And They All Fall Down

And They All Fall Down Amy Vierhile, DNP, RN, PPCNP BC Division of Child Neurology University of Rochester Medical Center Disclosures Research funding: Greenwich Pharmaceuticals, Biohaven, Teva, NINDS Advisory Board: Tris Pharma Epilepsy A seizure is a transient disruption of brain function due to abnormal and excessive discharges in brain cells Epilepsy predisposes a person to recurrent unprovoked seizures, defined as two or more unprovoked seizures. Unprovoked means that the seizures occur when an acute brain disturbance cannot be identified as the cause of seizure, in spite of neurological evaluation 1 in 26 people will develop epilepsy in their lifetime; 1% of the U.S population has epilepsy 1

Adults versus children Adults: over 18 more likely to develop due to a physical cause, such as a stroke, not likely to resolve completely, can be controlled with medication or devices Children: often idiopathic, 80% of children s seizures resolve, they can come off medication; this is due to brain plasticity EEG Sleep deprivation important Not all abnormal EEGs indicate seizures Normal EEGs can occur in people with seizures Generalized seizures are easier to pick up on an EEG May need 24 hour EEG or long term monitoring to capture an episode Sometimes EEG results can guide you to an MRI 2

This is an abnormal waking EEG due to the presence of generalized epileptiform abnormalities. During one discharge burst, there was a jerking movement of the legs and the patient leaned backwards. The EEG background was otherwise normal. These findings are consistent with a generalized epilepsy. This is an abnormal 24 hour ambulatory EEG due to the presence of: 1. Diffuse slowing of the background with lack of appropriate organization 2. Very frequent, independent, multifocal discharges emanating from the right central, bilateral parietal and occipital regions. The occipital discharges were substantially augmented by sleep. There were no electrographic seizures. These findings reflect multiple areas of increased epileptogenic potential, which include central, parietal and occipital regions with superimposed diffuse, moderate encephalopathy. Three events were noted during this 24 hour study. There were no electrographic correlates with these three events. IMPRESSION: This is an abnormal 24 hour ambulatory EEG due to: 1. A total of 18 electrographic seizures arising from the frontal region bilaterally with 2 different ictal patterns electrographically depending on whether they arose from wakefulness or from sleep. Some of these seizures may have had a right hemispheric predominance. 2. Rare, bifrontal spike and slow wave epileptiform discharges. These findings are consistent with a localization related epilepsy with ongoing seizure activity over the frontal regions bilaterally with a possible right hemispheric predominance. A total of 11 patient events were captured on this study, 8 of which correlated with seizures electrographically. The other 3 were not clearly associated with an ictal pattern although were obscured by prominent artifact. IMPRESSION: This is an abnormal waking and sleep EEG. There are focal epileptiform discharges on the left frontal area that was more discharged with drowsiness and sleep. These findings are consistent with results of previous studies. This is an abnormal waking EEG due to 1. Persistent delta slowing in the left hemisphere with loss of faster frequency activity. 2. Epileptiform discharges in the left frontotemporal region. 3. Diffuse background slowing in the right hemisphere. These findings indicate 1. Underlying structural/functional lesion in the left hemisphere 2. High epileptogenic potential in the left frontotemporal region. 3. The diffuse slowing in the right hemisphere is most likely related to a postictal state and sedating medications. This is an abnormal EEG due to frequent, diffuse, theta frequency slowing during wakefulness, with preservation of normal background organization. This finding is consistent with a mild, diffuse encephalopathy and similar to the results of previous study from 3/2/12. There are no epileptiform abnormalities. Neuroimaging should be considered. No seizures were seen in the recording. 3

Generalized seizures Discharges come from all parts of the brain at once More common than focal seizures Children are more likely to outgrow these Typically entire body is involved: stiffening, shaking, staring Grand mal (tonic clonic) are the most commonly recognized Infantile spasms Onset between 4 8 months of age More common in certain syndromes (Down Syndrome, tuberous sclerosis); important to determine the cause Subtle flexion and extension movements Occur more often upon awakening (naps and in the morning), often in clusters EEG is hypsarrhythmia total chaos Leads to developmental regression Treatment is ACTH, vigabatrin Absence seizures Most commonly seen in children ages 4 10 Short, often abrupt spells of arrest, lasting 10 15 seconds, cannot be interrupted Typical EEG pattern: 3 Hertz pike and wave Can be outgrown without medication; some people will have them along with other seizure types Usually treated due to frequency Can be provoked by hyperventilation Treatment is ethosuximide, valproic acid or lamotrigine 4

Tonic clonic seizures May start with tonic (stiffening) phase, then progress into alternating tonic and clonic (flexion) movements Last 3 minutes or less for most people; no brain damage No need to intervene usually: do not insert anything into the mouth, turn person on their side if they seem distressed Post ictal period of confusion, sedation can last a few minutes several hours Some patients will use rescue medication Treatment is levetiracetam, zonisamide, lamotrigine, valproic acid Other generalized seizures Myoclonic quick jerk, often in clusters Tonic stiffening Atonic drop seizures, usually collapse and recover quickly Juvenile Myoclonic Epilepsy (JME) onset in adolescence; morning jerks and early morning tonic clonic seizures Medications used to treat generalized seizures Most of these have approval to treat focal seizures as well Older medications Depakote (valproic acid), Lamictal (lamotrigine) have very widespread seizure control Newer medications: Keppra (levetiracetam), Zonegran (zonisamide) Specialized medications: Onfi (clobazam) and Banzel (rufinamide) are only for Lennox Gastaut Syndrome Less effective medications: Neurontin (gabapentin), Topamax (topiramate) 5

Depakote (valproic acid) Effective for many seizure types Common side effects: increased or decreased appetite, stomachache, sedation, hair loss, nausea, dizziness Less common side effects: pancreatitis, PCOS, thrombocytopenia Known teratogenicity with neural tube defects Need for hepatic panel, platelet count every 3 months for first year, then twice a year Women of childbearing potential should be weaned off; if they can t be, need 4 mg folic acid QD Drug interactions: lamotrigine, other AEDs, TCAs Pregnancy category: D Lamictal (lamotrigine) Great drug for many seizure types, non sedating Need to start low, go slow Common side effects: insomnia, decreased appetite, drowsiness Less common side effects: Stevens Johnson Syndrome Interacts with Depakote (need to start even slower) Good for mood stabilization as well Drug interactions: oral contraceptives (low LMT level), carbamazepine, Metformin Pregnancy category: C Zonegran (zonisamide) Good for occipital seizures Long half life, so wait 2 3 weeks between dose increases, can give only at night Common side effects: sedation, weight loss, dizziness Less common side effects: kidney stones, nausea, double vision Drug interactions: minocycline, SSRIs (can enhance CNS depressant effect) Pregnancy category: C 6

Keppra (levetiracetam) Broad spectrum, easy to dose (liquid and capsules) May worsen behavior, especially in small children; Vitamin B6, 100 mg per day thought to lessen that effect Not well tolerated by older adults can lead to agitation, confusion Common side effects: sedation, hypertension, behavior problems, headache, vomiting Less common side effects: abdominal pain Interactions: SSRIs (enhanced CNS effect) Pregnancy category: C Zarontin (ethosuximide) Used only for absence seizures Will make other seizure types worse Common side effects: sedation, stomach ache Less common side effects: aggression, ataxia, depression, agranulocytosis, leukopenia Interactions: amphetamines, minocycline, other AEDs Pregnancy category: C Topamax (topiramate) Good for migraine headaches, mood control, less benefit as an antiepileptic Need to increase fluids while on Topamax Warn patients about sudden onset of eye pain Common side effects: parasthesias, loss of appetite, word finding (dose related) Less common side effects: Wide angle glaucoma, kidney stones Interactions: Oral contraceptives, amitriptyline, metformin, amphetamines, thiazide diuretics Pregnancy category: D 7

Focal seizures Caused by a focus scar, cyst, tumor, heterotopia (undeveloped area) More difficult to pick up on EEG, often lifelong Many people who have benign MRI findings do not develop focal seizures May start with an aura (smell, taste, feeling) Onset is one side of the body, may or may not spread Consciousness is impaired, but often not fully May present as unusual episodes: déjà vu, confusion, night terrors Treatment of focal seizures May resect if focus found but often cannot resect Medications used to treat focal seizures are liver enzyme inducers, tend to interact with non inducers (i.e. carbamazepine and valproic acid) Carbamazepine, Dilantin, Phenobarbital Enzyme inducers tend to increase the rate in which the liver breaks down contraceptive hormones Can get tricky when combining several medications May need long term monitoring to determine if truly seizures Newer medications have fewer side effects Tegretol (carbamazepine) Older medication, newer cousin (oxcarbazepine) has fewer side effects Common side effects: sedation, weight gain, nausea, vomiting, dizziness, hyperactivity Less common side effects: aplastic anemia, Stevens Johnson Syndrome or allergic rash Teratogenicity with neural tube defects, cardiac and facial malformations Drug interactions: CYP3A4 inducers, oral contraceptives, macrolides, SSRIs Pregnancy category: D 8

Trileptal (oxcarbazepine) Cousin to carbamazepine, fewer side effects and interactions Less likely to cause allergic reaction Common side effects: dizziness, drowsiness, headache, double vision, tremor, ataxia, vomiting Less common side effects: diarrhea, emotional lability Drug interactions: oral contraceptives, most other AEDs Pregnancy category: C Dilantin (phenytoin) Older AED Small dose changes result in large blood level changes Common side effects: gum hypertrophy, sedation, mental slowing, ataxia Less common side effects: peripheral neuropathy, agranulocytosis Drug interactions: oral contraceptives, CYP3A4 inducers, acetaminophen, amphetamines, SSRIs, many medications Pregnancy category: D Vimpat (lacosamide) Newer AED, generally well tolerated and effective Common side effects: dizziness, fatigue, headache, nausea, vomiting, double vision, tremor Less common side effects: diarrhea, nystagmus Drug interactions: bradycardia causing agents, CYP3A4 inhibitors Pregnancy category: not yet determined 9

PNEA (psychogenic nonepileptic attacks) Can occur along with real epilepsy No EEG correlate Rarely hurt themselves Often a manifestation of anxiety Treatment: psychotherapy (tough love approach) and/or anxiety medication if needed MRI and CT scan Not needed for most seizure types, often done unnecessarily in ED Irradiation from CT scans is risk For focal epilepsy, MRI with and without contrast can be helpful Most of the time no focus is found Reassure patients that MRI and CT scan are not always helpful Seizure First Aid Main goal is to protect the patient from harm Time the seizure if possible Never put anything in a person s mouth Turn on their side if excess saliva Use rescue medication if prescribed clonazepam, Diastat, buccal midazolam 10

Other seizure information Restrict climbing over 5 feet unharnessed Encourage buddy approach for activities Consider medical alert bracelet Showers instead of baths, keep door unlocked Can t drive for 1 year seizure free, need to give up license when weaning medication For children, may need to ask for school transportation; 911 does not need to be called for each seizure Seizure Mimics Confusional Migraine Can be tricky to tease out, more common in children and teens Sudden, intense confusion, lasting from a few minutes to a day Slurred speech, drooping of one side of mouth, numbness in extremities, blurred vision, agitation Headache may precede or follow attack, usually not as long as confusion Episode followed by sleep No loss of consciousness Family history of migraine 11

Myoclonus Sudden involuntary jerking of a muscle or group of muscles May be benign or preceding symptom of a disease multiple sclerosis, Parkinson s disease, Alzheimer s disease Sleep myoclonus most common when drifting off to sleep If concerned about seizure, need to capture on EEG Syncope Temporary loss of consciousness due to lack of blood flow to brain May have twitching or limb jerking with syncope Often seen in adolescents during growth when standing up quickly they describe tunnel vision or gray/black vision, nausea, feeling warm and lightheaded Situational syncope caused by trigger, such as fear or needles or blood, fear, hunger, urination, exercise Treatment of syncope is increased salt in diet, increased fluids, move positions slowly Cardiology consult can be helpful Infantile gratification Starts in infancy, peaks around age 4 Usually girls, often with strong personalities Often mistaken for seizures or dystonia; can be interrupted Often treated with numerous medications without benefit Occur during down time when in the carseat, high chair, bored, excited Tensing and contracting of muscles, applying pressure with external surface, may be grunting, flushing, can lead to perineal irritation May be soothing 12

Conclusion Seizures always have some level of altered awareness They cannot be interrupted If a routine EEG does not answer the question, a longer one may be needed may need to capture an episode No need for MRI or CT unless focal epilepsy Check to make sure AEDs are not interacting with newly prescribed medications Questions? 13