B) Focal motor seizures. I) With elementary clonic motor signs. II) With asymmetric tonic motor seizures (e.g. supplementary motor seizures). III) With typical (temporal lobe) automatisms (e.g. mesial tempo- ral lobe seizures). IV) With hyperkinetic automatisms. V) With focal negative myoclonus. VI) With inhibitory motor seizures. Symptomatic (or probably symptomatic) focal epilepsies A) Lobar epilepsies They represent t he majority of focal epilepsies and are de \201 ned according to seizure semiology pointin g to an anatomic location. Since epileptic discharges can spread, it is important to relay on the very \201 rst manifestations of a seizure to correctly localize its onset. However , de \201 ning the area of onset of focal seizures based on clin- ical and surface EEG \201 ndings alone may be very dif \201 cult when neuroimaging is normal. In addition, de \201 ning lobar origin may be a misleading process, as the epileptogenic area may not respe ct the anatomic limits of the cerebral lobes, seizure spread from clinically silent areas m ay be rapid, producing unresponsiveness before subjective symptoms are reported or memorize d. Epilepsies and epileptic syndromes (Table 7. 6 ) Idiopathic focal epilepsies and syndromes A) Benign infantile seizures (non-familial) Watanabe described infants with complex partial seizures or par- tial seizures with secondary generalization, with normal develop- ment be fore seizure onset and a benign outcome ( 9 , 10 ). Clinical and EEG features are similar t o those of benign familial infantile seizures and of sporadic benign infantile seizures ( 1 1 ). B) Benign epilepsy of childhood with centrotemporal spikes (BECTS) BECTS is the most freque nt epilepsy syndrome in childhood. Age at seizure onset ranges between 3 \200\22313 years. Prognosis is excellent with remission by adolescence ( 12 ). Seizures are typically hemifa- cial, involving mainly the lips, tongue, and pharyngeal-laryngeal muscles. Sensory symptom s can involve the same body parts as motor phenomena. Sometimes the homolateral upper limb is involved. Attacks are typically sleep-related, appearing within the \201 rst 30 minute s after falling asleep in most cases. They often determine Temporal lobe epilepsies are the most frequent type of focal epi- lepsies ( 17 ). Onset is most often during childhood or early adult- hood. Seizures are simple or complex partial an d are of relatively long duration (1 \200\2232 min). Their onset is characterized by viscer al symptoms (rising epigastric sensation, thoracic constriction, hot or cold sensation, olf actory hallucinations) or by psychic or affective symptoms (dreamy state, d j -vu, d j -vecu), usually accompanied or followed by vegetative manifestations (pallor, \202 ushing , tachy- cardia, poly- or bradypnea, piloerection, mydriasis, or sweating). oxford textbook of epilepsy and epileptic seizures 76 Disruption of consciousness or \200\230unresponsiveness \200\231 appears later and can \202 uctuate. Sometimes it is not present at all even during automatisms that consist of or o-alimentary behavior (lip-smacking, swallowing) or gestural automatisms ( 18 ). Late somat omotor manifestations such as dystonic posturing of the upper limb con- tralateral to the e pileptic discharge may re \202 ect the ictal spread to extratemporal cortical regions or a ctivation of subcortical efferent systems. The postictal phase is characterized by prolonge d confu- sion, occasionally accompanied by walking. Aphasia is observed when the dominant h emisphere is involved. regions. Complex and frantic motor behavior with screaming and facial expression suggesting terror or rage are typical of anterior gyrus cinguli involvement. Interictal EEG can be normal, but it is sometimes characterized by widespread abnormalities that enable neither lateralization nor localization. Central region epilepsies are emanating from the perirolandic cortex ( 20 ). Typical signs include clonic motor or elementary sensory phenomena involving the contralateral hemibody w ith or without march. Secondary generalization is rare. Some of the seizures originating fr om this region can be precipitated by sensory or proprioceptive stimulations. Lateral or neocortical temporal lobe epilepsy is characterized by auditory hallucinations o r pseudovertigo and by aphasic speech arrest. Seizures originating from the opercular-insular region are char- acterized by oro-alimentar y manifestations such as mastication, swallowing, and salivation. Gustative hallucinations or illusions can be associated. Interictal or ictal abnormalities can be very mild. Seizure s arising from the insula may also mimic temporomesial ( 21 ) and frontal nocturnal hypermo tor seizures ( 22 ). Mesial temporal lobe epilepsy is manifested by a highly character- istic ictal pattern, inc luding epigastic sensation, psycho-motor arrest and staring, associated with mastication, l ip smacking, and later gestural automatisms in the upper limb homolateral to the epileptic discharges. Hippocampal sclerosis or other mesial tempo- ral lesions are frequently found o n magnetic resonance imaging (MRI). Interictal EEGs can be normal or show unilateral or bil ateral temporal abnormalities. Parietal lobe epilepsies are rare ( 23 ). Fast spreading of epileptic discharges to contigu ous lobes (frontal, temporal or occipital) can mask the initial signs or symptoms. Alterati on of high cortical functions such as limb movement sensation, and autoscopia (seeing of on e \200\231s own body image) can be the manifestation of non- dominant hemisphere involvemen t. In some patients unilateral spatial agnosia can only be recognized because they tend to look preferentially to one side that is homolateral to the epileptic dis- charge. Visual il lusions (macropsia, micropsia, metamorphospia) are present when posterior parietal cortex o r the parietal-occipital junction is involved. Clear rotatory vertigo is associated to an e pileptic discharge in the inferior parietal region or the tempo- roparietal junction. Slow rotation of the body towards the same side of the epileptic discharge and dystonic posturin g of the con- tralateral upper limb, is usually due to involvement of inferior parietal cor tex. Interictal and ictal EEG abnormalities may be widespread involving central, parietal, temporal, and occipital regions. Frontal lobe epilepsies are also frequent ( 19 ). Seizures are usually brief (seconds to te ns of seconds) and sleep-related. They are often manifested in clusters and are highly ster eotyped in the same patient. Tonic or postural manifestations can cause disabling drop atta cks. Consciousness disruption is highly variable but post-ictal recovery is usually fast. S ome patients describe psychic symptoms such as forced thought. Secondary generalization is common. Subjective symptoms are usually ill de \201 ned but cephalic, thoracic or abdomina l sensations are reported by some patients. Posterior frontal lobe seizures \200\224 seizures originating from the sup- plementary mot or area are characterized by speech arrest or palila- lia (involuntary repetition of the sa me words or sentences, when the dominant hemisphere is involved), followed by abduction and elevation of the upper limb contralateral to epileptic discharge and version of head and e yes to same side (fencing posture). When the dorsolateral convexity is involved, a contrala teral tonic posturing is observed. Frequently the discharge becomes bilateral with second- ary generalization. The involvement of Brodmann \200\231s area 4 pro- duces contralateral f ocal clonic movements. Speech arrest without loss of consciousness is observed when epilept ic activity spreads to the foot of the third frontal gyrus. Occipital lobe epilepsies are uncommon and dif \201 cult to correctly identify since rapid spreading of the epileptic discharge to contigu- ous lobes may mask initial symptoms ( 16 ). Elementary visual hal- lucinations (colored blobs, \202 ashes of light) associated to peri-ictal peripheral visual \201 eld de \201 cit (hemianopia) are typical and testify t he involvement of the contralateral pericalcarin region. Ictal or postictal headache are of ten observed. Eye movements are frequent in the course of the seizure. They can be phasic ( oculo-clonic movement or \200\230epileptic nystagmus \200\231) or tonic (slow version of t he eyes contralateral to the epileptic discharge). Occipital lobe seizures can rapidly prop agate to the frontal region, causing the patient to fall, or to the mesial temporal lobe. I nterictal EEG abnormalities involve the posterior regions and are usually enhanced during e ye-closure. Intermediate frontal lobe seizures are associated with a very fast spread of the epileptic discharge to other frontal lobe areas. Bilateral tonic manifestations appear suddenly, and can cause drop attacks. Axial tonic seizures with \202 exion of head and trunk associated with facial grimacing are typical. The patient can continuously moan and present apnoea, d ue to tonic diaphragm contraction. Anterior frontal lobe seizures have a longer duration than other frontal seizures. Secondar y generalization is rare. An automatic motor activity appears at seizure onset and involves either the upper limbs ( \201 ngers snapping, arms windmilling), or lower limbs (cycling, rhythmic \202 exion \200\223extension) or girdles (rocking of pelvis, sexual automatisms ). Verbal stereotypies are sometimes observed. Involvement of speci \201 c frontal areas c an be suspected when clus- tering of characteristic symptoms occurs. Forced thought or iso- lated alteration of awareness (frontal pseudo-absences) point to the fronto-polar area. Ol factory hallucinations with intense fear, brady- cardia and micturition suggest the involve ment of fronto-orbital B) Migrating partial seizures of early infancy It has been proposed that occurrence of almo st continuous migrat- ing focal seizures, combined with multifocal ictal EEG discharges, an d progressive deterioration of acquired skills, with onset in the \201 rst 6 months of li fe, represent an epilepsy syndrome ( 24 ). Seizures are markedly drug resistant and outcome is severe. The etiology is so far unknown, but it is likely that multiple aetiologies can cause a similar picture. CHAPTER 7 classification, clinical symptoms, and syndromes 77 Idiopathic generalized epilepsies Idiopathic generalized epilepsies (IGEs) are frequent and usually, but not necessarily, have a favorable outcome. Common charac- teristics to all IG Es syndromes include generalized tonic \200\223clonic, absence, and myoclonic seizures. The ir onset is from infancy to adolescence. A family history of epilepsy is frequent. IGEs hav e complex inheritance and are likely to be caused by the combined interaction of two or mor e genes ( 25 ). Sporadic cases are common and affected families are usually small ( 26 ). I nterictal EEG shows generalized spike-and-wave discharges at a frequency higher than 3 Hz. Drug treatment, especially valproic acid, is effective in most patients. GTCSs are observed in 90 % of patients and are sometimes pre- ceded by a crescendo of bilat eral jerks. Patients are most often brought to medical attention after a GTCS, while myoclo nic sei- zures can go unnoticed for months or even years. Ten to 15 % of patients also exhi bit brief absence seizures. Visual sensitivity is present in 30 \200\22340 % of patients. S leep deprivation and alcohol intake are precipitating factors, especially for GTCSs. Interi ctal EEG shows generalized or asymmetric polyspike-and-wave discharges at 3 Hz or faster. M yoclonic jerks are time-locked to polyspike-and-wave complexes. A characteristic increase i n polyspike-and-wave dis- charges is observed upon awakening ( 31 ). JME is genetically det er- mined. Three to 9 % of patients with JME carry dominant mutations of the EFHC1 gene, wh ich causes reversal of EFHC1 -induced neu- ronal cell death and EFHC1 -dependent increase o f R-type Ca + cur- rent ( 32 ). One family with dominant JME harbored a mutation in the 1 subunit of the GABA A receptor gene ( GABRA1 ) ( 33 ). A) Benign myoclonic epilepsy in infants (BMEI) This is a rare syndrome characterized by bri ef generalized myo- clonic seizures with onset during the \201 rst or second year of life in otherwise normal children ( 27 ). Valproic acid is effective in con- trolling jerks. Th e appropriateness of the term \200\230benign \200\231 for this syndrome is questioned as a number of children exhibiting the clinical presentation of the BMEI will subsequently deve lop behav- ioural or cognitive problems and, sometimes, GTCSs in adoles- cence. Ictal EEG s hows generalized polyspike-and-wave discharges, which are time-locked with the myoclonic je rks. Interictal abnor- malities are rare. Response to treatment (valproic acid, primidone, benzodi- azepines) is usually good but JME is a pharmaco-dependent type of epilepsy. Withdrawal of AEDs is associated to relapse in 9 0 % of patients, even late in life ( 34 ). Carbamazepine use in patients with JME is someti mes associated to seizure-worsening, rarely escalat- ing to status epilepticus ( 35 ). E) Epilepsy with generalized tonic \200\223clonic seizures only GTC seizures take mostly or exclusively place within 2 hours from awakening or during evening time relaxation. Sleep d eprivation, high alcohol intake, and induced arousals from sleep are all trigger- ing facto rs. Interictal EEG can be normal or show generalized spike/polyspike-and-wave discharges th at are increased upon awakening ( 31 ). As JAE, JME, and GMA represent a syndrome spectrum partially overlapping in age of onset, seizure types, and response to medication, the term IGEs with variable phenotypes has been suggested as all inclusive ( 4 ). B) Childhood absence epilepsy (CAE) This is a frequent form of IGE with onset in otherwise healthy school-aged children. Clinical presentation is with frequent typical absence seizur es. Interictal EEG shows normal background activity and generalized 3 Hz, spike-and-wave di scharges, which translate into a clinical seizure when their duration outlasts 4 \200\2235 seconds. Evolution is usually though not uniformly benign. Treatment with AEDs (valproic acid, ethos uximide, or lamotrigine) usually controls absence seizures, which tend to disappear in adol escence. Phenobarbitone and carbamazepine are not effective and can even increase seizure f requency ( 28 ). GTCSs can appear from adoles- cence in some patients. Late onset (above ag e 8), initial drug resist- ance, and photosensitivity are more likely to be associated with a less benign prognosis (see \200\230C) Juvenile absence epilepsy (JAE) \200\231 section) . Absence seizures are also observed in other rare syn- dromes, which are not included in t he classi \201 cation such as eye-lid myoclonus with absences and perioral myoclonus with absences. F) Epilepsy with myoclonic-astatic seizures (MAE) This syndrome has its onset between 6 mon ths and 6 years of age with a prominence in boys ( 36 ). A genetic predisposition is presen t, whose nosological boundaries are not well de \201 ned. Overlapping of some of the typic al clinical manifestations with those of other severe childhood epilepsies (Lennox \200\223 Gastaut syndrome (LGS) and Dravet syndrome) is possible. Affected children exhibit atonic o r myoclonic-atonic seizures and absence seizures with a clonic or tonic component. GTCSs ar e also present in most patients. Tonic seizures can appear late in the course, particularly in drug-resistant children. Non-convulsive myoclonic status with prolonged unre- sponsiven ess can be observed in around 30 \200\22335 % of children. Interictal EEG, normal at onset, shows generalized spike/polyspike- and-wave discharges later during the course. Outcome is unpre- dictable. Remission within a few months or years with normal cognition is possible even after a severe course ( 37 ). About 30 % of children experience an epileptic encephalo pathy with long lasting intractability and cognitive impairment. C) Juvenile absence epilepsy (JAE) This syndrome has its onset around puberty and is charac terized by rare typical absence seizures that cluster upon awakening ( 29 ). In 80 % of pat ients, GTCSs are also present. They are usually rare and tend to appear after sleep depriva tion, valproic acid is often effective. Ictal EEG can show either a typical 3 Hz or a faste r (4 \200\2235 Hz) spike-and-wave discharge. D) Juvenile myoclonic epilepsy (JME) This syndrome represents about 3 % of all patients wit h epilepsy referred to different epilepsy centers in Europe ( 30 ). Its onset is between ag e 6 \200\22325 years with a peak incidence between 12 \200\22317 years. Myoclonic jerks are always present. They usually involve the face and the upper limbs. Consciousness is retain ed but the patient can fall to the ground if the lower limbs are involved. They are typical ly experienced as tiny jerks that interfere with \201 ne motor tasks in the minutes that follow awakening. G) Epilepsy with myoclonic absences (EMA) This is a rare disorder (around 0.5 \200\2231 % o f all patients with epilepsy) with onset around age 7 ( 38 ). Children present with myoclon ic absences that appear several times per day, as episodes of interrup- tion of ongoing act ivity associated to rhythmic jerks in axial mus- cles causing a movement of shoulders, head and arms. Ictal EEG oxford textbook of epilepsy and epileptic seizures 78 re \202 ected from water surfaces, stroboscopic light in discos, and tel- evision (TV) or computer screens. Seizure types are GTC (84 % of patients), absence (6 % ), focal (2.5 % ), and myoclonic (1.5 % ) ( 48 ). EEG shows characteristics overlapping IGEs. The photosensit ivity range (the lowest and highest IPS frequency capable of generating a photoparoxysmal r esponse) should be carefully de \201 ned as it cor- relates with the likelihood experienci ng seizures. Antiepileptic drug (AED) treatment can indeed be ineffective if patients are v ery sen- sitive to light or indulge in self-inducing seizures. It is essential to teach pat ients to avoid causative stimuli and/or to occlude one eye in front of them. Dark lenses ca n sometimes be bene \201 cial. Some patients are exclusively sensitive to high-contrast pa tterns (blinds, wallpaper). In photosensitive occipital lobe epilepsy focal seizures can be induced by photic stimulation (video-games, TV) ( 49 ). They are characterized by visual s ymptoms lasting up to several minutes (colorful blobs, \202 ashing lights, followed by am aurosis), followed by vegetative symptoms and headache. shows generalized 3 Hz spike-and-wave discharges resembling those of CAE, synchronous with myoclonic jerks. Prognosis is vari- able ranging from poor with evolution to different seiz ure types and mental retardation to very good with remission. A combina- tion of different AEDs (valproic acid + ethosuximide or lamotrig- ine) is the most effective treatment ( 39 ) . Familial (autosomal dominant) epilepsies A) Benign familial neonatal convulsions Benign fam ilial convulsions of the neonatal and infantile period encompass distinct age-related disor ders. Benign familial neonatal convulsions are highly penetrant, with short lasting seizure s begin- ning between a few days of life and 3 months. A few patients will manifest isolate d seizures later in life. This disorder is associated with mutations of K + channel genes K CNQ3 and KCNQ2 ( 40 , 41 ). Benign infantile convulsions are characterized by seizures betw een 4 and 8 months of age, often occurring in families. There is no known gene defect. Beni gn familial neonatal-infantile seizures begin between day 2 and 7 months of age, and are as sociated with SCN2A gene mutations ( 42 ). B) Startle-induced epilepsy This affects infants, children, or young adults with static or progres- sive encephalopathy. It is rarely observed in individuals without a brain lesion. An unexpected noise or a sudden movement can induce a focal or generalized, usually tonic s eizure ( 50 ). Spontaneous seizures also occur in 40 \200\22395 % of patients. An epilep- t ogenic network involving the central cortex or the supplementary motor area is often demons trated. B) Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) This syndrome is characteriz ed by clusters of nocturnal motor sei- zures, which are often stereotyped and brief (5 seco nds to a few minutes). They vary from simple arousals from sleep to dramatic, often bizarre , hyperkinetic events with tonic or dystonic posturing. ADNFLE is inherited as an autosomal dominant trait; penetrance is estimated at 70 % . The genes known to be associated with AD NFLE are CHRNA4, encoding the 4 subunit of the neuronal nicotinic acetylcholine receptor (nAChR); CHRNB2, encoding the 2 subunit and CHRNA2, encoding for the 2 subunit ( 43 ). Progressive myoclonus epilepsies Progressive myoclonus epilepsies (PMEs) are a group of aet iologi- cally heterogeneous conditions characterized by multifocal and generalized myocloni c jerks, GTCSs, or clonic-tonic \200\223clonic sei- zures, photosensitivity, progressive co gnitive deterioration, and cerebellar and extrapyramidal signs. The different syndromes are identi \201 ed by age at onset and rate of progression of symptoms. They include Lafora d isease, Unverricht \200\223Lundborg disease, myo- clonus epilepsy with red ragged \201 br es (MERRF), early infantile, late infantile, juvenile, and adult ceroid-lipofuscinosis, and sialido- sis ( 51 ). Many patients with PMEs do not receive an aetiological diagnosis (see Chapter 6). C) Familial lateral temporal lobe epilepsy This epilepsy syndrome is characterized by clini cal manifestations usually beginning in childhood or adolescence as auditory halluci- natio ns, often with associated olfactory, vertiginous, or visual symptoms ( 41 ). Mutations of L GI1 / epitempin , the leucine-rich glioma- inactivated 1 gene have been identi \201 ed in many families ( 44 ). D) Generalized epilepsy with febrile seizures plus This term designates a spectrum of epile psy phenotypes including febrile seizures (FSs) and mild to severe generalized epilepsies ( 45 ). FSs are present in most of patients. FS + are fever-related seizures continuing over the age of 6 or in association to afebrile GTCSs. FS + may be associated with absence, myo clonic, or atonic seizures. Inheritance is autosomal dominant with 60 % penetrance, includ- ing FSs and FS + . Mutations of 1 and 1 voltage-gated sodium channel subunit genes ( SCN1B , SCN1A ) account for 17 % of gener- alized epilepsy with FS + ( 46 ). Genetic heter ogeneity is con \201 rmed by the \201 nding of mutations in the 2 subunit GABA A rece ptor gene ( GABRG2 ) in rare families ( 47 ). Epileptic encephalopathies Epileptic encephalopathies (EEs) are conditions in which seizure s, the epileptiform abnormalities, or both, contribute to the progres- sive disturbance in cerebral function. About 40 % of all epilepsies occurring in the \201 rst 3 years of life \201 t this de \201 nition ( 52 ). Epileptic encephalopathies may appear in children wi th normal early devel- opment and no brain lesions, in which case cognitive impairment is u sually attributed to epilepsy. When a clinically manifest epilep- togenic brain lesion is p resent, spread of epileptic activity to intact, remote areas is believed to amplify its cli nical consequences. Vigorous early treatment is often advocated. However, for most EEs there are no established endpoints for medical treatment and drug adjustments are empirically established. When an epilep- togenic lesion is present and drugs fail, surgical approach can be successful. They are described in Chapter 15 and include: Re \202 ex epilepsies A) Visual sensitive epilepsy Visual sensitive epilepsy is a common m anifestation of IGEs, but it can also be found in other generalized as well as focal epilep sies. In pure photosensitive IGEs, seizures are exclusively triggered by photic stimuli suc h as sunlight through tree foliage, sunlight \227\206 Early myoclonic encephalopathy and Ohtahara syndrome. \227\206 West syndrome. \227\206 Dravet syndrome. CHAPTER 7 classification, clinical symptoms, and syndromes 79 \227\206 Lennox \200\223Gastaut syndrome. References \227\206 Landau \200\223Kleffner syndrome. 1 . Commission on Classi \201 cation and Terminology of the International League Against Epilepsy . Proposal for a revised clinical and electroencephalographic class i \201 cation of epileptic seizures . Epilepsia 1981 ; 22 : 489 \200\223 501 . \227\206 Epilepsy with continuous spike waves during slow-wave sleep. \227\206 Early onset epileptic encephalopathies caused by single gene mutations ( ARX , CD KL5 , MECP2 , FOXG1 , STBPX , GLUT1 ). 2 . Commission on Classi \201 cation and Terminology of the International League Against Epilepsy . Proposal for revised classi \201 cation of epilepsies and epilep tic syndromes . Epilepsia 1989 ; 30 : 389 \200\223 99 . Conditions with epileptic seizures that do not require a diagnosis of epilepsy Transient an d reversible epileptogenic conditions are also known as situation-related seizures. They ar e not epilepsies stricto sensu, but seizures can relapse once the patient is re-exposed to the pro- voking factor. 3 . L ders H , Acharya J , Baumgartner C , Benbadis S , Bleasel A , Burgess R , et al . Se miological seizure classi \201 cation . Epilepsia 1998 ; 39 : 1006 \200\223 13 . 4 . Engel J Jr . A proposed diagnostic scheme for people with epileptic seizures and with e pilepsy: Report of the ILAE Task Force on Classi \201 cation and Terminology . Epilepsia 2 001 ; 42 : 796 \200\223 803 . 5 . Engel J Jr . Report of the ILAE Classi \201 cation Core Group . Epilepsia 2006 ; 47 : 1558 \200\223 68 . A) Febrile seizures (FSs) FSs are epileptic seizures that appear during a febrile illness, not associated to intracranial infection or other de \201 ned causes of sei- zures, in a n ormal child aged between age 3 months and 5 years. FSs are common and affect between 2 \200 \2235 % of children below age 5. Genetic factors are involved with both autosomal dominant and polygenic inheritance. During FSs most children have respiratory tract infections. Ther e is a signi \201 cant risk of occurrence in the 24 hours that follow the DPT (diphtheria, tetanus, and pertussis) vac- cine and in the 8 \200\22314 days following the MMR (measles, mumps and rubella) vaccine ( 53 ). 6 . Berg AT , Berkovic SF , Brodie MJ . Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classi \201 cation and Terminology, 2005 \200\2232009 . Epilepsia 2010 ; 51 : 676 \200\223 85 . 7 . Guerrini R . Classi \201 cation concepts and terminology: Is clinical description assertive and laboratory testing objective? Epilepsia 2010 ; 51 : 718 \200\223 20 . 8 . Guerrini R . Epilepsy in children . Lancet 2006 ; 367 : 499 \200\223 524 . 9 . Watanab e K , Yamamoto N , Negoro T , Takahashi I , Aso K , Maehara M . Benign infantile epilepsy with complex partial seizures . J Clin Neurophysiol 1990 ; 7 : 40 9 \200\223 16 . 10 . Watanabe K , Negoro T , Aso K . Benign partial epilepsy with secondarily Epilepsy will develop in only 5 % of children who have had FSs ( 54 ). Neuroimaging should be reserved to children with prolonged generalized seizures in infancy . Epilepsia 1993 ; 34 : 635 \200\223 8 . 11 . Saadeldin IY , Housawi Y , Al Nemri A , Al Hifzi I . Benign familial and non-familial infantile seizures (Fukuyama-Watanabe-Vigevano syndrome): a study of 14 cases from Saudi Ar abia . Brain Dev 2010 ; 32 : 378 \200\223 84 . postictal unresponsiveness or focal de \201 cits ( 55 ). Simple FSs appear after the \201 rst year of life and bear an excellent prognosis. They a re characterized by bilateral clonic or tonic \200\223clonic movements, lasting less than 1 5 minutes, not relapsing during the same febrile illness, and not followed by postictal de \201 cit. Seizures take place within 24 hours from fever onset at peak temperature or duri ng defervescence. EEG is not useful in evaluating children with simple FSs. Temperature con trol is the best prophylaxis. Subsequent epilepsy is observed in less than 2.4 % of cases. 12 . Lerman P . Benign partial epilepsy with centro-temporal spikes . In: Roger J , Bureau M , Dravet C , Dreifuss FE , Perret A , Wolf P (eds) Epileptic Syndromes i n Infancy, Childhood and Adolescence , pp. 189 \200\223 200 . London : John Libbey , 1992 . 13 . Gastaut H . Benign epilepsy of childhood with occipital paroxysms . In: Roger J , Bureau M , Dravet C , Dreifuss FE , Perret A , Wolf P (eds) Epileptic Syndrom es in Infancy, Childhood and Adolescence , pp. 201 \200\223 17 . London : John Libbey , 19 92 . Complicated FSs appear within the \201 rst year of life in children who frequently have a family history of epilepsy. They represent a febrile status epilepticus characterized by l ateralized, prolonged ( > 15 minute), clonic seizures, often relapsing during the same febr ile illness and followed by postictal paresis. Acute treatment with intrarectal diazepam sh ould be used to stop the seizures. A complete neurological work-up, including EEG lumbar pu ncture and neuroimaging, should be considered if an intracerebral infec- tion is suspected. Epilepsy can develop in up to 50 % of children, particularly if prolonged relapsing seizur es followed by postictal paresis are observed. 14 . Ferrie CD , Beaumanoir A , Guerrini R, Kivity S , Vigevano F , Takaishi Y , et al . Ea rly-onset benign occipital seizure susceptibility syndrome . Epilepsia 1997 ; 38 : 285 \200\223 93 . 15 . 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 . 16 . 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 89 . London : Saunders , 2000 . 17 . Bancaud J . S miologie clinique des crises pileptiques d \200\231origine temporale . Revue Neurologique 1987 ; 143 : 392 \200\223 400 . B) Benign neonatal seizures (BNSs) These are self-limited events without sequelae. 18 . Munari C , Bancaud J , Bonis A , Buser P , Talairach J , Szikla G , et al . Impairment of consciousness in temporal lobe seizures: a stereoelectroencephalographic stud y . In: Canger R , Angeleri F , Penry JK (eds) Advances in Epileptology: XI Epilepsy Intern ational Symposium , pp. 111 \200\2231 4 . New York : Raven Press , 1980 . C) Isolated seizures/isolated status epilepticus These can appear in a patient without a fa mily history of epilepsy and can remain an isolated event, not developing into an epilepsy syndrome. Isolated simple partial seizures can frequently be observed in adolescents. 19 . Bancaud J , Talairach J . Clinical semiology of frontal lobe seizures . Adv Neurology 1992 ; 57 : 3 \200\223 58 . 20 . 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