Neurology Flashcards
Child with severe trigonalcephaly. What is your management?
- sablage of metopic suture
- helmet
- place child face down
- craniostomy with removal of metopic suture
- observe
- craniostomy with removal of metopic suture
What is the most common reason for surgical intervention in a child who is born with a myelomeningocele:
a. Hydrocephaly
b. Chiari malformation
a. Hydrocephaly
Hydrocephalus with type 2 chiari in 80% (hydroceph is the reason to do surgery)
o Surgery within 1-7d (sooner if CSF leak), most require shunt, bladder catheterization, enemas
Which of the following is associated with spinal cord anomalies in a newborn:
a. anorectal anomalies
b. arthrogryposis
c. malrotation
d. dislocated hip
ANSWER: a. anorectal anomalies
Caudal regression syndrome (more common in IDM)
o anorectal malformation, kidneys, urinary tract, sacral abnormalities
b. arthrogryposis (congenital contractures)
6 mo old kid with vascular malformations of upper face. Complication you need to monitor?
a. Ipsilateral hearing loss
b. Cerebral AVM
c. Glaucoma
c. Glaucoma
Picture of a baby with right facial droop, able to wrinkle forehead, able to close eyes. Identify abnormality.
Asymmetric Crying Face (absence of depressor anguli oris muscle) - eye and forehead unaffected, compared to facial nerve palsy
A 4 month old child is observed to have positional plegiocephaly. What do you recommend to the parents?
a) encourage tummy time
b) refer to neurosurgery to rule out craniosynostosis
a) encourage tummy time
o 40% (highest incidence) at 4 months (babies can’t hold their own heads up until 4 months of age), then decreases - 3.3% at 24 months
o usually resolves by age 2-3 years
Child with facial nevus in V1 distribution. Came to the hospital with focal seizure. What is the CT head finding expected? Name 2 other complications.
Sturge Weber syndrome
- Abnormal blood vessel in brain (leptomeningeal angioma aka leptomeningeal capillary-venous malformation)
- Abnormal blood vessel of eye (leading to glaucoma)
- focal seizures
- hemiparesis
- developmental delay
- stroke like episodes
Four month infant who has recurrent extensor and flexor movements, preceded by a sharp cry, often in the early morning. Noted to have hypopigmented patches of skin. (1) What is your diagnosis of his acute problem? (1) What test would you use to confirm this ? What is his underlying condition (1) ? What test would you do to confirm this (1) ?
- infantile spasms
- EEG - hypsarrhythmia
- tuberous sclerosis
- MRI or genetic testing (TSC1 or TSC2 mutation)
Child with hypopigmented marks on his body and seizures. What is the diagnosis? How is it inherited? What test to you do to establish a diagnosis?
- tuberous sclerosis
- autosomal dominant with variable expression
- MRI +/- genetic testing
Neurofibromatosis – description of a kid with it. How many criteria need to be met to diagnose NF1? Name four of the criteria
Need two of seven:
- cafe au lait macules (6 or more)
- 2+ iris lisch nodules
- axillary/inguinal freckling
- 2+ neurofibromas
- optic gliomas
- bone changes - sphenoid dysplasia, cortical thinning of long bones
- first degree relative with NF1
A 3 and a half year old female had been developing normally, then over the past 6 months has been losing milestones. Which of the following diagnosis is this consistent with:
a. Tuberous sclerosis
b. GM-1 gangliosidosis
c. Adrenoleukodystrophy
d. MELAS
b. GM-1 gangliosidosis
GM1- neurodegeneration (ataxia, seizures, regression), HSM, cherry-red spot, skeletal deformities- AR
*Adrenoleukodystrophy- peroxisomal disorder (accumulation of VLCFA)- X-linked (boys)- females mildly
affected much later
List 4 features of Rett syndrome.
- acquired microcephaly
- hand wringing
- plateau and then regression of developmental milestones
- seizures
- scoliosis
Scenario of a child dying with metachromatic leukodystrophy who is palliative. You want to start opiods at home for pain control, What are four things/four principles you will have to consider when starting opioids?
- dosing will need to be individualized and titrated to effect
- correct dose is one that relieves pain with good margin of safety
- anticipate adverse effects (constipation, nausea, itching)
- consider PCA
A mom comes to see you about her 2 year old previously well child who is having daily episodes where her trunk is writhing, and she becomes flushed and diaphoretic. She also grunts, and breathes rapidly during these. Mom is sure she does not lose consciousness and if she talks to her daughter, she can shorten the duration of the episode. They happen when the child is in her car seat or watching t.v. What is your next step?
a. Refer to neurology for an EEG
b. GI imaging to r/o reflux
c. Reassure mom
d. Refer to a psychologist
c. Reassure mom
Masturbation may occur in girls 2-3 yr of age and is often associated with perspiration, irregular breathing, and grunting, but no loss of consciousness
Which is an indication (most specific) of seizure activity in a neonate?
- tachycardia
- abnormal eye movements
- irregular breathing
- irritability
- abnormal eye movements
Subtle- eye deviations, nystagmus, blinking, mouthing, abnormal extremity movements, fluctuations in heart rate, hypertension, apnea
A 3 month old baby with myclonic jerks of the head and arms. What is the most likely diagnosis:
- Juvenile myoclonic epilepsy
- Benign myoclonus of infancy
- Lennox-Gastaut
- Landau-Kleffner
- Benign myoclonus of infancy - myoclonic jerks of extremities in wakefulness/ sometimes sleep, similar to shuddering attacks (rapid tremor of head, shoulder, trunk lasting a few seconds, associated with eating, recurring many times per day). May be mistaken for infantile spasms, but EEG, MRI and development are normal, spontaneous remission within few months
* 3. Lennox-Gastaut → Triad: difficult to control seizures, slow spike-wave EEG during awake state, mental retardation
A child in status epilepticus. HR 220, respirations difficult to assess, BP 150/80. Unable to get IV access. What to do:
- Sodium nitroprusside
- Rectal diazepam
- Intubate
- IM Dilantin
- Rectal diazepam
* then monitor for respiratory depression (more common with diazepam than lorazepam)
A 7 year old girl has had 3 episodes over the past 3 months where she awakes from sleep, has twitching of her right upper lip and is unable to verbalize for 3 minutes. What is the likely diagnosis:
a. benign rolandic epilepsy of childhood
b. juvenile myoclonic epilepsy
c. parasomnias
a. benign rolandic epilepsy of childhood
- Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS)
A 7 year old girl has had 3 episodes over the past 3 months where she awakes from sleep, has twitching of her right upper lip and is unable to verbalize for 3 minutes. What is the treatment?
Rx: clobazam, carbamazepine (can induce myoclonus), or no treatment if mild and rare
Adolescent female noted to have ?seizure post soccer game. There is a family history of seizures and the GP starts her on phenobarb prophylactically. Despite this, she has a second episode. What would you do next?
a. EEG
b. ECHO
c. Holter for 24 hours
d. ECG
e. exercise ECG
d. ECG
children with new-onset seizure disorder should get an ECG to rule out LQT syndrome masquerading as a seizure disorder.
Cardiac syncope is usually sudden without the gradual onset and the symptoms that accompany vagal syncope
All of the following can cause seizures in the neonate EXCEPT:
a) hyperkalemia
b) hyponatremia
c) hypomagnesemia
d) hypocalcemia
e) alkalosis
a) hyperkalemia
alkalosis can cause decreased ionized calcium due to increased binding of calcium to albumin
Child with absence seizures. What do you tell the parents:
a) significantly increased risk of generalized tonic-clonic seizures in the future
b) should be seizure-free within 2 years
c) will have more absence seizures as time goes on
d) increased risk of developmental delay
e) prognosis is generally poor
a) significantly increased risk of generalized tonic-clonic seizures in the future
Onset 5-8y, Offset 10-12y
▪ <4y should evaluate for glucose transporter defect (low CSF glucose) gene- GLUT-1 def
What is the best medication for treating absence seizures:
a) valproic acid
b) clonazepam
c) carbamazepine
d) phenytoin
e) phenobarbital
a) valproic acid
Ethosuximide also a good option
3 month old with myoclonic jerks. No skin lesions. EEG shows disorganized background with intermittent discharges. What is the most likely diagnosis?
a. infantile spasms
b. benign myoclonus of infancy
a. infantile spasms (now called epileptic spasms - can happen past infancy)
- onset typically between 4-8 months of age
- underlying cause in ¾ of pt (perinatal, asphyxia, congenital, malformation, TS)
- hypsarrhythmia = high voltage b/l irregular high voltage spike + wave pattern
Parent of 2 yr with infantile spasms , DNR. Newly moved to your community. what is the management?
● Tx: vigabatrin first line (especially with TS) +/- prednisone, then maybe ACTH
o Awake and asleep EEG done 1,2, 4 wk after initiation of Vigabatrin to monitor patient’s response
o AE in higher doses: HTN, electrolyte imbalance, infections, high BG, glycosuria, gastric ulcers
Child presents with GTC of 10 minutes and is febrile. He has a history of febrile seizures. What questions do you ask to see if he is at risk of developing epilepsy (4). What is his absolute risk of developing epilepsy. If he had risk factors, what would his absolute risk be of having epilepsy?
Questions to ask: family hx of epilepsy, abnormal neurodevelopment, previous brain injury/damage, complex febrile seizures
- simple = <15 mins, 1sz in 24h, generalized; risk of recurrence of febrile sz 30-50%, risk of afeb sz 2% (general population 1%)
- risk of epilepsy if has risk factors: 20-50%
2 year girl who has episodes of abnormal breathing and movements. Occurs when watching TV or bored. Mum can decrease length of episodes when talking to her. Episodes last 5-6 min. She seems responsive throughout the episodes. What do you recommend to do to mum
Reassurance - this is self stimulation and is normal behaviour
Kid with facial twitch in the middle of the night. Seizure pattern?
A. Centro-temporal
b. 3 hz spike and wave
c. hypsarrhythmia
A. Centro-temporal
- seizures at night - think benign rolandic seizures/BECTS
- face twitching/can’t speak
- usually spontaneously resolve, no meds needed
Kid on longstanding phenytoin and valproic acid. Well controlled sz. Has gums so big he can’t eat, it hurts. What do you do:
a) Stop phen
a) Decrease phen
b) Stop valproic
c) Decease valproic
a) Stop phen
VPA: weight gain, tremor, alopecia, hepatic and pancreatic toxicity
Phenytoin: gingival hypertrophy, hirsutism, coarse facies, nystagmus, ataxia, liver toxicity
Child wakes up every night, ++ scared and screaming, no recollection in the morning. Parents are tired and frustrated. What to do?
a) wake him up 11:45 pm every night for a week
b) prescribe a benzo
c) refer to psychiatry
a) wake him up 11:45 pm every night for a week - kind of right… peds in review says to continue this for 2-4 weeks
Child has frightening awakenings, screams, cries. No recollection in the morning.
What is the most likely diagnosis?
a. nightmares
b. night terrors
b. night terrors
What is the most specific indication of seizures in neonate
a) tachycardia
b) abnormal eye movements
c) irregular breathing
d) Tachypnea
b) abnormal eye movements
Newborn, mom poor PNC, remote history of IVDU. Kid is having a seizure:
o Give pyridoxine
o Phenobarbital 20 mg/kg
o Morphine infusion
o Phenobarbital 20 mg/kg
*o Give pyridoxine - consider when seizures begin shortly after birth and are resistant to conventional antiepileptics
History of child waking up with garbled, confused speech. What would you expect on EEG?
a. normal EEG
b. 3 spikes/wave
c. centrotemporal spikes
d. hypsarrhythmia
c. centrotemporal spikes
What is a dysphasic aura?
least-common type of typical aura - described as an inability or difficulty to respond verbally. The patient afterwards will describe an ability to understand what is being asked, but cannot answer back . (previously called confusional migraine)
What are the two components of the symptoms seen with hypoglycemia?
- activation of autonomic nervous system and epinephrine release - anxiety, sweating, palpitations, tremor, nausea, vomiting
- cerebral glucopenia: HA, confusion, staring spells, diplopia, personality change, dysarthria, paresthesia, dizziness, lethargy, seizure, coma, stroke
Which medication do you choose to treat seizures with 3 Hz spike & wave pattern on EEG:
a) valproic acid
b) carbamazepine
c) clonazepam
d) phenytoin
a) valproic acid (absence seizures)
- ethosuximide is first line (as effective as VPA but less toxicity)
- second line is VPA and lamotrigine
8 month old child presents with seizures. He is dehydrated and his sodium is 121. What do use for treatment?
a) Ativan
b) 3% Saline
c) phenobarb
b) 3% Saline
Seizing child, decreased LOC. Best treatment?
a. intranasal midaz
b. iv valproic acid
c. iv phenytoin
d. Intubate
a. intranasal midaz
Which is the best treatment for tension headaches?
(a) acetaminophen
(b) ibuprofen
(c) codeine
(a) acetaminophen - better studied in children and better safety profile - ibuprofen is better for migraines
What is the most frequent cause of school absence in teenage girls:
(a) headache
(b) dysmenorrhea
(c) asthma
(d) sore throat
(b) dysmenorrhea
Teenager with migraine. Most likely to abort headache:
a. acetominophen
b. ibuprofen
c. caffeine
d. codeine
b. ibuprofen
Which of the following would be an indication for migraine prophylaxis:
- Headaches impact on daily activities
- Headaches are triggered by cold weather
- Headaches impact on daily activities
A 5-year-old boy has been experiencing constant headaches for the past 3 months. They are getting worse and are interfering with his functioning. Which diagnosis should you consider: a ) brain tumor b) migraine headaches c) tension headaches d) behavioral problem
a ) brain tumor (esp since constant)