SHAKE, RATTLE, & ROLL

Transcription

1 Epilepsy (Beyond seizure) 70% of seizures are idiopathic (cause not identified-likely genetic) +/- family history Cryptogenic-likely a cause, just not identified Symptomatic-cause identified Now it gets tricky.. Provoked vs Unprovoked But first, lets review the basics SHAKE, RATTLE, & ROLL Caroline Rich, RN, MSN, CPNP, C/PC Helen DeVos Children s Hospital Pediatric Neurology Seizure Types Simple partial (focal) NO altered consciousness can have aura. Sudden, brief, feelings of fear, anger, sadness, happiness, nausea. Sensation of falling or movement. Unusual feelings or sensations Altered hearing, taste, vision hard to explain déjà vu Can recall event Affects one hemisphere Focal motor Can progress to complex and/or generalized 2 Seizure Seizure Types-(pay attention I m going to quiz you in 3 minutes) Sudden stereotyped episode with change in motor activity, sensation, behavior, and/or consciousness Due to an abnormal electrical discharge in the brain-neurons (nerve cells) communicate with one another through tiny electrical impulses. Seizure happens when a LARGE number of cells send out an electrical charge at same time. This overwhelms the brain and results in a seizure. Complex partial (focal) Same as simple partial but WITH alteration in consciousness Can be preceded by aura Automatisms-lip smacking, chewing, swallowing 1-2 minutes Usually no recall Affect more of the brain than simple, but still limited to one hemisphere May seem aware, may be able to walk, will stare Postictal state Focal motor Can progress to generalized 4:3 Screen Ratio Template HDVCH October

2 WHAT IS DIFFERNCE BETWEEN SIMPLE PARTIAL AND Complex PARTIAL SEIZURE ALTERATION IN CONSCIOUSNESS Seizure Types Generalized seizures-can be convulsive or non convulsive -can be associated with epilepsy syndromes (JME, CAE, IS, LGS, BRE). Affect BOTH hemispheres. Positive loss of consciousness; no recall. Generalized seizure types 1. Absence (petit mal)-no aura, brief, few seconds, stare, appears vacant, very subtle, may pause mid sentence. Can be confused with daydreaming. No PI state, immediate return to baseline. * 2. Myoclonic rapid, brief contraction of muscles, +/- bilateral. Sudden jerks. Often occur during first few hours after waking. Quick recovery. Hypnagogic jerk-not seizure, happens when falling asleep 3. Tonic-rigid and stiff, 5-15 seconds. Usually fall backward. 4. Tonic-clonic-(grand mal)-initial contraction of muscles, may involve tongue biting, urinary incontinence, grunt, and apnea; followed by rhythmic muscle contractions 5. Atonic-abrupt loss of muscle tone, drop attacks, head drop, loss of posture, sudden collapse. Usually fall forward. Quick recovery 4:3 Screen Ratio Template HDVCH October

3 Case STUDY 1 John is a 9 yr old male, previously healthy without recent fever or URI symptoms. No history of trauma or concussion. John presents to your clinic today for evaluation of suspected seizure. Parents report that around 3 am, John wandered into their bedroom, he was making unintelligible sounds, but could follow simple commands. His eyes were open but he didn t seem to be looking at us. He had drool around his mouth, which was twitching. He then proceeded to have generalized tonic stiffening and clonic jerking of his extremities. What is your initial diagnosis? complex partial (focal) seizure EXTRA CREDIT---- secondary generalization First seizure-john 25,000-40,000 American children/year present with first seizure Was it a seizure Detailed description of event Medical history Family history Was it really the first seizure?? What tests are (not) needed? No reliable test (EEG +/- helpful) What treatment is (not) indicated? So everyone gets a freebie? You tell me. Some causes in kids.go AHEAD-PROVOKE ME Fever Metabolic derangements Brain infection-meningitis, abscess Brain injury-trauma Congenital brain defects Brain tumor Drug ingestion Stroke 4:3 Screen Ratio Template HDVCH October

4 UNPROVOKED SEIZURE Why do we care?? Work up, treatment, education, prognosis It changes everything Do we treat provoked seizure? That s a tough question to answer today Case 2 An 18 mos old girl is brought to the office/ed following a 90- second generalized tonic-clonic seizure. She has been ill with URI symptoms and a temp of 39C. On exam, her temp is 38.6C, is awake and playful, and has no focal neurological signs. FEBRILE SEIZURE Acute evaluation-some considerations Febrile seizures ABC s Place IV Labs-CBC, BMP CT head Lumbar puncture Primary focus is stabilization Very common (3-4%) Peak age months (range 6 mos-6 yrs) Seizure in association with a fever >38.5C Otherwise normal child +/- family history of epilepsy Nursing Role-Safety Febrile seizure Place on ground, side lying if possible; do not restrain Nothing in mouth ABC s Prepare to give rescue medication at 3-5 minute mark Rescue meds/ rescue plan from neurologist Memory recall pink elephant Details are important, but do not compromise your care Evaluate neuro response after seizure Check for symmetry, coordination, strength Hand grips, RAM, toe tapping, arm rolling, orientation/confusion Simple Generalized Duration <10 mins 1/24 hr Most common AED very rarely needed-if ever Complex Focal Duration>15 mins (status) >1/24 hr ~25 % of febrile seizures May need AED, rescue med usually provided 4:3 Screen Ratio Template HDVCH October

5 Case 2 What kind of seizure did she have? Simple febrile HOW DO YOU KNOW? Short duration GTC Once Febrile seizure-eval AAP guidelines (2011) In general, a simple febrile seizure does not usually require further evaluation, specifically EEG, blood studies, or neuroimaging With the data you have now, does she require a daily AED? No, but does she require any medicine? Consider rescue drug (diastat) Febrile seizure-eval Determine if complex vs simple Thorough history and exam Diagnostic studies considered LP should be considered if Clinical suspicion of CNS infection Young child (<6 mos), and/or persistent altered mental status, or failure to return to baseline Febrile seizures-management Fever control does NOT prevent febrile seizures AAP guidelines 2008 Febrile seizures-eval EEG Not recommended after a simple febrile seizure Not predicative of recurrence or future development of epilepsy Neuroimaging Not recommended Risk of radiation/sedation Other Blood tests considered only to identify source of fever Febrile seizures- prognosis Excellent, except: 30-50% risk of recurrence Doubles the risk of epilepsy, from 1% to 2% Simple febrile seizures do not lead to neurological impairment Expect normal academic achievement and social accomplishments Confirmed by multiple studies in US, Denmark, Finland, Taiwan Important for all health care providers to give the same optimistic and reassuring information 4:3 Screen Ratio Template HDVCH October

6 Case 1-John had another one Neuroimaging 1 in 26 Americans will develop epilepsy in their lifetime People with epilepsy can achieve greatness. Julius Caesar, Napoleon, Joan of Arc, Van Gogh, Socrates, Dostoevsky, Tchaikovsky, Danny Glover Does he have a tumor? A single seizure is an extremely rare presentation for most tumors Most kids do not need imaging Focal seizure-should image-structural lesion? Although incidence of lesions requiring acute intervention in children presenting with first seizure is ~2% Diagnostic work up Treatment-CNS 2003 EEG Optimal timing not clear-usually ok to do as outpatient If EEG done within hrs after seizure, more likely to be abnormal Some abnormalities, like post ictal slowing, may be transient and should be interpreted cautiously Normal EEG does not rule out seizure, but can be useful for diagnosis of seizure vs NES EEG can provide useful information, even when not actively seizing. Can be predictive of recurrence. LP NOT recommended routinely following a single unprovoked seizure After a single seizure, treatment with daily antiepileptic drug (AED) is almost never required. Risk of recurrence RISK VS benefit After single, unprovoked seizure If generalized with normal EEG and normal exam=25-40% chance within 2 years (either abnormal=50%, both 75%) If recurrence, 90% occur in first 2 years If focal with normal EEG and exam=50% chance within 2 years (either abnormal=75%, both >90%) Risk of second seizure (without treatment) is 30-35% Risk of third seizure (without treatment) is 70-75% Risk of fourth seizure (without treatment) is up to 90% Would you gamble with those odds? Pay attention Im going to quiz you in 2 mins 4:3 Screen Ratio Template HDVCH October

7 Diagnosis of epilepsy Educate family to describe spell Recurrent unprovoked seizures-more than one seizure 24 hrs apart A normal EEG does not rule out diagnosis of epilepsy-clinical history very important Body-whole, right, left, unsure? Movement-jerking, stiff, jerking and stiff, unsure? Eyes-up, closed, right, left, stare, stare and blink, no change, unsure? Skin color Accident-urine, stool, none Mouth-dry, drool, foam, tongue bite How often? After seizure-asleep, drowsy, alert, confused, paralyzed, vomiting LET S TALK ABOUT JOHN Safety Normal EEG Normal MRI Does he need daily AED? Yes WHY? Two unprovoked seizures What are the chances of third seizure without treatment? 70-75% Challenge for maintaining independence and participating fully in social interactions Michigan Law and driving-6 mos seizure freedom Parents perceive increased disability when a health care provide recommends restrictions Cognitively normal kids with epilepsy have the same rate of injuries as children without epilepsy ( Epilepsia 2006) Now what?? Family anxious. What if it happens again and we aren't near hospital? We need to empower them Education Rescue medication Rectal diazepam, oral clonazepam, intranasal midazolam Can I play sports? YES!!!! Encourage participation Contact sports are not precluded Swimming and water sports, horseback riding, gymnastics and harnessed climbing are safe with appropriate supervision Free climbing, sky-diving, and scuba diving are NOT safe Everyone should wear a helmet with bike riding. Kids with absence epilepsy are at high risk for accidents EPILEPSY FOUNDATION 4:3 Screen Ratio Template HDVCH October

8 AED Basics Goal of treatment is lowest dose that is effective, monotherapy, and least side effects. Our goal is 0 seizures and 0 side effects Lab considerations Compliance issues Bioavailability Some fun with drugs Levetiracetam -Keppra Oxcarbazepine -Trileptal Phenytoin -Dilantin Topiramate -Topamax Clobazam -Onfi Vigabatrin -Sabril Lacosamide -Vimpat Valproate Acid -Depakote Rufinamide -Banzel Zonisamide -Zonegran Ethosuximide -Zarontin Lamotrigine -Lamictal Phenobarb -Luminol Gabapentin -Neurontin Intranasal Midazolam 1.Intranasal midazolam also offers an effective treatment option for patients with prolonged seizures. Midazolam easily crosses the nasal mucosa and the blood-brain barrier, resulting in a rapid rise in both the plasma and the cerebrospinal fluid concentrations. 2.Compared rectal diazepam with intranasal midazolam and found intranasal midazolam faster in onset and more effective at seizure cessation (60% vs 87%). Compared with intravenous diazepam, intranasal midazolam had similar efficacy (92% vs 88%) and faster onset as a result of the lack of need to start an intravenous line 3. Intranasal midazolam and lorazepam are also safe for treating seizures outside the hospital setting Emergency Meds Dosing of nasal midazolam Ativan, diastat, versed, clonazepam-first line benzos!!! Give for seizure greater than 5 minutes Why at FIVE? Still seizing at 10 minutes repeat benzo but 911 or emergency equipment should be available (respiratory depression) Repeating benzos after second round will likely be ineffective receptors are saturated. Still seizing at 15 minutes hopefully en route--- IV Fosphenytoin, levetiracetam, phenobarbital Easy dosing all 20 mg/kg/dose Midazolam 5mg/ml Atomizer contains 10 ml Each spray =0.1 ml Five sprays is 2.5 mg Ten sprays is 5 mg Remove plastic cap Any head position Tip of atomizer into one nostril and spray, repeat in other nostril 4:3 Screen Ratio Template HDVCH October

9 Can t be too safe.. Learning differences W A T E R S A F E T Y Showers preferred, no tub bath unless direct supervision Unlock door, regulate hot water temperature While swimming designate specific supervisor-life jacket S A F E T Y - O T H E R Clothing irons Curling irons Stove top cooking Camp fires Top bunks Doors open outward Risk for decreased academic achievement, memory, behavior scores Despite seizure control Despite normal range IQ Learning problems predate seizures Not solely attributable to AED s Increased with disorganized, unsupportive home IEP or 504 usually necessary No child left behind Video games Learning differences Japanese cartoon triggers seizures in hundreds of children (Dec 1997). Only matters if photosensitive Bright ambient light Back up!!! ADHD: 38% of children with epilepsy Independent risk factor for trouble School, injuries, drug use, car accidents Stimulants NOT contraindicated Methylphenidate =safe and effective Atomoxetine=alternative Epilepsy Behavior 2011 Mental health Other concerns for kids with epilepsy D E P R E S S I O N I N D E T R O I T 1/3 screen positive Short mood and feelings questionnaire Of whom 1/3 accessed mental health care Epilepsia 2009 A N X I E T Y I N L. A. CAE or LRE: 5x more affective or anxiety d/o 20% suicidal ideation 1/3 accessed mental health care Epilepsia 2005 BONE HEALTH Epilepsy =risk for poor bone mineralization Seizures-risk for fall and fractures Complex problem Vitamin D deficiency 60% of all US children 75% of Michigan children with epilepsy Calcium AED s Co-morbid neuromotor dysfunction Lack of physical exercise Pediatric Neurology :3 Screen Ratio Template HDVCH October

10 Reproductive health Non epileptic seizures Teens with epilepsy are at high risk for unplanned pregnancy AED s can make hormonal contraceptives less effective VPA can cause PCOS Infants/toddlers/young kids GERD (sandifers), parasomnias, benign sleep myoclonus, exaggerated startle, breath holding spells, BPV Carbamazepine and VPA can cause neural tube defects All AED s teratogenic Recommend folic acid 5 mg daily What about Mom and Dad? Epilepsy affects whole family Up to 49% of mothers are depressed: Younger, less educated, lower family income Relational difficulties Worry Behavior problems in affected child (ADHD) Ask family how they are doing.. Epilepsia 2009 What about the sibs? Study of 127 sibs (5-18 yrs) of 78 patients with epilepsy 95% had witnessed a seizure and 79% think sib suffers during seizure 68% say sib gets more attention 42% say they feel responsible for sib, and curtail their own activities 45% tell their friends Seizure 2006 NES Adolescents-stress, anxiety, depression Stressful event, response to suggestion Occur in wakefulness, witnesses Convulsions are asynchronous, asymmetrical, waxing and waning, accelerating and decelerating; movements include pelvic thrust, flailing, and tremors Retained consciousness, NO PI state, may cry Intractable to AED or emergency meds Staring **during a spell of unresponsiveness, and increase in HR of 30% baseline is 97% predicative of ES At least one of the usual signs of GTCS (incontinence, tongue biting, falling) is reported by more than half of the patients with NES 4:3 Screen Ratio Template HDVCH October

11 NES Treatment History and clinical description are key Despite a normal EEG, normal neuro exam, developmentally healthy child---it is very difficult for families to accept diagnosis Anxiety is a real diagnosis Conversion disorder is common Counseling and/or psychiatry Often times these patients require more time than true epilepsy patients Need support from all involved with child Actually, kids with epilepsy can also have NES websites Epilepsy.com Purpleday.com Epilepsymichigan.com Thank you!!! Caroline.rich@helendevoschildrens.org 4:3 Screen Ratio Template HDVCH October