Epilepsy with seizures precipitated by light stimulation Visual sensitive (or photosensitiv e) epilepsies are characterized by seizures precipitated by environmental photic stimuli ( 79 ). The age at onset peaks at 11 years. Sensitivity to visual stimuli is associated with the inability of the visual cortex to process afferent inputs of high luminance and contras t through the normal mechanisms of cortical gain control ( 80 ). Photic-induced absences, myoclonic seizures, and GTCs are typ- ically observed in the idiop athic generalized epilepsies and in Dravet syndrome ( 81 ) but can rarely be observed in pa tients with other types of epilepsy. Single or repeated seizures while playing video games or in front of the television (especially with a 50 Hz screen) can be observed in children without a history of spontane- ous seizures. They manifest as either GTCSs or prolonged att acks with visual symptoms and vomiting ( 82 ). An outbreak was reported in Japan, where sev eral hundred children and adolescents experi- enced a seizure while watching television bro adcasting a popular cartoon ( 83 ). Self-induction is possible, especially in children with absences or myoclonic jerks who indulge in compulsively staring or blinking in front of li ght sources or contrasted patterns. Juvenile absence epilepsy starts around age 10 \200\22312. Its clinical manifestations and those of juvenile myoclonic epilepsy are par- tially overlapping. Absence seizures cluster upon awakening ( 63 ). GTCSs, often precipitated by sleep deprivation, occur in up to 80 % of patients and photosensitivity in 20 % ( 64 ). Absences tend to remit within a few years but GTCSs may recur, especially following sleep deprivation. The long-term prognosis is unc lear. In two early comparative trials, valproate and ethosuximide showed similar ef \201 cacy co ntrolling absence seizures ( 65 , 66 ). Lamotrigine was effective as monotherapy in a doubl e blind trial of new-onset absence seizures ( 67 ) and was also effective as an add-on trea tment of resistant absence seizures ( 68 ). However, such trials were not considered to pro vide suf \201 cient evidence to inform clini- cal practice ( 69 ). A more recent trial con \201 rmed previous results, showing that valproate and ethosuximide are more effective th an lamotrigine but also suggested that ethosuximide has fewer adverse attentional effects t han valproate ( 70 ). Some children may require combinations of drugs. If attacks are infrequent, preventive measures can be suf \201 cient. Video games should b e avoided. The triggering power of 50 Hz tel- evision screens is lowered by increasing the ambient light and by watching at a distance greater than 2.5 meters; 100 Hz television scr eens are much less provocative ( 84 ). If treatment is necessary, sodium valproate is the d rug of choice ( 85 ). Preliminary results have also been obtained with levetiracetam ( 86 ) . Polarized glasses or opti- cal \201 lters for screens have proven helpful in severe cas es ( 87 , 88 ). Epilepsy with myoclonic absences is a rare disorder (around 0.5 \200\2231 % of epilepsy) wi th onset between age 1 \200\22312 years ( 71 ). About 50 % of children have pre-existing co gnitive impairment. Myoclonic absences appear several times per day, as rhythmic jerking of shoul- ders, head, and arms. Impairment of consciousness is variable. The EEG \201 nding s are identical to those in childhood absence epilepsy. Evolution to different seizure type s with cognitive impairment occurs in about 60 % of children. A combination of valproate an d ethosuximide seems to be the most effective treatment ( 72 ). Myoclonic absences are also observed in symptomatic forms of epilepsies with variable aetiologies, where they represen t just one seizure type, often amongst others. Ketogenic diet The ketogenic diet is widely used to treat children with severe drug- resist ant epilepsies, especially the epileptic encephalopathies ( 89 ) but no comparative trials are available. The diet has shown some ef \201 cacy in a randomized clinical trial and is worth trying in drug- resistant cases ( 90 ), although no speci \201 c syndrome has shown to respond better than others and assessment of risk and bene \201 ts requires further stu dy. The mechanisms of action are not known but the predominantly fat nutrition maintains ke tosis in the long term and high concentrations of ketone bodies have been corre- lated with better seizure control. The diet may result too restrictive, and cause diarrhea, vitamin d e \201 ciency, renal stones, and cardiac complications ( 91 ). Juvenile myoclonic epilepsy (JME) represents about 2 % of epilep- sies ( 57 ) and is a gene tically determined syndrome (see Chapter 2). Peak incidence is between 12 \200\22318 years. The clinical presentation is often with myoclonic jerks which involve the proximal limbs a nd cause dropping of objects on awakening. GTCSs occur in 90 % of patients, with an average frequency of two per year ( 73 ), often fol- lowing sleep deprivation. It is not unusual t hat the myoclonic jerks are recognized for what they are only after a GTCS has occurred. Ab out 35 % of patients have absences and 5 % photic-induced sei- zures. Interictal EEG abnorm alities are characteristically activated upon awakening ( 74 ). AEDs control seizures in 85 % of patients ( 73 ). Surgical treatment Some of children who are refractory to AEDs may bene \201 t from surgic al treatment and the bene \201 ts of early intervention have been CHAPTER 16 principles of treatment of epilepsy in children and adolescents 191 progressive disorder, and in 31 % of those with malformations ( 97 ). These results are obt ained at the cost of worsening any pre-existing hemiplegia and visual \201 eld defect. He mispherotomy (hemispheric disconnection) is now preferred to hemispherectomy in an increas- ing number of centers. emphasized. However, the average delay from seizure onset to sur- gery is too long, ranging from 12 \200\22315 years ( 92 ). The aim of surgery is to reach complete control or to signi \201 - cantly reduce seizure f requency, while minimizing the harmful effects of epileptic activity and of AEDs on cogniti ve functions. Resective surgery, implies removal of the neuronal aggregate that is responsi ble for seizure generation; palliative or functional surgery aims at preventing or limiting propagation of seizure activity, with- out targeting seizure control. Palliative surgery Corpus callosotomy is a midline disconnection procedure, aimed at inhibi ting interhemispheric seizure spread in patients with disa- bling seizures with falls due t o bilateral synchronization. In order to limit disconnection symptoms, the anterior two-thi rds of the corpus are sectioned. Complete section can be carried out at a second operation if anterior resection is ineffective. Focal seizures may persist after the procedure but im pairment related to falling to the ground can be reduced ( 98 ). Identifying a child who can bene \201 t from surgery involves several steps. Medical intra ctability should be ascertained, possibly limited to the more appropriate drugs. Idiopathic and non-lesional genetic epilepsies should be ruled out as surgery is contraindicated in t hese cases. The level of seizure-related disability should be established on the basis of e ach patient \200\231s clinical presentation. Vagus nerve stimulation is used as an adjunct to treat drug-resist- ant epilepsy . A stimul ator is placed under the skin and emanates adjustable pulsed stimuli to an electrode that i s wrapped around the left vagus nerve ( 99 ). Interesting results have been obtained in chi ldren with severe epilepsies that are not treatable by surgery but epilepsy syndromes that are likely to bene \201 t electively are still undetermined. Adverse effects of hoarseness , cough, and pain are usually reasonably well tolerated. Resective surgery An intervention is only possible if no independent epileptogenicity is de tected outside the planned resection area and if any resulting neurological de \201 cit is no more invalidating than epilepsy itself. Clinical, video-EEG telemetry, neuropsychologic al assessment, and high-resolution MRI should provide a consistent localization of the epil eptogenic zone, i.e. the network of abnormally behaving neurons, usually distributed within a relatively large brain volume ( 93 ). The epileptogenic zone includes the ictal onset zo ne, which is the neurophysiologically de \201 ned cortical area in which seizures are init iated, but does not necessarily correspond to the epileptogenic lesion , which is anatomica lly defined. Defining the symptom- producing zone , i.e. the area of the brain that produce s initial clinical manifestations, the zone of functional alteration, which is outlined by functional techniques (e.g. by EEG, positron emission tomogra- phy (PET), or single-positro n emission computed tomography (SPECT)), and the irritative zone , i.e. the area over which paroxys- mal EEG activity is recorded, may help better delineate the epilep- togenic zone. Removal of an epileptogenic lesion, without de \201 ning and removing the epileptogenic z one ( \200\230lesionectomy \200\231), produces seizure control in a signi \201 cant propor tion of children ( 94 ) but delineation and removal of the entire epileptogenic zone, or at least the ictal onset zone, improves the outcome of lesionectomy. Surgery should be consid ered only very cautiously in the face of a normal high-resolution MRI scan ( 25 ). References 1 . Guerrini R , Belmonte A , Genton P . Antiepileptic drug-induced worsening of seizures i n children . Epilepsia 1998 ; 39 (suppl 3 ): 2 \200\223 10 . 2 . Chiron C , Dumas C , Jambaque I , Mumford J, Dulac O . Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis . Epilepsy Res 1997 ; 26 : 389 \200\223 95 . 3 . Picard F , Bertrand S , Steinlein OK , Bertrand D . Mutated nicotinic receptors responsible for autosomal dominant nocturnal frontal lobe epilepsy are more sensi tive to carbamazepine . Epilepsia 1999 ; 40 : 1198 \200\223 209 . 4 . Ambrosetto G , Tassinari CA . Antiepileptic drug treatment of benign childhood epilepsy with rolandic spikes: Is it necessary ? Epilepsia 1990 ; 31 : 802 \200 \223 5 . 5 . Cam \201 eld PR , Cam \201 eld CS . Treatment of children with \200\230ordinary \200 \231 epilepsy . Epileptic Disord 2000 ; 2 : 45 \200\223 51 . 6 . Deonna T , Roulet-Perez E . Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnorm alities: the continuing debate . Brain Dev 2010 ; 32 ( 9 ): 746 \200\223 52 . In cooperative children, functional MRI is used to map the motor and sensory cortex and is now preferred to the Wada int- racarotid amytal test for evaluating hemispheric language do mi- nance ( 95 ). In selected cases, MRI spectroscopy, EEG triggered functional MRI, ictal and interictal SPECT, and PET can help improve the surgical planning. Intracranial EEG reco rdings are reserved to cases in which doubts persist on the localization and extension of t he resection. Use of stereotactically implanted depth electrodes or subdural grids, depends on the preoperative hypoth- esis about seizure origin and propagation. 7 . Cam \201 eld CS , Cam \201 eld PR , Veugelers PJ . Death in children with epilepsy: a population-based study . Lancet 2002 ; 359 : 1891 \200\223 5 . 8 . Loring DW , Meador KJ . Cognitive side effects of antiepileptic drugs in children . Neurology 2004 ; 62 : 872 \200\223 7 . 9. Farwell JR , Lee YJ , Hirtz DG , Sulzbacher SI , Ellenberg JH , Nelson KB . Phenobarbita l for febrile seizures \200\224 effects on intelligence and on seizure recurrence . N Engl J Med 1990 ; 322 : 364 \200\223 9 . 10. Sulzbacher S , Farwell JR , Temkin N , Lu AS , Hirtz DG . Late cognitive effects of ear ly treatment with phenobarbital . Clin Pediatr 1999 ; 38 : 387 \200\223 94 . 11 . Riva D , Devoti M . Carbamazepine withdrawal in children with Anterior temporal lobectomy is the most common operation and provides the best results ( 29 ). Extratemporal cortical resections yield excellent results in well-selected lesional cas es. Seizure free- dom is higher after temporal resection (78 % ) than after extratem- poral or multilobar resections (54 % ) and in children with tumors (82 % ) than in those with co rtical malformations (52 % ) ( 96 ). Hemispherectomy (or hemispherotomy) controls seizures in about 82 % of children with acquired lesions, in 50 % of those with a previous symptomatic partial epilepsy: effects on neuropsychologic function . J Child Neuro l 1999 ; 14 : 357 \200\223 62 . 12 . Seidel WT , Mitchell WG . Cognitive and behavioral effects of carbamazepine in children: data from benign rolandic epilepsy . J Child Neurol 1999 ; 14 : 716 \200\223 23 . 13 . Bailet LL , Turk WR . The impact of childhood epilepsy on neurocognitive and behavioral performance: a prospective longitudinal study . Epilepsia 200 0 ; 41 : 426 \200\223 31 . oxford textbook of epilepsy and epileptic seizures 192 34 . Biraben A , Chauvel P . Falls in epileptic seizures with partial onset . In: Beaumanoi r A , Andermann F , Avanzini G , Mira L (eds) Falls in epileptic and non-epileptic seizures during childhood , pp. 125 \200\223 35 . London : John Libbey & Company Ltd , 1997 . 14 . Forsythe I , Butler R , Berg I , McGuire R . Cognitive impairment in new cases of epil epsy randomly assigned to carbamazepine, phenytoin and sodium valproate . Dev Med Child Neu rol 1991 ; 33 : 524 \200\223 34 . 15 . Lerman P . Benign partial epilepsy with centro-temporal spikes . In: 35 . Guerrini R , Parmeggiani L , Berta E , Munari C . Occipital lobe seizures . In: Oxbury JM , Polkey CE , Duchowny MS (eds) Intractable focal epilepsy: medical and surgical treatm ent , pp. 77 \200\223 88 . London : Saunders & Co , 2000 . Roger J , Bureau M , Dravet C , Dreifuss FE , Perret A , Wolf P (eds) Epileptic Syndromes i n Infancy, Childhood and Adolescence ( 2nd edn ), pp. 189 \200\223 200 . London : John Lib bey , 1992 . 16 . Dalla Bernardina B , Sgro V , Fejerman N . Epilepsy with central 36 . Williamson PD , Thadani VM , Darcey TM , Spencer DD , Spencer SS , Mattson RH. Occipit al lobe epilepsy: Clinical characteristics, seizure spread patterns, and results of surgery . Ann Neurol 1992 ; 31 : 3 \200\22313 . 37 . Verity CM , Hosking G , Easter DJ . A multice ntre comparative trial of sodium valproate and carbamazepine in pediatric epilepsy. The pae ditric EPITEG collaborative Group . Dev Med Child Neurol 1995 ; 37 : 97 \200\223 108 . temporal spikes and related syndromes . In: Roger J , Bureau M , Dravet C , Genton P , Tass inari CA , Wolf P (eds) Epileptic Syndromes in Infancy, Childhood and Adolescence ( 3rd edn ), pp. 181 \200\223 202 . London : John Libbey , 2002 . 17 . Bouma PA , Bovenkerk AC , Westendorp RG , Brouwer OF . The course of benign partial ep ilepsy of childhood with centrotemporal spikes: a meta-analysis . Neurology 1997 ; 48 : 430 \200\223 7 . 38 . de Silva M , MacArdle B , McGowan M , Hughes E , Stewart J , Neville 18 . Massa R , de Saint-Martin A , Carcangiu R , Rudolf G , Seegmuller C , Kleitz C , et al . EEG criteria predictive of cognitive complications in idiopathic focal epilepsy with rol andic spikes . Neurology 2001 ; 57 : 1071 \200\223 9 . BG , et al . Randomized comparative monotherapy trial of phenobarbitone, phenytoin, carbama zepine, or sodium valproate for newly diagnosed childhood epilepsy . Lancet 1996 ; 347 : 70 9 \200\223 13 . 39 . Gobbi G , Pini A , Bertani G , Menegati E , Tiberti A , Valseriati D , et al . Prospective study of \201 rst-line vigabatrin monotherapy in childhood partial epilepsies . Epilepsy Res 1999 ; 35 : 29 \200\223 37 . 19 . Parmeggiani L , Seri S , Bonanni P , Guerrini R . Electrophysiological characterization of spontaneous and carbamazepine-induced epileptic negative myoclonus in b enign childhood epilepsy with centro- temporal spikes . Clin Neurophysiol 2004 ; 115 : 50 \200\223 8 . 40 . Zamponi N , Cardinali C . Open comparative long-term study of vigabatrin vs carbamazepine in newly diagnosed partial seizures in children . Arch Neurol 1 999 ; 56 : 605 \200\223 7 . 20 . Gastaut H . Benign epilepsy of childhood with occipital paroxysms . In: Roger J , Drav et , C , Bureau M , Dreifuss FE , Perret A , Wolf P (eds) Epileptic Syndromes in Infa ncy , Childhood and Adolescence ( 2nd edn), pp. 201 \200\223 17 . London : John Libbey , 1992 . 41 . K lvi inen R, Nousiainen I , Mantyjarvi M , Nikoskelainen E , Partanen J , Partanen K , et al . Vigabatrin, a GABAergic antiepileptic drug, causes concentric visual \201 eld defects . Neurology 1999 ; 53 : 922 \200\223 6 . 21 . Ferrie CD , Beaumanoir A , Guerrini R , Kivity S , Vigevano F , Takaishi Y. Early-onse t benign occipital seizure susceptibility syndrome . Epilepsia 1997 ; 38 : 285 \200\223 93 . 42 . French JA , Kanner AM , Bautista J , Abou-Khalil B , Browne T , Harden CL , et al . Ef \201 cacy and tolerability of the new antiepileptic drugs I: treatment of new onset epile psy: report of the Therapeutics and Technology Assessment Subcommittee and Quality Standard s Subcommittee of the American Academy of Neurology and the American Epilepsy Society . Neu rology 2004 ; 62 : 1252 \200\223 60 . 22 . Panayiotopoulos CP , Michael M , Sanders S , Valeta T , Koutroumanidis M . Benign chil dhood focal epilepsies: assessment of established and newly recognized syndromes . Brain 20 08 ; 131 : 2264 \200\223 86 . 23 . Arzimanoglou A , Guerrini R , Aicardi J. Epilepsy in Children ( 3rd edn). 43 . Gilliam FG , Veloso F , Bomhof MA , Gazda SK , Biton V , Ter Bruggen Philadelphia, PA : Lippincott-Williams & Wilkins , 2004 . JP , et al . A dose-comparison trial of topiramate as monotherapy in recently diagnosed par tial epilepsy . Neurology 2003 ; 60 : 196 \200\223 202 . 44 . Privitera MD , Brodie MJ , M attson RH , Chadwick DW , Neto W , Wang S ; EPMN 105 Study Group . Topiramate, carbamazepin e and valproate monotherapy: double-blind comparison in newly diagnosed epilepsy . Acta Neu rol Scand 2003 ; 107 : 165 \200\223 75 . 24 . Guerrini R , Belmonte A , Veggiotti P , Mattia D , Bonanni P . Delayed appearance of i nterictal EEG abnormalities in early onset childhood epilepsy with occipital paroxysms . Br ain Dev 1997 ; 19 : 343 \200\223 6 . 25 . Guerrini R . Epilepsy in children . Lancet 2006 11 ; 367 : 499 \200\223 524 . 26 . Ha rvey AS , Berkovic SF , Wrennall JA , Hopkins IJ. Temporal lobe epilepsy in childhood: clinical, EEG, and neuroimaging \201 ndings and syndrome classi \201 cation in a cohort with new-onset seizures . Neurology 1997 ; 49 : 960 \200\223 8 . 45 . Guerreiro MM , Vigonius U , Pohlmann H , de Manreza ML , Fejerman N , Antoniuk SA , et al . A double-blind controlled clinical trial of oxcarbazepine versus phenytoin in childre n and adolescents with epilepsy . Epilepsy Res 1997 ; 27 : 205 \200\223 13 . 27 . Kuks JBM , Cook MD , Fish DR , Stevens JM , Shorvon SD. Hippocampal sclerosis in epile psy and childhood febrile seizures . Lancet 1993 ; 342 : 1391 \200\223 4 . 46 . Elterman RD , Glauser TA , Wyllie E , Reife R, Wu SC, Pledger G . A double-blind, randomized trial of topiramate as adjunctive therapy for partial-onset seizur es in children. Topiramate YP Study Group . Neurology 1999 ; 52 : 1338 \200\223 44 . 28 . Harvey AS , Grattan-Smith JD , Desmond PM , Chow CW, Berkovic SF . Febrile seizures an d hippocampal sclerosis: frequent and related \201 ndings in intractable temporal lobe ep ilepsy of childhood . Pediatr Neurol 1995 ; 12 : 201 \200\223 6 . 47 . Duchowny M , Pellock JM , Graf WD , Billard C , Gilman J , Casale E , et al . A placebo-controlled trial of lamotrigine add-on therapy for partial seizures in ch ildren. Lamictal Pediatric Partial Seizure Study Group . Neurology 1999 ; 53 : 1724 \200 \223 31 . 29 . Mohamed A , Wyllie E , Ruggieri P , Kotagal P , Babb T , Hilbig A , et al . Temporal l obe epilepsy due to hippocampal sclerosis in pediatric candidates for epilepsy surgery . Ne urology 2001 ; 56 : 1643 \200\223 9 . 48 . Glauser TA , Nigro M , Sachdeo R , Pasteris LA , Weinstein S , Abou- 30 . Hamer HM , Wyllie E , L ders HO , Kotagal P, Acharya J . Khalil B , et al . Adjunctive therapy with oxcarbazepine in children with partial seizures. The Oxcarbazepine Pediatric Study Group . Neurology 2000 ; 54 : 2237 \200\223 44 . Symptomatology of epileptic seizures in the \201 rst three years of life . Epilepsia 1999 ; 40 : 837 \200\223 44 . 31 . Vigevano F , Fusco L . Hypnic tonic postural seizures in healthy children provide evid ence for a partial epileptic syndrome of frontal origin . Epilepsia 1993 ; 34 : 110 \200 \22319 . 49 . Appleton R , Fichtner K , LaMoreaux L , Alexander J, Maton S, Murray G, Garofalo E . Gabapentin as add-on therapy in children with refractory partial seizures: a 24-week, multicentre, open-label study . Dev Med Child Neurol 2001 ; 43 : 269 \200\223 7 3 . 32 . Holthausen H , Hoppe M . Hypermotor seizures . In: L ders HO , Noachtar S (eds) Epile ptic Seizures: Pathophysiology and Clinical Semiology , pp. 439 \200\223 48 . Philadelphia , PA : Churchill Livingstone , 2000 . 50 . Allen JW , Oxley J , Robertson MM , Trimble MR, Richens A, Jawad SS . Clobazam adjunct ive treatment in refractory epilepsy . Brit Med J 1983 ; 286 : 1246 \200\2237 . 33 . Plazzi G , Tinuper P , Montagna P , Provini F, Lugaresi E . Epileptic nocturnal wandering . Sleep 1995 ; 18 : 749 \200\223 56 . CHAPTER 16 principles of treatment of epilepsy in children and adolescents 193 72 . Bureau M , Tassinari CA . Epilepsy with myoclonic absences . Brain Dev 51 . Keene DL , Whiting S , Humphreys P . Clobazam as an add-on drug in the treatment of re fractory epilepsy of childhood . Can J Neurol Sci 1990 ; 17 : 317 \200\2231 9 . 2005 ; 27 ( 3 ): 178 \200\223 84 . 73 . Thomas P , Genton P , Gelisse P , Wolf P . Juvenile myoclonic epilepsy . In: Roger J , Bureau M , Dravet C , Genton P , Tassinari CA , Wolf P (eds) Epileptic syndro mes in infancy childhood and adolescence ( 3rd edn), pp. 335 \200\223 56 . London :John Li bbey , 2002 . 52 . Berg AT , Lang \201 tt J , Shinnar S , Vickrey BG , Sperling MR , Walczak T , et al . How long does it take for partial epilepsy to become intractable? Neurology 2003 ; 60 : 18 6 \200\223 90 . 74 . Fittipaldi F , Curra A , Fusco L , Ruggieri S , Manfredi M. EEG discharges 53 . Engel J Jr . International League Against Epilepsy (ILAE) . A proposed on awakening: a marker of idiopathic generalized epilepsy . Neurology 2001 ; 56 : 123 \200 \223 6 . diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classi \201 cation and Terminology . Epilepsia 2001 ; 42 : 796 \200\223 803 . 75 . Nicolson A , Appleton RE , Chadwick DW , Smith DF . The relationship between treatment with valproate, lamotrigine, and topiramate and the prognosis of the idio pathic generalized epilepsies . J Neurol Neurosurg Psychiatry 2004 ; 75 ( 1 ): 75 \200\223 9 . 54 . Engel J Jr . Report of the ILAE classi \201 cation core group . Epilepsia 2006 ; 47 : 1558 \200\223 68 . 55 . Dravet C , Bureau M . Benign myoclonic epilepsy in infancy . Adv Neurol 76 . Noachtar S , Andermann E , Meyvisch P , Andermann F , Gough WB , 2005 ; 95 : 127 \200\223 37 . Schiemann-Delgado J . N166 Levetiracetam Study Group. Levetiracetam for the treatment of id iopathic generalized epilepsy with myoclonic seizures . Neurology 2008 ; 70 : 607 \200\223 16 . 56 . Guerrini R , Aicardi J . Epileptic encephalopathies with myoclonic seizures in infants and children (severe myoclonic epilepsy and myoclonic-astatic epilepsy) . J Clin Neurophysiol 2003 ; 20 : 449 \200\223 61 . 57 . Berg AT , Shinnar S , Levy SR , Testa FM . Newly diagnosed epilepsy in children: presentation at diagnosis . Epilepsia 1999 ; 40 : 445 \200\223 52 . 77 . Biraben A , Allain H , Scarabin JM , Sch ck S , Edan G . Exacerbation of juvenile myo clonic epilepsy with lamotrigine . Neurology 2000 ; 55 ( 11 ): 1758 . 58 . Guiwer J , Valenti MP , Bourazza A , Hirsch, E., Loiseau, P . Prognosis of 78 . Genton P , Gelisse P , Thomas P , Dravet C. Do carbamazepine and phenytoin aggravate j uvenile myoclonic epilepsy? Neurology 2000 ; 55 : 1106 \200\223 9 . idiopathic absence epilepsies . In: Jallon P , Berg A , Dulac O , Hauser A (eds) Prognosis of Epilepsies , pp. 249 \200\223 57 . Paris: John Libbey , 2003 . 79 . Kasteleijn-Nolst Trenite DG , Guerrini R , Binnie CD , Genton P. Visual sensitivity an d epilepsy: a proposed terminology and classi \201 cation for clinical and EEG phenomenolo gy . Epilepsia 2001 ; 42 : 692 \200\223 701 . 80 . Porciatti V , Bonanni P , Fiorentini A , Guerrini R . Lack of cortical 59 . Caraballo RH , Darra F , Fontana E , Garcia R , Monese E , Dalla Bernardina B . Absence seizures in the \201 rst 3 years of life: an electroclinical study of 46 cases . Epilepsia 2011 ; 52 : 393 \200\223 400 . 60 . Guerrini R , Sanchez-Carpintero R , Deonna T , Santucci M , Bhatia KP , contrast gain control in human photosensitive epilepsy . Nature Neuroscience 2000 ; 3 : 259 \200\223 63 . Moreno T , et al . Early-onset absence epilepsy and paroxysmal dyskinesia . Epilepsia 2002 ; 43 : 1224 \200\223 9 . 81 . Guerrini R , Genton P . Epileptic syndromes and visually induced 61 . Suls A , Mullen SA , Weber YG , Verhaert K , Ceulemans B , Guerrini R , et al . Early- onset absence epilepsy caused by mutations in the glucose transporter GLUT1 . Ann Neurol 20 09 ; 66 : 415 \200\2231 9 . seizures . Epilepsia 2004 ; 45 (Supp 1 ): 14 \200\2231 8 . 82 . Guerrini R , Dravet C , Genton P , Bureau M , Bonanni P , Ferrari AR , et al . Idiopathic photosensitive occipital lobe epilepsy . Epilepsia 1995 ; 36 : 883 \200 \223 91 . 62 . Wang D , Pascual JM , Yang H , Engelstad K , Jhung S , Sun RP , De Vivo DC . Glut-1 de \201 ciency syndrome: clinical, genetic, and therapeutic aspects . Ann Neurol 2005 ; 57 : 111 \200\2231 8 . 83 . Ishiguro Y , Takada H , Watanabe K , Okumura A , Aso K , Ishikawa T. A follow-up survey on seizures induced by animated cartoon TV program \200\230Pocket Monster \200\231 . Epilepsia 2004 ; 45 : 377 \200\223 83 . 63 . Wolf P , Inoue Y . Juvenile absence epilepsy . In: Roger J , Bureau M , Dravet C , Genton P , Tassinari CA , Wolf P (eds) Epileptic Syndromes in Infancy, Childhood and Adolescence ( 3rd edn), pp. 331 \200\223 4 . London : John Libbey , 2002 . 84 . Ricci S , Vigevano F , Manfredi M , Kasteleijn-Nolst Trenit DG. Epilepsy provoked by television and video games: Safety of 100 Hz screens . Neurology 1998 ; 50 : 790 \200\223 3 . 64 . Wolf P , Gooses R . Relation of photosensitivity to epileptic syndromes . 85 . Covanis A , Stodieck SR , Wilkins AJ . Treatment of photosensitivity . J Neurol Neurosurg Psychiatry 1986 ; 49 : 1386 \200\22391 . Epilepsia 2004 ; 45(Suppl 1) : 40 \200\223 5 . 65 . Sato W , White BG , Penry JK , Dreifuss FE, Sackellares JC, Kupferberg HJ . Valproic a cid versus ethosuximide in the treatment of absence seizures . Neurology 1982 ; 32 : 157 \200\22363 . 86 . Kasteleijn-Nolst Trenit DG , Marescaux C , Stodieck S , Edelbroek PM , Oosting J . P hotosensitive epilepsy: a model to study the effects of antiepileptic drugs. Evaluation of the piracetam analog, levetiracetam . Epilepsy Res 1996 ; 25 ( 3 ): 225 \200\223 30 . 66 . Callaghan N , O \200\231Hare J , O \200\231Driscoll D , O \200\231Neill B, Daly M . Co mparative study of ethosuximide and sodium valproate in the treatment of typical absence seizures (Pe tit Mal) . Dev Med Child Neurol 1982 ; 24 : 830 \200\2236 . 87 . Takahashi Y , Sato Y , Goto K , Fujino M , Fujiwara T , Yamaga M , Isono H , Kondo N . Optical \201 lters inhibiting television-induced photosensitive seizures . Neurology 2001 ; 57 : 1707 \200\223 73 . 88 . Kepecs MR , Boro A , Haut S , Kepecs G, Mosh SL . A novel 67 . Frank LM , Enlow T , Holmes GL , Manasco P , Concannon S , Chen C , et al . Lamictal ( lamotrigine) monotherapy for typical absence seizures in children . Epilepsia 1999 ; 40 : 9 73 \200\223 9 . nonpharmacologic treatment for photosensitive epilepsy: a report of three patients tested w ith blue cross-polarized glasses . Epilepsia 2004 ; 45 : 1158 \200\223 62 . 68 . Besag FM , Wallace SJ , Dulac O , Alving J, Spencer SC, Hosking G . Lamotrigine for the treatment of epilepsy in childhood . J Pediatr 1995 ; 127 : 991 \200 \223 7 . 89 . Cross JH , Neal EG . The ketogenic diet \200\224 update on recent clinical trials . 69 . Posner EB , Mohamed K , Marson AG . Ethosuximide, sodium valproate Epilepsia 2008 ; 49(Suppl 8) : 6 \200\223 10 . or lamotrigine for absence seizures in children and adolescents . Cochrane Database Syst Re v 2003 ; 4: CD003032 . 90 . Neal EG , Chaffe H , Schwartz RH , Lawson MS , Edwards N , Fitzsimmons G , et al . A randomized trial of classical and medium- chain triglyceride keto genic diets in the treatment of childhood epilepsy . Epilepsia 2009 ; 50 : 1109 \200\223 1 7 . 70 . Glauser TA , Cnaan A , Shinnar S , Hirtz DG , Dlugos D , Masur D , et al . Childhood A bsence Epilepsy Study Group. Ethosuximide, valproic acid, and lamotrigine in childhood abse nce epilepsy . N Engl J Med 2010 ; 362 : 790 \200\223 9 . 91 . Best TH , Franz DN , Gilbert DL , Nelson DP , Epstein MR . Cardiac complications in pediatric patients on the ketogenic diet . Neurology 2000 ; 54 : 2328 \200\223 30 . 71 . Bureau M , Tassinari CA . Epilepsy with myoclonic absences . In: Roger J , Bureau M , Dravet C , Genton P , Tassinari CA , Wolf P (eds) Epileptic Syndromes in Infancy, Childhood and Adolescence ( 3rd edn), pp. 305 \200\223 12 . London : John Libbey , 2002 . 92 . Duchowny M . Pediatric epilepsy surgery: the widening spectrum of surgical candidacy . Epileptic Disord 1999 ; 1 : 143 \200\223 51 . oxford textbook of epilepsy and epileptic seizures 194 97 . Devlin AM , Cross JH , Harkness W , Chong WK , Harding B , Vargha- 93 . L ders , H.O. , Engel , J. , Munari , C . General principles . In: Engel J (ed) Surgi cal Treatment of the Epilepsies ( 2nd edn ), pp. 137 \200\223 53 . New York : Raven Press , 1993. Khadem F , et al . Clinical outcomes of hemispherectomy for epilepsy in childhood and adole scence . Brain 2003 ; 126 : 556 \200\223 66 . 98 . Oguni H , Olivier H , Andermann F , Comair J. Anterior callosotomy in the treatment of medically intractable epilepsies: A study of 43 patients with a mean follow-up of 39 month s . Ann Neurol . 1991 ; 30 : 357 \200\22364 . 94 . Bourgeois M , Sainte-Rose C , Lellouch-Tubiana A , Malucci C , Brunelle F , Maixner W , et al . Surgery of epilepsy associated with focal lesions in childhood . J Neurosurg 1999 ; 90 : 833 \200\223 42 . 99. Crumrine PK . Vagal nerve stimulation in children . Semin Pediatr 95 . Barbier EL , Lamalle L , Decorps M . Methodology of brain perfusion Neurol 2000 ; 7 : 216 \200\223 23 . imaging . J Magn Res Imag 2001 ; 13 : 496 \200\223 520 . 96 . Wyllie E . Catastrophic epilepsy in infants and children: identi \201 cation of surgical candidates . Epileptic Disord 1999 ; 1 ( 4 ): 261 \200\223 4 . CHAPTER 17 Epilepsy in Learning Disability Tom Berney and Shoumitro Deb Introduction This chapter sets out what it is about people with learning disabil- ity (LD) and their services that affects their experience of epilepsy and its diagnosis and manageme nt. carer unusual importance in the clinical assessment; they may be the only people who unders tand what is going on. It also gives doc- tors an unusual responsibility in making decision s so that they need to ensure that, as far as possible, they have consulted all those conce rned. 1. What is it that distinguishes this group from the wider popula- tion and, in many countr ies, has led to the development of spe- cialist psychiatric and social services? Comorbidity The greater the degree of LD, the more likely there are to be other disabilitie s (including motor and sensory components) and other developmental disabilities, notably au tism and attention de \201 cit disorder. It means that a person with LD is likely to have a complex porridge of psychopathology that may extend to include any of the psychiatric dis orders found in the general population. Limited communication blocks descriptions of intern al, subjective experi- ences and perceptions making it dif \201 cult to diagnose disorders such as schizophrenia or obsessive \200\223compulsive disorder. There is a consequent risk of turning tentative inference and therapeutic trial into \201 rm diagnosis and unjusti \201 ed treatment. 2. How does this affect the diagnosis and management of epilepsy? The nature of learning disability The generic needs of people with LD and the services that developed in response have encouraged us to think of a discrete population. However, altho ugh cognitive ability (assessed by intelligence quo- tient (IQ)) has become the most widely used administrative charac- teristic, other qualities contribute to de \201 ning the grou p, particularly the person \200\231s ability to function and cope with everyday life (meas- ured by scales such as the Vineland Adaptive Behavior Scales) and this will vary with thei r circumstances, personality, and motivation. For example, someone with a mild degree of ge neralized LD may be unable to meet the academic demands of school but thereafter cope well enough with marriage and employment. Of those with LD, about 75 \200\22390 % will have it i n a mild degree (IQ = 55 \200\22370), about 1.1 \200\2231.6 % of the general population, th e exact \201 gure depending on the age and circumstances of the group ( 1 ). A much small er pro- portion, about 0.14 % of the population, will have a severe/profound LD (IQ <35). T heir etiology is known for 70 \200\22390 % of those with severe LD ( 2 ) but for only about 50 % of those with mild LD. Rather than a clear, de \201 ned psychiatric disorder, the result is often disturbed behav ior, ampli \201 ed by anxiety or discomfort, which can become suf \201 ciently intense, f requent, or severe as to threaten the physical safety of the individual or those around. At the least, the restrictive or aversive responses affect the quality of their life. All thi s is summed up in the term \200\230challenging behavior \200\231 ( 3 ). The prevalence of psychiatric disorder relates to LD and, indeed, is less frequent where epilepsy is present ( 4 \200\2236 ) although limited studies and variations in methodology make this hard to te ase out. For example, where there is both LD and epilepsy, the prevalence of a schizophreni form psychosis ranges from 24 % ( 7 ) to only 4.4 % ( 5 ) (as against 6 % in those with epi lepsy but of normal ability). The range may re \202 ect an additional factor however, the level of seizure control rather than simply the presence of epilepsy; psycho- sis being mor e frequent in those who have been seizure free for a period ( 8 ). There is also the sugges tion that, in these circumstances, psychosis might be associated with mild LD and depressio n with severe LD. The group is far from coherent, encompassing not only a wide range of age and ability but a lso a collection of developmental dis- abilities of varied form and intensity, giving great individual varia- tion. In common, though, are problems with communication and dependency as well as atypical emotional and behavioral responses. These may form a characteristic pat tern associated with the underlying cause of the LD as its behavioral phenotype. Communication can be very idiosyncratic with large discrepan- cies between verbal and non-v erbal as well as between receptive and expressive abilities. The patient is likely to have dif \201 culty in reporting any adverse effects from treatment as well as a limited capaci ty to understand its implications. This gives the parent or Prevalence In the community the prevalence of epilepsy in adults with LD is about 16 \200 \22326 % compared with 0.4 \200\2231 % in the general population oxford textbook of epilepsy and epileptic seizures 196 ( 9 \200\22311 ). The risk of epilepsy increases with the degree of LD ( 10 ) and the prese nce of other disorders but the effects of age and genetic syndrome are more variable ( 9 , 12 ). Adults with severe LD are 3 \200\2234 times as likely to have epilepsy as those with mild/moderate LD and, similarly, in childhood, 67 % of those with severe LD have epilepsy c ompared to 7 % of those with mild to moderate LD ( 13 ). The association means that any ove rall \201 gure will depend on the distribution of disability in the population surveyed, let alone those of age, etiology, and accompanying neurological disorders. For example, the presence of cerebral palsy in addition to LD, increases the prevalence 10-fold ( 14 ). \227\206 Prader Willi syndrome : the result of a failure to express (paternal) genes at 15 q11 \200\223q13. Seizures occur in up to a fourth of those with this syndrome and may be as sociated particularly with a deletion genotype (as against uniparental disomy) ( 30 ). \227\206 Angelman syndrome : seizures start in the \201 rst 3 years of life are varied, multiple, and dif \201 cult to control ( 31 , 32 ) \227\206 Autism : \226 Epilepsy can certainly make the symptoms of autism worse. The question became whethe r epilepsy might mimic autism, open- ing the way to therapeutic trials of antiepileptics fo r autistic symptomatology. These were unsuccessful and the present per- ception is that the two disorders may have common causation. Seizures begin before 5 years of age for 65 % of people with an LD and before 20 years for a further 30 % ( 15 ). Thereafter, the preva- lence of epilepsy remains relatively constant and, although increased mortality is related to seizure type and frequency, the cause of d eath seldom relates directly to the seizures ( 16 , 17 ). The epilepsies in LD tend to be r efractory, only a minority becoming seizure free. However, a retrospective study in a speci alist epilepsy center found signi \201 cant improvement over time with, as might be expect ed, the greatest shift in those who started with greatest seizure frequency. This found, in contrast with a Finnish study ( 18 ), 84 % remained seizure free, possibly because of a mo re cautious approach to wean- ing people off their medication (19). One recurrent lesson is that successful treatment, particularly in this population, depends on the clinicians \200 \231 degree of expertise in epilepsy ( 16 , 19 ). \226 A meta-analysis, although hampered by the variable method- ology and limited number of cases, con \201 rmed that the preva- lence of epilepsy in autism was associated with th e degree of LD and was probably more frequent in females ( 9 ). However, even after allowin g for LD, epilepsy is more frequent in autism compared to the general population. \226 Two categories of autism may be discerned; 80 \200\22390 % of people have primary/es sential autism where there is a greater herit- ability with a higher incidence in close rel atives, no signi \201 - cant dysmorphology, and a higher degree of ability. The other grou p, secondary/complex/syndromal autism, comprises 10 \200\22320 % and is associated with und erlying medical disorders, dysmorphology, and lower levels of ability; this group is more l ikely to have epilepsy and, then, a higher seizure frequency.