CHILDHOOD SEIZURES epilepsy and status epileptics

Childhood seizures

1. CHILDHOOD SEIZURES Moderator:Dr.Shreedhar.A Presenter:Dr.Guruswamy.N.T Date:26-02-2014

2. Seizures are the most common pediatric neurologic disorder 4% to 10% of children suffer at least one seizure in the first 16 years of life Children < 3 years of age have the highest incidence of seizures. Incidence decreases with increasing age Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

3. Epidemiologic studies reveal that approximately 1,50,000 children will sustain a first-time, unprovoked seizure each year, and of those, 30,000 will develop epilepsy Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

4. Definition: Seizure: transient occurrence of signs and/or symptoms resulting from abnormal excessive or synchronous neuronal activity in the brain Nelson Textbook of Pediatrics 19 th Edn

5. Definition: Epilepsy: disorder of the brain characterized by an enduring predisposition to generate seizures and by the neurobiologic,cognitive,psychological, and social consequences of this condition Nelson Textbook of Pediatrics 19 th Edn

6. Seizure disorder: general term , usually used to include any one of several disorders including epilepsy, febrile seizures, and possibly single seizures and seizures secondary to metabolic, infectious, or other etiologies (e.g., hypocalcemia, meningitis) Nelson Textbook of Pediatrics 19 th Edn

7. Status epilepticus: continuous or recurrent seizure activity lasting > 30 minutes without recovery of consciousness Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

8. Tonic seizures : are characterized by increased tone or rigidity (sustained contraction) Atonic seizures: are characterized by flaccidity or lack of movement during a convulsion. Clonic seizures: consist of rhythmic muscle contraction and relaxation

9. Myoclonus : shocklike contraction of a muscle, usually <50 msec in duration, may be isolated or may repeat but usually are not rhythmic Spasms (axial spasms): consist of flexion or extension of truncal and extremity musculature that is sustained for 1-2 sec, shorter than what is seen in tonic seizures, which last >2 sec. Postictal period: period of confusion, irritability, or fatigue following the seizure

10. CLASSIFICATION OF EPILEPTIC SEIZURES Focal Seizures Generalized Seizures

11. TYPES OF EPILEPTIC SEIZURES Nelson Textbook of Pediatrics, 19 th Edn

12. TYPES OF EPILEPTIC SEIZURES Nelson Textbook of Pediatrics, 19 th Edn

13. Partial Seizures account for approximately 40% of seizures in children Simple partial seizures : consciousness is not impaired Complex partial seizures: consciousness is affected

14. Focal (partial) seizures: Arise from a localized region of the brain Have clinical manifestations that reflect that area of brain. Both simple and complex partial seizures may progress to secondarily generalized seizures in up to 30% of children

15. Partial Seizures SIMPLE PARTIAL SEIZURES: Characterized by the development of localized motor or sensory symptoms without impairment of consciousness. These can take the form of sensory seizures (auras) or brief motor seizures Brief motor seizures are the most common and include -focal tonic, clonic or atonic seizures

16. Partial Seizures SIMPLE PARTIAL SEIZURES: Jacksonian seizure (Jacksonian march): type of frontal lobe seizure It only occurs on one side of the body It progresses in a predictable pattern from twitching or a tingling sensation or weakness in a finger, a big toe or the corner of the mouth, then  marches  over a few seconds to the entire hand, foot or facial muscles

17. Partial Seizures SIMPLE PARTIAL SEIZURES: Todd’s paralysis or Todd’s paresis postictal paralysis that can last minutes or hours,and sometimes longer

18. Partial Seizures COMPLEX PARTIAL SEIZURES: usually last 1-2 min Complex partial seizures are characterized by changes in perception and sensation, with associated alterations in consciousness Seizures tend to affect Eyes -a dazed look mouth -lip smacking and drooling abdomen -nausea and vomiting

19. Partial Seizures COMPLEX PARTIAL SEIZURES: Complex partial seizures might arise from any lobe  of the brain. most commonly arise from the temporal lobe A common associated brain abnormality is  mesial temporal sclerosis After the seizure, the patient can have postictal automatisms, sleepiness, and/or other transient focal deficits such as weakness or aphasia.

20. EPILEPSY SYNDROMES WITH PARTIAL SEIZURES Benign childhood epilepsy with centrotemporal spikes (BECTS) (Benign rolandic epilepsy): Inherited as an autosomal dominant trait Children between 3 and 13 years of age experience nighttime seizures during sleep initial phase of the seizure involves clonic activity of the face, including grimacing and vocalizations, which often wake the child from sleep

21. EPILEPSY SYNDROMES WITH PARTIAL SEIZURES BECTS: EEG : characteristic perisylvian spiking pattern can be seen MRI :Normal No therapy is needed because patients usually will outgrow these episodes by early adulthood Carbamazepine -frequent rolandic seizures

22. Lennox-Gastaut syndrome: onset between 3 and 5 years of age characterized by intractable mixed seizures with a combination of tonic, myoclonic, atonic, and absence seizures. Most of the children also have accompanying mental retardation and severe behavioral problems. EEG : irregular, slow, high-voltage spike pattern management is very difficult. Valproic acid is the drug used most commonly

23. Generalized seizures: Associated with the involvement of both cerebral hemispheres They may be Convulsive-with prominent motor activity or nonconvulsive Motor involvement, when present, is usually bilateral may also involve an altered level of consciousness

24. Generalized tonic-clonic seizures most common type of childhood seizure. Most tonic-clonic seizures have a sudden onset During the initial tonic phase, the child becomes pale, with dilation of the pupils, deviation of the eyes, and sustained contraction of muscles with progressive rigidity.

25. Generalized tonic-clonic seizures… Bladder or bowel incontinence is common. Clonic movements, involving rhythmic jerking and flexor spasms of the extremities, then occur. Mental status is usually impaired during the seizure and for a variable time after the seizure has ceased

26. ABSENCE SEIZURES Typical (simple)absence seizures: usually start at 5-8 yr of age characterized by a sudden cessation of motor activity, a brief loss of awareness, and an accompanying blank stare. Flickering of the eyelids may be seen. Episodes last less than 30 seconds not associated with a postictal period

27. ABSENCE SEIZURES… Complex (atypical) absence seizures : are usually associated with myoclonic activity in the face or extremities and an altered level of consciousness more difficult to treat usually accompanied by 1-2 Hz spike–and–slow wave discharges

28. Juvenile myoclonic epilepsy (Janz syndrome) Inherited as an autosomal dominant trait manifests in early adolescence (onset 12–18 years of age) Patients experience myoclonic jerks typically on awakening but may also have tonic-clonic (80%) or absence (20%) seizures.

29. Juvenile myoclonic epilepsy (Janz syndrome)… provoking factors include stress, alcohol, hormonal changes, or lack of sleep. EEG :pattern of fast spike-and-wave discharges Valproic acid is the drug of choice

30. Infantile spasms (West’s syndrome) present typically between 4 and 18 mo of age males affected more commonly than females Up to 95% of affected children are mentally retarded 20% mortality rate. Patients experience sudden jerking contractions of the extremities, head, and trunk.

31. Infantile spasms (West’s syndrome)… Jerking is spasmodic and often occurs in clusters. Episodes rarely occur during sleep. Up to 25% of patients have tuberous sclerosis. EEG :classic pattern of hypsarrhythmia (random high-voltage slow waves with multifocal spikes) Treatment with ACTH and prednisone has been used with some success

32. Pediatric conditions often mistaken for seizures Disorders with altered consciousness Apnea and syncope Breath-holding spells Cardiac dysrhythmias Migraine Paroxysmal movement disorders Acute dystonia Benign myoclonus Pseudoseizures Tics Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

33. Pediatric conditions often mistaken for seizures… Sleep disorders Narcolepsy Night terrors Sleepwalking Psychologic disorders Attention deficit hyperactivity disorder Hyperventilation Hysteria Panic attacks Gastroesophageal reflux (Sandifer’s syndrome) Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

34. Causes of seizures Infectious Neurologic or developmental Metabolic Traumatic or vascular Toxicologic Idiopathic or epilepsy Oncologic Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

35. Causes of seizures Infectious Brain abscess Encephalitis Febrile seizure Meningitis Neurocysticercosis Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

36. Causes of seizures… Neurologic or developmental Birth injury Congenital anomalies Degenerative cerebral disease Hypoxic-ischemic encephalopathy Neurocutaneous syndromes Ventriculoperitoneal shunt malfunction Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

37. Causes of seizures… Metabolic Hypercarbia Hypocalcemia Hypoglycemia Hypomagnesemia Hypoxia Inborn errors of metabolism Pyridoxine deficiency Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

38. Causes of seizures… Traumatic or vascular Cerebral contusion Cerebrovascular accident Child abuse Head trauma Intracranial hemorrhage Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

39. Causes of seizures… Toxicologic antihistamines, anticholinergics Isoniazid Lead, lithium, lindane Salicylates Theophylline Withdrawals – anticonvulsants Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

40. History and physical examination Detailed history Should focus on events immediately before the onset of the episode thorough description of the actual seizure

41. History and physical examination… Information to elicit includes Duration Movements Eye findings Cyanosis loss of consciousness presence of an aura Incontinence length of the postictal period any post-seizure focal neurologic abnormalities

42. History and physical examination… potential precipitating factors Trauma Ingestion Recent immunizations Fever or other systemic signs of illness

43. History and physical examination… k/c/o seizure disorder : Recent seizure was different from previous seizures typical seizure frequency for the patient medications the patient is taking Compliance with the medication regimen or any recent medication changes

44. History and physical examination… Additional history other significant medical problems neurologic disease presence of a VP shunt Developmental delay family history of seizures.

45. History and physical examination… Thorough physical and neurologic examination Vital signs Temperature Heart rate BP Head should be examined for Microcephaly dysmorphic features signs of trauma presence of a VP shunt In infants, a measurement of the head circumference bulging fontanelle -increased intracranial pressure

46. History and physical examination… Eyes papilledema retinal hemorrhages signs of meningeal irritation Hepatosplenomegaly metabolic or glycogen storage disease

47. History and physical examination… skin cafe´ au lait spots (neurofibromatosis) adenoma sebaceum or ash leaf spots (tuberous sclerosis) port wine stains (Sturge-Weber syndrome). Unexplained bruising suspicion of a bleeding disorder or child abuse

48. Diagnostic approach Laboratory testing: Afebrile seizure -should be guided by the history and physical examination. Rapid bedside glucose test drug level in patients taking anticonvulsant medications serum electrolytes, calcium, magnesium, ammonia, Total count, and toxicology screens -based on clinical suspicion

49. Diagnostic approach… Lumbar puncture: not indicated in patients who are alert and oriented after a first afebrile seizure should be considered neonatal seizures who have an altered mental status, signs of meningeal irritation, or a prolonged postictal period

50. Diagnostic approach… Neuroimaging: CT scan ,MRI focal seizure persistent seizure activity focal neurologic deficit neurocutaneous disorder signs of elevated intracranial pressure

51. Diagnostic approach… Neuroimaging: h/o trauma h/o travel to an area endemic for cysticercosis. Immunocompromising diseases (malignancy or HIV) Hypercoagulable states (sickle cell disease) Bleeding disorders MRI study is more sensitive than a cranial CT scan for the detection of certain tumors and vascular malformations

52. Diagnostic approach… Electroencephalography (EEG): When should an EEG be done? Recommended as a part of initial evaluation in all children presenting with an episodic event. Epileptiform abnormalities support a clinical diagnosis of seizure help in the diagnosis of specific syndromes and predict seizure recurrence Normal EEG does not rule out epilepsy or other underlying neurologic disorders EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

53. Diagnostic approach… When should an EEG be done? Before AED discontinuation, an EEG aids in predicting the risk of recurrence in most syndromes No place for routine follow-up EEGs in patients who are doing well. EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

54. Diagnostic approach… How should an EEG be done: should be recorded 3-4 days after the last seizure to avoid post-ictal slowing from interfering with the interpretation A sleep EEG after deprivation should be part of all routine recordings in children above the age of 3 years Omission of AED prior to EEG recording is not recommended EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

55. Febrile Seizures Age group of 6 and 60 mo temperature of 38°C or higher not the result of CNS infection or any metabolic imbalance occur in the absence of a history of prior afebrile seizures

56. Febrile Seizures… Simple febrile seizure: primary generalized usually tonic-clonic attack associated with fever lasting for a maximum of 15 min not recurrent within a 24-hour period. Complex febrile seizure: more prolonged (>15 min) Focal and/or recurs within 24 hr. Febrile status epilepticus: febrile seizure lasting >30 min.

57. Febrile Seizures…

58. Febrile Seizures… Any type of epilepsy can be preceded by febrile seizures Epilepsy syndromes typically start with febrile Seizures Generalized epilepsy with febrile seizures plus (GEFS+) Severe myoclonic epilepsy of infancy (SMEI-Dravet syndrome) Temporal lobe epilepsy secondary to mesial temporal sclerosis.

59. Febrile Seizures… Dravet syndrome: considered to be the most severe of the phenotypic spectrum of febrile seizures plus onset is in the 1st yr of life Characterized by febrile and afebrile unilateral clonic seizures recurring every 1 or 2 mo seizures are typically induced by fever

60. Febrile Seizures… Dravet syndrome: Differ from the usual febrile convulsions more prolonged more frequent come in clusters Subsequently start to occur with lower fevers then without fever. During the 2nd yr of life, myoclonus, atypical absences, and partial seizures occur frequently and developmental delay usually follows.

61. TREATMENT Febrile Seizures: Antiepileptic therapy , continuous or intermittent, is not recommended for children with one or more simple febrile seizures Parents- counselling about relative risks of recurrence of febrile seizures and recurrence of epilepsy Educated on how to handle a seizure acutely

62. TREATMENT Febrile Seizures: seizures lasting for >5 min acute treatment with diazepam,lorazepam, or midazolam Buccal or intranasal midazolam - often preferred by parents Febrile status epilepticus-IV benzodiazepines, phenobarbital,phenytoin, or valproate

63. TREATMENT Febrile Seizures: If the parents are very anxious intermittent oral diazepam (0.33 mg/kg Q 8 hr during fever) nitrazepam, clobazam, and clonazepam (0.1 mg/kg/day) Antipyretics – do not reduce the risk of having a recurrent febrile seizure

64. TREATMENT Febrile Seizures: Chronic antiepileptic therapy may be considered for children with a high risk for later epilepsy screening and treatment of iron deficiency anemia

65. Treatment of Seizures and Epilepsy DECIDING ON LONG-TERM THERAPY: After a first seizure ,treatment is usually not started if the risk of recurrence is low has normal neurodevelopmental status, EEG, and MRI

66. Treatment of Seizures and Epilepsy Treatment is started If the patient has abnormal EEG, MRI, development, and/or neurologic exam and/or a positive family history of epilepsy

67. Treatment of Seizures and Epilepsy COUNSELING: Educating the family and the child about the disease its management limitations it might impose and how to deal with them

68. INITIATING AND MONITORING THERAPY Guidelines for starting AED: Choose an agent that is effective for the particular type of seizure. If several drugs are available, use the drug that is least toxic. 2. Initiate therapy with a single agent. 3. Start at the low end of the dosage range. 4. Continue the same drug for at least long enough to reach a steady state, usually five times the half-life of the drug. Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

69. Side Effects Guidelines for starting AED: 5.Increase the dosage until seizure control is achieved or unacceptable side effects occur. 6. Consider adding another agent if the patient continues to have seizure activity. Aim for a goal of monotherapy by eventually eliminating the first drug Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277

70. CHOICE OF DRUG ACCORDING TO SEIZURE TYPE AND EPILEPSY SYNDROME In general, the drugs of first choice Focal seizures and epilepsies oxcarbazepine and carbamazepine Absence seizures - ethosuximide Juvenile myoclonic epilepsy valproate and lamotrigine Lennox-Gastaut syndrome valproate, topiramate, lamotrigine Infantile spasms ---ACTH

71. DOSES AND SIDE EFFECTS OF COMMON ANTIEPILEPTIC DRUGS Drug Daily dose Common side effects Phenobarbitone 2–6 mg/kg/d Hyperactivity, academic deterioration, reversal of sleep cycles Phenytoin 4–8 mg/kg/d Gum hyperplasia, hirsutism, ataxia, Stevens-Johnson syndrome, lymphoma Valparin 10-60 mg/kg /d Nausea, vomiting, loss of appetite, weight gain, irregular menstruation, alopecia, somnolence Carbamazepine 10-40 mg/kg/d Rash, hepatitis, diplopia, aplastic anemia, leukopenia EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

72. DOSES AND SIDE EFFECTS OF COMMON ANTIEPILEPTIC DRUGS Drug Daily dose Common side effects Oxcarbazepine 10–45 mg/kg/d Somnolence, vomiting,hyponatremia, seizure exacerbation Clobazam 0.4-1.2 mg/kg Behaviour changes, aggression, sleep disturbances, constipation, weight gain Levateracetam 10-60 mg/kg Headache,anorexia, fatigue, infection EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

73. CHOICE OF DRUG ACCORDING TO SEIZURE TYPE AND EPILEPSY SYNDROME… Monitoring: For the older AEDs, before starting treatment, baseline laboratory studies CBC, platelets, liver enzymes, kidney function tests and urinalysis are often obtained and repeated periodically

74. DISCONTINUATION OF THERAPY Discontinuation of AEDs is usually indicated when children are free of seizures for at least 2 yr AED therapy should be discontinued gradually, over a period of 3-6 mo. Abrupt discontinuation can result in withdrawal seizures or status epilepticus

75. Additional Treatment Patients who do not respond to antiepileptic drugs are candidates for steroids Intravenous gamma globulin or ketogenic diet Nelson Textbook of pediatrics, 19 th Edn

76. Steroids: Prednisone (2 mg/kg/day)are often used in epileptic encephalopathies such as West, Lennox- Gastaut, myoclonic astatic, continuous spike-waves in slow-wave sleep, and Landau-Kleffner syndromes. Nelson Textbook of pediatrics, 19 th Edn

77. IVIG: Effective in nonimmune-deficient patients with West, Lennox Gastaut, Landau-Kleffner, and continuous spike-waves in slow-wave sleep syndromes and possibly in partial seizures. Nelson Textbook of pediatrics, 19 th Edn

78. Ketogenic diet: Stringently controlled high fat and low protein/carbohydrate diet given with/without a restricted fluid intake to maintain ketosis on a long term basis EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

79. Ketogenic diet: believed to be effective in glucose transporter protein 1 (GLUT-1) deficiency pyruvate dehydrogenase deficiency myoclonic-astatic epilepsy tuberous sclerosis complex Rett syndrome severe myoclonic epilepsy of infancy (Dravet syndrome) infantile spasms Nelson Textbook of pediatrics 19 th Edn

80. Ketogenic diet: Adverse effects GI disturbances Acidosis Increased susceptibility to infections Drowsiness weight loss Nutritional deficiencies rarely- renal calculi and pancreatitis EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

81. Ketogenic diet: The diet should be considered a failure if there is no benefit in 3-6 months and it should be discontinued after this time. In responders, it should be continued for 2-3 year after which it is gradually tapered EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009

82. Epilepsy surgery Surgical interventions should be considered regardless of age, in children who have persistent, frequent seizures that are having an adverse impact upon their lives or are interfering with their cognitive and psychosocial development Uptodate.com

83. Epilepsy surgery Surgical approaches vagus nerve stimulation focal resections lobar or multilobar resections corpus callosotomy Hemispherectomy Multiple subpial transection Uptodate.com

84. References Nelson Text Book of Pediatrics, 19 th Edn Child Neurology, 7 th Edn, John H .Menkes Friedman MJ,Sharieff GQ. Seizures in Children. Pediatr Clin N Am 53 (2006) 257– 277 Blumstein MD, Friedman MJ. Childhood Seizures. Emerg Med Clin N Am25 (2007) 1061–1086 EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, IAP Guidelines for Diagnosis and Management of Childhood Epilepsy. INDIAN PEDIATRICS. VOLUME 46-AUGUST 17, 2009 Uptodate.com WHO.Epilepsy.A manual for Medical and Clinical Officers In Africa.2002

85. Thank you…