1. Pediatric Seizures An Overview

2. Childhood Seizures Evaluation Evaluation Classification Classification Diagnosis Diagnosis Treatment Treatment Mimics Mimics

3. Evaluation Frequency: 4-6/1,000 Frequency: 4-6/1,000 History History Focal or Generalized Focal or Generalized Duration, State of Consciousness, Triggers Duration, State of Consciousness, Triggers Aura, Behavior, Posture, Post-ictal State Aura, Behavior, Posture, Post-ictal State Examination Examination Vitals, HC, HSM, abnormal Neuro exam Vitals, HC, HSM, abnormal Neuro exam Skin exam, Retinal exam, hyperventilation Skin exam, Retinal exam, hyperventilation

4. Classification-Febrile Seizures 3-4% of population 3-4% of population Most common Most common Excellent prognosis Excellent prognosis 9 months to 5 years; peak 14-18 months 9 months to 5 years; peak 14-18 months Strong family history of febrile seizures Strong family history of febrile seizures Rapid rising temp, >38 degrees Celsius Rapid rising temp, >38 degrees Celsius Generalized Tonic/Clonic; <10 minutes Generalized Tonic/Clonic; <10 minutes If exam is normal, No further Work-up If exam is normal, No further Work-up Rectal Diazepam for recurrence Rectal Diazepam for recurrence

5. Febrile Seizures (2) Work up is necessary if: Work up is necessary if: More than one febrile seizure in 24 hours More than one febrile seizure in 24 hours Seizure last for more than 10 minutes Seizure last for more than 10 minutes Focal seizure characteristics Focal seizure characteristics Positive physical exam suggestive of infectious, structural, neurologic, congenital pathology Positive physical exam suggestive of infectious, structural, neurologic, congenital pathology

6. Classification-Partial Seizures Simple Partial Seizures Simple Partial Seizures Maintained Consciousness Maintained Consciousness Motor activity: Versive Seizures Motor activity: Versive Seizures Sensory: aura Sensory: aura Autonomic Autonomic No automatisms, No tics (can be suppressed) No automatisms, No tics (can be suppressed) EEG: spikes, sharp waves in a unilateral or bilateral or multifocal pattern EEG: spikes, sharp waves in a unilateral or bilateral or multifocal pattern Duration: 10-20 seconds Duration: 10-20 seconds

7. Partial Seizures (2) Complex Partial Seizures (impaired LOC) Complex Partial Seizures (impaired LOC) Simple partial seizure followed by LOC Simple partial seizure followed by LOC Consciousness impaired at onset of seizure Consciousness impaired at onset of seizure Aura: 1/3 of patients with PS Aura: 1/3 of patients with PS Automatisms: ¾ of patients with CPS Automatisms: ¾ of patients with CPS Following LOC into postictal phase, not recalled Following LOC into postictal phase, not recalled Infant: alimentary; Child: gestural, unplanned Infant: alimentary; Child: gestural, unplanned

8. PS (3): CPS (cont’d) Spreading of discharge throughout brain can lead to secondary generalization (tonic-clonic) Spreading of discharge throughout brain can lead to secondary generalization (tonic-clonic) EEG: Anterior temporal lobe sharp waves, focal spikes or multifocal spikes EEG: Anterior temporal lobe sharp waves, focal spikes or multifocal spikes Normal EEG in 20%; must use sleep deprived, prolonged techniques Normal EEG in 20%; must use sleep deprived, prolonged techniques Duration: 1-2 minutes Duration: 1-2 minutes Needs CT or MRI to rule out structural causes Needs CT or MRI to rule out structural causes

9. PS (4): BPEC Benign Partial Epilepsy with Centrotemporal Spikes (Rolandic Epilepsy) Benign Partial Epilepsy with Centrotemporal Spikes (Rolandic Epilepsy) Excellent Prognosis Excellent Prognosis Ages: 2 – 14; peak onset at 9 – 10 years old Ages: 2 – 14; peak onset at 9 – 10 years old Facial tonic-clonic symptoms Facial tonic-clonic symptoms Normal exam, possible positive FamHx Normal exam, possible positive FamHx One seizure: 20%; Repeated clusters: 25% One seizure: 20%; Repeated clusters: 25% Occurs during sleep: 75% Occurs during sleep: 75% EEG: repetitive spike in rolandic area, o/w nl. EEG: repetitive spike in rolandic area, o/w nl.

10. Classification: Generalized Absence Absence Simple: Cessation of activity with blank facial expression, flickering of eyelids Simple: Cessation of activity with blank facial expression, flickering of eyelids Usually after age 5, F>M, hyperventilation Usually after age 5, F>M, hyperventilation No aura, no postictal state, duration <30 seconds No aura, no postictal state, duration <30 seconds 3/sec spike, generalized wave discharge 3/sec spike, generalized wave discharge Complex: Associated motor symptoms Complex: Associated motor symptoms Myoclonic movements of face, fingers, extremities Myoclonic movements of face, fingers, extremities May have loss of body tone May have loss of body tone 2-2.5/sec spike and wave discharge 2-2.5/sec spike and wave discharge

11. Generalized (2) Generalized Tonic Clonic Generalized Tonic Clonic Focal Onset or De Novo Focal Onset or De Novo Aura can suggest origin Aura can suggest origin Tonic Contractions Tonic Contractions LOC, eyes roll back, cyanosis, apnea LOC, eyes roll back, cyanosis, apnea Clonic Contractions Clonic Contractions Rhythmic contraction/relaxation, loss of sphincter Rhythmic contraction/relaxation, loss of sphincter Post-ictal: 30 minutes to 2 hours Post-ictal: 30 minutes to 2 hours Truncal ataxia, hyperactive DTRs, Babinski’s Truncal ataxia, hyperactive DTRs, Babinski’s Vomiting, intense bifrontal headache Vomiting, intense bifrontal headache

12. Generalized (3); T/C (2) Triggers Triggers Low grade fever Low grade fever Fatigue Fatigue Stress Stress Drugs: Methylphenidate, psychotropics, etc… Drugs: Methylphenidate, psychotropics, etc… Duration: Few minutes Duration: Few minutes Idiopathic Idiopathic

13. Generalized (4) Myoclonic Epilepsies of Childhood Myoclonic Epilepsies of Childhood Repetitive seizures Repetitive seizures Brief, symmetrical contractions Brief, symmetrical contractions Loss of body tone—falling, slumping forward Loss of body tone—falling, slumping forward Benign Myoclonus of Infancy Benign Myoclonus of Infancy Myoclonic Epilepsy of Early Childhood Myoclonic Epilepsy of Early Childhood Complex Myoclonic Epilepsy Complex Myoclonic Epilepsy Juvenile Myoclonic Epilepsy Juvenile Myoclonic Epilepsy

14. Generalized (5); MEC (2) Infancy Infancy Neck, trunk, extremities Neck, trunk, extremities Normal EEG, Ends by 2 years, no meds Normal EEG, Ends by 2 years, no meds Early Childhood Early Childhood 6 months – 4 years 6 months – 4 years Favorable outcome, 50% seizure free Favorable outcome, 50% seizure free MR, social problems in the minority MR, social problems in the minority Positive EEG, possible genetic background Positive EEG, possible genetic background May have concurrent tonic/clonic or febrile seizures May have concurrent tonic/clonic or febrile seizures

15. Generalized (6); MEC (3) Complex Complex Poor prognosis Poor prognosis Focal or generalized seizures <1 year of age Focal or generalized seizures <1 year of age History: hypoxic-ischemic encephalopathy, microcephaly History: hypoxic-ischemic encephalopathy, microcephaly Positive EEG, less prominent FamHx Positive EEG, less prominent FamHx Refractory to meds Refractory to meds MR, behavioral problems in 75% MR, behavioral problems in 75% Lennox Gastaut syndrome Lennox Gastaut syndrome

16. Generalized (7); MEC (4) Juvenile Juvenile Between ages 12 – 16 Between ages 12 – 16 5% of all epilepsies 5% of all epilepsies Initial: Morning myoclonic jerks Initial: Morning myoclonic jerks Later: Morning Generalized Tonic Clonic szs Later: Morning Generalized Tonic Clonic szs Positive EEG: 4-6/sec irregular spike Positive EEG: 4-6/sec irregular spike Enhanced with photic stimulation Enhanced with photic stimulation Normal exam, lifelong meds (Valproic Acid) Normal exam, lifelong meds (Valproic Acid)

17. Generalized (8) Infantile Spasms Infantile Spasms Between 4 – 8 months Between 4 – 8 months Flexor, Extensor, or Mixed spasms Flexor, Extensor, or Mixed spasms Cryptogenic: 10-20%, normal work-up Cryptogenic: 10-20%, normal work-up Good prognosis Good prognosis Symptomatic: 80-90%, underlying pathology Symptomatic: 80-90%, underlying pathology Prenatal and Perinatal etiologies Prenatal and Perinatal etiologies MR 80-90% MR 80-90% Positive EEG: hypsarrhythmia pattern Positive EEG: hypsarrhythmia pattern

18. Diagnosis Minimum Minimum Blood glucose, calcium, mag, lytes, EEG Blood glucose, calcium, mag, lytes, EEG EEG techniques: 40% of EEGs are normal EEG techniques: 40% of EEGs are normal Sleep deprived, prolonged (72 hrs), photic Sleep deprived, prolonged (72 hrs), photic CSF CSF Infectious etiology suspected Infectious etiology suspected Radiologic: CT or MRI Radiologic: CT or MRI 1 Prolonged or intractable szs, neuro deficit, increased ICP Prolonged or intractable szs, neuro deficit, increased ICP High risk: Predisposing factors, focal sz <33 months High risk: Predisposing factors, focal sz <33 months

19. Treatment Treat after the first uncomplicated seizure with a negative work up—80% will NOT have another seizure Treat after the first uncomplicated seizure with a negative work up—80% will NOT have another seizure 2 Educate patient and family of possible long term use and side effects Educate patient and family of possible long term use and side effects May terminate meds after 2 seizure free years May terminate meds after 2 seizure free years Wean over 3-6 months due to possible recurrence or status. Wean over 3-6 months due to possible recurrence or status.

20. Treatment (2) Carbamazepine or Tegretol Carbamazepine or Tegretol Gen T/C, partial; watch leukopenia, LFTs Gen T/C, partial; watch leukopenia, LFTs Phenytoin or Dilantin Phenytoin or Dilantin Gen T/C, partial; watch SJS, rashes, lupus-like Gen T/C, partial; watch SJS, rashes, lupus-like Phenobarbital Phenobarbital Gen T/C; watch behavioral changes Gen T/C; watch behavioral changes Sodium Valproate or Valproic Acid Sodium Valproate or Valproic Acid Gen T/C, absence, myoclonic; watch LFTs, Reyes Gen T/C, absence, myoclonic; watch LFTs, Reyes ACTH ACTH Infantile spasms; watch glucose, BP, lytes Infantile spasms; watch glucose, BP, lytes

21. Treatment (3) Ketogenic Diet Ketogenic Diet Increases GABA inhibition of seizure activity Increases GABA inhibition of seizure activity Recalcitrant seizures Recalcitrant seizures Complex myoclonic epilepsy Complex myoclonic epilepsy Fat diet, restriction of CHO and protein Fat diet, restriction of CHO and protein Surgical Options Surgical Options Vagal Nerve Stimulator Vagal Nerve Stimulator 3 Ablation therapy Ablation therapy Intractable seizures Intractable seizures

22. Mimics BPV BPV Night Terrors Night Terrors Breath Holding Spells Breath Holding Spells Simple Syncope Simple Syncope Cough Syncope Cough Syncope Shuddering Attacks Shuddering Attacks Pseudoseizure Pseudoseizure Benign Paroxysmal Torticollis of Infancy Benign Paroxysmal Torticollis of Infancy

23. Bibliography Sharma, et. al, “Role of Emergent Neuroimaging…,” Pediatrics, Vol 111, January, 2003. 1 Sharma, et. al, “Role of Emergent Neuroimaging…,” Pediatrics, Vol 111, January, 2003. Shinnar, et. al, “Risk of Seizure Recurrence…,” Pediatrics, Vol 98, August, 1996. 2 Shinnar, et. al, “Risk of Seizure Recurrence…,” Pediatrics, Vol 98, August, 1996. Parker, et. al, “VNS in Epileptic Encephalopathies,” Pediatrics, Vol 103, April, 1999. 3 Parker, et. al, “VNS in Epileptic Encephalopathies,” Pediatrics, Vol 103, April, 1999.