Epileptic Seizures, Syndromes and Classifications

Epileptic Seizures, Syndromes and Classifications Randa Jarrar, MD Child Neurologist Phoenix Children's Hospital Clinical Assistant Professor, Department of Pediatrics University of Arizona Assistant Professor, Department of Neurology Mayo Graduate School of Medicine Phoenix, Arizona

Definitions

Diagnosis of Seizures A seizure is a sudden surge of electrical activity in the brain that usually affects how a person feels or acts for a short time.

Diagnosis of Seizures Potential Diagnosis of Seizures Seizures Reject (syncope, breath holding, loss of consciousness [LOC]) Acute cause? Yes Acute symptomatic No Unprovoked Febrile convulsions Only one Solitary seizure More than one Epilepsy Annegers. 1997.

Diagnosis of Epilepsy After two unprovoked seizures (>24 hours apart), the chance of having another is 73% at four years, versus 40-52% after a single unprovoked seizure. Seizures clustering within 24 hours confer approximately the same risk for later seizures as does a single seizure Hauser WA, et al. NEJM. 1998. Neligan A, et al. Handb Clin Neurol. 2012.

Diagnosis of Epilepsy A single unprovoked seizure after a remote brain insult: stroke, CNS infection or trauma A single seizure in a patient with a cortical malformation A patient with a seizure and an epilepsy syndrome with a high risk of seizure recurrence A patient with photosensitive epilepsy: seizures are provoked by lights?? Risk of a second unprovoked seizure is comparable to the risk for further seizures after two unprovoked seizures Hauser WA, et al. N Engl J Med. 1998. Hesdorffer DC, et al. Epilepsia. 2009.

New Changes An Operational Clinical Definition of Epilepsy: At least two unprovoked (or reflex) seizures occurring more than 24 hours apart; One unprovoked (or reflex) seizure and a probability of recurrence risk approximately 60% or more; Diagnosis of an epilepsy syndrome. International League Against Epilepsy. Available at: http://www.ilae.org/visitors/centre/definition-2014.cfm.

New Changes Advantages: Patient education and access to resources Could improve outcomes by sensitizing clinicians about the risk of recurrence after a single unprovoked seizure Comfort initiating treatment after one unprovoked seizure Physical injuries or social consequences Expand the opportunity for disease modifying interventions that prevent the progression of epilepsy and onset of comorbidities

New Changes Disadvantages: Insurability National health programs Stigma to a larger number of people Driving in certain countries, participation in sports

Status Epilepticus A condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures (after time point t 1 ). It is a condition, which can have long-term consequences (after time point t 2 ), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures. Trinka E, et al. Epilepsia. 2015.

Back to Classification

Why Classify? Understanding the classification of seizures/epilepsy is the first step towards the correct diagnosis Investigations pursued may be different depending on seizure types Specific seizure types or epilepsy syndromes respond better to specific medications or surgical approaches Prognosis differs; early knowledge of this allows focused treatment and lifestyle modifications for patients and families

Who Classifies International League Against Epilepsy (ILAE): The authoritative source of current and emerging knowledge, serving as a leading information resource in optimal clinical care and for finding innovative approaches to addressing the many issues that are encountered in assuring that no one s life is limited by epilepsy

Classification 1960: ILAE first published a classification system 1981: The last official update for seizures 1989: The last official update for the epilepsies 2010: A report by the ILAE Commission on Classification and Terminology recommended that changes be made in the current conceptualization, terminology, and definitions of seizures and epilepsy 2016: Proposal for seizure classification and Road Map for Classification of the Epilepsies http://www.ilae.org/visitors/centre/class-roadmap.cfm

Classification In between, there were many commissions, unsolicited advice, criticisms, invited critiques, etc Not a single classification system was felt to address the issues and concerns

Classification One has to be familiar with the old, the new, and the ugly

Classification of Seizures - 1981 Classified according to their: Clinical features Ictal EEG manifestation Interictal EEG features Commission on Classification and Terminology of the ILAE. Epilepsia. 1981.

ILAE Seizure Classification 1981 1981: Generalized Partial Simple Complex Unclassified Other Status Epilepticus Post-1997: Self-limiting Generalized Focal Continuous Generalized Focal Precipitating Stimuli for Reflex Seizures Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Engel J Jr. Epilepsia. 2001.

ILAE Classification of Epileptic Seizures Seizure Types PARTIAL SEIZURES SIMPLE PARTIAL SEIZURES Features EEG findings suggest focal onset Consciousness not impaired 1. With motor symptoms: Vocalization arrest of speech a. Focal motor b. Focal motor march (Jacksonian) c. Versive d. Postural e. Phonatory 2. With somatosensory or special sensory symptoms Simple hallucinations a. Somatosensory b. Visual c. Auditory d. Olfactory e. Gustatory f. Vertiginous 3. With autonomic symptoms or signs Epigastric sensations, pallor, sweating, flushing, piloerection, pupillary dilation 4. With psychic symptoms Disturbance of higher cortical function COMPLEX PARTIAL SEIZURES Consciousness impaired ABSENCE SEIZURES Typical absence Atypical absence MYOCLONIC SEIZURES CLONIC SEIZURES TONIC SEIZURES TONIC-CLONIC SEIZURES ATONIC SEIZURES *SWC = spike-wave complex Regular and symmetric 3-Hz *SWC on EEG Irregular slow SWC on EEG, abn Bkgrnd Polyspike or slow SWC on EEG Fast activity or slow SWC on EEG Low-voltage fast EEG Rhythm of less than 10 Hz on EEG Poly SWC or low-voltage fast Commission on Classification and Terminology of the ILAE. Epilepsia. 1981.

ILAE Seizure Classification 1981: Generalized Partial Simple Complex Unclassified Other Status Epilepticus Post-1997: Self-limiting Generalized Focal Continuous Generalized Focal Precipitating Stimuli for Reflex Seizures Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Engel J Jr. Epilepsia. 2001.

Issues with 1981 Classification Some seizure types (tonic or epileptic spasms) can have either a focal or generalized onset Lack of knowledge about the onset makes a seizure unclassifiable with the 1981 system A need to move away from preservation or alteration of awareness, responsiveness or consciousness as the only descriptor of a focal seizure (although it remains an important classifier) Some terms in current use do not have high levels of community acceptance or public understanding, such as psychic, partial, simple partial, complex partial, and dyscognitive. Some important seizure types are not included (myoclonic absence)

Classification Why change? Previous classifications are based on concepts that predate modern neuroimaging, genomic technologies and concepts in molecular biology Provide a common international terminology and classification New classification should reflect best knowledge but will not be arbitrary and should ultimately serve the purpose of improving clinical practice and research

ILAE Proposal for Revised Terminology for Organization of Seizures and Epilepsies 2010 *No NN sz International League Against Epilepsy. ILAE Proposal for Revised Terminology for Organization of Seizures and Epilepsies 2010. Available at: http://www.ilae.org/commission/class/documents/ilae%20handoutv10.pdf

ILAE Proposal for Seizure Classification 2016 Fisher RS, et al. Operational classification of seizure types by the International League Against Epilepsy. 2016. Available at: http://www.ilae.org. 2016

Major Changes in Classification, Terminology, and Concepts: For Seizures OLD TERMINOLOGY & CONCEPTS Partial: The first clinical and EEG changes indicate initial activation of a system of neurons limited to a part of one cerebral hemisphere Generalized: The first clinical changes indicate initial involvement of both hemispheres NEW TERMINOLOGY & CONCEPTS Focal seizures are conceptualized as originating at some point within networks limited to one hemisphere Generalized seizures are conceptualized as originating at some point within and rapidly engaging bilaterally distributed networks Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

Focal - Reconceptualized For seizures: Focal epileptic seizures are conceptualized as originating within networks limited to one hemisphere. These may be discretely localized or more widely distributed. Ictal onset is consistent from one seizure to the other with preferential propagation patterns Berg AT, et al. Epilepsia. 2010.

Major Changes in Classification, Terminology, and Concepts: For Seizures OLD TERMINOLOGY & CONCEPTS Partial: The first clinical and EEG changes indicate initial activation of a system of neurons limited to a part of one cerebral hemisphere Generalized: The first clinical changes indicate initial involvement of both hemispheres NEW TERMINOLOGY & CONCEPTS Focal seizures are conceptualized as originating at some point within networks limited to one hemisphere Generalized seizures are conceptualized as originating at some point within and rapidly engaging bilaterally distributed networks Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

Generalized - Reconceptualized For seizures Generalized epileptic seizures are conceptualized as originating at some point within, and rapidly engaging, bilaterally distributed networks. can include cortical and subcortical structures, but not necessarily include the entire cortex Although the onset may appear localized, the location and lateralization are not consistent from one seizure to the other Berg AT, et al. Epilepsia. 2010.

Major Changes in Classification of Focal Seizure Types OLD TERMINOLOGY & CONCEPTS Complex Partial: With impairment of consciousness Simple Partial: Consciousness not impaired Secondarily Generalized: Note that this was not the terminology used in the 1981 document but has come into common use NEW TERMINOLOGY & CONCEPTS No specific classification is recommended Seizures should be described accurately according to their semiologic features without trying to fit them into artificial categories Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

Focal Seizures Aware (without impairment of consciousness or awareness) Previous term: Simple partial With observable motor or autonomic components Eg, focal clonic, autonomic, hemiconvulsive With subjective sensory or psychic phenomena Aura - specific types Awareness: recall, responsiveness and consciousness maintained Fisher, et al. www.ilae.org. 2016. Berg AT, et al. Epilepsia. 2010.

Focal Seizures Unaware (where alteration of cognition is major feature) Previous term: complex partial Dyscognitive Fisher, et al. www.ilae.org. 2016.

Focal Seizures Evolving to bilateral, tonic clonic (convulsive) seizure Previous terms: partial seizure secondarily generalized; secondarily generalized tonic-clonic seizure With tonic, clonic, or tonic and clonic components Fisher, et al. www.ilae.org. 2016. Berg AT, et al. Epilepsia. 2010.

Focal Seizures Without impairment of consciousness or awareness Previous term: Simple partial With observable motor or autonomic components Eg, focal clonic, autonomic, hemiconvulsive With subjective sensory or psychic phenomena Aura - specific types Berg AT, et al. Epilepsia. 2010.

Focal Seizures Where alteration of cognition is major feature Previous term: complex partial Dyscognitive

Focal Seizures Evolving to bilateral, convulsive seizure Previous terms: partial seizure secondarily generalized; secondarily generalized tonic-clonic seizure With tonic, clonic or tonic and clonic components Berg AT, et al. Epilepsia. 2010.

Issues with the New ILAE Report on Seizures Not a real change but some conceptual changes Definitions are not given for the different seizures types, so what they are and what they need to include is not clear Changed complex to dyscognitive because they felt the term was misused or misunderstood when they could have emphasized its meaning Free text description of types of focal seizures may be fine in clinical practice but problematic for research, epidemiology

Issues with the New ILAE Report on Seizures NN seizures are still difficult to categorize Spasms were included but with no definition of their features No reflex epilepsy category No status epilepticus category

ILAE 1989 Classification of Epilepsies and Epilepsy Syndromes Two criteria utilized: Topography (generalized or localization-related) and etiology (idiopathic, symptomatic, cryptogenic) Commission on Classification and Terminology of the ILAE. Epilepsia. 1989.

ILAE 1989 Classification of Epilepsies and Epilepsy Syndromes Localization-related: Idiopathic, symptomatic, cryptogenic Generalized: Idiopathic, symptomatic, cryptogenic Epilepsies and syndromes undetermined whether focal or generalized With both generalized and focal: LKS, NNsz, SMEI, CSW Not clear if focal or generalized seizures Special syndromes: Situation related seizures isolated SE and febrile sz LKS=Landau-Kleffner Syndrome; NNsz=Neonatal Seizures; SMEI=Severe Myoclonic Epilepsy in Infancy; CSW=Continuous Spike Waves Commission on Classification and Terminology of the ILAE. Epilepsia. 1989.

ILAE 1989 Classification of Epilepsies and Epilepsy Syndromes Idiopathic presumed genetic with an age-related onset, clinical and EEG characteristics Symptomatic known or suspected disorder Cryptogenic unknown but presumed symptomatic Commission on Classification and Terminology of the ILAE. Epilepsia. 1989.

ILAE 1989 Classification of Epilepsies and Epilepsy Syndromes Localization related (focal, partial) Generalized Undetermined Idiopathic BECTS Childhood epilepsy with occipital spikes Primary reading epilepsy Symptomatic EPC Temporal epilepsy Frontal epilepsy Parietal epilepsy Occipital epilepsy Commission on Classification and Terminology of the ILAE. Epilepsia. 1989. Cryptogenic Idiopathic Symptomatic Cryptogenic NN familial convulsion B NN convulsions B myoclonic epilepsy of infancy CAE JAE JME GTC upon awakening Other generalized idiopathic epilepsies With activation LGS West Myoclonic astatic Epilepsy with myoclonic absences EME EIEE With specific etiology: Aicardi

ILAE 1989 Classification of Epilepsies and Epilepsy Syndromes Defined each of these epilepsy syndromes: Age of onset, clinical features, EEG features, and prognosis Commission on Classification and Terminology of the ILAE. Epilepsia. 1989.

Issues with the 1989 Classification Cannot be used by pediatricians or internists as classification requires EEG, video EEG Appeared better for pediatric epilepsies Some NN epilepsy syndromes were placed under generalized (benign familial NN convulsions) when they might include partial seizures

Issues with the 1989 Classification West syndrome/lgs was placed under generalized Idiopathic seemed to imply benign Secondarily generalized Symptomatic contrasts with subclinical MTS

2010 Major Changes in Classification, Terminology, and Concepts of Etiology OLD TERMINOLOGY & CONCEPTS Idiopathic: There is no underlying cause other than a possible hereditary predisposition Symptomatic: The epilepsy is the consequence of a known or suspected disorder of the central nervous system Cryptogenic: This refers to a disorder whose cause is hidden or occult. Cryptogenic epilepsies are presumed to be symptomatic NEW TERMINOLOGY & CONCEPTS Genetic: The epilepsy is the direct result of a known or presumed genetic defect in which seizures are the core symptom Structural/metabolic: There is a structural or metabolic condition that is associated with a substantially increased risk of developing epilepsy Unknown: The nature of the underlying cause is unknown; it may be due to an unidentified genetic, structural, or metabolic disorder Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

2010 Recommended Terminology for Etiology Use terms which mean what they say: Genetic Structural/Metabolic Unknown Berg AT, et al. Epilepsia. 2011.

Genetic Concept: The epilepsy is the direct result of a known or inferred genetic defect(s). Seizures are the core symptom of the disorder Evidence: Specific molecular genetic studies (well replicated) or evidence from appropriately designed family studies Genetic does not exclude the possibility of environmental factors contributing Berg AT, et al. Epilepsia. 2011.

Structural/Metabolic Concept: There is a distinct other structural or metabolic condition or disease present Eg, Tuberous sclerosis Evidence: Must have demonstrated a substantially increased risk of developing epilepsy in association with the condition Berg AT, et al. Epilepsia. 2011.

Unknown Concept: The nature of the underlying cause is as yet unknown Berg AT, et al. Epilepsia. 2011.

Classification: Old vs. New Organizational Structure for Epilepsies OLD TERMINOLOGY & CONCEPTS Localization-related (focal, partial): Epilepsies with focal seizures Generalized: Epilepsies with generalized seizures NEW TERMINOLOGY & CONCEPTS These terms were abandoned as overreaching categories for classifying epilepsies per se, as many syndromes include both seizure types; they may still apply in some, but not all instances (Dravet and West) Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

Classification: Old vs. New Organizational Structure for Epilepsies OLD TERMINOLOGY & CONCEPTS Hierarchically organized by localization-related, generalized, and undetermined Within those groups, by etiology (idiopathic, symptomatic, cryptogenic) NEW TERMINOLOGY & CONCEPTS No specific organization is proposed. Instead a flexible approach depending on needs is advocated Commission on Classification and Terminology of the ILAE. Epilepsia. 1981; Commission on Classification and Terminology of the ILAE. Epilepsia. 1989; Berg AT, et al. Epilepsia. 2010; Berg AT, et al. Epilepsia. 2011.

Classification of Epilepsy Syndromes Constellations Epilepsies associated with a structural/metabolic condition Epilepsies of an unknown cause Berg AT, et al. Epilepsia. 2011.

ILAE Proposal for Revised Terminology for Organization of Seizures and Epilepsies 2010 * The arrangement of electroclinical syndromes does not reflect etiology, ^ Not traditionally diagnosed as epilepsy + Sometimes referred to as Electrical Status Epilepticus during Slow Sleep (ESES) ** Forms of epilepsies not meeting criteria for specific syndromes or constellations International League Against Epilepsy. Available at: http://www.ilae.org/commission/class/documents /ILAE%20HandoutV10.pdf. Accessed July 2016.

Epilepsy Syndromes

Epilepsy Syndromes Why? Patients with epileptic seizures and their families are entitled to diagnosis, prognosis, and management that is specific and precise Medication choices (some AEDs may worsen certain seizure types) Defining the syndrome improves research, and ultimately improves treatment in all aspects

Epilepsy Syndromes Seizure type(s) EEG characteristics Age of onset and associated clinical findings Imaging (MRI, CT, etc.) Additional laboratory testing Etiology: Although it may not be uniform

Classification of the Epilepsies and Epilepsy Syndromes Epilepsy syndromes: Some syndromes represent a single disease eg, Autosomal dominant nocturnal frontal lobe epilepsy Some syndromes can be result of many diseases eg, West syndrome, Lennox-Gastaut syndrome Some diseases can manifest in different epilepsy syndromes eg, Tuberous Sclerosis Complex Some syndromes may have different outcomes eg, Infantile spasms

Childhood Epilepsy Syndromes Juvenile absence GTCS on awakening Childhood absence Rolandic epilepsy Lennox-Gastaut syndrome Simple febrile seizures Benign myoclonic epilepsy Infantile spasms EMEE/EIEE Neonatal seizures 0 5 10 15 20 25 JME (13-19) (1-2) (6 mo-1) (0-6 wk) (0-1 mo) (3-7) (6 mo-5) (1-8) (4-13) (10-15) (6-22) 0 5 10 15 20 25 Age (y) at Seizure Onset Pellock JM. Neurol Clin. 1993.

Benign vs Malignant Epilepsy Syndromes Benign: Typically have a good developmental outcomes Malignant: Typically have a poor outcomes Caveat: Not every malignant syndrome has a poor outcomes Not every benign syndrome has a good outcomes

Benign Syndromes: Benign vs Malignant Epilepsy Syndromes Childhood absence epilepsy Juvenile absence epilepsy Juvenile myoclonic epilepsy Benign childhood epilepsy with centrotemporal spikes (Rolandic) Malignant Syndromes: Infantile spasms Lennox-Gastaut syndrome

Childhood Absence Epilepsy Age of onset: 3-6 years Seizures very frequent Abrupt onset with loss of consciousness Abrupt end with resumption of activity No postictal period, child often unaware of seizure Automatisms, blinking can occur EEG Ictal: 3 Hz spike and wave, symmetric and synchronous Interictal: normal Induced by hyperventilation, occasionally photic stimulation

Absence Seizure: 3 Hz Spike and Wave

Juvenile Absence Epilepsy Age of onset: 7-17 years Lower seizure frequency than CAE; less frequent than every day, may be sporadic Majority (80%) have GTC seizures, upon awakening, which usually precede absences EEG: Ictal: 3.5-4 Hz generalized discharges Interictal: normal Typically responsive to therapy;?lifelong

Juvenile Myoclonic Epilepsy of Janz (JME) Mixed seizure disorder: Myoclonic Generalized tonic clonic (GTC) Absence seizures Familial disorder Onset usually in the second decade? Lifelong

Juvenile Myoclonic Epilepsy: Myoclonic Seizures Usually mild to moderate in intensity, may involve entire extremity rather than isolated muscles, generally bilateral Occur after awakening Aggravated by fatigue, sleep deprivation or EtOH May fall or drop objects Myoclonic status epilepticus can occur

Juvenile Myoclonic Epilepsy: Generalized Tonic-Clonic Seizures GTC or clonic-tonic-clonic seizures common 41/43 in series of Delgado-Escueta 83% in series of Asconape and Penry Myoclonic jerks precede GTC in majority, and may evolve into GTC Occur shortly after awakening or during early morning sleep Delgado-Escueta AV, Enrile-Bacsal F. Neurology. 1984; Asconape J, Penry JK. Epilepsia. 1984.

Juvenile Myoclonic Epilepsy: Absence Seizures Occur in a significant number: 40% on the series of Delgado-Escueta Usually occur in association with GTC or CTC seizures Most commonly occur shortly after awakening Delgado-Escueta AV, Enrile-Bacsal F. Neurology. 1984.

Juvenile Myoclonic Epilepsy: The EEG Interictal EEG: 3.5 to 6 Hz spike and wave and multiple spike and wave complexes With myoclonic seizures: Ictal EEG consists of 10-16 Hz rapid spikes with slow waves Photosensitivity common Abnormality may only appear in sleep

Benign Childhood Epilepsy with Centrotemporal Spikes Age of Onset: 3-13 years with peak at 7-8 years Seizure frequency typically low: 13% single seizure 66% infrequent 21% frequent (Lerman and Kivity) Nocturnal seizures only in 50%, both waking and sleep in 15%, waking only in 10%-20% Status epilepticus is rare Lerman P, Kivity S. Arch Neurol. 1975.

Clinical Characteristics of Benign Epilepsy with Centrotemporal Spikes Oral/Buccal/Lingual paresthesias Speech arrest Preservation of consciousness Sialorrhea Tonic or clonic facial movements Lerman P, Kivity S. Arch Neurol. 1975.

Benign Epilepsy with Centrotemporal Spikes: EEG Characteristics Interictal EEG: Distinctive, high amplitude, diphasic spike or sharp wave with prominent slow wave Midtemporal (T3,T4) and Central (C3,C4) Bilateral, independent Marked Sleep-Activation Horizontal Dipole present Lerman P, Kivity S. Arch Neurol. 1975.

Benign Occipital Epilepsy Age of Onset: 15 months to 17 years with peak between 5 and 7 years Symptoms and Course: Dependent on time when seizures occur: motor symptoms predominate when nocturnal, visual predominate if diurnal Seizure Duration and Frequency: Variable Headaches and vomiting occur - Migraine in the differential Lerman P, Kivity S. Arch Neurol. 1975.

Clinical Manifestations Benign Occipital Epilepsy VISUAL: Amaurosis, elementary or complex visual hallucinations Visual illusions including micropsia, metamorphopsia, or pallinopsia Rare in younger children MOTOR: Hemiclonic in 43%; CPS in 14% GTC in 13% Other in 25% (dysphasia, adversive seizures, dysesthesias) Lerman P, Kivity S. Arch Neurol. 1975.

Epilepsy Constellations Not exactly electroclinical syndromes Age of presentation is not a defining feature Have specific lesions or causes Have implications on treatment esp surgery Mesial temporal lobe epilepsy, hypothalamic hamartoma, Rasmussen Berg AT, et al. Epilepsia. 2010.

Problems with the New ILAE Report There is really no new classification, different organization May confuse the wider community Better to keep idiopathic and add genetic The category structural and metabolic may include epilepsies that are neither degenerative or autoimmune

Problems with the New ILAE Report Some clear epilepsy syndromes (CAE, etc) are mixed in the same bag as heterogeneous disorders (LGS) Didn t offer any new definitions or even definitions of the syndromes

Proposed Framework for Epilepsy Classification http://www.ilae.org/visitors/centre/class-roadmap.cfm

Other Terms Commonly Used Remote symptomatic Lesional/non-lesional Temporal/extratemporal

How Well Do These Systems Work Bodensteiner JB, et al. 1988: Classified 2219 descriptions of childhood seizures. Low overall agreement between observers, mainly because of poor details Ottman R, et al. 1993: Structured interview. Almost perfect agreement for partial seizures. Moderate to substantial agreement for generalized seizures Bodensteiner JB, et al. Epilepsia. 1988; Ottman R, et al. Neurology. 1993.

How Well Do These Systems Work Senanayke N. 1993: 1250 adult patients, only 5.4% couldn t be classified (BUT 65% were in undetermined group!!) Shah KN, et al. 1992: 483 children, 6% of seizures and 13.5% of epilepsy syndrome could not be classified Manford M, et al. 1992: When applied by GPs in England, only 33.6% were given specific diagnosis Senanayke N. Epilepsia. 1993; Shah KN, et al. Epilepsia. 1992. Manford M, et al. Arch Neurol. 1992.

How Well Do These Systems Work Several authors found that it works poorly in neonates mainly because consciousness could not be assessed.

Patients will Forever Divide their Seizures into Two Types Petit mal Grand mal

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