Paeds epilepsy Flashcards
What is an epileptic seizure?
Transient occurrence of signs &/or symptoms due to abnormal/excessive or synchronous neuronal activity in the brain
Unprovoked
What is epilespy?
A disorder of brain characterized by enduring predisposition to generate epileptic seizures & the neurobiologic, cognitive, psychological, and social consequences of this condition
What are epileptiform discharges?
spikes, polyspikes, sharp waves, or spike and slow-wave complexes without observed clinical seizures
Observed in 2-5% of children without epilepsy
How is an epilepsy history taken?
Do they have video?
Beginning, middle, end description
Seizure type
Syndrome
Cause
Relevant FH
Medical history
Impairment
Behaviour/emotional impact
Education
What are the 5 axes for classification of seizures?
1 Description of seizures (ictal semiology)
2 Seizure type (myoclonic absence)
3 Syndrome diagnosis
4 Aetiology (TS/Stroke etc)
5 Degree of impairment
What is a focal seizure?
Isolated to 1 hemisphere
What are the different types of generalised seizure?
- Tonic-clonic seizure: bilateral increased tone and then bilateral sustained rhythmic jerking
- Tonic seizure: Increased tone, most commonly happen while sleeping
- Atonic seizure: sudden loss of muscle strength
- Myoclonic seizure: shock-like jerks of muscles
- Epileptic spasms: type of seizure seen in infancy
What are generalised seizures?
Ones that rapidly engage bilaterally distributed networks
What are febrile seizures?
6m-5yrs
Immunisations – fever not the jab
1in 50 will have one
50% if fam hx/neurodeve/complex
?recurrent give buccal midaz within 2mins
What are infantile spasms?
A type of seizure that occurs in babies
The spasms look like a sudden stiffening of muscles, and the baby’s arms, legs, or head may bend forward
The seizures occur in a series of short spasms, about one to two seconds in length
What is Childhood absence epilepsy?
12% of childhood epilepsy
5 - 7 yrs
Frequent, typical absence seizures 100s/day lasting about 10s
90% disappear by childhood
What is a juvenile absence seizure?
10-12 yrs
Overlaps with juvenile myoclonic epilepsy
Absence seizures cluster upon awakening
photosensitivity in 20%
Long-term prognosis is unclear
What can precipitate a generalised tonic-clonic seizure?
Sleep deprivation (80%)
What is Benign Rolandic Epilepsy?
Most common childhood epilepsy (8 -23%)
3-13yrs age at onset, peak 9yrs
Daytime and night time seizures
Lateralised facial contraction/anarthria, dribbling/ grunting sound, no LOC +/- same upper limb – secondary generalisation can supervene
What are childhood epilepsy with centrotemporal spikes?
AKA CECT’s
Can occur day or night
What is sudden death in epilepsy?
When no other cause of death is found
It can be caused by things like pauses in breathing during a seizure which cause death
In which populations is epilepsy more common?
Downs Syndrome (8%)
Tuberous sclerosis (80-90%)
Angelman Syndrome (>80%)
Fragile X syndrome (13-40%)
What is Tuberous sclerosis?
A genetic disease that causes non-cancerous (benign) tumours to grow in the brain and several areas of the body, including the spinal cord, nerves, eyes, lung, heart, kidneys, and skin
What is Angelman Syndrome?
A genetic disorder that causes delayed development, problems with speech and balance, intellectual disability, and, sometimes, seizures
People with Angelman syndrome often smile and laugh frequently, and have happy, excitable personalities
What is Fragile X syndrome?
A genetic condition that causes a range of developmental problems including learning disabilities and cognitive impairment
Only males often affected
Affected individuals usually have delayed development of speech and language by age 2
What is Doose syndrome?
A seizure disorder characterized by frequent myoclonic and myoclonic-atonic seizures
What is West syndrome?
Previously known as Infantile spasms syndrome
Children have seizures and cognitive and developmental impairements
What is benign sleep myoclonus?
A self-limiting condition characterised by neonatal onset myoclonic jerks during non-REM sleep
What is Sandifer syndrome?
Unusual movements of your child’s head, neck and back
Can be an epilepsy mimic
What is cerebral palsy?
Most common motor disability in children
Causes motor, balance, and coordination problems because of damage to those areas of the brain
What is the difference between cerebral and Bells palsy?
Bell’s affects half of face
Cerebral affects whole body
What are some paediatric signs of cerebral palsy?
- Delayed milestones
Eg. not sitting by 8 months or not walking by 18 months - Seeming too stiff or too floppy (hypotonia)
- Weak arms or legs.
- Fidgety, jerky or clumsy movement
- Random, uncontrolled movements
- Muscle spasms
- Shaking hands (tremors)
What is muscular dystrophy?
A group of diseases that cause progressive weakness and loss of muscle mass
How does Duchenne’s muscular dystrophy progress?
Affects only boys, girls would get milder version
Need wheelchair by 12 yrs. By 20yrs they develop breathing problems. Die during 30’s
What is myotonic dystrophy?
Progressive weakness and wasting of muscles and it affects the electrical conductive system of the heart, lungs, swallowing, bowels, eyes, and brain
Patients live to their 40’s and usually die from respiratory or cardiovascular failure
What is Facioscapulohumeral muscular dystrophy?
Affects both men and women
Causes progressive weakness and wasting of face, shoulders, and humorous
Sign: sleeping with eyes slightly open, inability to squeeze eye tightly, inability to purse lips
What is Becker muscular dystrophy?
Like Duchenne’s jut less severe
Causes delayed walking and muscle cramps with exercise
Some may need wheelchair by 50yrs, but most have a normal lifespan
What is Limb-girdle muscular dystrophy?
Causes muscle weakness in hips, thighs and arm, back pain, and heart palpitations
Progress differently from person-to-person
What is spinal muscular atrophy (SMA)?
A genetic condition where the motor nerve cell in the spinal cord are attacked which causes weakness and atrophy of the spinal muscles
Affects child’s ability to crawl, walk, sit up, and control head movements
In severe cases, can affect breathing and swallowing
Which medication is prescribed for absence seizures in children?
Ethosuximide in girls of child bearing age
Sodium valproate when not child bearing
Lamotrigine can be used to increase effectiveness
Why can’t carbamazepine be used in childhood absence seizures?
Because it can aggravate them
