Frequently asked questions in Epilepsy

1. Frequently asked questionsin Epilepsy Enrique Feoli MD North East Regional Epilepsy Group October 2016

2. New patient visit for seizures • 26 year old woman with a history of two febrile seizures • In the past several 6 months, two episodes concerning for generalized tonic-clonic seizures. • Aura of odd hot, flushing sensation rising from the pit of her stomach • For the past few years, recalls episodes of similar auras followed by brief episodes of loss of awareness where she loses a period of time without having convulsions • No other medical history • Social alcohol • No tobacco or drugs • Married, no children but planning in the next few years • Works as an elementary school teacher • Medications: birth control and multivitamins • Exam: normal

3. Follow up visit • Inpatient continuous video-EEG: captured typical auras, episodes of losing time, and one generalized tonic-clonic seizure consistent with focal epilepsy of right anterior temporal region • MRI of the brain: right mesial temporal sclerosis • Treatment: Lamictal, tolerating well thus far without further seizures

4. She has several questions • Do I have epilepsy? • If so, why do I have epilepsy at my age? • Can I continue my normal daily activities, my job? • Are there activities or other medications I need to avoid? • Do I need to take special precautions as a woman? • Can I have healthy children? • Do I need to take medications forever? • Where can I find out more information?

5. What is epilepsy? • Two or more unprovoked seizures that occurred >24 hours apart • One unprovoked seizure and a probability of further seizures similar to the recurrence risk of having had 2 unprovoked seizures • Diagnosis of epilepsy syndrome Fisher et al, 2013 ILAE

6. Why do I have epilepsy? • As of 2013, about 2.9 million people had epilepsy in the US Hauser, et al. 1993, Epilepsia CDC

7. Common causes of epilepsy • Genetic • Channelopathies • Malformations of cortical development • Metabolic or mitochondrial • Cerebrovascular • Trauma • Infection • Autoimmune • Neoplasms Continuum, June 2013

8. How does this affect my life? Work

9. American Disabilities Act (ADA) • Prohibits employment discrimination against qualified individuals with disabilities • The “qualified individual” should be able to perform the job functions with or without reasonable accommodations • Accommodations need to be individualized due to variability in seizures and in job functions Epilepsy Foundation: www.epilepsy.com

10. Job considerations • Does the job involve flying, driving, or climbing up buildings/telephone poles, performing surgery, firearms? • Does the job require frequent change in the daily schedule and shift work? • Is there someone who works with you who can help if you have a seizure?

11. Law enforcement officers • No federal laws exist to restrict people with epilepsy from working as a LEO • However, certain medical requirements may need to be met in certain organizations • May not be ideal if • Person with epilepsy poses a “direct threat” to the safety of himself or others even with reasonable accommodations • If providing accommodations would pose an undue burden on the employer Epilepsy Foundation: www.epilepsy.com

12. Military • Does not have to abide by ADA rules • May need deployment to areas with limited medical care or limited medication availability • Will evaluate individual cases, especially if seizure free for >5 years OFF medications • Medical Review Board will evaluate if epilepsy develops after enlistment Epilepsy Foundation: www.epilepsy.com

13. Federal Aviation Administration (FAA) • Anyone with a diagnosis of epilepsy or “disturbance of consciousness without a satisfactory medical explanation of cause” are prohibited from obtaining a pilot’s license • Office of Federal Air Surgeon can make exceptions in certain situations if appealed • ie, if seizure free off medications for >10 years Epilepsy Foundation: www.epilepsy.com

14. Commercial driving license (CDL) • Federal Motor Carrier Safety Administration (FMCSA) • Prohibited if taking anti-seizure medications • May be allowed if seizure free for >10 years off medications if history of epilepsy • May be allowed if seizure free for >5 years off medications if history of a single unprovoked seizure • http://www.fmcsa.dot.gov/regulations/title49/section/391.43

15. How does this affect my life? DRIVING

16. DMV reporting states • CA, DE, NJ, NV, OR, PA

17. Driving in NJ • Mandatory physician reporting state, within 24 hrs • Recurrent convulsive seizures • Recurrent period of unconsciousness or impairment, • Loss of motor coordination due to conditions such as, but not limited to epilepsy in any of its forms which persist or recur despite medical treatment • Seizure free period: 6 months Epilepsy Foundation: www.epilepsy.com

18. Driving in NY • Not a physician reporting state • Seizure free period: 12 months • Exceptions: • 1) it was due solely to a physician-directed change in medication and the physician submits a statement to that effect • 2) the person submits a physician's statement confirming his knowledge of all such incidents and recommending licensing despite the medical history, because in his opinion the condition will not interfere with the safe operation of a vehicle and the Department’s medical consultant has no objection to such issuance Epilepsy Foundation: www.epilepsy.com

19. Seizure free interval requirement • Studies have not demonstrated consistent data in regards to risk of accidents related to seizures • Many states have no defined seizure free interval requirement • Most states that do have a requirement set it to 3-12 months of seizure freedom, median ~6 months • In a study comparing 50 drivers with epilepsy who had seizure- related crashes and 50 drivers with epilepsy who did not, compared to a shorter seizure free period… • If seizure free for >12 months: risk of MVA reduced by 93% • If seizure free for >6 months: risk of MVA reduced by 85% Krauss et al. 1999 Neurology

20. How does this affect my life? DAILY ACTIVITIES

21. Impact of alcohol on seizures • Risk of seizures increases in the setting of alcohol withdrawal • Binge drinking can also increase the risk of seizures • Anti-seizure medications may decrease the tolerance of alcohol • Alcohol may induce clearance of anti-seizure medications, making them less effective • Chronic alcoholism and alcohol related seizures may increase the risk of future unprovoked seizures and epilepsy Leach, et al. 2012 Epilepsia

22. Common triggers of seizures • Missing medications • Sleep deprivation • Stress • Excessive alcohol intake or alcohol withdrawal • Illicit drugs • Medications • antihistamines, antibiotics, certain pain medications, stimulant medications • many others, should discuss with your doctors first

23. Safety precautions • Avoid climbing up on ladders • Use the back burners when cooking • Use showers instead of baths • Lower the maximum temperature of the water to 110 degrees F in case a seizure occurs while bathing • Do not use power tools if seizures are not well controlled

24. Safety in sports/recreational activities • Swim and exercise with supervision • Always wear appropriate protective equipment when exercising • Downhill skiing is contraindicated with uncontrolled seizures • Scuba diving: • If seizure free for >4 years on stable AED therapy Almeida, et al. Epilepsia 2007

25. Women with epilepsy CATAMENIAL EPILEPSY PREGNANCY

26. Catamenial epilepsy • Seizures increase during certain phases of the menstrual cycle • Consistent two-fold increase in seizure frequency during these phases • More common with focal epilepsies • Increase can occur when the levels of estrogen are relatively higher than progesterone or when the levels are rapidly changing • May treat with additional medications during the specific phase or with progestin-only contraceptive agent Continuum, June 2013

27. Prenatal considerations • Many anti-seizure medications can interact with hormonal contraceptives • Hepatic inducers will decrease the efficacy of hormonal birth control • For the strong inducers, the risk of birth control failure outweighs the potential benefit of its use with the birth control • May consider alternative forms of contraception • Progestin implant • IUDs Continuum, June 2013

28. Folic acid Continuum, June 2013

29. Pregnancy considerations • Minor anomalies 6-20% of babies • 2.5 times the general populate rate • Major malformations 3.1-9% of babies exposed to AEDs • General population and women with epilepsy not on AEDs: 1.6-3.2% • Polytherapy increases risk of malformations Continuum, June 2013

30. Malformations associated with medications • Valproic acid: facial clefts, neural tube defects, hypospadias • Phenytoin: cleft palate • Carbamazepine: posterior cleft palate • Phenobarbital: cardiac malformations • Topiramate: oral clefts, hypospadias • * seizures during pregnancy can lead to fetal hypoxia, miscarriages, stillbirths Continuum, June 2013

31. Prognosis

32. How long will I have epilepsy? • Epilepsy is considered “resolved” if it was an age-dependent syndrome, and the patient has now passed the applicable age • Epilepsy is considered “resolved” if the patient has remained seizure free for 10 years with no anti-seizure medications for the last 5 years Fisher et al, 2013 ILAE

33. When to withdraw medications • In children <16 years of age • Discontinuing anti-seizure medications before 2 years of seizure freedom -> increased the risk of seizure recurrence by 34% • Risk increases with focal seizures, abnormal EEG, history of status epilepticus, low IQ, prior high frequency of seizures • No well conducted studies in adults to guide medication withdrawal Strozzi, et al. 2015 Cochrane Database

34. SUDEP Sudden Unexpected death in epilepsy

35. SUDEP By definition, (1) death is sudden and unexpected, (2) a clear cause of death must be absent, and (3) victims must have had epilepsy.

36. What Causes it Although the cause of death is unknown, irregularity in the heart rhythm. impaired breathing (apnea), increased fluid in the lungs (impairing the exchange of oxygen and carbon dioxide), and being face down on the bed. In many cases, death probably occurs after a seizure has ended.

37. Frequency 1:3000/year 1;300/year in patients with severe epilepsy. More frequent in men, age 20-40

38. Risk Factors frequent convulsive seizures early age of onset of epilepsy long duration of epilepsy higher number of antiepileptic medications, and at high doses frequent medication changes

39. Tips for prevention Patients should make sure to take the medications prescribed for them. Patients should visit with their doctor regularly, especially if convulsive seizures are not completely controlled.

40. Prevention Adult patients with a high likelihood of tonic-clonic seizures in sleep should be supervised whenever possible. (SUDEP is extremely rare in children with epilepsy and in other patients who are well monitored. In fact, supervision has emerged as a protective factor for SUDEP, independent of seizure control.)

41. Prevention Basic first aid should be provided during a seizure, including rolling the person onto one side, checking respiration and avoiding putting any object in the patient’s mouth. Family members and/or caregivers of patients with uncontrolled convulsive seizures should learn cardiopulmonary resuscitation.

42. Video Games Are video Games safe? Can Video Games cause epilepsy

43. Video Games 1981 The Lancet “space invader epilepsy” 1981 Jeavons (UK) Seizure by a hand held video game. 1983 Dahlquist (USA) first US video game sz report 1991 Nintendo starts putting warning on games. 1992 UK a child died following a seizure induced by video game. 1994 Ferrie, 35 cases, coined the term video game epilepsy 1997 Pokemon Incident.

44. Pokemon Incident An unplanned Experiment in Nature Pokemon incident in Japan Most Dramatic December 16, 1997. A rocket launch sequence of flashing red and blue lights changing at 12.5 sec for 4 sec. was shown on Tokyo TV. 685 children went to the hospital 560 of this children had seizures Rest had migraines, nausea, motion sickness.

45. Pokemon Incident 75 % of the children did not have epilepsy Given that 7 million children were watching the program, this suggests that 1 in 10,000 had a seizure in response to photic stimulation.

46. Terms Photic Induced Seizures: a seizure provoked by visual stimulation. Photoparoxysmal response, an abnormal EEG response to light or pattern, consisting of spikes, spike waves or slow waves, should not be confined to the occipital region, should not be confused with photic driving. Photosensitivity: abnormal response of the EEG to light or pattern consisting of a PPR. Photomyoclonic response: forehead and muscle twitching in response to the light flash, disappearing with eye opening

47. Video GamesMisnomers Video Game Epilepsy Photic Induced Epilepsy

48. Video Games Can Individuals with epilepsy, but with no known photoparoxysmal sensitivity safely play a video game? Millet and colleagues 1999 212 patients, with Epilepsy no EEG photic or pattern sensitivity Randomly assigned to a video game or leisure activity. Then crossover End point clinical seizure

49. Video Games 25 seizures 13 during video game, 12 during other activities. ANSWER IS Patients with Epilepsy with no know photoparoxysmal sensitivity MAY SAFELY play video games.

50. TV RECOMMENDATIONS View TV from > 8 feet View in a well-lit room, with a small lamp on top of the TV set. Do Not approach the TV to switch channels. Cover one eye if it is necessary to go near the TV Wear polarized glasses on sunny days to reduce flickering reflection from water. Play for one hour then take a break.