Epilepsy emergencies and how the prevent them

1. Epilepsy emergencies and how the prevent them Marcelo Lancman, MD Medical Director, Northeast Regional Epilepsy Group

2. Epilepsy emergencies • Breakthrough seizures • Seizure clusters • Prolonged seizures (status epilepticus) • Sudden unexpected death in epilepsy (SUDEP) • Seizure-related injuries • Preparedness plans

3. Epilepsy emergencies • Perspectives • Person with epilepsy (PWE) • Caregiver • Healthcare provider (nurse, MD) • How to recognize them? • What to do ? • How to prevent them? • How to prepare?

4. Breakthrough seizures • The severity and urgency depends on seizure type, seizure duration and external circumstances • Some seizures are more dangerous than others • Most seizures are self limited and there is no need for urgent intervention • Only a few need urgent care…and we need to be prepared for those

5. Breakthrough Partial Seizures • Simple partial (lower risk) • Sensory, motor, auditory, visual, psychic, autonomic • Complex partial (moderate risk)

6. Breakthrough Generalized Seizures • Absence (lower risk) • Atonic (higher risk) • Tonic (higher risk) • Clonic/myoclonic (moderate risk) • Tonic-clonic (higher risk)

7. Breakthrough seizures • Safety measures • When to worry? • When to call 911? • When to go to the hospital? • When to call your doctor? • When to use rescue medications?

8. Safety measures • Clear the way: keep other people out of the way • Move objects that could injure PWE • Important to keep calm and track the time and characteristics of the seizure • Check if there is any information regarding seizure or epilepsy type on bracelet or necklace • Turn PWE on his/her side to keep airway clear • Cushion head • Loosen any tight neckwear

9. Safety measures • Do not try to stop movements related to the seizure or hold down PWE • Do not put anything in the PWE’s mouth during a seizure • After the seizure, remain with PWE until awareness of surroundings is fully regained

10. When to worry • First time seizure (since you do not know this behavior) • Seizures lasting more than a few minutes (5?) • Repeated seizures without regaining consciousness • Increase in frequency of seizures • Different seizure types occur • PWE is injured, pregnant or with known associated medical condition • Seizure occurs in water • Difficulty breathing

11. What to do • When to call 911? (when you have any of the “worry” signs) • When to go to the hospital? (It is better to call 911 and have EMT trained personnel take care of the PWE) • When to call your doctor? (whenever you do not know what to do. However, if it an emergency call 911 first)

12. Breakthrough seizure-Rescue medications • What are rescue medications? • What are the side effects and possible complications? • Routes of administration: • Oral medications • Buccal • Sublingual • Rectal • Intranasal • Intramuscular • Intravenous

13. Breakthrough seizure-Rescue medications • What are rescue medications? • Medications that act very fast and can break seizures • They are usually not effective as routine antiepileptic treatment • It needs to be possible to administer them safely (oral, sublingual, rectal, nasal, intramuscular, intravenous) • They need to be available all the time to PWE and caregivers

14. Breakthrough seizure-Rescue medications • What are side effects • Sleepiness • May facilitate aspiration • Respiratory depression • May cause breathing problems

15. Oral rescue medications • Ativan, Valium, Klonopin • May be difficult to administer during a seizure • Very useful if there is an aura • Risk of injury by trying to put medication in mouth • Risk of aspiration • Never give liquids with medication during a seizure

16. Buccal rescue medications • Between gums and cheek • Problems: gagging, coughing and aspiration

17. Buccal route

18. Sublingual rescue medications • Klonopin wafers • Get absorbed faster than oral medications • May be difficult to administer during a seizure • Very useful if there is an aura • Risk of injury by trying to put medication in mouth • Risk of aspiration • Never give liquids with medication during a seizure

19. Sublingual route

20. Diazepam Rectal Gel • Used in acute or emergency situations to stop a seizure that will not stop on its own • Approved by FDA for use by parents and non-medical caregivers • State/school district regulations often govern use in schools

21. Emergency Treatment • Rectal Diastat • Clinically proven • Hard to give • Adults don’t like it • Can’t self administer

22. Intranasal route • Easy to administer • Increases production of nasal mucous and congestion

23. Intranasal Midazolam • Easy to give • Preferred route • Can be self-administered or given by caretaker • Under study

24. Rescue medications-Intramuscular and Intravenous • Valium, Ativan, Midazolam (Versed) • Rapid effect • Needs caregiver to be trained • Only in rare occasions • Midazolam IM

25. How to prevent them • The seizure threshold • What is the seizure threshold? • The amount of activity necessary to bring a seizure on. • We all have a seizure threshold • It is lower in PWE • What can change it? • The importance of knowing

26. How to prevent them • Seizure triggers: • Missing medication doses (pill organizers, alarms) • Alcohol and drugs • Stress • Environmental temperature • Lights • Fever/illness

27. How to prevent them • Seizure triggers: • Hormonal changes • Hyperventilation • Sleep deprivation • Medications and supplements (very important to discuss with your doctor every time you take any new medication for any reason or any supplements—many can provoke seizures) • Travel across time zones

28. Seizure clusters • What are seizure clusters • They start and stop but occur one after another • The can last a very long time • They can lead to injuries and complications • They need to be treated aggressively

29. Seizure clusters • Types of medications • Oral • Buccal • Sublingual • Rectal • Intranasal • Intramuscular • Intravenous

30. Status epilepticus • What is status epilepticus? • What to do? • Why? • What are the consequences if we do not act in a timely manner?

31. Status epilepticus • Formal Definition: seizures that do not stop for 30 minutes. Or they happen on and off without regaining consciousness between seizures • Practical definition: 5 minutes

32. Status epilepticus • Types • Partial motor status epilepticus • Generalized convulsive status epilepticus • Non-convulsive status epilepticus • Myoclonic status epilepticus

33. Partial motor status epilepticus • This prolonged seizure involves just one part of the brain. • The person is awake and talking/interacting normally, but has persistent rhythmic jerking on one side of the body, say the hand, arm or face. • It requires emergency treatment, but is not usually as life-threatening as other forms.

34. Generalized convulsive status epilepticus • This prolonged seizure involves the entire brain, and produces convulsive activity in all four extremities coupled with a lack of responsiveness. • This life-threatening condition requires urgent medical evaluation and treatment.

35. Non-convulsive status epilepticus • This seizure, which could involve part of the brain or the entire organ, is far less dramatic than generalized convulsive status epilepticus, and produces subtle symptoms such as blinking, staring or confusion – or no obvious signs at all. It is less dangerous than the generalized convulsive type, but still requires prompt recognition and treatment. • A continuous EEG recording is the only way to diagnose non-convulsive status epilepticus.

36. Myoclonic status epilepticus • Another seizure that involves the entire brain, this form produces prolonged jerking of all four extremities. • It is usually caused by a profound lack of oxygen to the brain due to heart dysfunction, but may also occur in those with myoclonic epilepsy.

37. Status epilepticus: Epidemiology • One of the most common life-threatening neurological disorders • Incidence: 50,000 to 200,000 cases annually in US. • Around 12% of patients with newly diagnosed epilepsy present with status epilepticus • Within 5 years of initial diagnosis of epilepsy, 20% of patients will have status epilepticus • Mortality rate: 3 to 53% (20%) • 55,000 deaths in U.S. per year

38. What can cause it? • Cause: Unknown in 25 to 40% of cases • Age: elderly > pediatric > adult

39. What can cause it? • Most common causes: • Missing medications • Stroke • Alcohol withdrawal • Metabolic disorders • Hypoxia • Infections • Tumors • Trauma

40. What are the consequences? • Effects on the body • Hyper-sympathetic state (increased HR, dysrhythmias, decreased cardiac output, increase in peripheral resistance, increase in BP followed by decreased BP) • Hyperpyrexia (increased body temperature) (central, infection or increase in muscle activity)- neuron damage • PH decreases • Hyperglycemia (increase in catecholamines)

41. What are the consequences? • Effects on the brain • Early stage: • Increased oxygenation • Increased blood flow (increased BP) • Late stage: • Decreased oxygenation • Decreased blood flow • High requirement of energy with low supply  brain injury • Decreased glucose and increased lactate

42. How is status epilepticus approached? • Clinical assessment: • Exam: trauma, infection • Drugs: ciprofloxacin, baclofen, flumazemil, interferon, ifosfamide, theophyline, isoniazid, alcohol withdrawal, cocaine • Infections: Mycoplasma pneumonia, cat-scratch encephalopathy, herpes simplex, AIDS • Tumor (metastases), cortical dysplasia

43. How is status epilepticusapproached? • Labs • Glucose level, electrolytes, CBC, toxic screen, LFTs, AEDs levels, urine, ABG (hyponatremia, hypernatremia, hypercalcemia, hepatic encephalopathy) • EKG • Others: mixedema, hyperparathyroidism • LP: if infection suspected • Neuroimaging • CT/MRI

44. How is status epilepticus approached? EEG and VEEG • Rule out psychogenic seizures • Classify type of SE

45. How is status epilepticus treated? • This is an emergency where time is of the essence • Steps: • Control of airway and ventilation • Arterial blood gas monitoring • EKG and BP monitoring • IV: glucose and Thiamine • Blood work: CBC, CPM, electrolytes and AED levels

46. Treatment • Pharmacological treatment • Benzodiazepines loading • Lorazepam • Diazepam • Phenytoin or Fosphenytoin loading • If no response: • Phobarbital, Depakon, Keppra, Vimpat • Refractory: • ICU: midazolam, propofol and pentobarbital

47. Status epilepticus-Conclusion • EMERGENCY SITUATION • TIME IS OF THE ESSENCE • DELAY IN TREATMENT COULD RESULT IN BRAIN DAMAGE OR DEATH

48. SUPED • What is SUDEP? • SUDEP stands for Sudden Unexpected Death in Epilepsy. • SUDEP could be the possible cause of death when there is no evidence of trauma or drowning and there is no other clear cause of death (Heart attack, etc.) • SUDEP is believed to be the cause of approximately 10% of seizure related deaths.

49. SUPED • Unfortunately, due to the unpredictable nature of SUDEP it remains an understudied phenomena. • Our understanding of this process is very limited and much remains to be investigated about these occurrences and what causes them. • Thankfully, SUDEP is relatively rare, occurring in about 1 out of 1000 patients with epilepsy per year, but its consequences can be catastrophic

50. Who is at risk? • All patients with Epilepsy are at some risk • Higher risk: • Long history of poorly controlled seizures (risk of 1 in 150) • Patients with generalized tonic-clonic seizures ‘Grand Mal’ • SUDEP also appears to typically affect younger adults with epilepsy. (Approximately 75% of all SUDEP deaths occur in individuals between 20 to 50 years of age) • Children, have a relatively lower risk of SUDEP.