EPILEPSY Neuroscience Nursing Orientation Johns Hopkins Epilepsy Center Rebecca E. Fisher RN BSN CNRN Alison Griffiths RN ASN

Seizure Disorder Definition A transient disturbance in cerebral function due to paroxysmal neuronal discharges Irritation and excitability Epilepsy Occurrence of two or more seizures

Prevalence Single Seizure Epilepsy 10% of the US population 1-2% of the US population by the age of 20. It reaches 3% by the age of 75 5 to 8 in 1000, of approximately 1.25 to 2 million people

Incidence Third most common neurological disorder 80% will respond well to treatment Intractable seizures 20-25% Febrile seizures in children 2-5% Close relatives of epilepsy patients have a threefold increase in incidence. 5-10% inherited

Etiology Primary (idiopathic) Secondary (organic) 50% of all epilepsy Birth trauma Head trauma Tumors Infections Biochemical CVA Degenerative Diseases

Terms Ictal Post Ictal Interictal The time during a seizure The time following a seizure Interictal The time between seizures

International Classifications of Seizures Primary Generalized Epilepsy Involves both hemispheres at the start of the seizure (usually starts deep within the brain) Partial Epilepsy Involves a focal area of the brain that may or may not spread

Primary Generalized Seizures Absence- brief staring spells (3-5 secs) Myoclonic- abrupt brief jerking of limbs Clonic- muscle contraction and relaxing Tonic- stiffening, extension of limbs Atonic- “drop attacks”

Partial Epilepsy Simple (consciousness is not impaired) Motor- abnormal movement of arm, leg, hand, and face Somatosensory or special sensory- epigastric feeling, visual symptoms, smell, and auditory Numbness or tingling in limb Autonomic- tachycardia, flushing, and respiration Psychic- déjà vu, and fear Aura is a simple partial seizure

Partial Epilepsy Complex (consciousness is impaired) Most common in adult epilepsy population May or may not start with simple partial May see staring lasting seconds to minutes May see semipurposeful repetitive movements (i.e. fumbling, lip smacking, swallowing, vocalizations, and wandering)

Evolving into Secondary Generalized Seizures May start as simple, or complex seizure, then progress to tonic-clonic movements.

Psychogenic Seizures Seizures that originate from an emotional disorder rather than paroxysmal neuronal discharges. EEG will be normal Patients can have a mixture of Epilepsy and Psychogenic seizure activity.

Possible clinical signs Asymmetrical or thrashing of limbs, pelvic thrusting, side to side head movement, gradual onset, multiple manifestations, prolong duration, purposeful activity, initiation or termination by suggestion, lack of amnesia, little postictal period.

Treatment Varies depending on psychiatric diagnosis Antidepressants Psychotherapy Relaxation techniques

First Aid Generalized Seizures Help to lying position with something soft under the head Position to side if possible Remove glasses and loosen tight clothing Clear area of sharp or hard objects Do not restrain of force anything in the mouth Suction airway only if necessary Time event, check vitals and neuro status, examine for injuries, monitor until return to baseline

First Aid Complex Partial Seizures Stay with patient and ensure safety Clear area of sharp and hard objects Do not restrain Monitor patient until return to baseline

Notification of MD New onset of seizures Change in seizure type and frequency or duration Seizure > 5 minutes Failure to return to baseline

Status Epilepticus A seizure that persists for a sufficient period of time ( 10 minutes) or is repeated frequently so that recovery between attacks does not occur. Diagnosis for status is at 30 minutes. Patients in status may appear conscious

Complications Shock Hypoxia ICP Acidosis Fever Arrythmia Hemorrhage Neuronal Death

Etiology Anticonvulsant withdrawal Acute metabolic disturbances (hypoglycemia, hyponatremia, hypocalcemia) CVA CNS infection CNS trauma Tumors

Treatment Anticonvulsant Start with Benzodiazepines Diazepam (Valium): 0.25mg/kg up to 20mg IV. Give slowly 1-2mg/min **May also give rectally (Diastat-rectal gel) Lorazepam (Ativan) 0.05mg/kg up to 10mg IV. Give slowly 2mg/min (this is the preferred medication because it lasts longer than the Valium in the body) Midazolam 5-10mg, well absorbed IM

Long-acting AED Phenytoin (Dilantin): 18-20mg/kg IV Do not give faster than 50mg/kg Use only with normal saline Monitor vital signs carefully Avoid IV infiltration (purple glove syndrome) Different institutions have different guidelines concerning the administration of this drug

Follow up with long-acting AEDs Fosphenytoin (Cerebyx): prodrug of phenytoin Water soluble (does not contain propylene glycol) fewer IV site and cardiac complications Dispensed in phenytoin equivalents (1 PE of fosphenytoin=1 mg of Dilantin) Loading dose 18-20 PE/kg Max rate is 150 PE/min IV with cardiac and BP monitoring May be given IM (large volumes i.e. 20ccs split in two IM sites)

Long-acting AED Fosphenytoin continued Side effects- same as phenytoin (hypotension, cardiac arrthymias, rash, dizziness, and itching groin) Each institution has specific guidelines concerning administration of this drug

Long-acting AED Phenobarbital: 10-20mg/kg Do not give faster than 50-100mg/min. Caution following BZD (increase risk of respiratory depression and hypotension)

Depacon (Valproate Sodium Injection) For use in Myoclonic Status and when unable to take po Valproic Acid Dispensed in Valproic Acid equalents (500mg po = 500mg IV) Administer over 60 minutes In 50ccs (Normal Saline, LR, or D5W) No more than 20mg/minute Give the same frequency as po Some institutions has a policy and procedure concerning administration

Side effects of Depacon Not like po-wt gain and hair loss Somnolence Dizziness Paresthesia Nausea H/A Pain at injection site

Other Treatments Petobarbital coma, propofol Supportive Care Airway Protection Lab tests (CBC, BMP, ABGs, AED levels) Fluids VS, EKG Drug Therapy Investigate Cause

Other options Keppra IV option coming in the near future

Diagnostic Studies History Physical Exam Blood work Epilepsy Protocol MRI-structural changes 3Tesla MRI fMRI- language function CT Epilepsy monitoring unit EEG (don’t seizure on demand) PET-metabolism changes WADA-side of language and memory dominance Neuropsych/Cognitive functioning testing MRS-biochemical SPECT-perfusion changes MEG-localization of interictal epileptiform activity with focal seizures used with MRI and EEG

Treatment Anticonvulsant Therapy Ketogenic Diet/Atkins Diet Vagus Nerve Stimulator Surgery

Potential Efficacy of AEDs Carbamazepine Simple Complex Complex Partial Tonic Clonic Phenytoin Simple Partial

Potential Efficacy continued Valproate Simple Partial Complex Partial Tonic-Clonic Atonic Myoclonic Atypical Absence Absence

Potential Efficacy continued Gabapentin Simple Partial Complex Partial Tonic-Clonic Lamotrigine Atonic Myoclonic Absence and atypica absence

Potential Efficacy continued Topiramate Simple Partial Complex Partial Tonic-Clonic Lennox-Gastaut Infantile Spasms Primary generalized

Potential Efficacy Continued Tiagabine Simple Partial Complex Partial Tonic-Clonic Infantile Spasms Levetiracetam Absence Myoclonic

Potential Efficacy Continued Oxcarbazepine Simple Partial Complex Partial Tonic-Clonic Zonisamide Absence Infantile spasms Myoclonic

Potential Efficacy Continued Pregablin - Simple partial - Complex partial

Dilantin Capsules 100mg, 30mg Brand name Dosing 200-500mg Half life 22 hours Blood levels 10-20 (if no side effects MDs may push the top level) High incidence of drug interaction with all other medications Side effects Ataxia Rash Blood changes Osteomalacia Cosmetic changes Dental changes

Tegretol, Tegretol XR, & Carbatrol Tabs 100mg, 200mg, 300mg (Carbatrol), & 400mg (Tegretol XR) Dose 400-2000mg Half life 10-25 hrs Levels 4-12 XR & Cabatrol BID dosing Side effects Weight gain GI upset Ataxia Blurred vision Decreased WBC Hyponatremia Hepatotoxicity

Depakene, Depakote, &Depakote ER Caps 125mg,250mg, 500mg Dose 500-4000mg Half life 8-12 hrs Blood levels 50-150 May be used for migraine management Interacts with Lamictal First line drug for myoclonic seizures (IV) Side effects Weight gain Tremors Uterine changes Sedation Disturb menses Hair loss H/A Dizziness Increase ammonia levels

Phenobarbital Tabs 15mg, 30mg, 60mg, & 100mg Dosing 30mg or 100mg Half life 72 hrs Blood levels 15-40 Side effects Sedation Hyperactivity Confusion Mood changes

Mysoline (Metabolizes to Primidone & Phenobarbital) Tabs 250mg Dose 500-1500mg Half life PRM 3-12hrs PBB 72 hrs Levels PRM 6-12 PBB 15-40 Side effects Sedation Hyperactivity Mood changes

Neurontin Caps 100mg, 300mg, & 400mg, Tabs 600mg & 800mg Dose 3600mg (no research telling how high to go) Dosing TID or QID Half life 5-8 hrs Blood levels-not done Use in management of migraines Side effects Sedation Ataxia Dizziness

Lyrica Capsules 25mg, 50mg, 75mg, 100mg, 150mg, 200mg, 225mg, 300mg Newest drug Dosage for Epilepsy 300-600mg/day Dosing BID Half Life No blood levels Medication is also used for pain management Side effects -Double vision -Ataxia -Edema -Weight gain - Dry mouth - Trouble concentrating

Benzodiazepines Valium Ativan Tranxene Klonopin Side effects Drowsiness Fatigue Ataxia Slurred speech Diplopia

Lamictal Tabs 25mg, 100mg, 150mg, & 200mg Dosage 300-500mg If miss a dose may double up on next dose Half life 14-103 hrs Blood levels 4-20 Depakote increases Lamictal Needs to be adjusted if birth control is added May make JME worse Side effect Rash (slow titration stops this) Depression Dizziness Somnolence H/A Blurred vision Nausea/vomiting

Topamax Tabs 25mg, 50mg, 100mg, & 200mg Half life 21 hrs Dose 200-400mg May increase Dilantin May decrease Carbatrol, Phenobarb, i.e. Side effects Memory problems Word finding difficulties Kidney stones Dizziness Ataxia Somnolence

Trileptal Tabs 150mg, 300mg, & 600mg Dose 900-2400mg Dosage BID Developed to improve on Tegretol’s side effects If Allergic to Tegretol 20% chance to become allergic to Trileptal Side effects Somnolence H/A Dizziness Rash Weight gain Alopecia Nausea Hyponatremia

Keppra Tabs 250mg, 500mg, & 750mg Dose 500-4000mg Dosing BID Blood levels are drawn but results depend on the pt a “good” level can be from 20s-30s Side effects Mood changes (use of vitamin B6) Lose of appetite Weight lose Diarrhea

Zonegran Caps 25mg, 50mg, & 100mg Dose 200-400mg and can be pushed higher Dosing BID or Daily Levels 10-30 Side effects This is a Sulfa drug Kidney stones Drowsiness Loss of appetite GI disturbance Mania Depression Dizziness Irritability

Ketogenic Diet High fat, low carbohydrate, limited protein diet Simulates metabolism of a fasting state (ketosis) Ketosis has an anticonvulsant effect Used in young children here at Johns Hopkins Epilepsy Center Currently Thomas Jefferson has a program for adults

Nursing issues Avoid medication preparations containing sugar Need daily sugarless multivitamin with iron and calcium Monitor urine ketones Give only allotted noncaloric liquids (may have diet decaffeinated soda)

Vagus Nerve Stimulator Stimulation wires placed around left vagus nerve in the neck. Subclavicular placement of transformer Nerve stimulus is typically on for 30 seconds and off for 5 minutes in cycles (like a buzz) Need to evaluate over 6 months Often palliative treatment (add on to current medications) Magnet swiped over the transformer will cause the buzz to occur longer in order to stop the seizure.

VNS continued Magnet held over the transformer will turn off the VNS. Once the magnet is removed it will turn back on. We in the Epilepsy Center can turn the generator completely off. Status may result from turning off the VNS May be around microwaves and cell phones, etc. Cannot be around MRI unless the VNS is turned off.

Epilepsy Monitoring Performed at large teaching hospitals Most Epilepsy patients respond well to medication Monitoring and visits to Epilepsy Centers is usually reserved for those pts whose seizures are unresponsive to medication

Epilepsy Monitoring Admission for 2-7 days to a special unit Epilepsy medications are reduced before and during admission (if seizures are infrequent) Clinical events (seizures) are recorded and EEG is reviewed and evaluated Type of Epilepsy is diagnosed and seizure onset is lateralized and localized (if possible)

Surgical Management Depth electrodes Epidural Electrodes Subdural Electrodes (Grid) Brain mapping Lobectomy Temporal most common, extratemporal resection Corpus Callosal Atonic or GTC Hemispherectomy

Epilepsy Surgery Some patients may need to have depths placed.

Epilepsy Surgery If not well localized pt may need to have bilateral strips placed to aid in localizing and lateralizing seizure onset

Epilepsy Surgery If pt seizure focus is in or near an area of high function then a subdural grid may be placed to help map area of function and aid in surgery design

Teaching the family and patient About their seizures First Aid Medication and Compliance Diagnostic tests, blood test, surgery Effects on Depression, Memory, Cognitive Function Disability and Work Emotional Support Assist in Problem Solving Their State Driving Laws Support Groups for all ages and their families

Pregnancy Counseling should be done prior to conception age Birth defects (increase by 1-2% above general population) Counseling on AEDs and contraception Interactions AED changes now prior to conception Seizure control on AEDs with only dosage changes during pregnancy AEDs with lowest Birth defect information Folic Acid daily (may be a higher dose) Neural tube development Planned pregnancies

Once Pregnant High Risk OB Increased number of clinic visits with Epilepsy Doctors Communication between OB and Epilepsy MD Teamwork Monthly blood levels with already predetermined target blood level(s) High level of communication with expected mother. (Teaching) Pregnancy Registries (Harvard, Lamictal and Keppra Registries)

Epilepsy Research at Johns Hopkins Neuropace Atkins for Adults Schwarz SP754 Progesterone

Not all patients are surgical candidates For these pts with intractable Epilepsy current research offers hope

Neuropace Surgical Implant A small generator is implanted with electrodes going to various seizure focci

Neuropace Strip or depth leads are placed (1 or 2 leads) A cranial defect is created for the device Each lead can send out an electrical signal to stop seizure activity

Neuropace Age 18-65 years 4 seizures per month or more Able to localize seizure focus 2 or more Epilepsy meds tried VNS will have to be turned off Live locally (lots of clinical visits)

Atkins trial for Adults Age 18 years or older 2 or more seizures per week No heart, kidney, cholesterol problems No major psychiatric problems Need to visit Johns Hopkins 4 times in 6 months Not have tried Ketogenic or Atkins diets prior to this trial

Atkins Pts are placed on a carefully planned Atkins diet and followed for 6 months to evaluated whether their seizure frequency is reduced. All labs, clinic visits and dietitian expenses are funded by the study

Schwarz SP754 Medication trial

Inclusion/Exclusion Inclusion (see attached) Age 16-70 years Partial onset seizures and or complex partial seizures Must have had partial onset seizures for at least 2 years Exclusion (See attached) Hx of drug and or alcohol abuse Medical or psychiatric condition Primary generalized seizures

Benefits Schwarz SP754 Patients are closely followed and evaluated All lab and clinic visits are fully funded by the study Helping a new medication to be developed

Progesterone Therapy for Women with Epilepsy Study Hypothesis: Adjunctive cyclic natural progesterone therapy significantly improves the course of epilepsy in women. Women ages 13-45 with intractable seizures that occur in relation to changes in reproductive hormone levels (catamenial epilepsy) despite trials of at least two AEDs

Inclusion Documentation of focal paraoxysmal EEG discharges 2 seizures/month during previous 3 months Stable optimal AEDs for 2 months Nl breast exams and PAP smears 9 months prior for all over 21 Menstrual cycle intervals between 23 and 35 days during 6 month prior Sexually active women will use barrier and/or spermicidal forms of contraception

Exclusion Pregnancy, lactation Progressive Neurological disorder Abnl Liver function test Major tranquilizer or reproductive hormones 3 months prior to study Sensitivity to natural progestrone Unable to document seizures or follow protocol Hx of thromboembolic, thrombophlebitis disorders, CVA Malignancy of breast, uterus or ovary Vaginal bleeding

If interested in participating in a study Call 410-955-4835 You will then be referred to the appropriate office

Bill of Rights for People Living with Epilepsy Sponsored by Novartis Pharmaceuticals Corporation

The goal of the Bill Of Rights is to help you: Encourage the patient’s involvement in managing many aspects of living with their epilepsy, including making informed choices and activity participating in decisions about care Empower them to ask questions and seek answers from their or their loved one’s healthcare team Help them access information and support resources Encourage them to speak up for their or their loved one’s rights and needs

A first-of-its kind initiative Designed to educate, empower, and increase understanding of epilepsy A guide to managing life with epilepsy that was developed by the community for the community It is not a legal document- these rights are aspirational goals that the epilepsy community is trying to achieve None of the information should be used as medical or legal advice

Goals of the Bill of Rights Goal #1: Educate and empower the people affected by epilepsy Goal #2: Increase understanding of epilepsy among the general public

Includes guidance on topics, such as: Social aspects of living with epilepsy Communicating with your healthcare team Current information on epilepsy and treatment options Rights at school Rights in the workplace

Enroll in the SHARE (Support, Hope, And Resources for Epilepsy) Program to receive Bill of Rights materials and additional information about living with epilepsy from Novartis Complete and mail your business reply card Visit www.EpilepsyBillofRights.com Call toll-free 1-877-6ERIGHTS

Resources Epilepsy Foundation of America 1-800-332-1000 www.epilepsyfoundation.org American Epilepsy Society 1-860-586-7505 www.aesnet.org American Association of Neuroscience Nurses www.aann.org

Resources continued MedicAlert Foundation 1-888-633-4298 www.medicalert.org Food and Drug Administration 1-888-INFO-FDA www.fda.gov