepsy. However, some children may present with independent autonomic seizures or seizures with mixed rolandic- autonomic manifestations including emesis as in Panayiotopoulos syndrome [18][19][20]
  • Atypical forms: Rolandic epilepsy may present with atypical manifestations such early age at onset, developmental delay or learning difficulties at inclusion, other seizure types, atypical EEG abnormalities.[21][22][23][24]

These children usually have normal intelligence and development.[2]

DiagnosisEdit

The diagnosis can be confirmed when the characteristic centrotemporal spikes are seen on electroencephalography (EEG).[25] Typically, high-voltage spikes followed by slow waves are seen.[26] 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. 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.[citation needed]

Evolution and prognosis Edit

The prognosis for rolandic seizures is invariably excellent, with probably less than 2% risk of developing absence seizures and less often GTCS in adult life [10][11][12][13][14][15] Remission usually occurs within 2–4 years from onset and before the age of 16 years. The total number of seizures is low, the majority of patients having fewer than 10 seizures; 10–20% have just a single seizure. About 10–20% may have frequent seizures, but these also remit with age. Children with rolandic seizures may develop usually mild and reversible linguistic, cognitive and behavioural abnormalities during the active phase of the disease.[27][28][29][30] These may be worse in children with onset of seizures before 8 years of age, high rate of occurrence and multifocal EEG spikes [31][32] The development, social adaptation and occupations of adults with a previous history of rolandic seizures were found normal.[33][34]

TreatmentEdit

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.[35] 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] Parental education about rolandic epilepsy is the cornerstone of correct management. The traumatizing, sometimes long-lasting effect on parents is significant.[36]

Genetics and disease mechanismEdit

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][37][38] Linkage studies have pointed to a possible susceptibility region on chromosome 15q14, in the vicinity of the alpha-7 subunit of the acetylcholine receptor.[39] 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.[40]


ReferencesEdit

  1. Kramer U (July 2008). Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review. J. Child Neurol. 23 (7): 785–90.
  2. 2.0 2.1 2.2 Wirrell EC (1998). Benign epilepsy of childhood with centrotemporal spikes. Epilepsia 39 Suppl 4: S32–41.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Chahine LM, Mikati MA (December 2006). Benign pediatric localization-related epilepsies. Epileptic Disord 8 (4): 243–58.
  4. Benign rolandic epilepsy. Retrieved August 8, 2008.
  5. Sidenvall R, Forsgren L, Heijbel J. Prevalence and characteristics of epilepsy in children in northern Sweden. Seizure 1996;5(2):139-46.
  6. Astradsson A, Olafsson E, Ludvigsson P, Bjorgvinsson H, Hauser WA. Rolandic epilepsy: an incidence study in Iceland. Epilepsia 1998 August;39(8):884-6.
  7. Bouma PA, Bovenkerk AC, Westendorp RG, Brouwer OF. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997;48(2):430-7.
  8. Larsson K, Eeg-Olofsson O. A population based study of epilepsy in children from a Swedish county. Eur J Paediatr Neurol 2006 May;10(3):107-13.
  9. Berg AT, Shinnar S, Levy SR, Testa FM. Newly diagnosed epilepsy in children: presentation at diagnosis. Epilepsia 1999 April;40(4):445-52.
  10. 10.0 10.1 Beaussart M. Benign epilepsy of children with Rolandic (centro-temporal) paroxysmal foci. A clinical entity. Study of 221 cases. Epilepsia 1972;13(6):795-911.
  11. 11.0 11.1 Loiseau P, Beaussart M. The seizures of benign childhood epilepsy with Rolandic paroxysmal discharges. Epilepsia 1973;14(4):381-9.
  12. 12.0 12.1 Lerman P, Kivity S. Benign focal epilepsy of childhood. A follow-up study of 100 recovered patients. Archives of Neurology 1975;32(4):261-4.
  13. 13.0 13.1 Panayiotopoulos CP. Benign childhood epilepsy with centrotemporal spikes or Rolandic seizures. In: Panayiotopoulos CP, editor. Benign childhood partial seizures and related epileptic syndromes.London: John Libbey & Company Ltd; 1999. p. 33-100.
  14. 14.0 14.1 Dalla Bernardina B, Sgro M, Fejerman N. Epilepsy with centro-temporal spikes and related syndromes. In: Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence (4th edition). 4th ed. Montrouge, France: John Libbey Eurotext; 2005. p. 203-25.
  15. 15.0 15.1 Panayiotopoulos CP, Michael M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131(Pt 9):2264-2286.
  16. Deonna T, Ziegler AL, Despland PA. Combined myoclonic-astatic and "benign" focal epilepsy of childhood ("atypical benign partial epilepsy of childhood"). A separate syndrome? Neuropediatrics 1986;17(3):144-51.
  17. Fejerman N, Caraballo R, Tenembaum SN. Atypical evolutions of benign localization-related epilepsies in children: are they predictable? Epilepsia 2000 April;41(4):380-90.
  18. Covanis A, Lada C, Skiadas K. Children with Rolandic spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome? Epileptic Disord 2003 September;5(3):139-43
  19. Caraballo R, Cersosimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007 June;48(6):1054-61.
  20. Specchio N, Trivisano M, Di C, V, Cappelletti S, Masciarelli G, Volkov J et al. Panayiotopoulos syndrome: A clinical, EEG, and neuropsychological study of 93 consecutive patients. Epilepsia 2010 October;51(10):2098-107.
  21. Datta A, Sinclair DB. Benign epilepsy of childhood with rolandic spikes: typical and atypical variants. Pediatr Neurol 2007 March;36(3):141-5
  22. Kramer U. Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review. J Child Neurol 2008 July;23(7):785-90.
  23. Wirrell EC, Camfield PR, Gordon KE, Dooley JM, Camfield CS. Benign rolandic epilepsy: atypical features are very common. Journal of Child Neurology 1995;10(6):455-8.
  24. Fejerman N, Caraballo R, Tenembaum SN. Atypical evolutions of benign localization-related epilepsies in children: are they predictable? Epilepsia 2000 April;41(4):380-90.
  25. Blueprints Neurology, 2nd ed.
  26. Stephani U (2000). Typical semiology of benign childhood epilepsy with centrotemporal spikes (BCECTS). Epileptic Disord 2 Suppl 1: S3–4.
  27. Neri ML, Guimaraes CA, Oliveira EP, Duran MH, Medeiros LL, Montenegro MA et al. Neuropsychological assessment of children with rolandic epilepsy: Executive functions. Epilepsy Behav 2012 August;24(4):403-7.
  28. Callenbach PM, Bouma PA, Geerts AT, Arts WF, Stroink H, Peeters EA et al. Long term outcome of benign childhood epilepsy with centrotemporal spikes: Dutch Study of Epilepsy in Childhood. Seizure 2010 October;19(8):501-6.
  29. Goldberg-Stern H, Gonen OM, Sadeh M, Kivity S, Shuper A, Inbar D. Neuropsychological aspects of benign childhood epilepsy with centrotemporal spikes. Seizure 2010 January;19(1):12-6.
  30. Danielsson J, Petermann F. Cognitive deficits in children with benign rolandic epilepsy of childhood or rolandic discharges: a study of children between 4 and 7 years of age with and without seizures compared with healthy controls. Epilepsy Behav 2009 December;16(4):646-51.
  31. Piccinelli P, Borgatti R, Aldini A, Bindelli D, Ferri M, Perna S et al. Academic performance in children with rolandic epilepsy. Dev Med Child Neurol 2008 May;50(5):353-6.
  32. Bulgheroni S, Franceschetti S, Vago C, Usilla A, Pantaleoni C, D'Arrigo S et al. Verbal dichotic listening performance and its relationship with EEG features in benign childhood epilepsy with centrotemporal spikes. Epilepsy Res 2008 March;79(1):31-8.
  33. Blom S, Heijbel J. Benign epilepsy of children with centrotemporal EEG foci: a follow-up study in adulthood of patients initially studied as children. Epilepsia 1982;23(6):629-32.
  34. Loiseau P, Pestre M, Dartigues JF, Commenges D, Barberger-Gateau C, Cohadon S. Long-term prognosis in two forms of childhood epilepsy: typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci. Annals of Neurology 1983;13(6):642-8.
  35. McAbee GN, Wark JE (September 2000). A practical approach to uncomplicated seizures in children. Am Fam Physician 62 (5): 1109–16.
  36. Valeta T. Parental attitude, reaction and education in benign childhood focal seizures. In: Panayiotopoulos CP, editor. The Epilepsies: Seizures, Syndromes and Management.Oxford: Bladon Medical Publishing; 2005. p. 258-61.
  37. Neubauer BA (2000). The genetics of rolandic epilepsy. Epileptic Disord 2 Suppl 1: S67–8.
  38. 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.
  39. 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.
  40. 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. 17 (9): 1171–1181.

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