Production by. Pediatric Epilepsy Class. Pediatric Epilepsy Class. Faculty Disclosure. Program Objectives. Accreditation

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1 Faculty Disclosure Carina Bruner, RN, BSN has disclosed no actual or potential conflicts of interest in relation to this educational activity. During this educational activity Carina Bruner will not be discussing the use of any commercial or investigational product not approved for any purpose by the FDA. Pediatric Epilepsy Class Carina Bruner, RN, BSN Clinical Educator Pediatric Epilepsy Program- St. Paul Children s Hospitals and Clinics of Minnesota Pediatric Epilepsy Class A lecture on pediatric epilepsy including brain anatomy, seizure classification, and seizure syndromes. Program Objectives Upon completion of this program, participants should be able to: Define Epilepsy Describe various seizure classifications and give examples of each Define and describe a variety of seizure syndromes Accreditation Children s Hospitals and Clinics is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center s Commission on Accreditation. Children s Hospitals and Clinics of Minnesota designates this educational activity for 1 continuing education hour. Production by Carina Bruner, RN, Sept. 19,

2 Pediatric Epilepsy Class What is a Seizure? Sudden, electrical discharge in the brain which results in an alteration in sensation, behavior, or consciousness Local disturbance vs. kindling A seizure requires the interaction of the abnormal area AND the surrounding community of cells Carina Bruner, RN, Sept. 19, Carina Bruner, RN, Sept. 19, What is Epilepsy? It is a physical condition not a mental illness or a sign of low intelligence 2 or more recurrent seizures that are unprovoked by fever Facts about Epilepsy About 1.5 million people in America have epilepsy The cause of epilepsy is unknown in 70% of all cases 25% of new cases occur in children under 14 years of age 80% of those with epilepsy are seizure free on medication Almost 10% of all individuals will have a single seizure at some time in their life and of those 10%, 50-75% will never have another seizure. Carina Bruner, RN, Sept. 19, Carina Bruner, RN, Sept. 19, Seizure Threshold The susceptibility of neurons surrounding neurons to interaction The balance of excitatory and inhibitory impulses influence seizure activity. When the balance is lost, a seizure occurs. Young children have lower seizure thresholds than adults. Margin of safety is largely determined by genetic makeup, however, anticonvulsant drugs increase the margin of safety and decrease the chance of a seizure occurring. Brain Anatomy Carina Bruner, RN, Sept. 19, Carina Bruner, RN, Sept. 19, Carina Bruner, RN, Sept. 19,

3 Anatomy of the Brain Cortex thinking part of the brain. The cortex contains four lobes: frontal, temporal, parietal, and occipital. Frontal responsible for personality and memory Temporal left side controls speech and right side controls higher functions such as spatial and muscle recognition Parietal making associations and interpreting sensations Occipital site of processing visual information Diagram of the Brain 1 13 Carina Bruner, RN, Sept. 19, Diagram of the Brain 2 Parts of a Seizure Aura the start of a partial seizure Ictus Latin word for stroke or attack. Physicians sometimes use this word for a seizure. Postictal Latin word for after the attack or seizure. During this time a child may fall asleep for a period of time. This period helps to quiet the excitability of the cortex and neurons are less likely to fire. Majority of seizure activity will have a postictal period. However, there are exceptions Seizure Classification Partial Seizures Partial seizures also called focal seizures Local disruptions of brain function where only part of the brain is involved There may be an abnormality in that part of the brain that causes a disruption to occur. For example, tumors, scars, or a tangle of blood vessels There are two types of partial seizures: simple partial and complex partial Carina Bruner, RN, Sept. 19,

4 Simple Partial Begins in one part of the brain and the disruption remains in that part of the brain Child does not lose consciousness Examples: finger twitching, nystagmus, facial pulling, eye fluttering 19 Complex Partial Seizure activity starts in one part of the brain and spreads to another part of the brain. Seizure generally spreads within the temporal lobe and affects consciousness. Consciousness is either altered or lost. Examples: entire side of body is jerking with facial pulling and nystagmus, gelastic seizures Gelastic Seizure Rare type of seizure that begins with a laugh or giggle Rarely becomes generalized Most commonly caused by a congenital tumor 20 Generalized Tonic Clonic Seizures Secondarily Generalized spreads from a complex partial seizure throughout the entire brain and alters consciousness Generalized affects the whole brain and alters consciousness Two types Nonconvulsive Convulsive Nonconvulsive Definition: alteration in consciousness but without jerking movements Convulsive Definition: alteration in consciousness and muscle movements like jerking or stiffening Examples of these two types generalized seizures include absence, myoclonic, atonic, tonic clonic, and febrile seizures Absence Seizures Uncommon type of seizure, starting suddenly and without warning Child displays a glazed look and stares off Usually lasts seconds, occasionally as long as 15 seconds, and ends as abruptly as it started. There is no confusion afterwards. May be induced through hyperventilation Most easily recognized at school. May be perceived as daydreaming by a child s teacher. This seizure type makes learning very difficult. Myoclonic Jerks Abrupt jerks of muscle groups An abrupt increase of tone in a muscle group will cause a sudden movement of that part of the body. Infants and children may experience a series of these jerks, constituting a special, serious form of epilepsy called infantile spasms. Myoclonic jerks can be serious because they are difficult to control and are often only one manifestation of a mixed seizure disorder Carina Bruner, RN, Sept. 19,

5 Atonic Seizures Sudden single events Sudden loss of tone or posture, manifested by a child suddenly going limp Myoclonic and atonic seizures probably originate in areas deep in the brain stem that control muscle tone. Tonic Clonic Seizures Most common seizure type in children Child initially stiffens (tonic) and simultaneously loses consciousness, eyes will roll back in the head, head goes back, and back arches. During this phase the chest muscles also contract, making it difficult for the person to breathe. After the initial phase, bilateral rhythmic jerking will occur (clonus). Jerking is rhythmic and rapid, slowing towards the end. The end of the jerking is usually accompanied by a deep sigh, after which normal breathing occurs and the child enters the postictal stage Febrile Seizures Most first seizures in a child less than 5 years old are brought on by fever. Most common seizures in childhood and occur in 3-4% of children Usually occur around 18 months and outgrown by the age of 5. Very uncommon after 5 or 6 years old. There is a 25-30% chance of recurrence after the first febrile seizure. If the first febrile seizure occurs after 18 months of age, in the first hour of an illness, without a high fever, and family hx of febrile seizures, chance of recurrence may be as high as 80%. 27 Subclinical Seizures Seizures with no clinical manifestations Only diagnosed with EEG May be considered a medical emergency if patient is in status epilepticus Status Epilepticus Continuous seizure activity without recovery between seizures Is considered a medical emergency and seizure activity must be stopped with medications such as Valium, Ativan, Versed, Fosphenytoin, or Dilantin 28 Nonepileptic Events No EEG correlate or physical cause May have some underlying psychiatric issue Children often have very little or no control over NES. These events occur due to difficulties a child has coping with various stressful experiences. Treatment of NEE events often involves identifying potential stressors and helping the child respond to stressors in different and more appropriate ways. 2:1 female: male ratio. Mean age of onset in patients on our unit: 13 years old, with range from 7-19 years old % of children diagnosed with NEE events have a history of sexual abuse. 29 Physical Manifestations of NEE s Movements are not rhythmic; random flailing or inconsistent parts of the body Repetitive movements may stop completely and then start again with renewed vigor. May last longer than epilepsy seizures Muscles are modifiable in presentation Avoidance of injurious behavior May be provoked by suggestion 30 Carina Bruner, RN, Sept. 19,

6 Seizure Syndromes Infantile Spasms Lennox Gastaut Tuberous Sclerosis Landau Kleffner Rasmussen s Syndrome Seizure Syndromes Infantile Spasms Interictal EEG pattern called hypsarrhythmia Mental Retardation and developmental delay Males are affected slightly more often than females. Peak onset is 4-8 months, rarely starts before 2 months. Even if left untreated, this form of epilepsy gradually disappears during the 2 nd to 4 th year of life. Occurs in clusters Spasms may be flexor or extensor. May be treated with ACTH 33 ACTH A form of steroid given twice a day by intramuscular injection Should only be given if the child has hypsarrhythmia ACTH can cause changes on an MRI looking similar to brain shrinkage. If a child is hypoxic at birth, the EEG will have a modified hypsarrhythmia, so child would not be started on ACTH in case of further brain damage caused by ACTH. ACTH has a 25% morbidity/mortality rate and can cause brain atrophy. 34 Lennox Gastaut Multiple types of seizures (2 or more seizure types)- one of which is the atonic type. Tonic and atypical absence seizures are common. Mental retardation or regression Average IQ is 66 Abnormal EEG No known causes High rate of injuries due to the tonic and atonic seizures Significant correlation between age of onset of seizures and mental deterioration Usually begins between 2-6 years old, often in children who previously had infantile spasms Tuberous Sclerosis An inherited condition where children may have white birthmarks (subtle and ash-leafed shape) on their skin and other skin lesions, mental retardation, and epilepsy. A diagnosis is indicated if multiple white spots appear on the skin. In the brain, cells may be abnormal and may form small tumors. These tumors may in turn cause seizures. Most common form of epilepsy in children with T.S. is infantile spasms and Lennox-Gastaut syndrome 80-90% of patients with T.S. have epilepsy Also may have autism and developmental delays. Multiple system genetic disorder: brain, skin, kidneys and heart. First drug of choice is Clobazam, then Felbamate Drug of choice is Vigabatrin. Carina Bruner, RN, Sept. 19,

7 Landau Kleffner Loss of receptive and expressive speech, first the understanding of language and later speech production Occurs more in boys than girls May or may not have seizures Diagnosis is made with sleep EEG showing periods of electrical status epilepticus If a child who previously had language loses language due to abnormal electrical activity in the speech area, he or she is said to have Landau Kleffner. Difficult to diagnose, often associated with autism 37 Rasmussen s Syndrome A focal, unilateral, encephalitis-like condition Intractable, progressive, unilateral motor seizures, usually associated with a progressive hemiparesis Declining cognitive function Sudden onset of intense, constant focal seizures, frequently revolving into a state called epilepsia partialis continua (continuous focal epilepsy) The body becomes allergic to some of its own brain cells on one side of the brain, and as it destroys them they become intensely epileptic. Onset is 10 years or younger in 85% of patients 38 Rasmussen s Syndrome- continued Seizures do not respond to any current anticonvulsant medications. Steroids have sometimes had a transient beneficial effect, as has the administration of large doses of IVIG. The only effective treatment is hemispherectomy. Removal of the affected half, if done early in the course of the disease, results in cessation of the seizures and permits normal intellectual development. Lasting effects include hemiparesis, visual field abnormality, and rehabilitation. 39 Thanks for viewing this presentation! To receive credit, please click the Post Test button below and complete the form. Diagnostic Studies EEG Telemetry electrodes placed on the surface of the head to record seizure activity Depth Electrodes ISAT/WADA (Intracarotid Sodium Amytal Test) sodium amytal is a fast-acting anesthetic agent that puts one hemisphere to sleep at a time. The reason for this test is to find each individual s speech/language and memory areas. If speech and memory are on the same side as seizure location, then the child must have a grid. Grid Placement 64 electrodes covered with silastic are placed on the surface of the brain to record electrical and seizure activity Stimulation studies 41 EEG Telemetry 42 Carina Bruner, RN, Sept. 19,

8 Grid Placement Treatment Anti-Epileptic Medications Ketogenic Diet Brain Surgery Vagal Nerve Stimulator Epilepsy Treatment Anti-Epileptic Medications Dilantin Zonisamide Carbatrol Trileptal Depakote Pregabalin Felbamate Status Medications Neurontin Valium Lamictal Ativan Topamax Fosphenytoin Keppra Ketogenic Diet A diet that is high in fat, adequate in protein, and extremely low in carbohydrates The diet causes the body to burn fat as its fuel, which produces a product called ketones. All meals on the ketogenic diet include heavy whipping cream and many include certain amounts of butter. All parts of the meal are measured out on a scale to the tenth of a gram. The child will fast until spilling moderate to large ketones in the urine, then the child may start to eat. We check urine ketones after every void and watch for symptoms of hypoglycemia. 1/3 of patients will have controlled seizures, 1/3 will have a Vagal Nerve Stimulator decrease in seizures, and 1/3 will have no success Carina Bruner, RN, Sept. 19,

9 Vagal Nerve Stimulator Pacemaker-like equipment that is surgically placed under the chest wall and an electrode is tunneled under the skin and wrapped around the vagus nerve. The VNS sends electrical impulses up the vagus nerve to the brain. Stimulating the VNS at the onset or during a seizure can decrease the severity and duration of the seizure. Programmable for the timing of the impulses, interval between them, and the amount of electrical discharge during the impulses Battery must be changed every 3-5 years in a minor surgical procedure VNS can decrease severity of seizures in intractable patients and those who are not candidates for surgery. Less than 5% are seizurefree with this treatment alone Brain Surgeries Topectomy removal of a piece of the brain lobe. May be any lobe. Lobectomy removal of a lobe of the brain, most commonly the temporal or frontal lobe Hemispherectomy(functional) The parietal and temporal lobes are removed and the frontal and occipital lobes are disconnected, but the disconnected lobes are left in the skull, reducing the need for a shunt and probable movement of the other hemisphere. Multiple Subpial Transection severing individual cell pathways on the surface of the brain Corpus Callosotomy severing of the corpus callosum 51 Corpus Callosotomy Usually done in two separate surgeries. The first surgery severs the first 2/3 of the CC, and the second surgery severs the last 1/3 of the CC approximately 3 months later. This surgery may prevent the spread of the seizure and abolish the generalized component. This surgery does not stop focal seizures. Usually surgery is done when an individual is severely handicapped by these generalized seizures and when the seizures are unresponsive to anticonvulsant medications. Children and adults are less likely to make substantial intellectual or motor improvements and seizure control is uncommon. This surgery is to improve quality of life. 52 Documentation 53 Seizure Documentation Description of the seizure-most important part of documentation is the onset of the seizure. -Before, During, After -Cue Word -Assess LOC -Assess tonicity -Keep patient safe Clinical Data Sheet pupil checks Seizure Data Sheet Event Record 54 Carina Bruner, RN, Sept. 19,