childhood epilepsy stefanie jean-baptiste berry, md pediatric epileptologist northeast regional...

Childhood Childhood EpilepsyEpilepsy

Childhood Childhood EpilepsyEpilepsy

Stefanie Jean-Baptiste Berry, MDStefanie Jean-Baptiste Berry, MDPediatric EpileptologistPediatric Epileptologist

Northeast Regional Epilepsy GroupNortheast Regional Epilepsy Group

What is Epilepsy?• 2 or more unprovoked seizures• Incidence <10 years old 5.2 to 8.1

per 1,000 (highest <1 year)• Causes: Brain malformations, birth

injury, infection, tumor & trauma; 69% with unknown cause

What is a seizure?• Abnormal and excessive electrical

activity of brain cells (neurons)• Seizure types: Generalized Focal (Partial) Focal with secondary

generalization

• Generalized Seizures: 1.) Generalized tonic-clonic (grand

mal)- Unconscious, whole body shaking; variable duration

2.) Absence (petit mal) – Staring, unawareness, brief (seconds)

3.) Myoclonic – Brief jerk of arm or leg

4.) Atonic – Sudden drop

• Focal (Partial) Seizures: 1.) Simple – Consciousness

preserved; twitching of one side of face or body, numbness, visual

2.) Complex – Impaired consciousness; twitching, head/eye deviation etc.

Testing• EEG – records brain activity;

seizures or potential for seizures• Video-EEG – prolonged; overnight

in hospital• MRI of brain – picture of brain;

look for abnormal structure

• Common EEG abnormalities:

1.) Slowing 2.) Spikes 3.) Seizures

Normal

Slowing

Spikes

Spikes

Seizure

Treatment 1.) Medication: • Trileptal, Tegretol, Keppra, Depakote, Lamictal

and Phenobarbital• Choice based on type of seizures, EEG findings,

side effects, age and sex• 2nd med may be added if seizures not controlled

2.) Ketogenic Diet – high fat and protein; low carb

3.) Surgery/Vagal Nerve Stimulator

Epilepsy Syndromes• Typical Absence • Juvenile Myoclonic Epilepsy• Benign Epilepsy in Childhood with

Centrotemporal Spikes (Rolandic Epilepsy)

Typical Absence• Generalized seizures• Sudden discontinuation of activity

with loss of awareness, responsiveness, and memory, with an abrupt recovery

• Most common in the first decade, particularly ages 5-7 years of age

Typical Absence

• Most patients with typical absence have normal neurological exams and intelligence scores

• Generalized spikes on EEG• Medications: Zarontin, Lamictal,

Depakote

Typical Absence• Average age when seizures stop is

10 years old• Typical absence seizures generally

have a good prognosis – resolves in approximately 80 percent of cases

Juvenile Myoclonic Epilepsy

• Myoclonic jerks, generalized tonic-clonic seizures, and sometimes absence seizures

• Usual age at onset of absence seizures is 7 to 13 years; myoclonic jerks, 12 to 18 years; generalized tonic-clonic seizures, 13 to 20 years

Juvenile Myoclonic Epilepsy

• More likely to have seizures with sleep deprivation and alcohol ingestion

• Risk for seizures is lifelong• Photic stimulation often provokes a

discharge. • Seizures are usually well-controlled

with medication (Depakote, Lamictal)

Benign Rolandic Epilepsy

• Onset is between 3 and 13 years • Peak age of onset is 7-8 years• Resolves by age 16• Normal intelligence amd

neurological exam• Seizures usually happen after

falling asleep or before awakening

Benign Rolandic Epilepsy

• One-sided numbness of the face, one-sided clonic or tonic activity involving the face, unable to speak, drooling

• No loss of consciousness• Can have secondarily generalized

tonic-clonic seizures

Benign Rolandic Epilepsy

• Spikes in midtemporal and central head region

• More spikes in drowsiness and sleep and 30% of cases show spikes only during sleep

Benign Rolandic Epilepsy

• No treatment is necessary in patients with infrequent, nocturnal, partial seizures

• If seizures are frequent and/or disturbing to patient and family, treatment with Tegretol or Trileptal

• Good prognosis

Seizure Safety • Lay child on floor on his/her side • Do not restrain• Nothing in the mouth• Diastat (rectal valium)• Call ambulance• May be confused or sleepy after

Seizures Precautions• Avoid heights >4 feet• No baths• Swimming should be supervised• Keep bathroom door unlocked• Teens – no driving X 1 year

Other• Good to inform school of child’s

condition• May play sports if seizures well

controlled• Videogames okay for most

Prognosis• Depends on seizure type• Usually treat at lest 2 years• Absence – 80% resolve• JME- respond well to treatment but

need meds for life• Neurologically abnormal often

difficult to control seizures

Prognosis• Injuries common in epilepsy

(Generalized tonic-clonic)• Lacerations, Fractures, Burns• SUDEP not very common (2.3

times more than general population)

Febrile Seizures• Not epilepsy• Often a family history• Seizures only occur with fever in

children age 6 months – 6 years• Up to 4% of children

• Simple – 1 brief seizure (genralized)• Complex – prolonged; more than 1;

focal• Developmental delay or family history

of epilepsy – more develop epilepsy• 1/3 have second (1/2 of that third

have third)

• Increase risk of recurrence if 1st before 18 months or lower temperature

• Increase risk of epilepsy if >3 febrile• Testing unnecessary with simple• Focal need MRI• EEG in high risk• Treatment usually not necessary

Resources• www.epilepsygroup.com• www.epilepsyfoundation.org• www.epilepsyadvocate.com• www.paceusa.org• www.epilepsy.com