Neurology 4th epilepsy(1)
- 1. NEUROLOGY Dr. Rami Abo Ali Neurology - Dr. Rami Abo Ali 1
- 3. TERMINOLOGY A seizure: is a paroxysmal event characterized by abnormal , excessive, hyper synchronous discharge of cortical neuron activity . Epilepsy: can be defined as a chronic seizure disorder or group of disorders characterized by seizures that usually recur unpredictably in the absence of a consistent provoking factor Convulsion : seizure with prominent alteration of motor activity Aura : a component of seizure which occurs before consciousness is lost and for which memory is retained afterwards; it localizes attack to the point of origin in the CNS 3 Neurology - Dr. Rami Abo Ali
- 4. TERMINOLOGY Tonic seizure: excessive motor outflow, giving rise to a tetanic state of the muscles involved. Atonic seizure: muscle tone drops to a very low values resulting in a sudden fall of the body Clonic seizure: a tonic seizure with periodic interruptions Tonic-clonic seizure: starts as a generalized tonic seizure and then interrupted during clonic phase and ending in complete relaxation. Myoclonic seizure: short involuntary contraction of one or more muscles (local or generalized) 4 Neurology - Dr. Rami Abo Ali
- 5. DEFINITION : Seizure is a paroxysmal, uncontrolled electrical discharge of neuron in the brain that interrupts normal function An epileptic seizure is electrophysiologically characterized by abnormal transient and excessive electrical discharge of cerebral neurons and clinically characterized by paroxysmal episodes of loss or excess of motor, sensory, autonomic or psychic functions with or without alteration in consciousness 5 Neurology - Dr. Rami Abo Ali
- 6. DIFFERENCE Seizure is a symptom and epilepsy is a syndrome. Though epilepsy begins first with a seizure, not all first seizures lead to epilepsy. Seizures may often occur in acute systemic conditions such as metabolic disturbances (hypoglycemia), drug toxicity (chloroquine) and drug withdrawal (diazepam) but usually they do not constitute epilepsy. 6 Neurology - Dr. Rami Abo Ali
- 7. HISTORICAL BACKGROUND Epilepsy derived from a Greek term: Epilambanei -to posses, to take hold of, to grab or to seize. Vedic period of 4500-1500BC :Ancient Indian Medicine refined and developed the basic concept of epilepsy Hippocrates 400 BC: ‘This is not a sacred disease rather disorder of brain’. He described some physical treatment of epilepsy and stated it is an incurable chronic illness. Ibn Sina 370 AH : describe epilepsy as a brain disease. 7 Neurology - Dr. Rami Abo Ali
- 8. CLASSIFICATION : Neurology - Dr. Rami Abo Ali 8 A. Simple partial seizures B. Complex Partial Seizures C. Partial Seizures evolving to secondary generalized seizures (tonic-clonic, tonic or clonic) A. Absence seizures B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-Clonic seizures F. Atonic seizures I. Partial (Focal)seizures II. Generalized seizures III. Unclassified epileptic seizures
- 9. CAUSES OF EPILEPSY In 30% cases cause can be determined. Rests (70%) are Idiopathic. Determined causes: Inherited genetic Acquired : trauma, Neurosurgery, Inflammatory, Metabolic , Infections, Tumor, Toxic disorders, drugs, etc. Congenital: inborn abnormality of metabolism. Withdrawal of drugs Alcohol ,Benzodiazepine , Barbiturates, Other Anti- Epileptics 9 Neurology - Dr. Rami Abo Ali
- 10. PATHOPHYSIOLOGY Nerve impulse propagates in the brain in a synchronous manner and for that the electrical potential reaches to zero. Any type of process which damages or cause irritation to the grey matter of the brain may cause activation or inactivation of neurons causes by unknown mechanism. This leads to sudden, excessive, synchronous discharge which results in an electrical potential. If the discharge remains localized it results in partial seizures or it may spread and involve the entire cerebrum causing generalized seizures. Imbalance of excitatory transmitters such as G-amino butyric acid (GABA) and selective central nervous system, calcium channel may be involved in the seizure disorders. 10 Neurology - Dr. Rami Abo Ali
- 11. PATHOPHYSIOLOGY The excessive neuronal disorderly discharge involving in the entire brain results in loss of consciousness, disturbances in sensation and conclusive movements. After peak of seizures there is decrease in frequency of neuronal discharge. It leads to the end of seizures . The seizure may be ending due to loss of cerebral energy reserves, local tissue anoxia, accumulation of toxic metabolites of neuronal metabolism and inhibitory neuronal feed back mechanisms. 11 Neurology - Dr. Rami Abo Ali
- 12. SIGNS AND SYMPTOMS "Blackouts" or periods of confused memory Episodes of staring or unexplained periods of unresponsiveness Involuntary movement of arms and legs "Fainting spells" with incontinence or followed by excessive fatigue Odd sounds, distorted perceptions, or episodic feelings of fear that cannot be explained. Strange sensations Visual hallucinations Emotional changes Muscle spasms Convulsions Other symptoms, depending on where in the brain the seizures begin. 12 Neurology - Dr. Rami Abo Ali
- 13. PARTIAL (FOCAL)SEIZURES CLINICAL MANIFESTATIONS Partial seizures or focal seizures are due to a small epileptic focus in the brain. They are divided into two main categories. a. simple partial seizure in which the seizure starts as a focal discharge and remains focal throughout without alteration of consciousness b. complex partial seizure when a seizure starts as a focal discharge, but consciousness is also altered or lost 13 Neurology - Dr. Rami Abo Ali
- 14. PARTIAL (FOCAL)SEIZURES (SIMPLE PARTIAL SEIZURES) Consciousness is fully preserved Motor disturbance may involve any body part Sensory disturbance , tingling , numbness, electrical shock like feelings Flashing light and colours, Simple hallucinations Changes in skin color, Blood pressure, Heart rate, Pupil size, Piloerection. Psychic manifestation: Dysphasic- when cortical speech area affected Dysmnestic (disturbance of memory) Cognitive symptoms- dreamy state, Affective symptoms- fear, depression, anger, irritability, elation(euphoria), erotic thoughts, Illusion of size, structured hallucination . 14 Neurology - Dr. Rami Abo Ali
- 16. PARTIAL (FOCAL)SEIZURES (COMPLEX PARTIAL SEIZURES) Also called (Psychomotor Seizures/Temporal lobe Epilepsy) Always involved impairment of consciousness. Majority originate in Temporal lobe (60%); but also originate another lobe – particularly Frontal(30%). May start as simple partial seizures then progress. Aura may be present-short live (few seconds) Automatism: Oro-Alimentary, Gestural, Ambulatory, Verbal , wild flailing movements, Responsive and Violent. Duration: < 3 minutes. 16 Neurology - Dr. Rami Abo Ali
- 17. 17 Neurology - Dr. Rami Abo Ali complex partial seizure repetitive movements, or "automatisms ,“ with perspiration autonomic aura
- 19. GENERALIZED SEIZURE These are seizures in which there is no evidence of an epileptic focus, either clinically or on EEG, as opposed to secondary generalized seizures. The epileptic discharge involves both cerebral hemispheres simultaneously from the onset of the seizures. Hence, consciousness is almost always impaired or lost at the onset of the attack 19 Neurology - Dr. Rami Abo Ali
- 20. GENERALIZED SEIZURE GENERALIZED TONIC-CLONIC (GRAND MAL) Convulsive seizures No Aura but have prodormal phase- general malaise Tonic phase- The tonic phase consists of rolling up of the eyes associated with stiffening of the limbs followed by clenching of the jaws, often resulting in injury to the tongue. Lasts 10 to 30 seconds Clonic phase: intermittent clonic movements of muscles , strenuous breathing, sweating, frothing of the mouth and excess salivation lasts for 1-2 minutes This is followed by a deep comatose state, which lasts for about 5 minutes Post ictal period: drowsiness, confusion, headache, deep sleep 20 Neurology - Dr. Rami Abo Ali
- 21. GENERALIZED TONIC-CLONIC (GRAND MAL) 21 Neurology - Dr. Rami Abo Ali
- 22. GENERALIZED SEIZURES TYPICAL ABSENCE SEIZURES (PETIT MAL) Occur almost exclusively in childhood or early adolescent. Sudden loss of consciousness and cease all motor only for a brief period often < 10 seconds. Suddenly appears blank and stares, fluttering of the eyelids, swallowing, flopping of the head. Attacks last only a few seconds (<10 sec) and often pass un-recognized. About 100-200 attacks may occur/day. Characteristic EEG : 3 per sec generalized spike and wave Attacks precipitate by fatigue, drowsiness, relaxation , photic stimulation or hyperventilation. 22 Neurology - Dr. Rami Abo Ali
- 23. TYPICAL ABSENCE SEIZURES (PETIT MAL) 23 Neurology - Dr. Rami Abo Ali
- 24. GENERALIZED SEIZURES MYOCLONIC SEIZURES Abrupt , very brief, involuntary flexion movements. Involve whole body or part of the body Occur most commonly at morning, shortly after walking. May occur in healthy people (physiological) No loss of consciousness is usually detectable 24 Neurology - Dr. Rami Abo Ali
- 26. GENERALIZED SEIZURES TONIC SEIZURES Tonic seizures consist of a sudden increase in muscle tone in the axial or extremity muscles, or both, producing a number of characteristic postures. Consciousness is usually partially or completely lost Prominent autonomic phenomena occur. Postictal alteration of consciousness is usually brief, but it may last several minutes. Tonic seizures are relatively rare and usually begin between 1 and 7 years of age 26 Neurology - Dr. Rami Abo Ali
- 28. GENERALIZED SEIZURES ATONIC SEIZURES Atonic seizures consist of sudden loss of muscle tone. The loss of muscle tone may be confined to a group of muscles, such as the neck, resulting in a head drop. Alternatively, atonic seizures may involve all trunk muscles, leading to a fall to the ground . 28 Neurology - Dr. Rami Abo Ali
- 29. GENERALIZED SEIZURES CLONIC SEIZURES Clonic seizures occur almost exclusively in early childhood. The attack begins with loss or impairment of consciousness associated with sudden hypotonia or a brief, generalized tonic spasm. This is followed by 1 minute to several minutes of bilateral jerks, which are often asymmetric and may appear predominately in one limb During the attack, the amplitude, frequency, and spatial distribution of these jerks may vary greatly from moment to moment. In other children, particularly those aged 1 to 3 years, the jerks remain bilateral and synchronous throughout the attack. Postictally , recovery may be rapid, or a prolonged period of confusion or coma may ensue. 29 Neurology - Dr. Rami Abo Ali
- 31. CHILDHOOD ABSENCE EPILEPSY (CAE) JUVENILE MYOCLONIC EPILEPSY (JME) INFANTILE SPASMS/WEST SYNDROME LENNOX-GASTAUT SYNDROME (LGS) 31 Neurology - Dr. Rami Abo Ali EPILEPSY SYNDROMES
- 32. CHILDHOOD ABSENCE EPILEPSY (CAE) GENERALIZED EPILEPSY Onset between 4-10 years (peak onset 5-7 years) Frequent typical absence seizures: short staring spells (less than 20 seconds), occasionally with other features: automatisms of hands or mouth, eye fluttering Neurological development is normal More prevalent in girls (60-70% affected patients are girls) Onset of seizures often accompanies a decline in school performance Seizures brought out by hyperventilation 32 Neurology - Dr. Rami Abo Ali
- 33. CHILDHOOD ABSENCE EPILEPSY (CAE) TREATMENT OF CAE Ethosuximide (only useful in this disorder) Lamotrigine Valproic acid These are considered equivalent Can try in refractory cases: clobazam, levetiracetam, topiramate, zonisamide PROGNOSIS OF CAE Generally good. Seizures remit in up to 95% of cases Increased risk of other epilepsies Children can have learning/cognitive difficulties even after seizure remission occurs 33 Neurology - Dr. Rami Abo Ali
- 34. JUVENILE MYOCLONIC EPILEPSY (JME) GENERALIZED EPILEPSY 3 seizure types: myoclonic jerks (cardinal symptom) absences convulsions Myoclonic jerks are more typical in the morning Onset between 8-24 years (peak onset 12-18) Patients are very sensitive to sleep deprivation and alcohol consumption 34 Neurology - Dr. Rami Abo Ali
- 35. JUVENILE MYOCLONIC EPILEPSY (JME) TREATMENT OF JME First line: Valprioc acid Lamotrigine (may make myoclonus worse) Other agents: Levetiracetam is useful of convulsions and myoclonus Topirmate and zonisamide are useful for convulsions clobazam is good for everything PROGNOSIS OF JME Usually people are on medication lifelong Occasionally seizures do remit, but most people elect to stay on medications If seizures are poorly controlled, it can cause cognitive impairments, but if well controlled, many people can live normal lives Advise against sleep deprivation/alcohol 35 Neurology - Dr. Rami Abo Ali
- 36. INFANTILE SPASMS/WEST SYNDROME Has its own classification Epileptic encephalopathy Clinical triad of clinical spasms (myoclonic tonic) hypsarrhythmia on EEG developmental regression Can be idiopathic or caused by structural /metabolic defect : Tuberous sclerosis (TS), perinatal stroke, Sturge Weber, cortical migration abnormalities Typical age of onset 3-18 months (peak incidence 6-9 months) 36 Neurology - Dr. Rami Abo Ali
- 37. INFANTILE SPASMS/WEST SYNDROME TREATMENT/PROGNOSIS Adrenocorticotropic hormone (ACTH), Vigabitrin (first line in TS), topirmate (if there are focal seizures as well), clobazam Most children have long term neurodevelopmental problems, which are worse the longer it takes to initiate treatment Some evolve to a Lennox-Gastaut picture as they get older 37 Neurology - Dr. Rami Abo Ali
- 39. LENNOX-GASTAUT SYNDROME (LGS) MIXED EPILEPSY Often severe epileptic encephalopathy Multiple seizure types: tonic, myoclonic, atonic, atypical absence, focal seizures that can generalize EEG hallmark: “slow” generalized spike-wave 2 hertz-usually at onset of disease, EEG can evolve over time, can also have other focality Many children with LGS have evolved to that from infantile spasms 39 Neurology - Dr. Rami Abo Ali
- 40. LENNOX-GASTAUT SYNDROME (LGS) TREATMENT/PROGNOSIS Can be medication resistant Broad spectrum agents preferred: lamotrigine, valproic acid, topiramate, clobazam, runfinamide Long term neuro-developmental problems /encephalopathy 40 Neurology - Dr. Rami Abo Ali
- 42. EFFECTS OF PREGNANCY ON EPILEPSY The effects of pregnancy on epilepsy is uncertain. All anticonvulsants interfere with folic acid metabolism. Folic acid deficiency has been associated with neural tube defects and other congenital malformations. Pre-conception treatment with folic acid (5 mg daily), along with use of the smallest effective doses of as few AEDs as possible, may reduce the risk of fetal abnormalities. 42 Neurology - Dr. Rami Abo Ali
- 43. EFFECTS OF EPILEPSY ON PREGNANCY Relatively resistant to short episodes of hypoxia and there is no evidence of adverse effects of single seizures on the fetus. No increased risk of miscarriage or obstetric complications in women with epilepsy unless a seizure results in abdominal trauma. Incidence of fetal malformations, intrauterine growth restriction (IUGR), oligohydramnios, preeclampsia and stillbirths is increased. Birth defects are increased by two fold. This could be related to the severity of the disease with its genetic predilection and also due to the anticonvulsants. The malformations include- cleft lip and/ or palate, mental retardation, cardiac abnormalities. Limb defects and hypoplasia of the terminal phalanges. 43 Neurology - Dr. Rami Abo Ali
- 44. MANAGEMENT Pre- pregnancy counselling: Control of epilepsy should be maximized prior to pregnancy with the lowest dose of the most effective treatment that gives best seizure control. Review of antiepileptic drugs (AED) should taken into account the risk of teratogenesis and other adverse neurodevelopment effects. If a decision is taken to stop treatment, AEDs should be withdrawn slowly in order to reduce the risk of withdrawal associated seizures. This is particularly important for benzodiazepines and phenobarbitone. 44 Neurology - Dr. Rami Abo Ali
- 45. MANAGEMENT The current recommendations are to stop driving from the commencement of the period of drug withdrawal and for a period of six months after cessation of treatment, even if there is no recurrence of seizures. All women receiving AEDs should be advised to take pre-conception folic acid (5mg/day) 45 Neurology - Dr. Rami Abo Ali
- 46. ANTENATAL MANAGEMENT The dose of the chosen drug should be kept as low as possible and to be monitored regularly from the serum level. The commonly used drugs are: Phenobarbitone 60-100mg daily in two to three divided doses. Phenytoin 150-300mg daily in two divided doses. Carbamazepine 0.8-1.2g daily in divided doses. Folic acid daily prior to conception, continues throughout pregnancy, as there is also a small risk of folate-deficiency anemia Relatives, friends and/or partners should be advised on how to place the women in the recovery position to prevent aspiration in the event of a seizure. Vitamin K 10 mg daily must be given orally in the last 2 weeks. 46 Neurology - Dr. Rami Abo Ali
- 47. INTRAPARTUM MANAGEMENT The risk of seizures increases around the time of delivery . Women with major convulsive seizures should deliver in hospital. Anticonvulsant medication continue throughout the labor regular review by the obstetric team is indicated. If seizures recur, short-acting benzodiazepines are administered. The women should not be left alone in labor, and dehydration ,hyperventilation and exhaustion should be avoided as they can trigger a seizure. The birth can be spontaneous facilitated by the midwife. Following obtaining informed consent from the women , vitamin K should be administered to the baby promptly after birth to protect against AED induced hemorrhage disease. Caesarean section is only required if there are recurrent generalized seizure in late pregnancy or labor . 47 Neurology - Dr. Rami Abo Ali
- 48. POSTNATAL MANAGEMENT In the first 24 hours of birth the women has an increased risk of a seizure and so should remain in hospital. Breast feeding is encouraged. The baby should be carefully observed and any concern reported to the pediatrician immediately . Advice should be given about safety when caring for the baby in case of maternal seizure. 48 Neurology - Dr. Rami Abo Ali