Module 2 (a) Pediatric Neurology - Seizures Flashcards
Seizure Definition
- Transient occurrence of signs and/or symptoms due to an abnormal excessive or synchronous neuronal activity in the brain
Epilepsy Definition
- At least 2 unprovoked seizures occurring more than 24 hours apart
- One unprovoked seizure and a probability of further seizures
- Diagnosis of epilepsy syndrome
EEG Info
- Recording of electrical activity along the scalp produced by the firing of neurons in the brain
- EEG alone CANNOT make or refute a diagnosis of epilepsy
- Sleep deprivation preferred
Definitions of Seizure Activity
- Tonic — Sudden tension of skeletal muscles
- Clonic — Rapid contraction and relaxation of muscles (Convulsions)
- Myoclonic — Brief involuntary twitching of a muscle or muscle group
- Atomic — Sudden loss of tone (Drop Seizures)
- Ictal — During the event
- Interictal — Between events
- Postictal — After the event
- Automatisms — Set of brief, unconscious behaviors
Most Common Seizures?
- Focal seizures are most common type of seizure
- Most common focal seizure is a Focal seizure w/out impaired awareness
Etiology of Seizures
- Genetic
- Structural — Physical problem in the brain.
- Congenital Ex: Cortical displasia or tubular sclerosis
- Acquired Ex: Scar tissue from a stroke or trauma - Metabolic — Ex: Glucose transport deficiency (Mitochondrial disorders)
- Immune — Inflammation resulting in seizures. Ex: Encephalitis
- Infectious — CNS infection Ex: HIV
- Unknown — MOST COMMON TYPE
Seizure Prognosis and Outcomes
- Remission of seizures w/ Medication management is 70% in childrenTEST — 30% of seizures are refractory to medication
- 50% of seizures are limited to childhood
- Goals of care
- Decrease seizure frequency as much as possible
- Improve quality of life
Risks for Intractable Seizures
- Continued seizures while on medication
- Family medical hx of epilepsy
- Symptomatic epilepsy - Ex: Stroke that left damage
- Low IQ
Key Components of Seizure Hx.
- Birth Hx — Hypoxia or trauma?
- Hx of CNS infection or TBI?
- Fam hx of epilepsy
- HPI — Focus on context
- Sleep deprivation
- stress
- Menstrual cycle
- Infection/illness
- Patients w/ epilepsy — Missed doses of AED - Seizure Description — Take video if possible
Types of Seizures
- Generalized — Motor and Non-motor
- Both sides of the brain (Both hemispheres) are impacted **TEST
- Motor Example — Ex: General Tonic-Clonic
- Non-Motor — Ex: Absence seizure - Focal Seizures (Partial) — Motor and Non-motor
- Aware (simple)
- Impaired Awareness (Complex)
Generalized Seizures
-Info
- Bilateral at onset — BOTH hemispheres are impacted
- Bilateral motor manifestations
- Bilateral EEG changes - Can be motor or non-motor
Focal Seizures
-Consciousness Maintained
- Motor — Jerking movements of one part of the body, tonic movements (“Fencing posture”)
- Aura — Focal seizure w/ sensory or psychic symptoms
- Automatic — Changes in BP, HR, bowel function, etc.
—Temporal Lobe epilepsy is the MOST COMMON type of focal Epilepsy’s**
-Starts with a seizure aura — Considered part of the seizure
Focal Seizures
-Alteration of Awareness
- Behavioral arrest lasting 30-120 seconds
- Stare into space
- Automatisms are common
- Unaware and unresponsive
Epilepsy Syndromes
-Definition
- Distinct clinical entity defined as a complex of clinical features, signs and symptoms that together define a distinctive recognizable clinical disorder.
Epilepsy Syndromes
-Types encountered in Primary Care?
- benign focal epilepsies of childhood
- Childhood absence epilepsy
- Juvenile myoclonic epilepsy
- Infantile spasms
Benign Focal Epilepsies of Childhood
-Common Types?
- Limited to childhood
- Occur in developmentally and neurologically typical children
- Most common types:
- Benign epilepsy w/ centrotemporal spikes (BECTS) — Also known as benign Rolandic epilepsy (BRE)
- Benign occipital epilepsy
- Panayiotopolous syndrome
Benign Focal Epilepsies of Childhood
-Benign Epilepsy w/ Centrotemporal Spikes (BECTS)
- 10-20% of all childhood epilepsies
- Family hx of epilepsy is fairly common
- School-age children w/ normal development
- Most seizures occur at night/ upon awakening
—Often in the face — Twitching, numbness/tingling, drooling, difficulty speaking - EXCELLENT prognosis w/ infrequent seizures and LOW risk for status epilepticus
- Antiepilépticos drugs often NOT recommended
- If meds are used, consider weaning after 1 year seizure free - May be associated w/ mild cognitive impairment
Benign Focal Epilepsies of Childhood
-Benign Occipital Epilepsies 2 Types
- Benign occipital epilepsy of childhood
- Panayiotopoulos syndrome
-Can have features of both types
-Characteristic EEG findings
—Occipital spikes
Benign Focal Epilepsies of Childhood
-Benign Occipital Epilepsy
(Panayiotopoulos Syndrome)
- Occurs in YOUNGER children (<5 yrs old w/ range of 1-14 yrs)
- Seizure Sx’s include
—Vomiting, syncope, and prominent autonomic Sx’s (Pallor, miosis, incontinence, coughing, hyper-salivation, tachycardia
—Typically nocturnal and last longer than 5 minutes
—1/3 to 1/2 last longer than 30 minutes ** This would be STATUS Epilepticus
Benign Focal Epilepsies of Childhood
-Benign Occipital Epilepsy of Childhood
- Mean age at onset is 8-9 years
- Seizure characteristics
—Visual Sx’s (Blindness, visual hallucination)
—May also have hemiclonic activity, automatisms, migraine-like headache, versive movements
—Shorter duration — < 5 minutes - Typically occur during the day*
Absence Epilepsy
- GENERALIZED seizures**
- Sudden, profound impairment of consciousness w/out loss of body tone
- Duration approximately 10 seconds
- High frequency — (10+/day)
- May be subtle
- Characteristic EEG finding
Absence Epilepsy 1st line Tx?
- Ethosuxamide (Zarontin) — FIRST LINE TX
Childhood Absence Epilepsy
- Onset between 4-10 yrs
- Put to 20% have hx of febrile seizures
- Almost 50% have a first or second decree relative w/ seizures
- Increased Risk for GTCS
- High incidence of psychiatric comorbidities — ADHD, Anxiety
Juvenile Absence Epilepsy
- Less common than childhood onset
- Peak age of onset 10-12 years
- Seizures are typically longer and more severe impairment in consciousness
- Usually a life long disorder
Juvenile Myoclonic Epilepsy
- Most diagnosed between age 12-18 yrs And otherwise healthy kids
- About 50% have FMH of generalized seizures
- Get good hx of presence of other seizure types like absence seizures
- LIFE LONG disorder
- At risk for cognitive, behavioral, and social difficulties
- Increased risk for anxiety, mood disorder, and personality disorder
- Overall favorable prognosis
Juvenile Myoclonic Epilepsy
-Treatment
- Valproate is FIRST LINE treatment
- Lifestyle
- Sleep
- Alcohol/drug consumption
- Medication non-adherence
Infantile Seizures
- 90% onset < 1 year old
- Developmental regression after onset
- Initial seizures infrequent and isolated — Eventually frequent and occur in clusters
- Hypsarrhythmia on EEG
Infantile Spasms
-Seizure Description
- Sudden, BRIEF contractions of >/= 1 muscle group (neck, trunks, extremities) followed by a longer, less intense tonic phase
- Can be flexor, extensor, or mixed flexor-extensor
- Typically symmetric and synchronous
- Can occur in clusters lasting several minutes
- Most frequent just after WAKING , and almost exclusively occur in the DAYTIME
- May be followed by motor arrest or decreased responsiveness
Infantile Spasms
-Prognosis
- POOR prognosis and early recognition is critical
- Differential diagnosis
- GERD
- Exaggerated/hyperactive reflexes
- Benign myoclonus of infancy
- Shuddering attacks
- Benign paroxysmal torticollis
Seizures and Fever
- Often a child’s first seizure will occur in the setting of fever
- Three possibilities
- Nervous system infection
- Underlying seizure disorder
- Febrile Seizure
Febrile Seizures
- 2-4% of children <5 yrs
- Diagnostic Criteria
- Seizure w/ temp >100.4
- No CNS infection/inflammation
- Absence of acute systemic metabolic abnormality that may produce convulsions
- No Hx of previous febrile seizures
Febrile Seizures
-Risks
- High fever
- Viral infection
- Recent immunization — DTP, MMR, MMR-V
- FMH febrile seizures
Other Risks
- Prenatal exposure to nicotine
- Iron insufficiency
- Allergic rhinitis/Atopic disease
MAXIMUM Height of fever is greatest contributing risk
Febrile Seizures
-Simple Vs Complex
- Simple
- Generalized, Duration <10-15 minutes, No recurrence w/in 24 hrs - Complex
- Focal onset, Duration >15 minutes and >1 in 24 hrs
- Febrile status epilepticus - Duration >30 minutes
Febrile Seizures
-Diagnostic Criteria
- MUST r/o CNS infection and underlying structural abnormality
- Altered LOC
- Meningismus
- Petechiae
- Tense or bulging fontanelle
- Focal differences in muscle tone, strength or movements
- Post-I tal drowsiness > 10 minutes
Febrile Seizures
-Work up & Education
- No work up necessary if — Simple febrile seizure and NORMAL neurological exam
- Education
- Role of antipyretics
- Risk for recurrence of febril seizures
- Risk for subsequent epilepsy - Provide at-home benzodiazepine for seizure >5 minutes
Status Epilepticus
-Definition
- Single seizure lasting >30 minutes OR a cluster of seizures w/out return to baseline lasting >30 minutes
- Duration >5 minutes should be treated as Status Epilepticus
Status Epilepticus
-Rescue Meds
- Rectal Diazepam
2. Bucal or intranasal Midazolam
Non-Pharmacologic Treatment of Seizures/Epilepsy
- Educate on Seizure Precautions
- Lifestyle habits
- Avoiding head injury
- Water safety
- Emergency seizure plan - Assess for/Treat anxiety and mood disorders
- Address cognitive issues
- Make sure they have a neurology provider
- Adolescents
- Alcohol avoidance
- Transition to adulthood
- Driving and Sex (Some Seizure meds can interfere with birth control and they are teratogenic)
Anti-Epileptic Drugs (AED)
- First-time unprovoked seizure
—Typically NOT treated with AED
—Tx withheld until a pattern of recurrence is established — AED’s used w/ remote symptomatic etiology and focal seizure w/ abnormal EEG - Second unprovoked seizure
—Typically started on AED
—Exception: Infrequent and/or “mild” seizures
Antiepilépticos Drug Goals
- Goals include:
- Minimal seizures
- Maximal Quality of life
- Few S/E - General Principles
- Low and slow
- Monotherapy preferred
- High index of suspicion for AE’s
AED: Implications for Primary Care?
- Bone health
- Screen for Vit D deficiency and use supplemental calcium and vit D - Psychiatric/behavioral health screening
- Depression
- SUICIDALITY
- ADHD
- Learning issues - Adherence to AED
AED’s
-Potential Serious/Life Threatening Reactions?
- Stevens-Johnson Syndrome SJS and Toxic epidermal necropsy is (TEN)
- Drug reaction w/ eosinophilia and systemic Sx’s (DRESS)
- AED + RASH = STOP IMMEDIATELY
Stopping AED Therapy
- Children: Consider w/drawal after 2 years w/out seizures — Always SLOW taper over several weeks to a few months
- Risk for return of seizures:
- Motor or cognitive deficit
- Abnormal EEG at time of DC
- Symptomatic epilepsy
- Stopping AED before 2 yrs seizure free - ALWAYS Taper the AEDs
Adolescent Females on AEDs
- ALL on AED require FOLIC ACID supplementation
- Valproate or carbamazepine = 4mg/day
- All other AEDs = 0.4 - 0.8 mg/day - Decreased efficacy of ALL hormonal contraceptives
- Use at least 50 mcg of estrogen
- Long-acting reversible contraception recommended ** - “Morning-After Pill” also affected by AED
- Two doses of levonorgestrel 1.5 mg separated by 12 hrs is recommended
Adolescent Females on AEDs
-Pregnancy
- > 90% of women w/ epilepsy have good outcomes
- May need AED DC’d or dose adjusted during pregnancy — Under guidance of neurology
- Seizures (Especially GTCS) thought to be harmful to fetus**
AED’s that are Teratogenic
- Congenital Malformations
- Phenobarbital
- Phenytoin
- Valproate
- Topiramate
- Carbamazepine - Neuro-cognitive Effects/Autism
- Valproate
- Phenytoin
- Carbamazepine
- Phenobarbital
Syncope Vs Seizures
Syncope is the biggest differential w/ seizures
- Sudden, brief LOC w/ loss of postural tone w/ spontaneous recovery
- In children and adolescents, almost always benign
- WORRY IF
- Occurs during or after athletic event
- Cardiac risk factors
- Concurrent eating disorder - Seizures typically last longer and include prolonged tonic-clonic activity and/or post-ictal phase
Syncope Causes?
- Vaso-Vagal Syncope —Precipitating event + Prodrome
- Orthostatic Hypotension — Syncope that occurs w/ postural change
- Toxic Exposure — Secondary to decreased cardiac output or sudden LOC
- Hypoglycemia — Preceded by hunger, weakness, diaphoresis
Breath Holding Spells
- Onset age 6-24 months; resolves by 5 years
- Triggered by emotional insult — Pain, anger, fear
- Can have associated brief posturing or tonic clonic motor activity
- May be associated w/ IRON DEFICIENCY — Check serum ferritin and a CBC