PC of Pediatrics: Quiz #2- Neuro (Part 1: Seizures)

What's the rule for sports sign offs in children with a seizure disorder?

- Anything with impairment in consciousness or activity, should be a red flag for sports participation - No set guidelines for seizures and sports participation

What is Epidiolex and what can it treat?

- Approved for Dravet syndrome and Lennox-Gastaut Syndrome - A plant based formulation of CBD; does not contain THC Seizures may be drastically reduced but only schedule V for DS and LGS

Tell me more about focal seizures in terms of: awareness, motor vs non motor, any progression?

- Can be with awareness or impaired awareness - Motor or non-motor - Focal seizures may progress to bilateral tonic clonic (previously known as secondary generalized)

What diagnostic testing/ imaging is done as part of a seizure evaluation?

- Consider labs based on individual circumstances such as dehydration, toxicology, etc. Lumbar puncture usually not needed (since afebrile) - EEG is required part of the evaluation, usually performed outpatient --- Abnormal EEG is useful in confirming an event, but is not sufficient to make a diagnosis --- Absence of abnormal EEG can't rule out seizures Routine imaging not done - Only performed if abnormalities on neuro exam or concern for focal seizures or concern for localization related epilepsy - Also consider if motor or cognitive impairment for unknown reason, child < age 1, EEG that not consistent with primary generalized epilepsy or benign focal epilepsy of childhood

When trying to assess if a kid had a seizure, what are some important questions to ask to rule out other causes such as breath holding spells etc.?

- Crying before seizure often due to breath holding --> need to differentiate between seizure vs. breath holding - Prolonged return to baseline is often seen with seizure rather than syncope whereas if someone faints due to vasovagal event they will return to baseline rapidly - Absence seizures - cannot be stopped by calling the child's name or touching them --- In absence seizures, child will have a staring episode and CANNOT respond to touch or name. --- If child is day-dreaming, they will respond to stimuli Seizures are usually stereotyped (each one is like the previous one), random (occur at any time of the day or night), and are not usually precipitated by specific environmental, psychological, or physiological events. - However, JME may be worsened by bright flickering lights

What are some other signs of seizure type activity that parents may not put together with a seizure?

- Episodes of staring - Waking up with bite marks on cheeks or inside of tongue - Bedwetting - Dropping things

How common is CAE? What is the age range for onset? Generalized or focal?

- Generalized seizure disorder - Very common - Presents between ages 4-10. Mean age of onset is 6

What's an additional consideration we need to remember in prescribing AEDs in adolescent females?

- If taking hormonal contraception, AED may lower efficacy of hormonal contraception - Also: hormonal contraception can lower serum levels of some AEDs (eg, lamotrigine) - Teratogenicity seen especially with topirimate, valproate, phenobarbital, and phenytoin - Folate supplementation is recommended

It is important to differentiate childhood absence epilepsy from juvenile absence epilepsy. What are some of the ways to do so?

- JAE is a generalized epilepsy that will start at later age, around 10-12 years old - Features include: myoclonic seizures in 1/5 of patients with higher incidence of generalized tonic clonic seizures

What are some potential long term effects of a febrile seizure?

- Maybe ADHD - Slightly higher risk of epilepsy compared to general population (1%) Risk of epilepsy: If simple FS: 2-3%; complex - 5%

Tell me about potential co-morbidities, progression and risk factors for CAE

- More common in females with a family history of seizures or PMH of febrile seizures - Look for ADHD - kids with childhood absence epilepsy may have this - Kids usually grow out of CAE, however it can progress to JAE --> this is not typical

Do we do any imaging for simple febrile seizures?

- Not used in routine evaluation for simple FS --- No published data supported or negating need for imaging. Risk of radiation from CT and MRI

When do we screen for the HLA-B*1502 allele and why?

- Screen for HLA-B*1502 allele in genetically at-risk populations (ie, those with Asian ancestry) before initiating treatment with oxcarbazepine and carbamazepine. - Having this allele puts kids at risk for medication-induced rash

What's the word on vaccinations in kids with seizure disorders?

- Slight increased risk of febrile seizure on the day of DTP vaccine and 2 weeks after MMR vaccine - Children with seizure disorder are NOT at increased risk of seizure following vaccines - Children with stable epilepsy should receive all vaccines - A personal or family history of seizures is a precaution for MMRV vaccination (recent study found an increased risk for febrile seizures in children 12-23 months who receive MMRV compared with MMR and varicella vaccine) - DTaP and Tdap may be deferred IF progressive neuro d/o, uncontrolled epilepsy increased infantile spasms

What's a typical presentation of a kiddo with childhood absence epilepsy?

- Sudden rapid onset of impairment of consciousness that is accompanied by blinking/staring without loss of tone and resolves abruptly - Kids stop what they are doing. Are not confused or tired afterwards. - No jerking movements, head nods, or focal findings on exam - Often go unnoticed / 10-100/day; can be provoked by hyperventilation - Events can be subtle and occur many times per day

What do you do after a simple febrile seizure?Do we recommend meds?

- Treatment with continuous or intermittent anticonvulsants NOT recommended. Provide reassurance - the seizures are NOT harmful & do NOT significantly increase risk of developing epilepsy. - Antipyretic: no benefit whether given preventatively or sporadically does not effect outcome

What sort of parent education should you give after their child has a febrile seizure? Include helpful data, chances of it happening again etc.

- 3-5% of children are likely to have a febrile seizure - There is often a familial pattern -- more common in kids with family history of febrile seizures Likelihood of recurrence: - After 1x: there is a 30% chance of another febrile seizure - More likely to happen again if the child is younger (less than 18 months) - If less than 1 years old, the risk of repeat seizure can go up to 50% - If young age, relatively low fever, family history, brief duration between onset of fever and seizure, and multiple seizures during the same episode --> higher likelihood of another seizure (about 70%) - There is NO long-term effect of a simple febrile seizure --> they do NOT lead to neurological problems or developmental delays. Reassure parents that these are benign, common events.

How do antiepileptic med doses change as children age? Do we need to be monitoring drug levels?

- AEDs medication dose is usually increased as child grows although there are exceptions - Some drugs require monitoring of levels. This is usually done by neuro. Neurology usually goes by how the child is doing on the medication as opposed to the actual level. - In general, dosing is increased until seizures stop, adverse effects occur, or serum levels reach a high or supra-therapeutic range without a significant improvement seizure frequency - Recommendation is to treat with medications for 2 years. There can be some variation on this. It depends on why the child is on medication and what the abnormality is

What are some safety restrictions to consider in kids with seizure disorders?

- Activity restrictions with climbing and swimming if seizures consist of LOC or loss of awareness - Safety considerations for children with seizures when showering - should give kids privacy, but don't lock door, etc. and ensure somebody else is home - Driving restrictions are determined differently in each state

How common is BRE/BECTS? Do we know the cause? Most kids will be between ____-____ years old, but can occur anywhere between ages ____-____

15-23% of childhood epilepsies= BRE/BECTS --> Very commonly seen in primary care The cause is unknown, maybe genetic --> About 50% have a family history of seizure disorder Most kids will be between 7-9 years old, but can occur anywhere between ages 3-13

Define: Epilepsy

2 or more seizures more than 24 hours apart that are unprovoked The seizures may be due to genetic, structural, metabolic, immune, infectious, or unknown causes

A key feature of a febrile seizure is that the child returns to baseline within ___ minutes. What does "baseline" entail?

A key feature of a febrile seizure is that the child returns to baseline within 2 minutes. After seizure, child's eyes should be open and they should be interacting in a normal way

25,000 to 40,000 of children have their first non-febrile seizure every year. After a child's first unprovoked seizure without acute cause: If the child is neurologically normal & no history of prior neurologic illness there is a ____% risk of another seizure in the next year and a _____% risk over the next 3 years

After a child's first unprovoked seizure without acute cause: If the child is neurologically normal & no history of prior neurologic illness there is a 25% risk of another seizure in the next year and a 45% risk over the next 3 years

Case #4: 8 yo boy with weird facial movements - Right sided facial twitching 1 week ago that lasted for 1 minute. No change in LOC. Afterwards, mouth drooping to the right. He was trying to talk during the episode but had difficulty. He states that he was trying to say, "what is wrong with me?" He was seen in the ED and discharged home to follow up with neurology - No prior seizure symptoms and no risk factors for seizures - There is no history of headaches, other seizure-like episodes, CNS infection, weakness, loss of consciousness, head injury or other abnormal movements What's your diagnosis?

Benign, focal epilepsy with centro-temporal skies (BRE/BECTS) Centro-temporal spikes on EEG- typical spike pattern seen with BRE that is autosomal dominant inherited

Case #2: 2 year old boy with an episode of LOC - No significant PMH, 38 week nl devp - Episode yesterday of running at the park and was pushed and fell on his arm and leg and screamed and then lost consciousness briefly. He woke up after a few seconds and was crying and acting like himself. - PE wnl, other than a scab on his knee and elbow Diagnosis?

Breath holding spell Typical story is toddler who is scared or hurt that takes a deep breath in and then begins to looks pale or cyanotic with maybe a few clonic jerks. Will awaken quickly after event and have normal physical exam.

Breath holding spells are very common vasovagal-type events in kids ages ____ months to ____ years

Breath holding spells are very common vasovagal-type events in kids ages 6 months to 6 years

Case #5: 8 year old female - Frequent daydreaming in class & blinking - Classmates are complaining they don't like sitting near her at snack because she is messy. Her older brother has ADHD and her father has a history of tics. Parents thought she likely has ADHD and tics as well. - No PMH including nl devp, no attention issues in past. During the visit when she was playing with a toy she stopped for a few moments and the toy fell to the ground. She picked it up and started playing again. Otherwise her exam was wnl. Diagnosis?

Childhood Absence Epilepsy (CAE)

Treatment and prognosis for BRE/BECTS?

Consider treating if frequent episodes, usually goes away after 2 years with or without treatment. The prognosis is favorable

How do you diagnose BRE/BECTS?

EEG

Is the EEG in kids with JME typically normal or abnormal?

EEG is usually abnormal (in 75% of patients) --> especially overnight (closer to 100% of EEGs are abnormal)

True or false: Unlike nonfebrile seizures, there is often no postictal phase with confusion/ agitation/ drowsiness with febrile seizures

False As with nonfebrile seizures, the postictal phase can be associated with confusion or agitation and drowsiness. Prolonged drowsiness is not typical for simple febrile seizure and should prompt consideration of an alternative etiology (eg, meningitis, structural brain pathology) or ongoing seizure activity

True or false: ADHD is one of the most common comorbid conditions with seizure disorders but stimulants are contraindicated

False Yes, common comorbidity but stimulants are NOT contraindicated

True or false: There are no typical EEG patterns that are diagnostic of childhood absence epilepsy

False: There is a typical EEG pattern in Absence Epilepsy and it is key in diagnosing (2-5 hertz spike in wave) Imaging is not indicated if this EEG pattern is detected

Case #1: 18 m.o female with generalized tonic-clonic seizure - No significant PMH with normal development. Woke up Saturday with fever pulling on ear. Later that AM, while changing diaper - eyes rolling back, unresponsive with shaking of her arms & legs lasting about 2 minutes - Afterwards: crying, consolable by parents - In ED: watching a video on her parents phone and drinking a cup of juice and had a nl exam with the exception of right otitis media Diagnosis?

Febrile seizure

Febrile seizures usually occur from ______ months to _____ years of age

Febrile seizures usually occur from 6 months to 5 years of age

What are the classifications for seizures based on location in the body?

Focal- partial, coming from one part of the brain Generalized- both sides of the body Unknown May be hard to determine generalized after just one seizure

Febrile seizures can be an early sign of two genetic seizure disorders. What are they?

Generalized epilepsy with febrile seizures plus (GEFS): usually autosomal dominant inheritance Severe myoclonic epilepsy of infancy (Dravet syndrome): another genetic epilepsy with a well-known preponderance for seizures with fever in early childhood - Outcome of Dravet isn't good - Developmentally kid seems okay at first. Then we usually see decline

What's a typical presentation for juvenile myoclonic epilepsy?

Generally happens in healthy teens, characterized by triad of myoclonic jerks, general clonic tonic, & absence events. Seizures usually happen when they first wake up (within the 1st hour) or if they are sleep deprived. May also see other characteristics like feeling out of it, dropping things, etc. Consciousness is preserved.

What are some non-medication based potential treatments for pediatric seizures?

Ketogenic diet: often hard for kids due to extreme dietary restrictions Nerve stimulation treatment: device implanted to help stimulate the vagus nerve

There are two types of febrile seizures: simple and complex Tell me about complex febrile seizures

Less common (about 20% of febrile seizures) If you meet ANY of the below criteria, the seizure is complex: - Prolonged (>15 minutes) - Focal - one area or side of the body - Recurs within 24 hours

Management of febrile seizures Step 1: Identify the cause of the fever How do we do this?

Lumbar Puncture- - Consider LP when you are concerned about meningitis - If child recently had antibiotics, always be concerned about meningitis. Classic meningitis signs & symptoms might not be present because meningitis could be partially treated with antibiotics - Always be concerned about meningitis in unvaccinated kids - If any concern for meningitis, refer to ED for further testing Labs- - Labs sometimes are sometimes done to identify cause of fever

Would you get an MRI if the EEG and symptoms are diagnostic of JME?

MRI is not required if the EEG is definitive - If someone has juvenile myoclonic epilepsy, we are not worried about tumors or abnormal areas of the brain because the seizures are generalized, not from a specific area. - Really only do imaging when worried about focal seizures - Guidelines don't recommend that everyone with seizure gets imaging

True or false: We know the exact cause of febrile seizures

Nope The cause of febrile seizures is not well defined. Not due to rapid rise in fever, inflammation, metabolic abnormalities, or intracranial inflammation

Do we do EEGs for simple febrile seizures?

Not in a neurologically healthy child

Who do we refer to neuro, a kid with a simple febrile seizure, a complex febrile seizure or both?

Only kids with complex febrile seizures

What should you do in primary care if a patient presents to you and you are highly suspicious of JME?

Order an EEG first and then refer to neuro

What are some potential problems associated with phenytoin, primidone, carbemazepine, phenobarbital and valproate in kids? What can we do to help reduce this issue?

Potential problems with bone density and mineral metabolism - Ensure adequate calcium (combined diet & supplement to achieve 1000 mg/day in younger patients & vitamin D (400-800 IU) - Measure serum D levels to determine if vitamin D intake is sufficient to maintain levels above 30 ng/mL

Always refer kids with a potential febrile seizure if they have a prolonged return to baseline. What symptoms would you want to look out for that would signify a delayed return to baseline?

Presence of persistently open & deviated eyes is an important clinical feature of ongoing seizure activity

What do we tell parents if their kid had a breath holding spell? Do we refer? If so, to who?

Provide reassurance to parents- kids usually outgrow by 3-4 Do not need to refer to neuro. Consider cardiology referral

When is medical marijuana considered for seizures?

The only indication is for intractable epilepsy (if the child has failed 2 or more drugs) If a child has failed two options, you always want to look at other options MM is legal in CT, NY, NJ, RI and recreationally legal in VT, MA

True or false: Antiepileptic drugs are associated with an increased risk of depression and suicidality

True

True or false: Benign focal epilepsies of childhood BECTS, typically will have motor symptoms involving the face with no impairment in consciousness

True

True or false: Generalized seizures are split into motor or non-motor

True

True or false: Febrile seizures are the most common type of seizure in pediatrics

True Usually caused by a virus

What's panayiotopoulous syndrome?

Usually in younger children (< 5 years) with prominent autonomic features: vomit, syncope, nocturnal, not prolonged - Commonly vomit and then pass out. May have some other autonomic symptoms - Usually brief seizures that don't last long - Benign condition

Most effective treatment for JME?

Valproate (usually lifelong)

How do we treat CAE?

Zarontin (Ethosuximide)

Talk to me about EEGs in regard to how they do or do not dictate seizure med prescribing or seizure diagnoses

- Used to detect abnormal epileptiform discharges. Done on most kids who present with signs of seizures. - A child does NOT need to have a seizure to reveal epileptiform activity. EEG is done to detect abnormal discharges, which may or may not be present. - You can have a seizure disorder even if your EEG is normal. EEG sensitivity varies. Often with focal seizures the abnormality comes from too deep in the brain for the EEG to pick up. - Always go by history, over testing. Can use medications (if necessary) even if EEG is normal - However, children with abnormal EEG do not always require seizure medications (there can be false positives, family history of EEG abnormalities, etc.) - EEGs will not necessarily normalize after child with seizure disorder is given medications A summary: EEGs and medication prescribing and diagnosing seizures may go together, or they may not.

There are two types of febrile seizures: simple and complex Tell me about simple febrile seizures

- Usually last 3-4 minutes, always under 15 minutes - Generalized: the whole body is involved - Doesn't recur within 24 hours (i.e. if child has 2 seizures within 24 hours, it is not simple) - Quickly return to baseline

What's benign occipital epilepsy of childhood?

- Visual symptoms prior to seizure onset, daytime, frequent, brief - Mean age: 8-9 years old - May have hemiclonic (one side of the body movement) - Seizures happen more frequently and occur during the daytime. These kids are often treated, however is usually benign. - Meds used to address frequency

Case #3 Older sister heard a noise and found patient on floor with eyes rolled back. Event lasted two minutes and was incontinent of urine. She opened her eyes afterwards but was very sleepy in the ambulance. Brought to ED where she began waking up and was then awake and alert. This was her first episode concerning for seizure. Of note, she has been having episodes of dropping things and unclear staring episodes. Exam in ED: normal. Diagnosed with first generalized tonic-clonic seizure and told to follow up with PCP and neuro. What are some questions to ask when taking a history about a seizure?

- When did the episode occur and activity? - Has it happened more than once? - What was child doing before it occurred? - Medications? Supplements? Vaping? - What else is going on medically? Fevers? Head injury? - Other medical events occurring? Sick with viral illness? - Timing and prior activity - Relaxing? Randomly? When crying or upset? - Focal signs or symptoms? Eye deviation? Changes in vision, hearing? Headaches? - Description of color, movements and loss of consciousness - Eyes open or closed? Quick or slow return to baseline? - What did the event look like? Movement back and forward? One side? Both sides? - Behavior after the event? - Also: PMH, FH, consanguinity

BRE/BECTS seizures usually happen when?

At night or on awakening Can present with secondary generalized tonic clonic seizure during sleep