1. Epilepsy Diagnosis of epilepsy PÉCSI TUDOMÁNYEGYETEM SZEGEDI TUDOMÁNYEGYETEM „AZ ÉLETTUDOMÁNYI-KLINIKAI FELSŐOKTATÁS GYAKORLATORIENTÁLT ÉS HALLGATÓBARÁT KORSZERŰSÍTÉSE A VIDÉKI KÉPZŐHELYEK NEMZETKÖZI VERSENYKÉPESSÉGÉNEK ERŐSÍTÉSÉRE” TÁMOP-4.1.1.C-13/1/KONV-2014-0001

2. I. Definition and classification of epilepsy definítion klassification differential diagnostic procedure II. Diagnostic and differentialdiagnostic methods of epilepsy III. Epileptic seizures signs and symptoms klassification IV. Diagnosis of epileptic syndromes TÁMOP-4.1.1.C-13/1/KONV-2014-0001

3. Dg: Epilepsy – definition of epilepsy Etiology Patomechanism Seizures Syndromes Therapy Prognosis Structural abnormalities Molecular – ion, neurotransmitter, receptor – membrane-integrity – metabolism, energy supply Cellular – action potential generation and propagation – synaptic processing (chemical, electrical) Networks Genetic ILAE, Fisher et al., Epilepsia 2005;46(4):470-472 Excitation (Glutamat) Inhibition (GABA) EPILEPSY: GROUP OF HETEROGEN DISORDERS TÁMOP-4.1.1.C-13/1/KONV-2014-0001

4. at least one epileptic seizures I. The definition of epilepsy requires the occurence of at least one epileptic seizures. an enduring predisposition to generate epileptic seizures II. Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures. complex neurobiological state III. Epilepsy is a complex neurobiological state - more than potential recurrence of seizure – associated with – neurocognitive and/or psychological symptomes – and sotial disturbances (patients with epilepsy may suffer from stigma). TÁMOP-4.1.1.C-13/1/KONV-2014-0001 THE CONCEPTUALLY DEFINITION OF EPILEPSY (ILAE, 2005)

5. A PRACTICAL CLINICAL DEFINITION OF EPILEPSY (ILAE, 2014) Epilepsy is a disease of the brain defined by any of the following conditions: 1. At least two unprovoked (or reflex) seizures occurring more than 24 hours apart; 2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years; 3. Diagnosis of an epilepsy syndrome. Epilepsy is considered to be resolved for individuals who had an age- dependent epilepsy syndrome but are now past the applicable age or those who have remained seizure-free for the last ten years, with no seizure medicines for the last five years. TÁMOP-4.1.1.C-13/1/KONV-2014-0001

6. Ad I. Ad I. Clarification of seizure symptoms ♦ Anamnesis: auto-, and heteroanamnesis ♦ Video: home-made video, video-EEG monitoring Ad II. Ad II. Phenomenons significantly associated with epileptic mechanisms: ♦ Neurologic examination – neurologic deficits ♦ Brain MRI, according to epilepsy protocol – epileptogenic lesion ♦ EEG, video-EEG examination – epileptiform discharges ♦ „Anything else, that is neccessary” epileptic risk factors, febrile seizure, genetic alteration ECG – arrhytmia; laboratory parameters – metabolic disorders acute imaging modalities (CT,MR) – acute CNS lesion Ad III. Ad III. Assesment of psychosotial status ♦ Neurocognitive and psychiatric examination – memory deficit, psychiatric symptomes ♦ Sotial anamnesis –sotial status BASIC DIAGNOSTIC CONSIDERATIONS TÁMOP-4.1.1.C-13/1/KONV-2014-0001

7. Paroxysmal clinical event Paroxysmal clinical event = / ≠ ?? Epileptic seizure = / ≠ = / ≠ ??Epilepsy ?? Epileptic syndrome ? Antiepileptic medication / Antiepileptic medication / epilepsy surgery epilepsy surgery KEY ISSUES TÁMOP-4.1.1.C-13/1/KONV-2014-0001

8. Paroxysmal clinical event Paroxysmal clinical event = / ≠ ?? Epileptic seizure = / ≠ = / ≠ ??Epilepsy ?? Epileptic syndrome ? Antiepileptic medication / Antiepileptic medication / epilepsy surgery epilepsy surgery DIFFERENTIAL DIAGNOSIS I. Common differential diagnostic events: - psychogenic non- epileptogenic event - synkope - REM slepp disorders - migraine - TIA TÁMOP-4.1.1.C-13/1/KONV-2014-0001

9. First-line questionnaire to differenciate syncope and epileptic seizure Prepared by: Department of Neurology, University of Pécs (2010), based on the recommendations of „Journal of the American College of Cardiology:40;142-148 (2002)”. 1. Is there a sign of tongue-biting after the seizure? y:2 n:0 2. Was there a sensation of „deja vu” or „jamais vu” before the seizure? y:1 n:0 3. Was a tonic head version observed during the seizure? y:1 n:0 4. Was any of the following signs observed during the seizure: loss of contact, uncommon limb position, repetitive muscle contractions, amnesia? y:1 n:0 5. Was there confusion and/or restlessness observed after the seizure? y:1 n:0 6. Have the patient remained concious during the attack y:-2 n:0 7. Was excessive perspiration observed before the seizure? y:-2 n:0 8. Can the seizure be correlated with long-time standing? y:-2 n:0 9. Have the patient shouted or formed unarticulated voices during the attack? y:1 n:0 EPI >= 1 (Indicates epilepsy if the sum of scores are over 1) Syncope < 1 (Indicates syncope if the sum of scores are below 1) TÁMOP-4.1.1.C-13/1/KONV-2014-0001

10. The most common causes of convulsive syncope require immediate intervention, because these are potentially life- threatening conditions, e.g. : I. metabolic disturbances ■ hyponatremia ■ hypokalemia II. Cardiovascular feilure: ■ major arrhytmia ■ heart attack ■ pulmonary embolia ■ hypovolemic shock Based on above, these are non-neurologic patient and should be directed to the Emergency department immediately. (Remark: convulsive syncope may also be evoked by vasovagal mechanism.) brain hipoxia and decreased global cerebral blood flowconvulsive syncope All types of brain hipoxia and decreased global cerebral blood flow can induce convulsive attack, known as convulsive syncope. TÁMOP-4.1.1.C-13/1/KONV-2014-0001

11. Paroxysmal clinical event Paroxysmal clinical event = / ≠ ?? Epileptic seizure = / ≠ = / ≠ ??Epilepsy ?? Epileptic syndrome ? Antiepileptic medication / Antiepileptic medication / epilepsy surgery epilepsy surgery TÁMOP-4.1.1.C-13/1/KONV-2014-0001 DIFFERENTIAL DIAGNOSIS II.

12. I. Provoked epileptic seizure (known factors behind the seizure, e.g. drog abuse) II. Acute symptomatic seizure (symptom of a serious illness) 1, CNS related e.g. herpes encephalitis, acute hemorrhage, sinus thrombosis, subarachnoid bleeding 2, non-CNS related e.g. liver failure, hypoglycemia, hypoxia III. Unprovoked epileptic seizure, as the manifestation of epilepsy Spontaneous seizure, without any serious acute disease. ACCORDING TO THE ORIGIN OF THE EPILEPTIC SEIZURE: TÁMOP-4.1.1.C-13/1/KONV-2014-0001

13. SEMIOLOGICAL SEIZURE CLASSIFICATION Ictal symptomes can be produced by epileptic interference of one of the following four „spheres” (Lüders et al, 1998): 1, sensorial sphere ▬ sensorial signs ▬ aura 2, autonomic sphere ▬ primarily autonomic symptome ▬ autonome seizure 3, consciousness sphere ▬ alteration of consciousness ▬ dialeptic seizure 4, motor sphere ▬ motor phenomena ▬ motor seizure The semiological seizure classification identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. TÁMOP-4.1.1.C-13/1/KONV-2014-0001

14. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - comsist exclusively of subjective symptomes - occure at the beginning of seizure - duration: from seconds to minutes - auras are subdivided into the following subgroups: TÁMOP-4.1.1.C-13/1/KONV-2014-0001

15. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - Patients present objective vegetative signs (bradycardia, asystolia); as the leading signs - quite rare - almost every seizure is accompanied by some kind of autonomic symtom. TÁMOP-4.1.1.C-13/1/KONV-2014-0001

16. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - Patients present episodic alterations of consciousness, cognitive deficit; as the leading sign. - also called as „absence-like seizure” - these can be generalized or focal - incidence: absence-epilepsy, FLE (TLE) TÁMOP-4.1.1.C-13/1/KONV-2014-0001

17. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events Further classification based on the leading mortor symptoms : A, dominated by unnatural, simplex- homogenous motor events B, dominated by complex motor events (automatisms) tipical to epilepsy TÁMOP-4.1.1.C-13/1/KONV-2014-0001

18. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - characterized by irregular muscle contractions - quite often generalized - consciousness can be maintained - duration: from seconds to hours. TÁMOP-4.1.1.C-13/1/KONV-2014-0001 Video starts in five secs.

19. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - The tonic contraction of the muscles of the extremities, trunk or neck. - In FLE bilateral tonic asimmetric seizure TÁMOP-4.1.1.C-13/1/KONV-2014-0001

20. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - regular, repetitive series of short-duration muscle contractions - this can be focal (the sign of lateralization) or generalized TÁMOP-4.1.1.C-13/1/KONV-2014-0001

21. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - long-lasting, tonic, unnatural motor phenomenon - with version of eyes, head and sometimes the trunk - sign of lateralization TÁMOP-4.1.1.C-13/1/KONV-2014-0001

22. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events -automatisms: relatively complex motor events, may mimic appropriate expressions according to the given situation. - oral / manual – TLE - disturbances in consciousness „dreamy state” TÁMOP-4.1.1.C-13/1/KONV-2014-0001 Video starts in five secs.

23. Semiological seizure classification Epileptic seizure Auras Somatosensory aura Visual aura Auditory aura Gustatory aura Olfactory aura Autonomic aura Abdominal aura Psychic aura Autonomic seizures Dialeptic seizures Motor seizures Simple motor seizures Moyclonic seizures Tonic seizures Clonic seizures Tonic-clonic seizures Epileptic spasms Versive seizures Complex motor seizures Automotor/psychomotor seizures Hypermotor seizures Gelastic seizures Special seizures Atonic seizures Negative myoclonic seizures Hypomotor seizures Aphasic seizures Non-epileptic paroxysmal events - complex, rapid series of movements - their appearance are impulsive and vivid, bizarre - The muscles of the trunk and the proximal parts of the extremities are affected - duration: less than a minute - origin: frontal lobe (orbitofrontal region) TÁMOP-4.1.1.C-13/1/KONV-2014-0001 Video starts in five secs.

24. Skalp-EEG: 10 - 20 and 10 - 10 electrode system routine EEG / EEG with sleep devrivation / video-EEG monitoring EEG (electroencephalography) TÁMOP-4.1.1.C-13/1/KONV-2014-0001

25. Left temporal lobe epilepsy ~ ictal EEG Ictal EEG: seizure onset zone TÁMOP-4.1.1.C-13/1/KONV-2014-0001

26. ♦ spike ♦ spike ♦ sharp wave ♦ sharp wave ♦ spike-and-wave komplex ♦ spike-and-wave komplex ♦ poly-spike ♦ poly-spike ♦ polyspike-and-wave komplex ♦ polyspike-and-wave komplex ♦ slow-sharp-wave komplex ♦ slow-sharp-wave komplex EPILEPTIFORM DISCHARGES TÁMOP-4.1.1.C-13/1/KONV-2014-0001

27. Sharp wave, left temporal Interictal EEG: irritative zone TÁMOP-4.1.1.C-13/1/KONV-2014-0001

28. slow-sharp-wave komplex, generalized Interictal EEG: irritative zone TÁMOP-4.1.1.C-13/1/KONV-2014-0001

29. HS Post-stroke state Disgenesis Subep. heterot. cavernom MR: epileptogenic lesion Disgenetic tumor Malignant tumor Post-encephalitis TÁMOP-4.1.1.C-13/1/KONV-2014-0001

30. Paroxysmal clinical event Paroxysmal clinical event = / ≠ ?? Epileptic seizure = / ≠ = / ≠ ??Epilepsy ?? Epileptic syndrome ? Antiepileptic medication / Antiepileptic medication / epilepsy surgery epilepsy surgery Important features of a syndrome include: - the type of seizure(s) + EEG pattern - age at onset - precipitating factors / prognosis - physical and mental signs - epileptogenic lesion / genetic - response to treatment TÁMOP-4.1.1.C-13/1/KONV-2014-0001 CLASSIFICATION OF EPILEPTIC SYNDROMES

31. Samuel Auguste A. D. Tissot Traité de L’Epilepsie (1770) Idiopathic epilepsy: „a disease of its own kind” Symptomatic epilepsy: related to known etiology Dichotomy 1.: „type of genesis” ↔ idiopathic ↔ symptomatic Dichotomy 2.: „localisation of origin” ↔ focal / localisation related ↔ generalised Focal epilepsy: the seizures are originated from a well- circumscribed area. Generalized epilepsy: the seizures can be originated from any brain regions. (ILAE 1989) TÁMOP-4.1.1.C-13/1/KONV-2014-0001

32. Localisation related epilepsy Generalized epilepsy Idiopathic epilepsy Symptomatic epilepsy 1 month 10 year old Reading epilepsy Juvenile absence epilepsy Juvenile myoclonic epilepsy Epilepsy with grand mal seizures on awakening Genetic reflex epilepsies ESES Severe myoclonic epilepsy in infancy Doose syndrome Absence epilepsy in elderly Childhood absence epilepsy Lennox-Gastaut syndrome Epilepsy with myoclonic absence Benign childhood centro- temporal epilepsy Temporal lobe epilepsy Fontal lobe epilepsy Parietal lobe epilepsy Occipital lobe epilepsy Rasmussen encephalitis Benign childhood occipital epilepsies Landau- Kleffner s. 1 year old NS Abbreviations: NS: neonatal seizures ESES: Epilepsy with continouos SW during SWS EME: Early myoclonic encephalopathy EIEE: Early infantile epileptic encephalopathy BNFC: Benign neonatal familial convulsions BMEI: Benign myoclonic epilepsy in infancy BNFC BMEI EIEE EME West syndrome AD nocturnal FLE AD-TLE with auditory features AD focal epilepsy with variable foci Progressive myoclonic epilepsy TÁMOP-4.1.1.C-13/1/KONV-2014-0001

34. genetic-structural-metabolic Based on the underlying etiology, epilepsy can be divided into groups of genetic-, structural-metabolic- and unknown-origin. TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

35. CHILDHOOD ABSENCE EPILEPSY TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

36. SEIZURE SEMIOLOGY: 1, dialeptic (absence-like) seizure 2, primary generalized tonic-clonic seizure (GTCS) NEUROLOGICAL STATUS: normal MR: normal EEG: spike-and-wave koplexes (3/sec), generalized AGE AT ONSET : 4-8 y ETIOLOGY: genetic (T-Typ calcium-chanal abnormaliies) / epileptic disturbance of thalamo-cortical network ANTIEPILEPTIC DRUG: ethosuximid TÁMOP-4.1.1.C-13/1/KONV-2014-0001 CHILDHOOD ABSENCE EPILEPSY

37. BENIGN EPILEPSY WITH CENTROTEMPORAL SPIKES TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

38. SEIZURE SEMIOLOGY: somatosensory aura > motor / clonic seizure, one sided > sGTCS NEUROLOGICAL STATUS: normal MRI: negativ Interictal EEG: sharp wave, centro-temporal AGE AT ONSET: 4-6 y TÁMOP-4.1.1.C-13/1/KONV-2014-0001 BENIGN EPILEPSY WITH CENTROTEMPORAL SPIKES

39. CHILDHOOD OCCIPITAL LOBE EPILEPSY TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

40. CHILDHOOD OCCIPITAL LOBE EPILEPSY: PANAYIOTOPOULOS / GASTAUT SYNDROMES SEIZURE SEMIOLOGY: - autonome aura > autonome seizure > motor seizure > sGTCS - visuale aura (lateralizing sign) / motor seizure > sGTCS NEUROLOGICAL STATUS: normal MRI: negative EEG: ii: sharp wave, okcipital AGE AT ONSET: 3-6 y TÁMOP-4.1.1.C-13/1/KONV-2014-0001

41. JUVENILE MYOCLONIC EPILEPSY / JANZ SYNDROME TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

42. ANTIEPILEPTIKUM: Despite the fact that AE medication can result in long- term seizure-free status, discontinuation of the therapy, even after 5 years of seizure-free state, results in the reappearance of the seizures in 75-100% of the cases. SEIZURE SEMIOLOGY: 1, myoclonic seizure, generalized 2, primary GTCS NEUROLOGICAL STATUS: normal MRI: negativ AGE ET ONSET: 13-20 y PREVALENCE: 3-6% of epilepsies EEG: ii: P-SPW-K, generalizált Fotoparoxizmális reakció előfordul TÁMOP-4.1.1.C-13/1/KONV-2014-0001 JUVENILE MYOCLONIC EPILEPSY / JANZ SYNDROME Video starts in five secs.

43. Paroxysmal clinical event Paroxysmal clinical event = / ≠ ?? Epileptic seizure = / ≠ = / ≠ ??Epilepsy ?? Epileptic syndrome ? Antiepileptic medication / Antiepileptic medication / epilepy surgery epilepy surgery riteria of epilepsy surgery: Criteria of epilepsy surgery: - accurate diagnostic of epilepsy syndrome - pharmacoresistant epilepsy farmakorezisztencia - accurate localisation of epileptogenic zone and eloquent areas - chance of benefitting from surgery - motivation of patients Medical treatment according to epilepsy syndrome TÁMOP-4.1.1.C-13/1/KONV-2014-0001

44. This problem was also outlined by ILAE, by forming a separate category, called as „Distinctive contellations/surgical syndromes”. TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

45. TEMPORAL LOBE EPILEPSY (HS) TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

46. SEIZURE SEMIOLOGY : abdominal aura / mnestic aura > psychomotor seizure > sGTCS NEUROLOGICAL STATUS: normal with neurocognitive disturbances MRI: hippocampal sclerose Interictal EEG: SW temporal Ictal EEG: seizure onset: temporal AGE AT ONSET : 10-20 y TEMPORAL LOBE EPILEPSY (HS) TÁMOP-4.1.1.C-13/1/KONV-2014-0001 Video starts in five secs.

47. RASMUSSEN-SYNDROME TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

48. Case report: 31 year-old female  therapy-resistant hemispheric epilepsy  left-sided clonic signs, SE  brain MRI: only the right side is affected  biopsy: Rasmussen encephalitis?  tumor diagnostics are negative  no systemic autoimmune disease  liquor IgG index 0.65, OGP  fluctuating clinical signs: relapse?  provokated by fever  Plasma exchange: remission hippocampus Brainstem nuclei, Purkinje cells AMPAr neg NMDAr neg GABA B r neg VGKC neg LGI1 neg GAD neg sharp wave right zentral TÁMOP-4.1.1.C-13/1/KONV-2014-0001

49. This category contains heterogenic, but rather common disorders. Epileptic syndromes caused by structural-metabolic alterations: TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

50. FRONTAL LOBE EPILEPSY TÁMOP-4.1.1.C- 13/1/KONV- 2014-0001

51. FRONTAL LOBE EPILEPSY Case report: right-sided FLE 32 year-old female Onset of epilepsy: at 6 years of age Hypermotor-tonic seizure – GTCS Ictal EEG: T4>F8, F8>T4 Interictal EEG: SWs T4, T6, F8 Kortikale repraesentation des Schluckaktes – Klinische Neuropysiologie 2009 Hypermotor R TÁMOP-4.1.1.C-13/1/KONV-2014-0001 EEG-seizure onset Video starts in five secs.

52. Paroxysmal clinical event Paroxysmal clinical event Epileptic seizure Epilepsy Epilepsy Epileptic syndrome Epileptic syndrome Antiepileptic medication / epilepsy surgery epilepsy surgery 5-10% 0.5-1% ? 20-30% Prevalence EPIDEMIOLOGY TÁMOP-4.1.1.C-13/1/KONV-2014-0001

53. Thank you for your attention! TÁMOP-4.1.1.C-13/1/KONV-2014-0001

54. What are the principal symptoms of a psychomotor seizure? 1, tonic stretching, tonic elevation of arms 2, unilateral rhytmic cloni in the arms 3, staring eyes, freezing of movements (arrest), distal automatisms in the extremities, oral automatisms 4, eye deviation, amnesia on the seizure TÁMOP-4.1.1.C-13/1/KONV-2014-0001 QUIZ QUESTION 1

55. What are the principal symptoms of a hypermotor seizure? 1 tonic stretching, tonic elevation of arms 2, autonomic symptoms, dystonic posture of the hands, long-lasting postictal confusion 3, fast, automatic movements involving the trunk muscles, vocalizations 4, staring, amnesia on the seizure TÁMOP-4.1.1.C-13/1/KONV-2014-0001 QUIZ QUESTION 2

56. Which clinical symptoms are characteristics to the juvenile myoclonic epilepsy? 1, abnormalities on the neurological examination, psychomotor and clonic seizure, focal cortical dysplasia on the MRI, spike-and-slow wave pattern on the EEG 2, normal neurological examination, myoclonic seizures, focal cortical dysplasia on the MRI, non-lateralized frontal sharp waves on the EEG 3, normal neurological examination, myoclonic and generalized tonic-clonic seizures, normal MRI, no EEG abnormalities 4, none of above TÁMOP-4.1.1.C-13/1/KONV-2014-0001 QUIZ QUESTION 3

57. Which clinical symptoms are characteristics to the temporal lobe epilepsy? QUIZ QUESTION 4 1, abnormalities on the neurological examination, psychomotor and tonic seizures, focal cortical dysplasia on the MRI, temporal spike-and- wave pattern on the EEG 2, normal neurological examination, psychomotor seizure, hippocampal sclerosis on the MRI, temporal sharp waves on the EEG (they can be contralateral to the hippocampal sclerosis) 3, normal neurological examination, generalized myoclonic and secondarily generalized tonic-clonic seizures, normal MR, temporal sharp waves (unilateral) 4, none of above TÁMOP-4.1.1.C-13/1/KONV-2014-0001

58. SUMMARIZING (VIDEO PRESENTATION) TÁMOP-4.1.1.C-13/1/KONV-2014-0001

59. REFERENCES Panayiotopoulos CP. Principles of therapy in the epilepsies. In: A clinical guide to epileptic syndromes and their treatment. Springer Healthcare Ltd., 2010. Rosenow F, Lüders H. Presurgical evaluation of epilepsy. Brain 2001;124:1683-700. Rosenow F, Lüders HO (Eds). Presurgical assessment of the Epilepsies with Clinical Neurophysiology and Functional Imaging. Vol 3 (Handbook of Clinical Neurophysiology). Elsevier, 2004, pp:187-200. Lüders HO, Acharya J, Baumgartner C, Benbadis S, Bleasel A, Burgess R, Dinner DS, Ebner A, Foldvary N, Geller E, Hamer H, Holthausen H, Kotagal P, Morris H, Meencke HJ, Noachtar S, Rosenow F, Sakamoto A, Steinhoff BJ, Tuxhorn I, Wyllie E. Semiological seizure classification. Epilepsia 1998;39:1006-13. Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE, Engel J Jr, Forsgren L, French JA, Glynn M, Hesdorffer DC, Lee BI, Mathern GW, Moshe SL, Perucca E, Scheffer IE, Tomson T, Watanabe M, Wiebe S. A practical clinical definition of epilepsy. Epilepsia 2014;55(4):475-82.

60. Dr. Csilla Gyimesi M.D., Ph.D., med.habil. Prof. Dr. József Janszky, M.D., Ph.D., D.Sc. Peer reviewer: Prof. Dr. Sámuel Komoly, M.D., Ph.D., D.Sc. TÁMOP-4.1.1.C-13/1/KONV-2014-0001