Generalize: loc

Motor vs non motor

Focal important to distinguish between aware vs altered
awareness

Vocabulary:
Tonic : continuous stiffness

Clonic : fast and slow component

Atonic: without movement, sudden drop

Myoclonic: abduction movements , muscle jerk

Typical seizure: interrupts a child and can’t get them out of

BFNE: can begin in the first week of life

Epilepsy syndrome- 20% of epilepsy in childhood

SEIZURE VS EPILEPSY VS OTHER
 Seizure mimickers
 Seizure
o Transient occurrence due to abnormal excessive neuronal activity In the brain
 Epilepsy: the tendency to have repeated unprovoked epileptic seizures
o Epilepsy syndromes (make up 20% of)
NON EPILEPTIC EVENTS
1. Breath holding spells ,GERD, syncope, migraine, vertigo, movement disorder ect
SEIZURE CAUSES
2. Head trauma, brain tumor, toxins, infections, vascular, metabolic disturbance, systemic
EPILEPSY
3. SYNDROMES (make up 20-25% of epilepsy causes)
a. Infantile spasms
i. Most common cause is tuberous sclerosis
ii. West syndrome
b. Absence epilepsy of childhood
i. 4-8 years old
c. BECTS/Benign Rolandic
i. Most common partial epilepsy during childhood
ii. Autosomal dominant
iii. When? Early morning hours

Value of EEG
 How often is it abnormal in someone with epilepsy?
o Better sensitive when its done when the patient is wake and asleep (30-90%)
 Sensitivity increase during sleep
 Abnormal in someone who dosent have it
o False positive: 300 people
 Don’t have epilepsy
 Specificity is better in a child with normal cognition
What is important in the history?
1. Setting, Sequence: motor and autonomic, Sequelae
a. Need a first hand witness
2. Stereotypical: same each time
3. paroxysmal
a. comes and goes, over a period of minutes
4. Unpredictable (can have a trigger)
5. Interrupt the child
6. The event itself cannot be interrupted
Non epileptic events
1. Neonates
a. Sleep myoclonus

2. Infant
a. Breath holding
b. Anoxic syncope
c. Sandifers
d. Shuddering
3. Child
a. Day dreaming
b. Sleep parasomnias
c. Tics
d. Paroxysmal torticollis
e. Vertigo
f. Dyskinesie or ataxia
4. Adolescent
a. Syncope
b. Pseudo seizures
5. Any age
a. Cardiac arrythmia (QT interval)

Non epileptic events

1. Sleep myoclonus (benign condition)
a. Setting: only occur when he is asleep
b. Event: stereotypical
c. It can be interrupted
d. Myoclonic activity during sleep is common
2. Breath holding spell
b. Setting: Only occurs when child is upset, frightened or has an injury
c. Hypoxia Intensely cyanosed-> stiff tonic posture->
i. Associated with :Brain stem hypoxia
ii. Why? Perfusion oxygenation mismatch, during this event oxygen is
d. Event: stereotypical
e. Can be interrupted
f. Sequence reflect brain stem hypoxia
i. Perfusion oxygenation mismatch, during this event oxygen is, blood should be diverted
to the upper part when oxygen comes in but goes to bottom lung
6. Sandiferd syndrome
a. Events exclusively with feeding
b. No epileptic event
c. Present with epilepsy query
7. Reflex anoxic Syncope
a. Pathophysiology: Due to brief cardiac arrest
b. Presents with as query seizure
c. Stiffness of the neck and twitching of the hand
8. Shuddering spell
a. Stiffens arms and legs, remains aware
 b. Lateral head movement, side to to side
c. Stereotypical movement: it’s the same each time
d. Provoked by seeing food coming
9. Movement disorder: Childhood tics
a. Common
b. Can be distracted and stop the activity
10. Paroxysmal torticollis
a. Movement of neck looks like torticollis
b. Stereotypical
c. Interrupts the child
11. Syncope
i. Myoclonic arrhythmic movement of all 4 limbs
1. Unconscious
ii. You need to know the sequele and setting
iii. Only happens during Valsalva when they hold their breath
iv. Eye lid myoclonus, twitching
12. Pseudo seizure: Non epileptic event
a. Illness behaviour
b. Arrythmia
c. Pelvic thrusting
d. Functional neurological problem
e. In adolescents with epilepsy 15% of them will have non epileptic attack disorders in addition to
their epilepsy
SEIZURE
Febrile seizure: seizure in a child aged 6 months to 5 years associated with a fever caused by infection outside
the central nervous system

 Case 1 febrile seizure Febrile seizure: hard to distinguish between

febrile seiz and meningitis, should have low threshold for doing a
lumbar puncture
 When to admit? Is it just a febrile seizure or is it anything else eg
meningitis?
o NB febrile seizures are usually benign vs bacterial meningitis
which can present with seizures/high mortality rate
o CNS infections: meningitis, encephalitis, prions, toxo, TB
o Is due to infection outside the CNS

When to admit?
o Complex febrile like picture more likely to have something like meningitis
o Infant less than 18 because they cant tell you the symptoms, hard to tell: meningitis or
headache
o Dosen’t make a complete recovery >1 hour
o Parents are very anxious
 CASE 2: Complex febrile seizure

Risk of epilepsy is 1/10 because she has 2 risk factors-

first degree relative and febrile seizure

Febrile seizure are epileptic seizures but they aren’t

considered epilepsy because children out grow febrile
seizures

Seizures without temperature?

Developing epilepsy

1. Development: abnormal development-> 50% of the time you will develop epilepsy
2. Complex febrile
3. Epilepsy in a first degree relative

Risk of child developing future epilepsy in children with febrile seizures

 No seizure history: 1/200

 Febrile seizure
o No risk factors 1/110
o One risk factor 1/50
o two or more risk factors 1/10
o risk of child with status 1/5

Febrile seizures

Most common cause of status in child

Symptomatic of something happening at the time

Most common symptomatic seizure in childhood

Other cause of symptomatic epileptic seizures:

Seizures that occur are temporary vs epilepsy: unprovoked

Respond to acute treated that we would use for

Most common cause of symptomatic epileptic seizures

Febrile seizures, meningoencephalitis, head injury

Other causes of symptomatic epilepsy

EPILEPSY (only 1/5 have an abnormal MRI, most causes are genetic and small amount are metabolic)

1. due to a lesion
a. problem with brain development
i. neuronal migration disorder
b. acquired injury to the brain
i. HSV
1. Temporal lobe
ii. Trauma
iii. Stroke during the antenatal period (1/2000)
2. non lesional (90% of the time, ie cant find anything on imaging)
a. genetic
i. Dravet syndrome: SCNIA
ii. GEFS: SCNIA, SCNIB
iii. Neonatal BFNE KCN02/
b. metabolic
i. hypocalcaemia
ii. hypoglycemia

T1 MRI: lesion on left

hemisphere /cortical dysplasia
The neuron begins life at the edge of
the ventricle and migrates out to form
6 layer cortex-> if migration through
white matter dosent occur properly
are common dysplasia that are
associated with epilepsy
-an area of cortex that extends from
the ventricle to the surface of the
brain
of Cortex hasn’t migrated properly
 Embryology: Cortical grey matter starts out near ventricle and migrates out near glial fibres to
form the 6 layer cortex

tuber sclerosis (commonest identifiable cause of infantile spams)

– AD, TSCI gene mutation (hamartin) and TSC2 (tuberin)

– Infantile spasms
– Cutaneous findings
– Facial angiofibroma’s: skin coloured firm papule
o Nasal/labial, chin
o Secondary mutation induced by UV light
o Progress in size and number in the first decades of life
o Ash leaf macules
o Facial angiofibroma’s occur across the cheeks and nose, and tend to spare the
upper lip
 Not present till after the age 4-5
o Retina hamartomas
o Seizures
o Pulmonary
o Corticle tubers-> that can become calcified
o Cardiac rhabdomyoma
– angiofibroma
– multiple hypo macules
– Infantile spasm

STURGE WEBER SYNDROME

– Sporadic neurological disorders

– Too many capillaries
– Associated with PWS
– Associated with ocular abnormalities and neurological
o Glaucoma
o Seizures/motor/learning disability
– Abnormal vasculature

– Local atrophy over the surface of the angioma which results in epilepsy and focal weakness
– Glaucoma

CT

-car crash

-seizure

Phentoi and phenobartbitol

1. SWB
a. local shrinkage, provoking seizures
2. CT brain
a. White lesions
i. White on CT: calcium or blood
3. HSV infection
a. Injury to temporal lobes
b. Liquefying brain injury
4. Clonic jerking effecring
a. Presenting second day of life
b. Middle cerebral artery vascular accident on the CT brain
c. Required phenobarbital first few weeks of life to settle seizures
d. Came back without illness or fevers
e. Scar from acquire brin injury
f. 1/2000 babies
g.