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You’ve Got Nerve: Clinical Pearls of Epilepsy. Akilah Mikel, MSN, FNP-BC Pediatric Specialists of Virginia Neurology Nurse Practitioner. No Disclosures. Objectives. Describe seizures and epilepsy according to standard classification Discuss common types of seizures
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You’ve Got Nerve: Clinical Pearls of Epilepsy Akilah Mikel, MSN, FNP-BCPediatric Specialists of Virginia Neurology Nurse Practitioner
Objectives • Describe seizures and epilepsy according to standard classification • Discuss common types of seizures • Explain the diagnostic evaluation of seizures • Gain some familiarity with anti-seizure treatment • Discuss safety and management of seizures
Seizures have been documented since the earliest civilizations • An account of a boy with epileptic-like seizures is recorded in 3 of the 4 gospels of the bible using phrases like “possessed by a spirit”, “a spirit seizes him (Matthew 17:14–18; Mark 9:14–29; Luke 9:38–42) • The word epilepsy comes from the Greek verb epilambanein (over seize), derived from the notion that gods take hold or “seize” a person
Definitions • Seizure – a disturbance in the electrical activity of the brain • Epilepsy – two or more unprovoked seizures separated by more than 24 hours • Proposed ILAE (International League Against Epilepsy) definition – one seizure and risk for additional seizures >60%
Epilepsy Epidemiology • 2.2 million people in the United States and more than 65 million people worldwide • 150,000 new cases of epilepsy are diagnosed in the United States annually • 1 in 26 people in the United States will develop epilepsy at some point in their lifetime
New Onset Seizure Evaluation • Electrolytes? • Lumbar puncture? • EKG? • Neuroimaging? • EEG? • Other?
Seizure Differential Diagnosis • Syncope • Pseudo-seizures • Breath-holding • Sandifer syndrome-related to reflux • Migraine • Movement Disorder • TIA • Sleep myoclonus
History • Pre-monitory • Ictal • Post-ictal • Other spells • Tongue biting • Incontinence
Neuroimaging • Almost always indicated for seizure evaluation • MRI (Magnetic Resonance imaging) preferred 3 Tesla (T) epilepsy protocol • Look for signs of tumors, stroke, infection, structural abnormalities in the brain such as hydrocephalus and vascular malformations that would indicate a cause for seizures
Neuroimaging • Immediate noncontrast CT is possibly useful for emergency patients presenting with seizure to guide appropriate acute management especially where there is an abnormal neurologic examination, predisposing history, or focal seizure onset.
EEGElectroencephalogram • A non-invasive procedure that detects abnormalities in the brain waves, or in the electrical activity of the brain. • It records the brains electrical activity as a series of traces. Each trace corresponds to a different region of the brain • Activation procedures: Photic stimulation (flashing lights) and hyperventilation (breathing fast for 3 min)
EEG Electrodes consisting of small metal discs with thin wires are pasted onto the scalp.
EEG • Useful for evaluating new onset seizure: • Prognosis • Focal vs. generalized • Epilepsy syndrome • 25-60% chance of catching abnormal discharges on routine EEG • A negative EEG does not exclude the possibility of seizures • Prolonged video EEG when indicated
Generalized Seizures-affect the entire brain • Tonic Clonic (rigid/jerking) • Absence (starring spell) • Other motor including: • Myoclonic (muscle jerks) • Atonic (drop seizure) • Epileptic Spasms
Focal seizure-affect part of the brain • Aware: Alert, involuntary movement, odd taste/smell, fear/happiness, sweating/goose bumps • Impaired awareness: Aura, unconscious, lip smacking, meaningless movements, picking at clothing, wandering around, repeating words
Examples of Seizures Generalized seizure: Focal Seizure:
Febrile Seizures • 2-5% of children (Hauser 1975, Nelson 1978, Offringa 1991) between 6 months and 6 years of age • Simple vs complex • Complex: • Duration > 15 minutes • Focal features • ≥ 2 in 24 hours
Febrile Seizures: management • Anti-pyretics do not decrease risk for febrile seizures with subsequent illness • However, they (acetaminophen) may decrease risk for febrile seizures within the same illness (9% vs 24%) (Murata et al., 2018)
Febrile Seizures: Prognosis • Overall, 1/3 have recurrence of FS • ~10% have ≥3 FS • 1% without risk factors will develop epilepsy by age 7 • Compared to 10% with 2/3 risk factors – complex, preexisting neurological abnormality, family history of afebrile seizures Nelson & Ellenberg (1978)
Infantile Spasms • Clinical – usually trunk flexion and extremity extension in clusters • 50-60% continue to have seizures (mostly LGS) • 71-81% MR • West syndrome = spasms, psychomotor deterioration, and hypsarhythmia • Tx Corticotropin (ACTH) or high dose steroids, alternatively Vigabatrin
Absence seizures • Starring spells • <30-40 seconds • No post-ictal • Multiple per day • 3 Hz spike-wave • Can be triggered by hyperventilation
Benign Epilepsy with Centrotemporal Spikes (BECTS) • Onset 3-14 years old; most outgrow by 16 years • Most common focal childhood epilepsy • Seizures from rolandic cortex • 80% have < 6 seizures • Classically, unilateral face, tongue, and/or hand/arm clonic jerking/ parasthesia in early morning or while asleep – may be on either side; often retained awareness but expressive aphasia in ictal/ post-ictal phase • Seizures may generalize • Central-temporal spikes, activated by drowsiness/ sleep
Juvenile Myoclonic Epilepsy • Most common generalized epilepsy in adolescents • Myoclonic, absence, and GTC seizures usually after morning awakening, provoked by sleep deprivation, alcohol • Myoclonic jerks necessary for diagnosis, predominate in upper limbs • 4-5 Hz generalized polyspike and slow wave discharges; photosensitive • Most easily treated; 16% pharmacoresistant
Pseudo-seizure • Also known as: psychogenic non-epileptic seizures (PNES), conversion disorder, functional neurologic disorder • A non-epileptic seizure: no abnormal electrical activity in the brain correlating with the event • Tx: Cognitive behavioral therapy, psychiatric evaluation, PT/OT
Pseudo-seizure • Risks in children: history of abuse, depression, anxiety, school phobia, mood disorders, separation anxiety, ADHD, panic disorder, PTSD, cognitive impairment, learning disability, epilepsy • Video EEG is the gold standard for diagnosis • Common reason for admission to EMU; about 20-30% with refractory “seizures” have been misdiagnosed and most are PNES
Status Epilepticus • Single seizure ≥ 30 minutes • Multiple seizures without regaining consciousness • Impending status epilepticus (Chen JWY and Wasterlain CG, 2006) or early status epilepticus (Shorvon S, 2001) = 5 minutes • Status epilepticus: • Convulsive • Non-convulsive • Incidence 20 episodes per 100,000 per patient-year • 1/3-1/2 present with SE as their first seizure
Status Epilepticus Etiology • Missed medications (history of epilepsy) • Febrile status epilepticus • Meningitis/ encephalitis • Electrolyte disturbance, hypoglycemia • Traumatic brain injury • Toxin/ overdose • Stroke, Tumor, other lesion • Eclampsia
SUDEP • Sudden Unexpected Death in Epilepsy • Each year about 1 in 1000 adults and about 1 in 4,500 childeren with epilepsy dies from SUDEP • No known cause of SUDEP • Risk increases with poorly controlled epilepsy (particularly GTC seizures) • Best prevention is to control seizures
Management • ABCs • High flow O2 via non-rebreather mask • Do not put anything in the mouth • Do not restrain the patient, but ensure they are in a safe place • May need to help keep the airway open • Turn patient on his/her side when possible to prevent aspiration • Check blood sugar, electrolytes (more important if no history of epilepsy)
New Onset Seizure: To Treat or Not to Treat? • Effect on long-term prognosis • Generally do not treat first unprovoked seizure • Recurrence risk- 50% within 2 years after 1st unprovoked
Common Anti-Epileptic Drugs • Medications: • Broad-spectrum – levetiracetam, lamotrigine, valproic acid, phenobarbital, etc • Focal – oxcarbazepine, carbamazepine, phenytoin, etc • Absence – ethosuximide
New AEDs • Cannabidiol (Epidiolex-Lennox Gastaut, Dravet syndrome) • Brivaracetam (Briviact-Focal) • Steripentol (Diacomit-Dravet syndrome)
Medication Management • To avoid side effects, medications are gradually titrated to a goal dose based on weight. There is a risk for seizure during this titration period. • Ensure that medications are taken at the same time twice daily (use alarm/pill box for reminders) • Labs are generally checked at trough levels • Unless otherwise instructed contact your neurology team with every seizure
Medication Management • Vaccinations are generally recommended including the flu shot (except for the nasal spray) • Please do not stop medications abruptly without contacting your neurology team • If seizure free for 2 years, your neurologist may discuss weaning seizure medication if patient has a normal EEG
Non-medication therapies • Vagus Nerve Stimulator (VNS)-prevents seizures by sending regular, mild pulses of electrical energy to the brain via the vagus nerve • Deep Brain Stimulation (DBS)-electrodes placed in target areas in the brain provide brain stimulation to help stop the spread of seizures • Ketogenic Diet- 3-4 gms of fat for every 1 gm carb and protein
Vagus Nerve Stimulator • Magnet can be swiped once a minute 3-5 times • Needs to be turned off if getting MRI