1. Epilepsy and convulsion syndromes

2. Epilepsy is a chronic disorder, which is characterized by the presence of:  Epileptic focus  Recurrent attacks with various clinical signs  Personality disorders between attacks  Some specific paraclinical signs

3. Epilepsy as a disease should be differentiated from:  Epileptic reaction  Epileptic syndrome

4. Epileptic reaction – is the response of the brain to the strong external and internal damaging factors (such as brain hypoxia, severe alcohol intoxication, electro – shock, insulin shock and so on). The main clinical features of epileptic reaction are abortive seizures or general tonic – clonic seizures.

5. Epileptic syndrome – is characterized by recurrent epileptic attacks on the background of pathologic focus in brain. The attacks are variable and depend on the localization of focus. Focal symptoms are obligatory in this case.

6. Risk factors  Inheritance  Organic brain diseases  Prenatal (infections – cytomegalovirus, rubella, toxoplasmosis, toxicosis of pregnancy, diet disturbances)  Perinatal (physical trauma, child birth anoxia, metabolic disorders, neonatal infection)  Postnatal (infections, trauma, dehydration, toxins)  Disorders of brain function. Sleep disorders.  Paroxysmal states in childhood  Newborns seizures  Febrile seizures  Affective – respiratory seizures

7. Pathology  The results of organic diseases  The results of epileptic process  Each epileptic attack causes hypoxic changes in brain and leads to the development of encephalopathy

8. Pathologic physiology  There is a group of neurons with pathologic activity, which is called epileptic focus  There is the ability to enforce and spread the activity  Weakness of anti - epileptic protection. (It is provided by caudal parts of the brain)

9. Pathophysiology  Presence of neurons’ group with pathological activity (epifocus)  Ability to spread activity  Weakness of system of antiepileptic defense

10. Neurochemistry of epilepsy  Disorders of balance between glutamate (exciting neurotransmitter), GABA (inhibitory neurotransmitter) that leads to desynchronization.  Disorders of K / Na pump

11. Neurochemistry of epilepsy  Disorders of balance between glutamate and GABA.  Disorders of K / Na pump

12. Immunology of epilepsy  There is increasing content of anti– brain antibodies. The primary attack causes disturbances of HEB.  The result is production of antibodies and CIC that are fixed in brain tissue and encourage its lesion in new zones.

14. Classification of epileptic attacks  Partial epileptic  Simple  Simple motor  Focal motor without march  Focal motor with march  Adversive  Postural  Phonatory simple  Simple sensory  Somatosensory (with and without march)  Visual, acoustical, gustatory, smell)  Simple autonomic – visceral  Simple with psychiatric disorders  Aphatic  Dysmnestic  With thinking disturbances (ideatory)  Emotional – affective  Hallucinatory

15. Classification of epileptic attacks  Complex  Temporal pseudoabsance  Automatisms  Simple with generalization (secondary general)  General attacks  Absance  Typical  Atypical  Myoclonic  Tonic – clonic  Tonic  Clonic  General atonic  Non classified  Epileptic status

18. Clinical features A. General seizures.  Epileptic general tonic – clonic attack (grandmal) usually begins with short initial stage that lasts several seconds. The last can manifest as:  Bilateral general muscle jerks  Loss of consciousness  Autonomic changes  Enlargement of pupils

19. Types of epileptic tonic attacks  Axial – body and facial muscles are involved in attack. There is spasm of respiratory muscles and breathing stop at expiration.  The same signs plus less involvement of extremities muscles.  Global means involvement of body and extremities muscles in the same way.

20. Clonic epileptic attacks  general typical clonic attacks are often observed in newborn babies.  loss of consciousness  autonomic disorders  rhythmic clonic seizures  between the attacks of clonic muscles jerking movements there is muscle hypotonia  If the attacks last 1–2 min the consciousness recovers quickly. But these attacks can last 4–5 min and even more. Then after the attack coma can develop.

21. petit mal seizures  Most petit mal seizures last only a few seconds. Most commonly they involve staring episodes or "absence spells." The episodes may:  Occur many times a day  Occur for weeks to months before being noticed  Interfere with school and learning  Be mistaken for lack of attention or other misbehavior

22.  Unexplained difficulties in school and learning difficulties may be the first sign of petit mal seizures.  During the seizure, the person may:  Stop walking and start again a few seconds later  Stop talking in mid-sentence and start again a few seconds later

23.  The person usually does not fall during the seizure.  Immediately after the seizure, the person is usually:  Wide awake  Thinking clearly  Unaware of the seizure

24. Specific symptoms of typical petit mal seizures may include:  Changes in muscle activity, such as:  No movement  Hand fumbling Hand fumbling Hand fumbling  Fluttering eyelids  Lip smacking  Chewing

25. Changes in alertness (consciousness)Changes in alertness (consciousness), such as: Changes in alertness (consciousness)  Staring episodes  Lack of awareness of surroundings  Sudden halt in movement, talking, and other awake activities  May be triggered by hyperventilation or flashing lights, in some cases hyperventilation

26. Without seizure attacks Complex absance can be divided into myoclonic, atonic, tonic and with automatisms.  Myoclonic absance - loss of consciousness, rhythmic bilateral myoclonus in muscles of face and upper extremities. There are jerking movements in eyelids, periorbital muscles, mouth edges, eye bulbs. The patient can loose some objects which he is holding in his arms  Atonic absance - decreasing of postural tonus, hanging head and sudden drops  Tonic absance is associated with looking of eyes upwards. There is domination of either extensor or flexor component, symmetric or asymmetric

27. Without seizure attacks  Absance with automatism can be the sign of focal attack and absance. The main condition for automatism is incomplete loss of consciousness.  Typical absance are associated with bilateral symmetric complexes “top – waves” with frequency 3 per sec in frontal–central lobe.  Atypical absance begin slower and last longer. Symptoms are similar but muscle activity changes may be more noticeable.

28. Focal attacks Groups of focal attacks:  Simple focal  Complex focal  Focal attacks with secondary generalization.

29.  Simple motor  Focal motor without march  Focal motor with march  Adversive  Postural  Phonatory simple  Simple sensory  Somatosensory (with and without march)  Visual, acoustical, gustatory, smell)  Simple autonomic – visceral  Simple with psychiatric disorders  Aphatic  Dysmnestic  With thinking disturbances (ideatory)  Emotional – affective  Hallucinatory

30.  Complex  Temporal pseudoabsance  Automatisms

31.  Simple -- not affecting awareness or memory  Complex -- affecting awareness or memory of events before, during, and immediately after the seizure, and affecting behavior

32. Symptoms:  Motor  Abnormal muscle contraction muscle contractionmuscle contraction  Muscle contraction/relaxation (clonic activity) -- common  Affects one side of the body (leg, part of the face, or other area)  Abnormal head movements  Forced turning of the head

33.  Staring spells, with or without complex, repetitive movements (such as picking at clothes) -- these are called automatisms and include:  Abnormal mouth movements  Lip smacking  Behaviors that seem to be a habit  Chewing/swallowing without cause

34.  Sensory (somatosensory, visual, acoustical, olphactory, taste attacks and epileptic attacks of dizziness)  Autonomic Abdominal pain or discomfort NauseaSweating Flushed face Flushed face Dilated pupils Rapid heart rate/pulse Rapid heart rate/pulse

35.  Psychiatric that depends on focus localization  Sensation of deja vu  Changes in mood or emotion Changes in mood Changes in mood

36.  Epileptic discharges in motor speech center speech disorders or involuntary vocalization – involuntary repetition of words are observed.  As for visual, acoustical, olphactory, taste attacks and epileptic attacks of dizziness they can manifest as simple disorders or complex illusion or hallucinations.

37. Intensive Video-EEG-Monitoring

39. Severe Pediatric Epilepsy Disorder

40. Photostimulation of attack

41. Treatment The main principals :  emergency  accordance to stages  following On the way to hospital:  To release breathing air ways  Digitalis drugs  Sibazonum 0.01g

42. Treatment In ambulance:  Tracheobronchial tree drain  Sibazonum 30 ml in 150 ml of physiological solution, in 10 min we add the medication up to 100 – 120 mg  Magnesiii sulfas 25% 10.0 in glucose 40 %  Anesthesia with nitrous oxide  Dosed anesthesia  Aminazinum 25% 1-2 ml  Atropinum 0.1% 1.0 s/c  Cardiac, antihistamine, diuretics  Natrii tiopentali 1g in 10 ml of physiological solution

43. Treatment Epileptic status  To provide permeability of respiratory airways  To evaluate the function of heart – vascular and respiratory systems  To provide free way to veins  Lorazepam 4mg i/v or Diazepam 10 mg In the hospital  To take blood for analysis  % of urea, electrolytes  liver function  % of glucose  % of blood gas  etiology of attack – hypoglycemia – 50% solution of glucose 50 mg  at alcohol abuse – Tiaminum

44. Treatment Next half an hour:  to introduce the medication through the naso–gastral tube  Fentoin 18 mg per kg or Phenobarbitalum 15 mg per kg i/v by drop 100 mg per kg In 30 minutes:  General anesthesia  EEG

45. Surgical methods of treatment:  Resections  Anterior temporal lobectomy  Selective amygdalohypocampoectomia  Calosotomia  Hemisphereectomia  Stereotaxic  Destruction of deep temporal structures. This procedure on lateral part normally decreases seizures, on medial ones – aggression.

46.  Radio – surgical with γ – knife. γ – waves from 201 sources are focused on certain distance. The effectiveness of this procedure is 70 – 80 %.  Electrostimulative – stimulation of certain structures:  Nucleus dentatus, the caput of nucleus caudatus  Stimulation of n. vagus – this method is one of the newest one. It is indicated at partial seizures with secondary generalization.

50. EEG–fMRI and ESI monitoring of epileptic activity

51. Peri-rolandic epilepsy – surgical technique

53. Treatment of patients with epileptic status

54. Measures Time, min Treatment 0-5 Definition of state of living functions. Oxigenotherapy. 6-10 Intravenous infusion of physiological solution. Checking of tempeature, BP, biochemical blood analysis. Intravenous injection of Tiaminum 100 mg, then 50 ml 40 % glucose.

55. Time, min Treatment 11-15 Intravenous injection of Diazepam 0,2 mg per kg (speed 5 mg per min). Injection is repeated every 5 min. General dose is not more then 20 mg 16-45 Intravenous injection by drops of Fenitoin in dose 20 mg per kg (speed 50 mg per min). While injection ECG is made, BP is checked. At changes on ECG or BP injection is stopped. If attacks continue we can add 5 – 10 mg per kg of Fenitoin.

56. Time, min Treatment 46-59 If attacks continue we make endotracheal intubation, then use Fenobarbitalum in dose 20 mg per kg (speed 100 mg per min) 60-90 If attacks continue we introduce patient in barbiturate coma. It is recommended to start usage of Fenobarbital in dose 5 mg per kg up to stopping of epileptic activity on EEG.Then continue usage of 0,5-3,0 mg per kg per hour to stop recurring attacks. From time to time we decrease speed to prove ourselves that the attacks are absent. While infusion we check EEG, ECG, BP and breathing functions.