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  1. 1. What Spell is This? Marcos J. Valdez, M.D. Pediatric Neurology
  2. 2. What Spell is This? <ul><li>At the conclusion of the activity, the participant should be able to: </li></ul><ul><ul><li>Be familiar with the classification of seizures </li></ul></ul><ul><ul><li>Distinguish seizure from epilepsy </li></ul></ul><ul><ul><li>Identify some of the clinical manifestations </li></ul></ul><ul><ul><li>Distinguish between seizure and other disorders that can present similarly </li></ul></ul><ul><ul><li>Learn different causes of seizures </li></ul></ul>
  3. 3. What Spell is This? <ul><li>At the conclusion of the activity, the participant should be able to: </li></ul><ul><ul><li>Be familiar with the different treatment modalities </li></ul></ul><ul><ul><li>Learn First Aid for Seizures </li></ul></ul>
  4. 4. What Spell is This? <ul><li>An overview of the following aspects of diagnosis and treatment will be discussed: </li></ul><ul><ul><li>Incidence and Prevalence </li></ul></ul><ul><ul><li>Patient evaluation </li></ul></ul><ul><ul><li>Risk of Recurrence Following a First Seizure </li></ul></ul><ul><ul><li>Factors Influencing Remission Rates </li></ul></ul><ul><ul><li>Treatment </li></ul></ul><ul><ul><li>Withdrawal of Antiepileptic Drugs </li></ul></ul>
  5. 5. Incidence and Prevalence <ul><li>Epileptic seizure: paroxysmal event of the central nervous system characterized by abnormal cerebral neuronal discharges with or without a loss of consciousness </li></ul><ul><li>Epilepsy: condition characterized by a tendency for recurrent epileptic seizures (two or more) unprovoked by any known proximate insult </li></ul>
  6. 6. Incidence and Prevalence <ul><li>The risk of any individual having an epileptic seizure in their lifetime is approximately 10% </li></ul><ul><li>The prevalence of epilepsy requiring protracted medical treatment is only < 1% </li></ul>
  7. 7. Patient Evaluation <ul><li>Evaluation should include a complete history, thorough general medical and neurological examination, and pertinent laboratory examinations. </li></ul>
  8. 8. Patient Evaluation <ul><li>HISTORY: A precise and extensive history is the most crucial step in the evaluation </li></ul><ul><li>A good description of the event and symptoms and signs surrounding the event must be obtained </li></ul>
  9. 9. Patient Evaluation <ul><li>Pertinent information includes: </li></ul><ul><ul><li>Precipitating factors </li></ul></ul><ul><ul><li>Determination of the Presence of an Aura and its Description </li></ul></ul><ul><ul><li>Description of the event </li></ul></ul><ul><ul><li>Post Event Signs and Symptoms </li></ul></ul><ul><ul><li>Effect of Consciousness </li></ul></ul><ul><ul><li>Duration of Event </li></ul></ul><ul><ul><li>History of Previous Events </li></ul></ul>
  10. 10. Patient Evaluation <ul><li>It is important to determine common risk factors: </li></ul><ul><ul><li>Abnormalities of pregnancy, birth, and development </li></ul></ul><ul><ul><li>Febrile seizures </li></ul></ul><ul><ul><li>Meningitis or encephalitis </li></ul></ul><ul><ul><li>Head trauma with loss of consciousness </li></ul></ul><ul><ul><li>Drug and alcohol abuse </li></ul></ul><ul><ul><li>Predisposing medical/neurological conditions </li></ul></ul>
  11. 11. Patient Evaluation <ul><li>It is important to determine common risk factors: </li></ul><ul><ul><li>Family history </li></ul></ul><ul><ul><li>Prescribed medications </li></ul></ul><ul><ul><li>Birthmarks or skin lesions </li></ul></ul><ul><ul><li>Exposure to toxins </li></ul></ul><ul><ul><li>Metabolic or nutritional disturbances </li></ul></ul><ul><ul><li>Vascular disturbances </li></ul></ul>
  12. 12. Physical and neurological examination <ul><li>A thorough general physical and neurological examination are required at the time of presentation </li></ul><ul><li>The clinician must search for evidence of an underlying systemic or neurological disorder </li></ul>
  13. 13. Laboratory Examination <ul><li>The following investigations should be performed: </li></ul><ul><ul><li>Serum studies: electrolytes, calcium, magnesium, phosphorus, complete blood count, toxic substance screen, alcohol level </li></ul></ul><ul><ul><li>Electroencephalogram </li></ul></ul><ul><ul><li>MRI of the brain </li></ul></ul><ul><ul><li>Lumbar puncture (if suspect central nervous system infection, inflammation, or bleeding </li></ul></ul>
  14. 14. Differential Seizure Diagnosis <ul><li>Seizure Classification </li></ul><ul><ul><li>Partial seizures </li></ul></ul><ul><ul><ul><li>Simple partial </li></ul></ul></ul><ul><ul><ul><li>Complex partial </li></ul></ul></ul><ul><ul><ul><li>Secondary generalized </li></ul></ul></ul><ul><ul><li>Generalized seizures </li></ul></ul><ul><ul><ul><li>Absence </li></ul></ul></ul><ul><ul><ul><li>Myoclonic </li></ul></ul></ul><ul><ul><ul><li>Tonic </li></ul></ul></ul><ul><ul><ul><li>Clonic </li></ul></ul></ul><ul><ul><ul><li>Tonic-clonic </li></ul></ul></ul><ul><ul><li>Unclassified seizures </li></ul></ul><ul><ul><ul><li>Infantile spasms </li></ul></ul></ul><ul><ul><ul><li>Febrile seizures </li></ul></ul></ul><ul><ul><li>Non-epileptic seizures </li></ul></ul>
  15. 15. Differential Seizure Diagnosis <ul><li>Partial seizures: are characterized by a focal disruption of cerebral electrical activity </li></ul><ul><li>They produce epileptiform discharges in a localized area of a cerebral hemisphere </li></ul><ul><li>Conscious contact is maintained in simple partial seizures and is lost in complex partial seizures </li></ul>
  16. 16. Differential Seizure Diagnosis <ul><li>Generalized seizures: involve simultaneous disruption of electrical activity of both cerebral hemispheres </li></ul><ul><li>May present in various combinations in a given patient </li></ul><ul><li>Represent the majority of the genetically determined seizure disorders </li></ul>
  17. 17. Differential Seizure Diagnosis <ul><li>Non-epileptic seizures </li></ul><ul><ul><li>Non-epileptic seizures represent 25-33% of the patients referred for closed circuit TV/EEG monitoring </li></ul></ul>
  18. 18. Differential Seizure Diagnosis <ul><li>Conditions resembling seizures and epilepsy </li></ul><ul><ul><li>Syncopes </li></ul></ul><ul><ul><li>Cardiac Arrhythmias </li></ul></ul><ul><ul><li>Pseudoseizures </li></ul></ul><ul><ul><li>Paroxysmal torticollis </li></ul></ul><ul><ul><li>Movement disorders </li></ul></ul><ul><ul><li>Gastroesophageal reflux </li></ul></ul><ul><ul><li>Migraines </li></ul></ul><ul><ul><li>Breath-holding spells </li></ul></ul><ul><ul><li>Sleep disorders (sonambulism, night terrors) </li></ul></ul><ul><ul><li>Attention deficit-hyperactivity disorder (ADHD) </li></ul></ul>
  19. 19. Predictors of Recurrence and Remission <ul><li>Following a single unprovoked seizure, the risk of recurrence varies from 16% to 61% </li></ul><ul><li>The second seizure usually occurs within the following year </li></ul><ul><li>Prognostic factors that increase the risk of recurrence include: remote symptomatic etiology, initial partial seizure, and an abnormal EEG </li></ul>
  20. 20. Predictors of Recurrence and Remission <ul><li>The probability of remission varies from 41-75% across studies </li></ul><ul><li>Favorable prognosticators for remission are younger age of onset and generalized seizure disorder, especially tonic-clonic seizures </li></ul>
  21. 21. Treatment <ul><li>Medications </li></ul><ul><li>Surgery </li></ul><ul><li>Ketogenic diet </li></ul><ul><li>Vagus nerve stimulator </li></ul>
  22. 22. Treatment <ul><li>Medications </li></ul><ul><ul><li>Phenobarbital </li></ul></ul><ul><ul><li>Phenytoin </li></ul></ul><ul><ul><li>Carbamazepine </li></ul></ul><ul><ul><li>Valproic acid </li></ul></ul><ul><ul><li>Ethosuximide </li></ul></ul><ul><ul><li>Lamotrigine </li></ul></ul><ul><ul><li>Topiramate </li></ul></ul><ul><ul><li>Lorazepam </li></ul></ul><ul><ul><li>Diazepam </li></ul></ul>
  23. 23. Treatment <ul><li>Treatment with antiepileptic drugs (AED’s) can result in life threatening idiosyncratic reactions, teratogenicity, cognitive impairment, systemic toxicity, and adverse behavioral effects </li></ul><ul><li>The physician should discuss all the associated risks with the patient and the family prior to treatment </li></ul>
  24. 24. Treatment <ul><li>If the risk of further seizures outweighs the risks of treatment, then AED’s therapy is needed </li></ul><ul><li>The primary intent of AED therapy is to provide cessation of seizures without associated adverse effects </li></ul><ul><li>Clinical indications for laboratory examinations include: clinical evidence of toxicity, suspected non-compliance, drug interactions, and changes in seizure frequency </li></ul>
  25. 25. Treatment <ul><li>The physician should initiate therapy with a single AED </li></ul><ul><li>The medications should be increase until seizure control is obtained or the patient is experiencing clinical toxicity </li></ul><ul><li>Any patient who has failed and adequate trial of AED’s should be referred to an epilepsy center to receive a comprehensive evaluation </li></ul>
  26. 26. Treatment <ul><li>Withdrawal of Antiepileptic Medication </li></ul><ul><ul><li>50-75% of patients will become seizure free without or with minimal side effects to their AED’s </li></ul></ul><ul><ul><li>After the patient has been seizure free for 2 years, AED’s withdrawal is contemplated </li></ul></ul><ul><ul><li>An EEG is recommended prior to withdrawal </li></ul></ul><ul><ul><li>Seizure relapse usually occurs within the first year following AED withdrawal </li></ul></ul>
  27. 27. Treatment <ul><li>Vagus Nerve Stimulator (VNS) </li></ul><ul><ul><li>Mild electrical pulses applied to the left vagus nerve in the neck send signals to the brain </li></ul></ul><ul><ul><li>Automatic intermittent stimulation </li></ul></ul><ul><ul><li>Magnet use allows the patient/caregiver </li></ul></ul><ul><ul><ul><li>On-demand stimulation </li></ul></ul></ul><ul><ul><li>Simple in office programming </li></ul></ul><ul><ul><li>Assured compliance </li></ul></ul>
  28. 28. Treatment <ul><li>Vagus nerve stimulator (VNS) </li></ul><ul><ul><li>6 to 11 year battery life </li></ul></ul><ul><li>Historical Overview of VNS Therapy in Humans </li></ul><ul><ul><li>1988 First human implant </li></ul></ul><ul><ul><li>1994 European community approval </li></ul></ul><ul><ul><li>1997 US (FDA) commercial approval </li></ul></ul><ul><ul><li>2004 >30,000 patients treated worldwide </li></ul></ul>
  29. 29. Treatment <ul><li>Vagus Nerve Stimulator (VNS) </li></ul><ul><li>Adverse events (occurring in > 5% of patients) </li></ul><ul><ul><li>Hoarseness 37% </li></ul></ul><ul><ul><li>Throat pain 11% </li></ul></ul><ul><ul><li>Coughing 6% </li></ul></ul><ul><ul><li>Dyspnea 6% </li></ul></ul><ul><ul><li>Tingling 6% </li></ul></ul><ul><ul><li>Muscle pain 6% </li></ul></ul>
  30. 30. Treatment <ul><li>Ketogenic Diet </li></ul><ul><ul><li>Rigid, carefully controlled, medically supervised diet, requiring hospital admission for initiation </li></ul></ul><ul><ul><li>Appears to be equally effective for all seizure types </li></ul></ul><ul><ul><li>Most effective in children; not commonly used in adults </li></ul></ul>
  31. 31. Treatment <ul><li>Ketogenic Diet </li></ul><ul><ul><li>Diet consists of high ratio of fat to protein and carbohydrate </li></ul></ul><ul><li>Efficacy </li></ul><ul><ul><li>54% of patients experience >50% seizure reduction at 3 months </li></ul></ul><ul><ul><li>10% have seizure freedom at 1 year </li></ul></ul>
  32. 32. Treatment <ul><li>Ketogenic Diet </li></ul><ul><li>Side effects may include: </li></ul><ul><ul><li>Hyperlipidemia </li></ul></ul><ul><ul><li>Constipation/diarrhea </li></ul></ul><ul><ul><li>Vitamin deficiencies </li></ul></ul><ul><ul><li>Kidney stones (6.2%) typically uric acid </li></ul></ul><ul><ul><li>Weight loss </li></ul></ul><ul><ul><li>Growth retardation </li></ul></ul>
  33. 33. Status Epilepticus <ul><li>Partial or generalized seizures usually have a duration of 1-2 minutes </li></ul><ul><li>Status epilepticus is defined as: more than 30 minutes of continuous seizure activity or two or more sequential seizures without full recovery of consciousness between seizures </li></ul><ul><li>Status epilepticus is a Medical Emergency </li></ul>
  34. 34. Seizure precautions <ul><li>No tub baths </li></ul><ul><li>No unsupervised swimming by a trained personnel in case of an emergency </li></ul><ul><li>No other activities that may put the person’s life in danger </li></ul><ul><li>No driving at least for 6 months (policy by DPS) from the last event </li></ul>
  35. 35. First Aid for Seizures <ul><li>Keep the patient safe by moving away hard, sharp, or hot objects </li></ul><ul><li>Put something soft but flat under the patient’s head </li></ul><ul><li>Turn the patient’s head on a side for optimal airway patency </li></ul><ul><li>DON’T put anything in the patient’s mouth </li></ul><ul><li>DON’T introduce your fingers in the patient’s mouth </li></ul>
  36. 36. First Aid for Seizures <ul><li>DON’T try to hold the patient’s tongue (it can’t be swallowed) </li></ul><ul><li>Activate EMS if this is the first seizure activity </li></ul><ul><li>DON’T restrain movement </li></ul><ul><li>DON’T try to give liquids or medications during or just after the seizure </li></ul><ul><li>Reassure when consciousness returns </li></ul>
  37. 37. Questions?