Pediatric Seizure - Swartz Flashcards
What is a seizure?
A phenomenon of heightened neuronal excitability and depolarization which spreads to neighboring neurons, columns, gyri, lobes to produce a loss of function with or without obvious clinical manifestations.
What is the new definition of epilepsy?
- At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart.
- One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrency risk after 2 unprovoked seizures (at least 60%), occurring over the next 10 years.
Diagnosis of an epilepsy syndrome involves what?
Constellation of signs and symptoms with known etiology, pathophysiology and outcome.
Neonatal seizures and pediatric febrile seizures are different from adult seizures. They involve what?
Unique time period, etiologies and semiologies ( progression of clinical signs that occur during the course of a seizure, behavior of seizure).
Do pediatric epileptic encephalopathies have a unique and different course from adult?
Yes.
Metabolic syndrome and genetic syndromes are both what?
Age dependent.
How are pediatric seizures similar to adult seizures?
- Importance of ictal recordings for diagnosis and prognosis - CEEG
- Importance of semiology – the clinical characteristics of the seizures – for diagnosis and prognosis
- Need for sensitive neuroimaging
CT- 50%; MRI – 80% - Need for rapid initiation of treatment
What is the overall prevalence of epilepsy in children?
5%
Describe the epidemiology of epilepsy in children.
- Increased in teen years in third-world countries
- Studies may include acute symptomatic seizures and over-represent incidence
- Incidence 0.2% at birth. Drops to 0.1% age three and stabilizes at .05%. (USA), Prevalence 0.7%
- Life time incidence of epilepsy - 1/26.
What are acute symptomatic seizures?
Seizure following head trauma within 24 hours.
Neonatal seizures occur when?
Up to 28 days post term but usually in the first week of life.
How can you differentiate the motor phenomena of seizures from release phenomenon?
By whether the movement is induced by stimulation such as noise, tactile, passive movement or if the movement is stopped by repositioning.
If neonatal seizures are diagnosed, what is the therapy?
- specific treatment
2. AED’s
What type of seizures are unique to childhood?
Febrile seizures and epileptic encephalopathies.
What types of motor phenomenon are there?
- focal clonic
- focal tonic
- generalized tonic
- myoclonic - can be epileptic or non-epileptic
- spasms - epileptic
- motor automatisms
Describe focal clonic motor phenomenon.
These are epileptic, repetitive rhythmic jerking of a limb, face or trunk
Describe a focal tonic motor phenomenon.
These are epileptic, sustained posturing of a limb, eye deviation, asymmetric trunk
Describe generalized tonic motor phenomenon.
These are non-epileptic, are sustained, symmetic posturing of the muscles of the limbs, neck and trunk. Can involve flexors, extensors or both. A common cause is GERD
Describe motor automatisms.
These are non-epileptic movements of the eye, or mouth. They can be semi purposeful or complex purposeless movements.
What are some causes of neonatal seizures?
- Hypoxic-ischemic pre-or perinatal insult
- Infection – septicemia, meningitis, meningo-encephalitis, HIV
- Intracranial hemorrhage - premature
- Congenital CNS abnormalities
- Electrolyte disturbance – Ca++, Na+,Glu, Mg++ (babies more likely to have seizures due to this reason)
- Inborn errors of metabolism - pyridoxine
- Toxins – bilirubin,
- medications, drugs in mother
- Genetic – BNFC (benign neonatal familial convulsions), chromosomal, Leigh’s,
What if the treatment of neonatal seizures?
- Treat etiology – hypocalcemia, hypomagnesemia, hypogycemia, hyponatremia, pyridoxine
- Treat with an AED (ASD)
- Brief and infrequent focal motor, focal tonic or myoclonic seizures may not need AED
- Withdraw AED two weeks after the last seizure if EEG is negative
Medications for seizures include?
- Levetiracetam - first line Tx
- Phenobarbital
- Phenytoin
- if still seizing - treat with Ativan or Midazolam plus high does phenobarbital or DPH
- Newer AED’s such as Lacosamide may work
Febrile seizures are?
The most common seizure of childhood - are generalized tonic clonic seizures.
Describe some characteristics of febrile seizures.
- Occurs ages 3 mo to 5 years – 2-5% in USA and Europe. Up to 14% in Guam
Median age 18-22 mo.
“Not caused by an infection of the CNS and not associated with prior non-febrile seizures and not meeting criteria for any other acute symptomatic seizure. - In most T>39o.
- Usually in first 24 hrs of an illness
- Simple = last less than 10 minutes, generalized at onset AND do not recur in first 24 hours – 85% of all FS
- Complex = longer than 10 min or focal in onset or recur within 24 hours
CNS infections can cause seizures as well as high temperatures. In the case of infections such as meningitis, the situation is a medical emergency while febrile seizures are benign. Describe how a seizure should be evaluated in this context.
- Was it simple or complex?
- Does anything suggest meningitis?
- Was the child neurologically normal beforehand?
- Could something else have caused the seizure?
- EEG not needed. Routine blood work not typically useful. Neuroimaging not needed in normal child
- LP? Should be done in every child with a first complex febrile seizure or in any child > 18 mo with meningeal signs.
- LP should be done in children
How are febrile seizures treated?
- Symptomatically. If they are prolonged then can use Benzodiazepines - ativan and midazolam.
- If recurrent - can use rectal valium
What are the risk factors for febrile seizures?
Age (6 mo – 3 yrs) Degree of temperature elevation FS in 1st or 2nd degree relatives Family hx of afebrile seziures Slow development of child Maternal smoking and EtOH during pregnancy Day care attendance
What are the risk factors for recurrent febrile seizures?
First at <1 yr old Family Hx of retardation FS following low grade or brief fever Epilepsy in 1st degree relative Complex febrile seizures Neurodevelopmental abnormalities Attendance at day care
Those with recurrent febrile seizures may be at more risk of developing epilepsy later on. What risk factors contribute?
- abnormal neonatal history
2. family history of retardation
What are epileptic encephalopathies? Give examples.
Syndromes of recurrent seizures associated with regression of developmental milestones. Examples are:
Ohtahara Syndrome Dravet Syndrome West Syndrome Lennox Gastaut Syndrome Landau Klefner ESES
What is Ohtahara’s syndrome?
- Seen in neonatal period; 10 da – 3 mo .
- Seizures are tonic spasms, drop attacks and partial. Rare myoclonus can also occur.
- Cryptogenic = imaging normal in 15%.
Some inborn errors of metabolism. - Symptomatic – abnormal imaging. 85% -hemimegencephaly or just atrophy
- Poor Px – 100% intellectually disabled (profound MR) with severe seizures
What is another name for Otahara’s syndrome?
Early infant epileptic encephalopathy or EIEE.
What is the EEG pattern for Otahara’s syndrome?
EEG is characterized by suppression - burst patterns.
What is the treatment for Otahara’s syndrome?
- ACTH
- Steroids
- valproic acid
- klonadine
- Topiramate
- Clobazam
- felbimate
What is Dravet’s syndrome (Severe myoclonic epilepsy of infancy or SEI)?
- Unilateral clonic seizures, febrile and afebrile, in the first year of life in previously normal infant
- Recur at 6-8 wk intervals. 3. May cause status epilepticus – convulsive or subtle, Followed by absence then partial seizures.
- Family Hx + 25-50% - epilepsy or Febrile Seizures
- Appears to be the severe end of the GEFS+ (generalized epilepsy with febrile seizures +) spectrum
Dravet’s syndrome is associated with what molecular defects?
Associated with molecular defects in three sodium channel subunit genes and a gamma-aminobutyric acid (GABA) subunit gene.
What is the pattern on EEG with Dravet’s syndrome?
Generalized spike waves or slow spike waves seen in sleep. The background is normal at onset but deteriorates with time.
Describe some other characteristics of Dravet’s syndrome including treatment.
- MRI – normal
- intellectual decline develops in 50%
- Can be stopped if seizures controlled
- Rx = VPA, CLBZ, TPM, Bromide, Benzo, KD, FELB
What are some seizure meds?
- VPA - valproic acid
- CLBZ - clobazam
- TPM - topiramate
- KD - klonadine
- bromide
- benzodiazepines
- FELB - felbimate
What should not be given for Dravet’s syndrome?
Sodium channel blockers - these make the condition worse.
What is West syndrome (Severe encephalopathic epilepsy in infants or SEEI)?
- Characterized by infantile spasms – brief bilaterally symmetic contraction of muscles of neck, trunk and extremities
- Subtle or generalized
3-100’s a day
Occur on sleep transition or with stimulation - Onset in first 4-8 mo of life 4. 90% have some degree of cognitive and neurologic disability
- Treatment – ACTH, Steroids, VGB (vigabatrin), CLB, KD, TOP, RUF (rufinamide)
- PX – 5% normal outcome; severe impairment 67%; cryptogenic better than symptomatic (38% vs 5% nearly normal)
What are some causes of West syndrome?
- head injury
- CNS infection
- intracranial hemorrhage
- dysgenesis
- tuberous sclerosis
- inborn errors of metabolism
What is the pattern on EEG for West syndrome?
EEG – hypsarrhythmia, Slow waves or slow SW, BS, rarely normal
Can be misdiagnosed as Morrow reflex, colic, startle responses
85% symptomatic; 15% cryptogenic (with or without abnormal MRI)
What is Lennox Gastaut syndrome?
- an epileptic encephalopathy
2.Triad of slow spike-wave on EEG, mental retardation and mixed seizure types – myoclonic jerks, atypical absences and drop attacks (tonic or atonic). - Mean age = 2 y/o, usually preschool (10% of sz up to age 5) but 1-14 y/o = range.
Males>females - Drop attacks have “electrodecremental response” on EEG
What is Acquired epileptic aphasia or Landau-Klefner syndrome?
- Ages 3-9 y/o, Males > Females
- EEG shows multifocal SW
- Child develops verbal agnosia and decreased spontaneous speech
- Remits before age 15
- Etiology unknown
- Seizures may not be prominent feature
- Treatment – steroids, KD, felbamate, early speech therapy. 40-50% recover to a normal life.
What is Electrical status epileptics during sleep?
- EEG – some bursts of SW while awake become continuous in sleep (1.5-2Hz)
- Seizures – partial, generalized, atypical absence
- Normal development until onset – may return to near normal with control of S/W
- Rx - ? CBZ, TOP, ACTH, VPA
What are Absence seizures?
- Bihemispheric initial involvement of 3 Hz GSW lasting 3-10 sec
- Average age of onset 5.7 yrs (3-9 y/o)
Childhood absence(3-5 y/o) – strong genetic, girls>boys, self limited
Juvenile absence – begins around puberty. Boys=girls, SW 4 Hz
Juvenile myoclonic epilepsy- 12-18 y/o, rarely remits, shorter bursts - Risk factors – febrile sz; family Hx
- Medications – ETX (Ethuxamide), LTG (lamotrigine), LEV (Levetiracam), VPA, TOP, ZON (zonisamide)
- Typical = normal IQ, faster SW (3-3.5 Hz), normal background
65% remit by puberty; 15% evolve to JME;
Atypical – slower SW – 1.5-3Hz, abnormal background, lower IQ’s,
Absence seizures are the only indication for what med?
Ethuxamide.
What is Juvenile myoclonic epilepsy?
- Most common epilepsy syndrome
Possibly 20% of all epilepsies - Hallmark is myoclonic jerks, fast GSW (4-6 Hz) normal IQ and development
- Lifelong (maybe 10% remit)
Onset 5 – absence in 20%, 8-10 – myoclonus, 15 – GTC -60% - Rx – LEV, VPA, LTG, TOP, ZON, CLNZ, LAC?
- Several associated gene loci – myoclonic gene involved in spindle body directing cell division and neuronal migration
What is Benign focal epilepsy of childhood or BFEC (also called Rolandic epilepsy)?
- 13-23 % of all childhood epilepsies =MOST COMMON EPILEPSY
- Onset 3-13 y/o, peak 7-8 years
- Normal NE, IQ, MRI and EEG background
- GTC’s occur in sleep, 15% have in day time also
Partial seizures are unilateral paresthesias +/- clonus of face, lips, tongue. Also salivation, speech arrest but preserved consciousness.
EEG – centro-temporal spikes, high amplitude, triphasic - Genetic predisposition
- Remit by age 16
- Similar condition is Benign Epilepsy of Childhood with Occipital Paroxysms:
Ictal visual symptoms, amaurosis, hallucinations
High amplitude diphasic occipital spikes.
Eye opening suppresses spikes.
What is Panayiotopolus syndrome?
Benign occipital; spikes occipital or anterior
With autonomic onset, autonomic status
What is Atypical benign partial epilepsy of childhood?
CTSW bilateral. Focal ones produce focal atonias, when they generalize produce atonic drops. Nocturnal seizures = Rolandic, GTC or jerks. EEG in sleep = ECSWS. All recover
What is Hypothalamic Hamartomas or Gelastic epilepsy?
- Boys > Girls. Peak age 2-3. 2. Seizures can appear throughout adulthood
- Laughter without emotion, 10-30 sec, frequent
- Can have autonomic, complex partial GTC as well.
- Can be associated with precocious puberty, behavioral and cognitive difficulties
- rare
