Childhood Seizures: A Comprehensive Overview

1. Childhood Seizures: A General Review Vanessa Ruppe RN,MSN,APRN Pediatric Neurology: Epilepsy Div. Feb. 28, 2015

2. Objectives • Understand the Classification of different Seizure types • Know important questions to obtain during the H&P • Know the differential Diagnoses • Be able to describe various seizure presentations • Know specific treatments for various seizure types • Be aware of possible side effects to various AEDs • Understand seizure safety

3. What is a seizure? • An abrupt, abnormal electrical discharge from neurons in the brain causing a transient alteration of: • Movement • Sensation: noises, visions, flashes, spots, smells, dizziness, light headed -- Autonomic Function: sweating, incontinence, elevated HR, flushing, nausea, vomiting, goose bumps, dilated pupils. -- Psyche: fear, dream state, pleasure, anger, detachment from time.

4. Differential Diagnosis • Syncope or breath holding spells • Migraine/Complex Migraine • Benign Paroxysmal Vertigo • Reflux, Sandifer syndrome • Self stimulation, Stereotypies, Tics, Benign Myoclonus, dyskinesias • Night terrors or parasomnias • Psychogenic/Functional Seizures

5. Helpful Questions • Is the movement suppressible? • Is the event distractible? • Were the movements jerking or thrashing? • Are the events always provoked? • Is there tongue biting during the event and if so, where? • Was there urination or loss of stool? • Does it only happen during exercise? • What do the eyes do during the seizure? • Does it happen only during certain times of the day? • Does it only happen upon standing? • What is the child like after the event? • Does the child retain consciousness during the event? • Does the child remember the event?

6. Additional Questions • History: illnesses, trauma, development. • Past medical history • Family history of seizures • Then, of course….. • Physical Exam

7. Definition of Epilepsy • A diverse set of chronic neurological disorders characterized by seizures. • Operationally, it is defined as an individual who has had two or more unprovoked seizures

8. General Guidelines for Therapy: • Correct classification of seizures leads to correct AED selection • Treat when the benefit of therapy outweighs the risks • 2 year commitment to medication

9. Risks & Benefits of Treatment Treatment Benefits: • decrease likelihood of another seizure - ↓ risk of injury, pychological stigma - ↓ risk of status epilepticus Treatment Risks: • effects on school performance, behavior • allergic reactions, systemic toxicity • costs of medication, office visits, labs

10. Principles of Management • Consider toxicity, side effect, cost • Oral suspension or sprinkle formulations are preferred in young children and infants

11. Seizure Classification Partial(Focal) Primary Generalized Simple Complex Absence Myoclonic Atonic Convulsive Tonic Clonic Tonic-clonic Clonic-tonic-clonic May secondarily generalize

13. Common AEDs and Side Effects • Phenobarbital: sedation, cognitive dysfunciton, hyperkinesis • Carbamazepine (Tegretol): ataxia, dizziness, sedation, rash • Valproic Acid (Depakote): weight gain, thrombocytopenia, tremor, liver toxicity, teratogenic to fetus, can be a mood stabilizer or cause aggression • Phenytoin (Dilantin): hirsutism, gingival hyperplasia, ataxia • Ethosuximide (Zarontin): GI distress, nausea, headache, drowsiness.

14. AEDs and Side Effects Cont’d • Levetiracetam (Keppra): psychotic behavior, irritability • Lamotrigine (Lamictal): Rash • Topiramate (Topamax): sedation, kidney stones, decreased sweating, tingling, appetite suppression, word finding difficulty. • Oxcarbazepine (Trileptal): hyponatremia, dizziness.

15. AEDs and Side Effects Cont’d • Lacosamide (Vimpat): dizziness, headache, nausea, diplopia, fatigue • ACTH: irritability, HTN, cushingoid, • Vigabatrin (Sabril): peripheral vision loss • Clobazam (onfi): sedation, aggression

16. Sophia • 3-week-old infant with no apparent perinatal complications • Hypotonia • Slow feeder, poor suck • Several spells per day of staring with slight jerking movements

17. Neonatal video

18. Neonatal Seizures • Most common cause for neonatal seizures is hypoxic-ischemic encephalopathy • Intracranial hemorrhage (subarachnoid in term, germinal matrix in preterm) • Metabolic disturbances (hypoglycemia, hypocalcemia, drug withdrawal, amino acidemias, organic acidurias, urea cycle defects) • Infection (TORCH) • Malformations of cortical development • Benign neonatal or infantile familial convulsions

19. Clinical Seizures in Newborns • Subtle • Tonic • Clonic • Myoclonic

20. Eye deviation • Fixed stare or blinking • Repetitive mouth or tongue movements • Apnea • Pedaling or tonic posturing of limbs • Hiccups Have to occur while awake and asleep Cannot be suppressed or provoked

21. Why do neonatal seizures present so differently? • The immature CNS cannot sustain a synchronized, well-orchestrated generalized tonic-clonic seizure • Synaptogenesis is not complete • Deficient myelenation in the cortical efferent systems

22. Treatment of Neonates with Seizures • Phenobarbital 3-4mg/kg/d • Levetiracetam (Keppra) 20mg/kg/day Divided BID

23. Hank • 7-month-old infant with no prior medical history. • Now with episodes described as “startle reflexes” commonly occurring in clusters • Not responsive to maintenance phenobarbital

25. Infantile Spasms Often at presentation there is a history of normal pregnancy, labor, delivery…. Developmentally normal and then around 5 mos or so, starts with “episodes”. By that time, losing developmental milestones, episodes occur in clusters, mostly upon awakening but increasing in frequency to as much as every 2 hours.

26. Spasms Semiology • Brief bilateral contractions of neck, trunk, extremities, often occurring in clusters (flexor, extensor, or mixed) that often occur upon awakening or falling asleep • “Jack-knifing” or like an abdominal crunch • Some can appear as facial grimace or with focal features (might be harder to recognize)

27. Treatment Adrenocorticotropin hormone (ACTH)ACTH high dose (150u/m2/day) • Packaged as 5 mL multi-dose vial containing 80 USP units per mL. Dose based on BSA • Give full dose for 2-4 weeks, then ¾ dose for 1 week, then ¼ dose for one week, then off

28. Burden imposed by ACTH • HIGH cost ($28,000/vial)!!!! • mode of administration (IM injection).

29. Infantile Spasm Treatment Cont’d Prednisolone: Start at 15mg tid (45mg daily) for 2 weeks, then check EEG If persistent clinical spasms at 1 week, increase prednisolone to15mg qid (60mg daily) for 1 week - if EEG normalizes, wean off over 2 weeks -if EEG does not normalize, wean off over 2 weeks and consider alternate treatment, either with ACTH or with standard AEDs If EEG normal at 2 weeks, wean off: 15mg bid x1 wk, then 15mg daily x1 wk, then off Vigabatrin (Sabril) Topiramate clonazepam valproate ketogenic diet

30. Prognosis 50-90% of patients develop other seizure types. In general, symptomatic IS are more likely to develop other seizure types than those with cryptogenic IS (57.5 versus 35.3%). Approximately 27-50% of patients with IS develop Lennox-Gastaut syndrome

31. Teddy • 9-year-old boy with long-standing history of intractable epilepsy with tonic-clonic, tonic, myoclonic, atypical absence and atonic seizures • Moderate mental retardation • Frequent injuries from falls

33. Lennox-Gastaut Syndrome • Onset in early childhood • Many underlying etiologies, often cryptogenic • Multiple seizure types including • Atypical absence, generalized convulsive, atonic, myoclonic, partial • Negative neurodevelopmental impact • Mental retardation • Slow spike and wave (2 hertz)

34. Atonic • Generalized seizure • No warning, abrupt onset • Injuries common • Very difficult to treat. Broad Spectrum AEDs • May respond well to VNS • Corpus callosotomy may prevent falls

36. Robert • 4-year-old boy with 3-week history of recurrent episodes of sudden fear or panic • Initial frequency of 2-3 per day, now increased to 1 per hour or more • Maintenance of consciousness • LMD felt these were not seizures

37. Simple Partial Seizures

39. Brain Anatomy Parietal Frontal • Sensory • Planning • Organizing • Emotions • Motor • Speech Occipital • Vision Temporal • Smell • Sound • Short term memory Cerebellum • Coordination

40. Treatment of Simple Partial Seizures • Oxcarbazepine (Trileptal) • Lacosamide (Vimpat) • Topiramate (Topamax) • Levetiracetam (Keppra) • Valproic Acid (Depakote)

41. Tiffany • 12 y/o Hispanic girl with 1-year history of frequent spells with several daily • Diagnosed with “pseudoseizures” • Maintenance of consciousness • Events are stereotypical with extension of the right arm and turning to the left, followed by agitated movements and vocalization with an abrupt recovery

42. Frontal Lobe Epilepsy • Reflecting the large area of the frontal cortex and its diverse functions, frontal lobe epilepsy can manifest in many forms. Seizure manifestations generally reflect the area of cortex involved, which is not necessarily the site of origin of the seizure.Frontal lobe seizure types include: • Complex partial seizures are often characterized by hypermotor behaviors (proximal limbs, tonic). These can produce bizarre-looking episodes that may be mistaken for psychogenic nonepileptic seizures.

43. Frontal lobe seizure types include • Complex partial seizures: • hypermotor behaviors (proximal limbs, tonic). These can produce bizarre-looking episodes that may be mistaken for psychogenic nonepileptic seizures. • bicycling automatisms as well as pelvic thrusting and other sexual automatisms. Tonic posturing and head and eye deviation (version), usually contralateral to the side of the seizure focus, can occur in some patients. • Vocalizations are also very common.

44. frontal absence seizures manifest with staring, trance-like states. These seizures originate from the frontopolar or medial frontal regions. • more prolonged than other seizure types, often lasting several minutes, sometimes hours, or even days

45. video

46. Rachel 12 yo girl gets a feeling of nausea and upset stomach, stares blankly, fidgets with her hands, speech is intact but patient is somewhat confused during event which lasts 2-3 minutes and has evolved with head deviation, stiffening and convulsion of body.

47. Video

48. Complex Partial Aura is common • Represents the ictal onset • Stereotypical, but may be difficult to describe • Alteration of awareness • Automatisms • Postictal confusion • May secondarily generalize

49. Jack • 8yo boy. Spell where he awoke early in the am, noted inability to speak and drooling profusely. Woke his dad who was sleeping next to him and dad noted his face was pulled to the R. Event lasted approx 3 minutes. • 2 events in last two weeks. Identical to above, lasting 2-3 minutes. • Third seizure occurred during wakefulness and involved the right face and arm, slurred speech, with subsequent secondary generalization

50. Benign Epilepsy with Centro Temporal Spikes (BECTS) Benign Rolandic Epilepsy • Age 3-13y (Peak 7-10 years) • Most commonly= brief partial motor seizures of face, pharyngeal muscles. • Usually Nocturnal • Seizures are usually infrequent • Remits near puberty onset