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In \202 ammatory and immunological diseases of the central 11 . Arzimanoglou A , French J , Blume WT , Cross JH , Ernst JP , Feucht M , et al . Lennox -Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial met hodology . Lancet Neurol 2009 ; 8 : 82 \200\223 93 . nervous system . In: Shorvon SD , Andermann F , Guerrini R (eds) The Causes of Epilepsy. Co mmon and Uncommon Causes in Adults and Children , pp. 585 \200\22392. Cambridge: Cambridge University Press , 2011 . 33 . Dalmau J . Status epilepticus due to paraneoplastic and 12 . J z wiak S , Kotulska KS . Gene table: monogenic determined nonparaneoplastic encephalitides . Epilepsia 2009 ; 50(Suppl 12) : 58 \200\223 60 . neurocutaneous disorders . Eur J Paediatr Neurol 2010 ; 14 : 449 \200\223 51 . 13 . Orlova KA , Crino PB . The tuberous sclerosis complex . Ann N Y Acad 34 . Shorvon SD . Historical introduction: The causes of epilepsy in the pre-molecular era (1860 \200\2231960) . 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The genetics of neurodevelopmental disease . Curr Opin 38 . Shorvon S . Handbook of epilepsy treatment . Oxford : Wiley Blackwell , Neurobiol 2011 ; 21 : 197 \200\223 203 . 2010 . This page intentionally left blank CHAPTER 7 Classi \201 cation, Clinical Symptoms, and Syndromes Renzo Guerrini and Carmen Barba The classi \201 cation of the epilepsies and of epilepsy syndromes has been a topic of muc h concern, and controversy for decades. In 1981 the International League Against Epilepsy ( ILAE) Commission on Classi \201 cation and Terminology proposed an International Classi \201 cation of Epileptic Seizures ( 1 ). Seizures were classi \201 ed as partial and gener alized (Table 7. 1 ). Seizures were de \201 ned as partial if the \201 rst clinical and electroencephalographic (EEG) signs indi- cated that initial activation was limited to part of one cerebral hemisphere. Partial seizures were classi \201 ed in simple or complex on the basis of whether or not awareness was impaired during the attack. Seizures were conside red as generalized if the \201 rst clini- cal and EEG changes indicated the initial invol vement of both hemispheres. Since 1989, various attempts of revision of the 1989 classi \201 cation system have been m ade. In 1998 L ders and colleagues called for a seizure classi \201 cation based exclusiv ely on ictal clinical semiology, either as reported by the patients or observers or as docu mented directly during video monitoring ( 3 ). The classi \201 cation proposal was based o n the assumption that ictal symptoms were produced Table 7.1 International classi \201 cation of epileptic seizures Partial (focal, local) seizures I) Simple partial seizures (without impairment of consciousness): With motor signs With sensory symptoms With autonomic symptoms or signs With psychic sympto ms To complement the Classi \201 cation of Epileptic Seizures, in 1989 the ILAE Commission on Classi \201 cation and Terminology pro- posed a Classi \201 cation of Epilepsies and Epi leptic Syndromes ( 2 ), conceived as a tool for characterizing epilepsies as different cond i- tions and diseases responsible for seizure recurrence. However, since current knowledge of the mechanisms of epileptogenesis was considered too limited to draw a classi \201 cati on based upon patho- genesis, the Commission adopted a syndromic classification. A syndrome was considered as a group of signs and symptoms cus- tomarily occurring in association, in cluding seizure types, clinical background, neurophysiological and neuroimaging \201 ndin gs and, often, outcome (Table 7. 2 ). According to symptoms, epilepsies were classi \201 e d as generalized and partial (or focal). Generalized epilepsies were de \201 ned as charac terized by generalized seizures, bilateral motor manifestations, and generalized interictal and ictal EEG discharges. Partial epilepsies were those characterized by sei- zures origin ating from a circumscribed brain region, and by clini- cal manifestations consistent with a focal onset of the epileptic discharge, with or without subsequent spread, and by focal ic tal or interictal EEG abnormalities. The 1989 Classi \201 cation also divided the epilepsi es by etiology, into two broad categories: idiopathic and symptomatic epilepsies. Idiopathi c epilepsies were de \201 ned by absence of any brain lesions, normal background EEG activ ity and interictal generalized spike and wave discharges. They were consid- ered to be due to a genetic predisposition or to a speci \201 c mode of inheritance. Symptomatic epilepsi es were considered the expres- sion of a focal or diffuse brain lesion as demonstrated by c linical history, structural neuroimaging, EEG \201 ndings, or biological tests. II) Complex partial seizures (with impairment of consciousness) III) Simple partial onset f ollowed by impairment of consciousness: With simple partial features followed by impaired consciousness With automatisms With impai rment of consciousness at onset With impairment of consciousness only With automatisms IV) Partial seizures evolving to secondarily generalized seizures: Simple partial seizures evolving to generalized seizures Complex partial seizures evolving to generalized seizures Simple partial seizures evolving to complex partial seizures evolving to generalized seizur es Generalized seizures I) Absence seizures: Absences Atypical absences II) Myoclonic seizures III) Tonic seizures IV) Atonic seizures V ) Clonic seizures VI) Tonic \200\223clonic seizures Unclassi \201 ed epileptic seizures (lack of information) Reproduced from Commission on Classi \201 cation and Terminology of the International Leag ue Against Epilepsy. Proposal for a revised clinical and electroencephalographic classi \201 cation of epileptic seizures. Epilepsia 1981; 22:489 \200\223501. With permission from Wiley. oxford textbook of epilepsy and epileptic seizures 72 Table 7.2 International classi \201 cation of epilepsies and epileptic syndromes by epileptic interference of one of four \200\230spheres \200\231: sensorial, con- sciousn ess, autonomic and motor. Accordingly, seizures were clas- si \201 ed in four main categor ies: auras, dialeptic, autonomic, and motor seizures. EEG \201 ndings were taken into acc ount to differenti- ate between epileptic and non-epileptic events ( 3 ). This approach, ho wever, was criticized because, it was suggested, different epilep- tic syndromes, having di fferent outcomes, can be associated with the same types of seizures and appropriate managem ent of a given patient requires that the physician de \201 nes the epileptic syndrome. In 2001 the ILAE Task Force on Classi \201 cation and Terminology proposed a \200\230new dia gnostic scheme for people with epileptic seizures and with epilepsy \200\231 ( 4 ), organiz ed in \201 ve axes (Table 7. 3 ). That report recommended the terms of \200\230simple \200\231 and \200\230complex \200\231 be avoided in classifying speci \201 c seizure type s, while ictal impairment of consciousness be mentioned when occurring in individual sei- z ures. The terms \200\230partial \200\231 and \200\230cryptogenic \200\231 were discourage d as they conveyed ambiguous implications and replaced with \200\230focal \200\231 and \200\230probably symptomatic \200\231, respectively. The 2001 Task Force warned against the previous dichotomy between the concepts of \200\230partial \200\231 versus \200\230gener alized \200\231 abnormalities, which might lead to neglect a variety of conditions between focal and generalized epi- leptogenic dysfunctions such as diffuse hemispheric or multifoca l abnormalities ( 4 ). Finally, the concept of epileptic encephalopathy was introduced, to designate a group of conditions in which the epileptiform activity may contribute to progre ssive cerebral dysfunction. Localization-related (focal, local) epilepsies and epileptic syndromes I) Idiopathic: Benign childhood epilepsy with centrotemporal spikes Childhood epilepsy with occipital paro xysms Primary reading epilepsy II) Symptomatic: Chronic progressive epilepsia partialis continua of childhood Epilepsy characterized by sei zures with speci \201 c modes of precipitation Syndromes of great individual variability w hich are based mainly on seizure types and other clinical features as well as anatomic loca lization and etiology III) Cryptogenic: Cryptogenic epilepsies are presumed to be symptomatic and the etiology is unknown Generalized epilepsies/epileptic syndromes I) Idiopathic: Benign neonatal familial convulsions Benign neonatal convulsions Benign myoclonic epilepsie s of infancy Childhood absence epilepsy (pyknolepsy) Juvenile absence epilepsy Juvenile myo clonic epilepsy Epilepsy with grand mal (GTC) seizures on awakening Other idiopathic genera lized epilepsies not de \201 ned above Epilepsy with seizures precipitated by speci \201 c modes of activation In 2006, the ILAE Task Force on Classi \201 cation and Terminology ( 5 ) proposed new crit eria for the identi \201 cation of speci \201 c seizure types and epilepsy syndromes (see Tables 7. 4 and 7. 5 ), with epilep- tic seizures being classi \201 ed according to patho physiologic mecha- nisms, neuronal substrates, response to AEDs, and ictal EEG patterns. Ep ileptic syndromes were classi \201 ed according to seizure type, age of onset, interictal EEG pattern, progressive nature, pathophysiological mechanisms, anatomical substrates, aeti ologi- cal categories, genetic basis, and associated interictal signs and symptoms. In 2006 , the ILAE Task Force concluded that because II) Cryptogenic a or symptomatic: West syndrome Lennox \200\223Gastaut syndrome Epilepsy with myoclonic-astatic seizures Epil epsy with myoclonic absences III) Symptomatic: Non-speci \201 c etiology Early myoclonic encephalopathy Early infantile encephalopathy wi th suppression-bursts Other symptomatic generalized epilepsies not de \201 ned above Speci \201 c syndromes Epilepsies/epileptic syndromes undetermined as to whether focal or generalized With both partial and generalized seizures: Table 7.3 Proposed diagnostic scheme for people with epileptic seizures and with epilepsy Neonatal seizures Severe myoclonic epilepsy in infancy Epilepsy with continuous spike-waves during sleep Acquired epileptic aphasia (Landau \200\223Kleffner syndrome) Other undetermi ned epilepsies not de \201 ned above Axis 1: Ictal phenomenology, from the Glossary of Descriptive Ictal Terminology, can be use d to describe ictal events with any degree of detail needed Without unequivocal generalized or focal features Axis 2: Seizure type, from the List of Epileptic Seizures. Localization within the brain an d precipitating stimuli for re \202 ex seizures should be speci \201 ed when appropriate Special syndromes Situation-related seizures: Febrile convulsions Isolated seizure/status epilepticus Seizure s due to acute metabolic or toxic factors Axis 3: Syndrome, from the List of Epilepsy Syndromes, with the understanding that a syndro mic diagnosis may not always be possible Axis 4: Etiology, from a Classi \201 cation of Diseases Frequently Associated with Epilept ic Seizures or Epilepsy Syndromes when possible, genetic defects, or speci \201 c patholog ic substrates for symptomatic focal epilepsies a Use of the term \200\230Cryptogenic \200\231 is now discouraged, and should be substitut ed by \200\230presumed symptomatic \200\231. Reproduced from Commission on Classi \201 ca tion and Terminology of the International League Against Epilepsy. Proposal for revised cla ssi \201 cation of epilepsies and epileptic syndromes. Epilepsia 1989; 30:389 \200\22399. With permission from Wiley. Axis 5: Impairment, this optional, but often useful, additional diagnostic parameter can be derived from an impairment classi \201 cation adapted from the WHO ICIDH-2 Reproduced from Engel J Jr. A Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy: Report of the ILAE Task Force on Classi \201 cation and Terminology. E pilepsia 2001; 42:796 \200\223803. With permission from Wiley. CHAPTER 7 classification, clinical symptoms, and syndromes 73 Table 7.4 Epilepsy syndromes by age of onset Table 7.5 Seizure types Neonatal period Self-limited epileptic seizures Benign familial neonatal seizures Early myoclonic encephalopathy Ohtahara syndrome Infancy Migrating partial seizures of infancy West syndrome and infantile spasms Myoclonic epilepsy in infancy Benign infantile seizures Dravet syndrome Myoclonic encephalopathy in non-progr essive disorders I. Generalized onset A. Seizures with tonic and/or clonic manifestations B. Absences C. Myoclonic seizure types D. Epileptic spasms E. Atonic seizures II. Focal onset (partial) A. Local 1. Neocortical 2. Hippocampal and parahippocampal Childhood B. With ipsilateral propagation to: Early onset benign childhood occipital epilepsy (Panayiotopoulos type) Epilepsy with myoclo nic astatic seizures Benign childhood epilepsy with centrotemporal spikes Late onset childh ood occipital epilepsy (Gastaut type) Epilepsy with myoclonic absences Lennox \200\223Gasta ut syndrome Epileptic encephalopathy with continuous spike-and-wave during sleep, including Landau \200\223Kleffner syndrome Childhood absence epilepsy 1. Neocortical areas (includes hemiclonic seizures) 2. Limbic areas (includes gelastic seiz ures) C. With contralateral spread to: 1. Neocortical areas (hyperkinetic seizures) 2. Limbic areas (dyscognitive seizures with or without automatisms [psychomotor]) D. Secondarily generalized 1. Tonic \200\223clonic seizures 2. Absence seizures 3. Epileptic spasms (unveri \201 ed) Adolescence Juvenile absence epilepsy Juvenile myoclonic epilepsy Progressive myoclonus epilepsies Less speci \201 c age relationship Autosomal-dominant nocturnal frontal lobe epilepsy Familial temporal lobe epilepsies Mesial temporal lobe epilepsy with hippocampal sclerosis Rasmusse n syndrome Gelastic seizures with hypothalamic hamartoma III. Neonatal seizures Status epilepticus I. Epilepsia partialis continua (EPC) II. Supplementary motor area (SMA) status epilepticus III. Aura continua IV. Dyscognitive f ocal (psychomotor, complex partial) status epilepticus V. Tonic \200\223clonic status epile pticus VI. Absence status epilepticus VII. Myoclonic status epilepticus VIII. Tonic status epilepticus Special epilepsy conditions Symptomatic focal epilepsies not otherwise speci \201 ed Epilepsy with generalized tonic \200\223clonic seizures only Re \202 ex epilepsies Febrile seizures plus Familial focal ep ilepsy with variable foci Reproduced from Engel J Jr. Report of the ILAE Classi \201 cation Core Group. Epilepsia 20 06; 47:1558 \200\22368. With permission from Wiley. the terms of generalized and focal be abandoned, as this dichotomy was considered no longer meaningful except for some forms of epilepsy such as, for example, many of the encephalopa thic con- ditions observed in infants and young children and some of the neurodegenerative disorders of later life. Considering etiology, the terms idiopathic, symptomatic, and crypt ogenic were consid- ered unworkable and from the perspective of recent knowledge prompted b y molecular genetics and neuroimaging, the Commission proposed the terms of genetic (epilep sy is a direct result of a genetic cause) and structural and metabolic (epilepsy is the sec ondary result of a separate structural or metabolic condi- tion). These Commission proposal s have prompted abundant con- troversies and criticisms. The suggestions for updating termi nology and concepts or amending the list of syndromes were, in fact, rather limited. A numb er of concept categories had already been recognized and updated in the 2001 and 2006 Task force reports ( 4 , 5 ) and had been reinforced over time ( 7 ). For example, the term \200\230cryptogenic \200\231 had been discouraged since 2001 ( 4 ). On the other hand, the main syndrome categories introduced in 1989 still appear reliable for clinical and epidemio logical purposes. Adding a class of genetic epilepsies to the aetiological classi \201 cat ion may be appropri- ate to those epilepsies that are a direct result of a known genetic de fect, in which seizures are the core symptom of the disorder. Conditions with epileptic seizures that do not require a diagnosis of epilepsy Benign neonatal seizures Febrile seizures Reproduced from Engel J Jr. Report of the ILAE Classi \201 cation Core Group. Epilepsia 20 06; 47:1558 \200\22368. With permission from Wiley. the 1989 Classi \201 cation of epilepsy syndromes and epilepsies was generally accepted an d workable, it would not be discarded unless, and until, clearly better classi \201 cation s had been devised. The Task Force suggested retaining the terms of \200\230focal \200\231 and \200\230generalized, \200\231 due to the prevalent usage and therapeutic implications . Conversely, the term cryptogenic was again discouraged as it was unclear whether it shoul d be limited to conditions that are probably symp- tomatic, as originally intended, or to a ll conditions with unknown etiology, as habitually meant by epidemiologists. Various suggestions for revising terms and concepts of the 1989 Classi \201 cation were pr oposed in 2010 ( 6 ). Although no adequate knowledge was considered to be available to prop ose a new classi \201 - cation of the epilepsies new terms and concepts appeared to better re \202 ect current knowledge. For instance, it was recommended that oxford textbook of epilepsy and epileptic seizures 74 Epileptic seizures Generalized onset A) Seizures with tonic and/or clonic manifestations However, for the great majority of patients, the categories of idio- pathic and symptomatic still have solid implications for diagnosis, management, and prognosis, and allow a large number of patients to be categorized in easily recognizable syndromes ( 7 ). In view of the uncertainty related to the most recent proposed amendments to the classi \201 cation system, we will describe here the main symptom categories and epileptic syndrom es as listed in the 1989 classi \201 cation and revised in the 2001 and 2006 Task force re ports ( 8 ) (Table 7. 6 ), however introducing the most relevant amongst the most recently formulated concepts. I) Clonic seizures: clonic seizures are fast rhythmic events (1 \200\2232 Hz), often associated with impaired consciousness. II) Tonic seizures: the mechanism of tonic seizures is probably not the same as that of the tonic phase of generalized tonic \200\223 clonic seizures. Generalized tonic seizures typi cally occur in Lennox \200\223Gastaut syndrome and occasionally in epilepsy with myoclonic astatic (or myoclonic-atonic) seizures. III) Generalized tonic \200\223clonic seizures (GTCSs) have sudden onset with immediate los s of consciousness. There is a brief tonic phase (10 \200\22330 seconds) with whole body to nic contraction, associated with a loud scream and vegetative symptoms such as tachycardia, midriasis, increased blood pressure, and apnoea. Tongue biting if present, is produced at this stage. The clonic phase lasts around 30 seconds \200\224 1 minute and is charac- teri zed by bilateral clonic jerks that gradually decrease in intensity and frequency. The posti ctal phase, which can last for several minutes up to hours, is characterized by initial myd ria- sis, body relaxation, hypotonia, and sleep. Urination if present, takes place at this stage. Finally the patient gradually recovers and appears confused, presenting sometimes wi th automa- tisms, headache, and muscle aches. Table 7.6 Epileptic syndromes and epilepsies Idiopathic focal epilepsies A) Benign infantile seizures (non-familial) B) Benign childhood epilepsy with centrotempora l spikes C) Early and late onset idiopathic occipital epilepsy Symptomatic (or probably symptomatic) focal epilepsies A) Lobar epilepsies B) Migrating partial seizures of early infancy Idiopathic generalized epilepsies A) Benign myoclonic epilepsy in infancy B) Childhood absence epilepsy C) Juvenile absence e pilepsy D) Juvenile myoclonic epilepsy E) Epilepsy with generalized tonic \200\223clonic se izures only F) Epilepsy with myoclonic astatic seizures G) Epilepsy with myoclonic absences B) Myoclonic seizures Myoclonic seizures are manifested as brief symmetrical muscular jerks of variable intensity. Proximal muscles such as girdle muscles are mostly involved. During stronger attacks, there is possibility of the patient falling over, but quickly recovering . The patient is usually conscious during the jerks. Myoclonic seizures may often be trigge red by photic stimulation. Familial (autosomal dominant) epilepsies A) Benign familial neonatal convulsions and benign familial infantile convulsions B) Autoso mal dominant nocturnal frontal lobe epilepsy C) Familial lateral temporal lobe epilepsy D) Generalized epilepsies with febrile seizures plus C) Absences Typical absence seizures are brief (5 \200\22312 seconds). They appear mostly i n children and are clinically characterized by sudden inter- ruption of ongoing activity an d staring straight ahead or drifting upwards. There is complete loss of awareness during th e seizure. The onset and offset is sharp. Possible associated manifestations include slight rhythmic (3 Hz) eyelid myoclonus, slight decrement or increment of muscle tone, simple ges tural automatisms (if the absence is of long duration), and, rarely, vegetative symptoms (u ri- nary incontinence, pupil dilatation, pallor, \202 ushing, tachycardia, change in bloo d pressure). Absence seizures can be easily produced if the child is asked to hyperventilat e. Concomitant EEG abnor- malities are typical generalized spike-and wave discharge at 3 Hz . Re \202 ex epilepsies A) Idiopathic photosensitive occipital lobe and other visual sensitive epilepsies B) Startl e epilepsy Epileptic encephalopathies A) Early myoclonic encephalopathy and Ohtahara syndrome B) West syndrome C) Dravet syndrome D) Lennox \200\223Gastaut syndrome E) Landau \200\223Kleffner syndrome F) Epilepsy with co ntinuous spike waves during slow-wave sleep Progressive myoclonus epilepsies A) Lafora disease B) Unverricht \200\223Lundborg disease C) Mitochondrial encephalopathy D) Late infantile, juvenile, and adult ceroid-lipofuscinosis E) Sialidosis D) Epileptic spasms These consist of a brief (0.5 \200\2232 second) tonic contraction of th e neck and trunk in \202 exion, extension or in a mixed \202 exed-extended posture. The y occur most commonly in clusters upon awakening. Each cluster consists of several spasms t he intensity and frequency of which follow an increasing-plateau-decreasing pattern. Theref ore the \201 rst spasms in a cluster can be barely visible, presenting a forced opening o f the eyes or slight nodding of the head. Ictal EEG is char- acterized by pseudo-periodic s low polyphasic EEG discharges that are concomitant to spasms. EEG activity related to spasm s can also Seizures not necessarily needing a diagnosis of epilepsy A) Febrile seizures B) Benign neonatal seizures C) Isolated seizures/isolated status epilep ticus D) Drug or other chemically-induced seizures Reprinted from Guerrini R. Epilepsy in children. The Lancet 2006; 367:499 \200\223524. Copy right (2006), with permission from Elsevier. CHAPTER 7 classification, clinical symptoms, and syndromes 75 arousal with ictal anarthria, while the child is fully aware. Secondary generalization can supervene but is rare. Typically parents are awakened by a grunting sound. They \201 nd t he child dribbling pro- fusely and unable to speak. Interictal EEG shows typical high ampli tude, slow biphasic centrotemporal spikes, with a character- istic tangential dipolar distr ibution. Spikes greatly increase in frequency and often become bilateral during sleep. be a bilateral electrodecremental pattern. Electromyographic activ- ity from deltoid and ne ck muscles shows a characteristic rhomboid pattern during the spasm, usually lasting 0.5 \200\2232 seconds. E) Atonic seizures Atonic seizures are characterized by decrease or complete inhibi- tion o f postural tone. They manifest as head nodding, dropping of the jaw or of a limb, or falls. The patient can then lie motionless on the ground or promptly resume the posture. Pure ato nic seizures are rare. Ictal EEG is usually characterized by a generalized slow spike-and-w ave discharge. AED treatment can be avoided if seizures are brief and limited to sleep. If treatment is ne cessary carbamazepine or valproate are preferred ( 8 ). C) Idiopathic childhood occipital epilepsy (ICOE) ICOE was originally described by Gastaut in 1982 as an idiopathic partial epilepsy with visual seizures (illusions, elementary hallu ci- nations, ictal blindness) evolving either to hemiclonic or complex partial seizures, wi th or without secondary generalization, with age of onset between 15 months and 17 years. I n around 25 % of Gastaut \200\231s patients, a migraine-type postictal headache was reporte d ( 13 ). Interictal EEG showed normal background activity, with high amplitude, unilateral or bilateral, synchronous or asynchro- nous, occipital spike-and-wave discharges, facilita ted by eye clo- sure. Outcome was generally favorable with remission before adulthood. Focal onset (For a detailed description see \200\230A) Lobar epilepsies \200\231 section.) A) Focal sensory seizures. I) With elementary sensory (visual, somatosensory, vestibular, olfactory, gustatory, or aud itory) symptoms as produced by activation of primary sensory cortices (e.g. occipital and p arietal lobe seizures). II) With experiential symptoms. These are complex, formed, distorted and/or multimodal sens ory symptoms, usually implying seizure initiation in association cortices, such as the temp oro-parieto-occipital junction. A subset of children with ICOE present brief or prolonged sleep- related seizures character ized by tonic eye and head deviation, vomiting, and eventually hemiclonic or secondary gene ralization ( 14 ) The age at seizure onset is between 2 \200\2238 years. Prognosis is excel lent with remission by the age of 12 and with most children having suffered only one seizur e. This form of early onset idio- pathic occipital epilepsy is also referred to as \200 \230Panayiotopoulos syn- drome \200\231 ( 15 ) or benign childhood seizure susceptibility s yndrome. The EEG abnormalities in this syndrome are not as speci \201 c as those of BECTS; in fact similar abnormalities can be observed in cryptogenic or symptomatic focal epilepsi es with less favorable prognosis ( 16 ). 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.