Neurology Flashcards
Most common type of pediatric seizure
Febrile seizure
what percentage of patients will have recurrence of simple febrile seizure
1/3
What is the biggest risk factor for febrile seizures
first or 2nd degree relative with history of febrile seizure
Simple febrile seizure includes what 4 criteria
- generalized
- <15 min
- does not recur in 24 hours
- no post-ictal abnormalities
Complex febrile seizure includes what 4 criteria
- focal
- > 15 min
- > or equal to 2 in 24 hours
- focal neurological deficits
what is the risk of the general population for developing epilepsy? after a simple febrile seizure what is your risk?
1%
2-3% (2x baseline)
What are 4 factors that increase your risk of epilepsy after a simple febrile seizure
- Family history
- complex febrile seizure
- age < 1
- developmental delay
febrile convulsions >90 minutes have been associated with?
increased risk of medial temporal sclerosis
what is the recurrence risk after 1 unprovoked seizure?
after 2 unprovoked seizures?
40%
80%
What percentage of children with epilepsy will outgrow their disorder?
50%
Name 5 treatable neonatal seizures
- Pyridoxine dependent seizures
- Pyridoxal phosphate dependent seizures
- biotinidase deficiency
- folinic acid responsive seizures
- glucose transporter type 1 syndrome
What will you see on EEG for infantile spasms
Hypsarrythmia
How do you treat infantile spasms (3)
Vigabatrin, ACTH, high dose oral steroids
Name 2 syndromes associated with infantile spasms
TS and Down syndrome
What is the classic EEG finding for absence seizures
3 hx spike and wave
What do you use to treat Absence seizures
Ethosuximide first line
valproic acid second line
What percentage of children will outgrow absence seizures by adolescence
70%
What is the name of the seizure that presents with nocturnal focal seizures of semi-face lasting 1-2 min without loss of consciousness
Benign rolandic epilepsy
What will you see on EEG for Benign rolandic epilepsy
Centro-temporal spikes
At what age do seizures stop for Benign rolandic epilepsy
15 yo
What is JME (Juvenile Myoclonic Epilepsy)
onset late in childhood/early adolescence
jerking in the morning (myoclonus)
later present with GTC seizure and can have absence seizures
tx: valproic acid or lamotrigine
what is the prognosis of Juvenile Myoclonic Epilepsy
Lifelong epilepsy requiring treatment
What is the general dosing for phenobarbital
3-5mg/kg/day at bedtime
what is the most common adverse event for phenobarbital
sedation
why do you not use valproic acid <2 years of age
increase risk of liver toxicty
If mitochondrial or metabolic disorder causing seizures, may trigger drug-induced liver failure
Name 2 contraindications to carbamazepine or phenytoin use
myoclonus/ absence seizures
Name one contraindication to valproic acid use
metabolic disease
Name side effects of valproic acid (8)
weight gain, hair loss, PCOS, thrombocytopenia, LFTS, pancreatitis, hyperammonia, tremor
Name 5 side effects of phenytoin
gingival hypertrophy, coarsening of facies, hirsutism, liver toxicity, SJS, ataxia
Name 4 side effects of carbamazepine
SJS, ataxia, agranulocytosis, SIADH
Name 1 side effect of Ethosuximide
agranulocytosis
Name 3 side effects of Topimax (topiramate)
metabolic acidosis, weight loss, kidney stones*
cognitive dysfunction, fever, glaucoma, hypohidrosis
Which anticonvulsant has the greatest risk of SJS
Lamotrigine
Name 2 side effects of Keppra
behavioural changes- aggression/ “keppra rage”
suicidal ideation
Breath holding spells are associated with
Iron deficiency anemia
By what age do breath holding spells resolve
100% resolve by 8 years pf age
Describe benign infantile myoclonus
sudden brief asymmetrical myoclonic jerks, only during sleep
What are the diagnostic criteria for pediatric migraine?
mnemonic SULTANS
5 or more attacks
lasting 1-72 hours
2/4 of the following characteristics or more:
S- severity (moderate-severe pain)
U- unilateral or bilateral (frontotemporal not occipital)
T- Throbbing/pulsating quality
A- Aggravated by activity or causing avoidance of physical activity
1 of these:
N- Nausea with or without vomiting
S- Photophobia AND phonophobia
Cannot be explained by another disorder
Name 4 childhood migraine variants
cyclic vomiting
abdominal migraine
paroxysmal torticollis of infancy
paroxysmal vertigo
what is a retinal migraine
transient episodes of blindness or scotoma
what is hemiplegic migraine
rare aura transient hemiplegia (unilateral weakness) followed by headache
basilar migraine- now called migraine with brainstem aura
transient ataxia and or cranial nerve deficits
vertigo, tinnitus, diplopia, blurred vision, scotoma, ataxia, occipital headache
Diagnostic criteria for tension headache
A. At least 10 episodes of headache occurring on < 1 day/mo on average (<12 days/yr) and fulfilling criteria B to D
B. Lasting from 30 min to 7 days
C. At least 2 of the following 4 characteristics:
1. Bilateral location
2. Pressing or tightening (nonpulsating) quality
3. Mild or moderate intensity
4. Not aggravated by routine physical activity, such as walking or climbing stairs
D. Both of the following:
1. No nausea or vomiting
2. No more than 1 of photophobia or phonophobia
E. Not better accounted for by another ICHD-3 beta diagnosis
What is the criteria for medication overuse headache
headache present for more than 15d/month for longer than 3 months and intake of simple analgesic on more than 15d/month and/or prescription medication (triptans or combination medications) for >10 d/month
often presents as chronic daily headache
Headache due to raised ICP present with
postural (worse in the morning and when lying down)
progressive worsening severity
optic disk edema +/- cranial VI palsy (papilledema on fundoscopy)
Causes of headache due to raised ICP (4)
tumor
sinus venous thrombosis
meningitis/encephalitis
pseudotumor cerebri (TCA use, steroids, obesity, SLE, hyper/hypothyroidism)
When should you image a child with headache? (8)
sudden onset of severe h/a (worst ever of their life) focal abnormalities on neurological exam pain that wakes patient from sleep paint worst in mornings +/- vomiting change in chronic headache type acutely progressively worsening headache papilledema
Nelsons:
abnormal neurologic exam
abnormal or focal neurologic signs/symptoms
seizures or very brief aura (<5min)
unusual headache in children (hemiplegic, basilar, cluster headache)
headache in children <6 or child who cannot adequately describe headache
headache worst on first awakening or awakens child from sleep
migrainous headache in a child with no family history of migraine
brief cough headache in a child or adolescent
Name lifestyle modifications/ supplements for headache management
sleep exercise avoid caffeine regular meals avoid triggers magnesium, riboflavin, coQ10
Name one treatment for Pseudotumor cerebri
Diamox
What is the gene deletion for Duchenne Muscular Dystrophy
xp21 deletion
What is the dystrophin gene
structural protein in muscle cell