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Day 1_Avanzini -Epilepsy Seizure and Classification.pptx
- 1. EPILEPSY AND SEIZUE CLASSIFICATION GiulianoAvanzini Istituto Neurologico C. Besta Milano. Italy 21° San Servolo Advanced Epilepsy Course July 21-August 1 San Ser and new volo , Venice Italy Bridging basic with Clinical Epileptology (8) Contribution of non neuronal cells to epilepsy and new therapautic strategies
- 2. “There are twoways of investigating diseases, and two kinds of classification corresponding thereto, the empirical and the scientific. The former is to be illustrated by the way in which a gardener classifies plants, the latter by the way in which a botanist classifies them.” Johns Hughlings Jackson (1874) On classification and on methods of investigation” 1874
- 3. The gardener arrangeshis plants as they are fit for food, for ornament etc. His object is the direct application of knowledge The botanist classification (the classification properly so-called) is rather for the better organisation of existing knowledge and for discovering the relations of new facts, it is also of utilitarian value, but not directly Classifications reflect the state of knowledge in a scientific field.
- 6. Taxomic Rules Classifiers: Reflectbiological classes conceptual justification an directly impact clinical managment (utilitarian justification): 1. Main seizure classes 2. Seizure types 3. Level of consciousness Descriptors: represent key seizure characteristic which aid seizure managment when combined with other clinical data. 4. Semeiological features
- 7. DESCRIPTORS FOR FOCALAND UNKNOWN FOCAL/GENERALIZED SEIZURES 1. ELEMENTARY MOTOR PHENOMENA 2. COMPLEX MOTOR PHENOMENA 3. SENSORY PHENOMENA 4. COGNITIVE AND LANGUAGE PHENOMENA 5. AUTONOMIC PHENOMENA--------------------- 6. AFFECTIVE (EMOTIONAL) PHENOMENA 7. INDESCRIBABLE AURA • 5. AUTONOMIC PHENOMENA Cardiovascular ○ Ictal asystole ○ Ictal bradycardia ○ Ictal tachycardia • Cutaneous/thermoregulatory ○ Flushing ○ Piloerection ○ Sweating • Epigastric • Gastrointestinal ○ Borborygmi ○ Flatulence ○ Hypersalivation ○ Nausea/vomiting ○ Polydipsia ○ Sialorrhea ○ Spitting • Pupillary ○ Miosis ○ Mydriasis • Respiratory ○ Apnea ○ Choking ○ Hyperventilation ○ Hypoventilation • Urinary ○ Incontinence ○ Urinary urge
- 8. Main changes introducedin Present Classification • 1. “Onset” is removed from the names of the main seizure classes. 2. A distinction is made between classifiers and descriptors, based on taxonomic rule. 3. Consciousness is used as a classifier instead of awareness, with consciousness operationally defined by awareness and responsiveness. 4. The motor vs. non motor dichotomy is replaced by observable vs. non observable manifestations. 5. The chronological sequence of seizure semiology is used to describe seizures, rather than relying solely on the first sign. 6. Epileptic negative myoclonus is recognized as a seizure type.
- 9. Juvenile Myoclonic Epilepsy(JME) Janz Syndrome Collegamento (2) a 14 dia.21.JME.lnk
- 10. Fp2-Avg P4 -Avg F4 -Avg C4-Avg T6-Avg F8-Avg T4-Avg O2-Avg Fp1-Avg P3-Avg F3-Avg C3-Avg T5-Avg F7-Avg T3-Avg O1-Avg Fz-Avg Cz-Avg Pz-Avg 50 V R Deltoid L Deltoid 50 ms F4 -Avg F3 -Avg R Deltoid L Deltoid 20 ms -7 5 V 7 5 V C 25 V B A
- 11. A cortical “focus”of spike-and-wave discharges SWDs are initiated in the facial somatosensory cortex in GAERS and propagate to other cortical areas and to the thalamus. Polack, Guillemain, Hu, Deransart, Depaulis and Charpier, J. of Neurosci June 2007
- 12. VPM VPL 18.4 8.8 29.3 3.1 9.9 Hindpaw UpperLip Nose “FOCUS” SmI Thalamus B.whole seizure 4.9 A. first 500 msec VPM 2.9 8.1 11.7 30.0 4.3 6.1 VPL UpperLip Nose “FOCUS” SmI Thalamus Hindpaw 20-30 40-50 30-40 70-80 50-60 60-70 Association (%) Meeren, Pijn, van Luijtelaar, Coenen and Lopes da Silva, J. Neurosci 2002,22:1480-95 Cortico-Cortical. Intra-Thalamic and Cortico-Thalamic relations
- 13. CONSCIOUSNESS • Consciousness: awarenessof self, of his own mental contents and of the objects to which it turns (Descartes Kant, Hegel, Jaspers, Husserl) • The source of every type of human experience or knowledges is somebody’s content of consciousness and is therefore not accessible to any type of objectve analysis (Hartwig Kuhlenbeck) • If we metaphorically describe consciousness as the only window trough which we can observe the world then when we look through this window we cannot see it (Peter Gloor) • You are conscious when you are actively interacting with the world (Alva Noe)
- 14. EPILEPTIC SEIZURES &EPILEPSIES • An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. • Epilepsy is a chronic condition of the brain characterized by an enduring propensity to generate epileptic seizures, and by the neurobiological, cognitive, psychological, and social consequences of this condition.
- 15. ILAE EPILEPSY (EPILEPTICSYNDROMES) CLASSIFICATION IDIOPATHIC SYMPTOMATIC Generalized Generalized Partial/localization related Partial/localization related
- 16. IDIOPATHIC PARTIAL EPILEPSIES •The concept of benign partial epilepsies (BPE) that emerged in the late fifties and early sixties (Bancaud et al 1958, Nayrac and Beaussard 1958, Faure and Loiseau 1960, Lombroso 1967) introduced a novel view in the pathophysiological concepts founded by John Hughlings Jackson. • Besides epilepsies due to focal epileptogenic lesions and “functional” epilepsies due to a dysfunction of diffusely projecting “centrencephalic” structures a new category of epilepsies due to a dysfunction affecting a cortical area in which no evidence of lesion was demonstrable was then established.
- 17. These localization-related epilepsiescould not be attributed to an epileptic focus in an anatomic sense but rather to an age- related hyperexcitable condition of a given cortical region, most frequently the sensory-motor and visual ones, with no detectable structural alteration.
- 18. EPILEPSY SYNDROME A complexof signs and symptoms that define a unique epilepsy condition with different etiologies. This must involve more than just the seizure type; thus frontal lobe seizures per se, for instance, do not constitute a syndrome.
- 19. Idiopathic epilepsy syndrome:A syndrome that is only epilepsy, with no underlying structural brain lesion or other neurologic signs or symptoms. These are presumed to be genetic and are usually age dependent. Symptomatic epilepsy syndrome: A syndrome in which the epileptic seizures are the result of one or more identifiable structural lesions of the brain. Probably symptomatic or cryptogenic epilepsy syndrome: syndromes that are believed to be symptomatic, but no etiology has been identified.
- 20. CURRENT CLASSIFICATION OFTHE EPILEPSIES (1) IDIOPATHIC EPILEPSIES GENERALIZED • Benign neonatal (familial) seizures • Benign myoclonic epilepsy in infancy • Childhood absence epilepsy • Juvenile myoclonic epilepsy • Juvenile absence epilepsy • Generalized epilepsy with tonic-clonic seizures (on awakening, during sleep, at random) PARTIAL • Benign childhood epilepsy with centro-temporal spikes • Childhood epilepsy with occipital paroxysms
- 21. SYMPTOMATIC/CRYPTOGENIC EPILEPSIES GENERALIZED • earlyinfantile encephalopathies with suppression burst • infantile spasms (West syndrome) • severe myoclonic epilepsy in infancy • Lennox-Gastaut syndrome Progressive epileptogenic encephalopathies (ceroidolipofuscinoses, sialidoses, Lafora disease, Unverricht- Lundborg disease etc.) PARTIAL • mesial temporal lobe epilepsy • others (defined according to the location of the epileptogenic zone) CURRENT CLASSIFICATION OF THE EPILEPSIES (2)
- 22. SITUATION RELATED EPILEPSIES: •Reflex epilepsies • Febrile seizures STATUS EPILEPTICUS • Non age related status epilepticus • Rare age-related syndromes presenting with electrical or clinical status CURRENT CLASSIFICATION OF THE EPILEPSIES (3)