Neurology Flashcards
1
Q
Child with severe trigonalcephaly. What is your management?
- sablage of metopic suture
- helmet
- place child face down
- craniostomy with removal of metopic suture
- observe
A
- craniostomy with removal of metopic suture
2
Q
What is the most common reason for surgical intervention in a child who is born with a myelomeningocele:
a. Hydrocephaly
b. Chiari malformation
A
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
3
Q
Which of the following is associated with spinal cord anomalies in a newborn:
a. anorectal anomalies
b. arthrogryposis
c. malrotation
d. dislocated hip
A
ANSWER: a. anorectal anomalies
Caudal regression syndrome (more common in IDM)
o anorectal malformation, kidneys, urinary tract, sacral abnormalities
b. arthrogryposis (congenital contractures)
4
Q
6 mo old kid with vascular malformations of upper face. Complication you need to monitor?
a. Ipsilateral hearing loss
b. Cerebral AVM
c. Glaucoma
A
c. Glaucoma
5
Q
Picture of a baby with right facial droop, able to wrinkle forehead, able to close eyes. Identify abnormality.
A
Asymmetric Crying Face (absence of depressor anguli oris muscle) - eye and forehead unaffected, compared to facial nerve palsy
6
Q
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
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
7
Q
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.
A
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
8
Q
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) ?
A
- infantile spasms
- EEG - hypsarrhythmia
- tuberous sclerosis
- MRI or genetic testing (TSC1 or TSC2 mutation)
9
Q
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?
A
- tuberous sclerosis
- autosomal dominant with variable expression
- MRI +/- genetic testing
10
Q
Neurofibromatosis – description of a kid with it. How many criteria need to be met to diagnose NF1? Name four of the criteria
A
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
11
Q
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
A
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
12
Q
List 4 features of Rett syndrome.
A
- acquired microcephaly
- hand wringing
- plateau and then regression of developmental milestones
- seizures
- scoliosis
13
Q
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?
A
- 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
14
Q
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
A
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
15
Q
Which is an indication (most specific) of seizure activity in a neonate?
- tachycardia
- abnormal eye movements
- irregular breathing
- irritability
A
- abnormal eye movements
Subtle- eye deviations, nystagmus, blinking, mouthing, abnormal extremity movements, fluctuations in heart rate, hypertension, apnea
16
Q
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
A
- 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
17
Q
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
A
- Rectal diazepam
* then monitor for respiratory depression (more common with diazepam than lorazepam)
18
Q
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
a. benign rolandic epilepsy of childhood
- Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS)
19
Q
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?
A
Rx: clobazam, carbamazepine (can induce myoclonus), or no treatment if mild and rare
20
Q
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
A
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
21
Q
All of the following can cause seizures in the neonate EXCEPT:
a) hyperkalemia
b) hyponatremia
c) hypomagnesemia
d) hypocalcemia
e) alkalosis
A
a) hyperkalemia
alkalosis can cause decreased ionized calcium due to increased binding of calcium to albumin
22
Q
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
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
23
Q
What is the best medication for treating absence seizures:
a) valproic acid
b) clonazepam
c) carbamazepine
d) phenytoin
e) phenobarbital
A
a) valproic acid
Ethosuximide also a good option
24
Q
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
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
25
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
26
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%
27
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
28
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
29
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
30
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
31
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
32
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
33
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
34
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
35
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)
36
What are the two components of the symptoms seen with hypoglycemia?
1. activation of autonomic nervous system and epinephrine release - anxiety, sweating, palpitations, tremor, nausea, vomiting
2. cerebral glucopenia: HA, confusion, staring spells, diplopia, personality change, dysarthria, paresthesia, dizziness, lethargy, seizure, coma, stroke
37
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
38
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
39
Seizing child, decreased LOC. Best treatment?
a. intranasal midaz
b. iv valproic acid
c. iv phenytoin
d. Intubate
a. intranasal midaz
40
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
41
What is the most frequent cause of school absence in teenage girls:
(a) headache
(b) dysmenorrhea
(c) asthma
(d) sore throat
(b) dysmenorrhea
42
Teenager with migraine. Most likely to abort headache:
a. acetominophen
b. ibuprofen
c. caffeine
d. codeine
b. ibuprofen
43
Which of the following would be an indication for migraine prophylaxis:
1. Headaches impact on daily activities
2. Headaches are triggered by cold weather
1. Headaches impact on daily activities
44
```
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)
45
A 10-year-old child has a history of migraine headaches. Which drug should he take at home for symptomatic relief from headaches:
a) acetaminophen
b) codeine
c) gravol
d) sumatriptan
e) maxeran
a) acetaminophen
- ibuprofen would be better option
- sumatriptan not approved for kids under 12
46
Which of the following is not prescribed for migraine prophylaxis:
a. propranolol
b. methylsegide
c. sumatriptan
d. phenytoin
e. amitriptyline
c. sumatriptan → abortive not prophylactic
- prophylaxis indicated for >1/wk and disabling (missing activities) >1/month
- some of these are old (methylsegide and phenytoin)
- propranolol and amitriptyline are used for prophylaxis; flunarizine is a CCB that is very effective
- neutraceuticals: Coenzyme Q10, riboflavin, Mg
47
Describes a child with headaches, emesis, acne, being teased at school. Exam shows papilledema. Diagnosis?
Idiopathic intracranial hypertension (pseudotumour cerebri) secondary to isotretinoin
48
List 3 reasons to image a child with headaches
- focal neurologic abnormalities on exam
- waking from sleep
- early morning vomiting
- headache improved with sitting up, worse with lying flat
- aura <5 minutes or >60 minutes
- brief headache only associated with bending forward or coughing
- occipital headache
- instantaneous "worst headache ever"
49
A 10 year old boy presents to your office with a history of waking in the mornings with a headache for the past month. He occasionally vomits with the headache. Over the past week he has started to complain of double vision, and feels that he is unsteady on his feet. What is your diagnosis?
posterior fossa tumour (most common are medulloblastoma, cerebellar astrocytoma)
50
Kid with abdominal migraine. Family history of migraines. What are two periodic syndromes associated with migraine development as an adult.
- cyclic vomiting syndrome, recurrent abdominal pain
| - benign peroxysmal vertigo
51
Child with headaches interfering with function. What to prophylaxis with?
a. Amitriptyline
b. Pimozide
c. Some type of SSRI (I think Effexor)
d. Valproic acid
a. Amitriptyline
52
What is the most common cause of childhood headaches.
a) Migraine
b) Myopia
c) Sleep disorder
a) Migraine (most frequent recurrent HA brought to medical attention)
- and tension type headaches also common childhood HA
53
What medications can be used for the treatment of status migrainosus?
all IV - use in ED or inpatient setting
- prochlorperazine, metoclopramide (can get EPS - have diphenhydramine ready)
- ketorolac
- dihydroergotamine
- valproate sodium injection
54
Which type of brain injury is most commonly seen in shaken baby syndrome?
a. epidural hemorrhage
b. subdural hemorrhage
c. intraventricular hemorrhage
b. subdural hemorrhage - most common abusive head injury
55
Sickle cell anemia, which is true about strokes?
a. usually subclinical strokes
b. clinical stroke
a. usually subclinical strokes
- they are ischemic, not hemorrhagic
- mgmt: blood transfusion to get hemoglobin close to but not >100
56
A 1 month old has a droopy lower left lip. The forehead moves normally. What is the problem:
1. centrally mediated facial nerve palsy
2. peripherally mediated facial nerve palsy
3. congenital absence of the mouth angle depressor muscle
3. congenital absence of the mouth angle depressor muscle
57
Guillain Barre has been associated with which of the following infections?
a. e coli gastro
b. shigella
c. campylobacter
c. campylobacter
- occurs about 10 days following specific GI/resp infections
o GI: campylobacter jejuni , helicobacter pylori
o Resp: mycoplasma
58
14 year old boy with Duchenne’s, who is in a wheelchair, has recently seen his FVC fall from 30 to 21% predicted. What symptom will he most likely be complaining of?
a. Headache early in morning
b. Headaches in the afternoon
c. Tingling of his fingers
d. Dyspnea with exertion
a. Headache early in morning
59
In a child with myopathy, which of the following could help distinguish Dermatomyositis from Duchenne’s MD?
a) proximal muscle weakness
b) rash on face and knuckles
c) abnormal muscle enzymes
d) onset before age 5 years
e) more commonly affects girls than boys
b ) rash on face and knuckles
*d) onset before age 5 years (both usually after 5)
60
3 yo with proximal muscle weakness. You suspect Duchenne. How do you confirm the dx?
a. Dystrophin assay
b. Biopsy
c. Molecular testing
c. Molecular testing
| - blood PCR for dystrophin gene - if positive can defer biopsy
61
Child with proximal muscle weakness and decreased DTR
a. Congenital myopathy
b. Peripheral neuropathy
c. Spinal cord
d. Neuromuscular junction problem
a. Congenital myopathy
62
Kid with bilateral proximal muscle weakness. Dysphagia, bilateral ptosis, difficulty with upward gaze. Slow onset. Give the Dx. Gave a whole bunch of normal blood work, creat, TSH, CK was normal.
Myasthenia gravis
63
Child with suspected Duchenne muscular dystrophy. What is diagnostic on biopsy (be specific)? What 2 things do you want to know to help with genetic counseling?
1. lack of detectable dystrophin in myofibrils, endomysial connective tissue proliferation, increased connectivity between muscle fibres
2. X-linked, 2/3 inherited - need to know if mother is a carrier, if plan to have more kids, consider if mom has sisters who have or are planning to have kids
64
Duchene muscular dystrophy 7 year old. List 4 complications you will monitor for.
- scoliosis
- cardiomyopathy and heart failure
- weakness of swallowing muscles and aspiration
- restrictive lung disease
- contractures
65
Infant with symptoms of myotonic dystrophy described. Give diagnosis. (for practice, give 4 signs/symptoms of myotonic dystrophy)
Generally normal at birth, may have hypotonia
- characteristic facies: inverted V shaped mouth, thin cheeks, high palate, concave temporalis muscles
- DISTAL muscle wasting (different from most muscular dystrophies)
- myotonia - very slow relaxation of muscles after contraction (e.g. the handshake grip that can't let go)
- cataracts
- heart block, hypothyroidism
66
Most specific finding in duchenne’s
a. pelvic girdle weakness
b. fasciculations
c. distal muscle weakness
a. pelvic girdle weakness
67
17 year old male with Duchenne’s MD. His FVC has gone from 30% to 21%, what does he complain of?
a) AM somnolence
b) PM somnolence
c) dyspnea with activities
a) AM somnolence - due to hypoventilation at night
68
Child with suspected Duchenne’s Muscular Dystrophy. What is the best test? (that was the exact wording...)
a. serum dystrophin assay
b. biopsy
c. molecular studies
d. EMG
b. biopsy
- reasonable to do molecular studies first (if positive you have the diagnosis and biopsy is not necessary), BUT if negative and still suspect DMD then need to do biopsy - detects the 30% of cases that do not have abnormality on PCR
69
An 8 year old girl is in a motor vehicle accident and has had significant pain in her left upper extremity. Her symptoms are consistent with neuropathic pain. What treatment do you recommend?
a) opioids
b) gabapentin
c) NSAIDs
b) gabapentin
70
Newborn with an Erb’s palsy. Which is true?
a. extension at the wrist
b. preserved grasp
c. symmetric moro
b. preserved grasp
71
Baby born with inability to open one eye and pupillary constriction - which nerves are likely to have been injured at birth?
a) C5,C6,C7
b) C5,C6,C7,C8,T1
c) C7,C8,T1
c) C7,C8,T1 - T1 involvement leads to Horner's (pupillary constriction)
*a) C5,C6,C7 → Erb’s - 80% recover (best prognosis)
b) C5,C6,C7,C8,T1 → Erb’s + Horner’s
C8, T1 --> Klumpke's - forearm supinated, wrist and fingers flexed, grasp reflex lost, "claw hand", ptosis and miosis
72
You are discussing with a mother the prognosis of her child born at 31 weeks gestation. There was thin meconium at birth. APGARs were 8 and 9. Head ultrasound showed a small intraventricular hemorrhage at one week of age. She is concerned because he is not yet walking and has stiff legs.
1. the child likely has spastic diplegia which is often associated with prematurity and intraventricular hemorrhage
2. The history is unusual in that children with CP usually have a history suggestive of birth asphyxia
3. IVH would cause PVL which would result in hemiplegia
4. there will be progressive decline in his development
1. the child likely has spastic diplegia which is often associated with prematurity and intraventricular hemorrhage
73
In which of the following diseases is there persistence of the asymmetric tonic neck reflex and 9 months of age:
a. Down syndrome
b. Werdnig-Hoffman disease
c. Spastic cerebral palsy
c. Spastic cerebral palsy
74
What is the most common cause of spastic diplegia:
a. Prenatal brain injury
b. Periventricular leukomalacia
c. Birth asphyxia
b. Periventricular leukomalacia
75
Ex-29 week prem with hyperreflexia and gross motor delay. Had history of Grade II IVH and PVL. Advise the mother about the likely cause of CP in this child. What would you see on a CT that is specific to this?
1. likely spastic diplegia secondary to PVL which occurred as a complication of prematurity
2. CT: necrotic lesions in periventricular white matter
76
10 mo. old with Cerebral Palsy. (3) 3 signs/symptoms clinically that might confirm CP
- spasticity
- scissoring of legs, palming of thumb
- persistence of primitive reflexes
- brisk DTRs
- swallowing difficulties
77
Description of an 8 month old child who has right hand preference and spasticity of his extremities . Birth history was uneventful. What is the most likely diagnosis? What would you recommend in your management?
1. hemiplegic spastic cerebral palsy
2. multidisciplinary approach with PT and OT, may need SLP
- MRI to rule out other diagnoses (tumour)
- mgmt/prevention of contractures - stretching, bracing, achilles tenotomy, botox injections
78
2 year old M you recently diagnosed with spastic diplegia. APGAR at 1min was 6. Nothing else to note. Parents want to know the cause:
a) His normal APGARs rule out birth asphyxia as the cause
b) Likely pre-natal insult
c) Likely perinatal asphyxial insult
d) Can’t say because you cannot diagnosis CP at the age of 2
b) Likely pre-natal insult
79
10 month old has diagnosis of spastic diplegia. Apgars were 6 at one minute, 8 at 5 minutes. Parents want to know about the possibility of birth asphyxia causing his spastic diplegia. You tell them:
a) His apgar scores rule out the possibility of birth asphyxia
b) The etiology of spastic diplegia is usually related to antenatal event
c) He’s too young for the diagnosis of CP
b) The etiology of spastic diplegia is usually related to antenatal event
80
Toddler with "description of breath-holding spells". Your presumptive diagnosis is breath-holding spells. What treatment, if any, would you recommend to mom?
1. try to intervene before child becomes overly distressed (e.g. time out); ignore breath holding behaviour (do not incentivize); reassurance - no increased risk of epilepsy even if had seizure with breath holding
- rule to iron deficiency
81
A 12 month old child has had several episodes of crying, followed by cyanosis and then a few seconds of generalized convulsions. These convulsions resolve spontaneously and his behaviour is normal post-ictally. What is the most likely etiology?
a) infantile spasms
b) breath holding spells
c) febrile seizures
d) myoclonic epilepsy
b) breath holding spells
82
Which of the following is least associated with increased intracranial pressure?
a. meningitis
b. encephalitis
c. TCA overdose
d. Intracranial bleed
e. Tumour
c. TCA overdose
83
```
A 12 year old child comes to you with a 2 week history of severe headaches awakening her from sleep in the morning. She now has with an acute onset of diplopia. On examination she has papilledema and a left 6 th cranial nerve palsy. CT and MRI of her head are normal What
diagnosis do you consider first:
a. Lyme disease
b. Atypical GBS
c. Intracranial hypertension
```
c. Intracranial hypertension
84
Girl with acne has pseudotumour cerebri. Which is the most likely etiologic drug?
a. OCP
b. Tetracycline
c. Topical tretinoin
d. Clindamycin
e. EMG
b. Tetracycline
85
6 year old who has a history of diplopia, headache and ataxia. Where is the lesion? (1) What are the two most likely brain tumours for the lesion
1. posterior fossa brain tumour
| 2. medulloblastoma, cerebellar astrocytoma
86
Child with headaches and papilledema – no other focal signs, physical exam normal, no fever... cant remember all the details... what is the most likely cause of his headache?
Need to rule out mass or vascular lesion - MRI/MRA; if normal then likely IIH
87
A child has developed ataxia, a head tilt and diploplia. What is most likely?
a. Craniopharyngioma
b. Brainstem glioma
c. Posterior fossa hematoma
b. Brainstem glioma
88
7 year old boy with diplopia, head tilt and ataxia. Which is the most likely lesion?
a. Brainstem glioma
b. Cerebellar astrocytoma
c. Posterior fossa hemorrhage
d. Craniopharyngioma
a. Brainstem glioma
- epidemiologically cerebellar astrocytoma is most common posterior fossa tumour but brainstem glioma specifically associated with double vision as key presenting symptom, as well as ataxia and head tilt
89
Kid eats like crazy, very skinny, runs up to hug you. What does this kid have?
a. diencephalic syndrome
b. emotional deprivation
b. emotional deprivation - per Hamilton review - but depending on additional info given, this really could be in keeping with diencephalic syndrome
90
12 year old M with recent change in behaviour, irritability, daily headaches and a change in his vision. What is the most important thing to consider?
a. Brain Tumour
b. Drug use
c. Psychiatric Diagnosis
a. Brain Tumour
91
6 y o with severe headache collapses and has progressive posturing. After intubation, what would you do?
a) mannitol IV
b) urgent CT scan
a) mannitol IV
- concern for raised ICP because of posturing - need to treat presumptively if you think someone is coning and not wait for imaging to confirm
92
Kid with signs of increased ICP, teased at school because of obesity and acne. PE reveals papilledema, MRI head normal. No sexual activity. What is the most likely cause?
a. oral contraceptive
b. minocycline
b. minocycline
93
6 y/o boy with 2 weeks of sudden onset of OCD behaviours. Which infectious agent would you be concerned about:
1. Strep pneumonia
2. Group A Strep
3. E. Coli
4. H. Flu
5. Echovirus
2. Group A Strep
- PANDAS: Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infection: pediatric autoimmune disorder after strep infection - molecular mimicry, abrupt OCD, tics, emotional lability
94
A child has developed motor tics and symptoms of OCD. Which organisms is associated with this?
a. S. pneumonia
b. Group A strep
b. Group A strep
95
Boy with recurrent pharyngitis. Episodic jerking of shoulders and head. Most likely diagnosis?
a. Rheumatic fever
b. Transient tic disorder
c. Tourettes
d. Some sort of seizure disorder
b. Transient tic disorder
| - episodic jerking sounds more like tic than chorea, but consider ARF depending on description - could be Syndeham's
96
Kid presents with facial tics. What would support a diagnosis of Tourette’s?
a. tics are present for 6 months
b. family history of tics
c. attention-deficit, hyperactivity disorder
b. family history of tics
| * a. tics are present for 6 months ( No, need to be present for >1yr to make dx )
97
8 year old girl with subtle choreiform movements and emotional lability for 2 weeks. All tests including a throat swab are normal. What is the likely diagnosis.
1. Huntington’s Chorea
2. Sydenham’s Chorea
3. Lupus
2. Sydenham’s Chorea
SLE is a cause of chorea, though not the typical presenting complaint
98
Parents bring their 18 month old son to see you because of concern about head banging. What treatment, if any, do you offer (1)?
● Reassurance, avoid triggers, don’t scold; ignore behaviour so there is no secondary gain in getting parental attention
99
Child presenting with writing difficulties and seems moody. What does she have?
a. Lupus
b. Sydenham’s chorea
c. Tourettes
d. Hungtinton’s disease
b. Sydenham’s chorea
chorea + emotional lability - chorea can impact writing ability
100
Picture of a CT scan of the head with intracranial calcifications. What is the diagnosis?
prenatal CMV infection; also could be HIV
101
Kid with hearing loss. Test most likely to help with etiology?
a. + CMV in urine
b. MRI
a. + CMV in urine
- depending on age - in a newborn, yes (hearing loss is not associated with postnatally acquired CMV infection)
- most common cause conductive hearing loss is middle ear fluid
102
List 4 absolute contraindications to LP
- thrombocytopenia, DIC
- space occupying lesion in brain
- cellulitis over LP site
- cardioresp instability
- spinal anomalies
103
Child presents with ataxia and inability to sit up two weeks after having chicken pox.
A) What is the diagnosis?
B) How do you differentiate this from meningoencephalitis? List three.
```
A) post infectious acute cerebellar ataxia - expect improvement within 1 wk of symptom onset and full recovery in 3 months
B) afebrile
- no meningismus
- normal LOC
- normal mental status
- CSF
```
104
15 yr old girl has a headache, then syncope at school for several minutes. She is brought to hospital. Can’t walk because of numbness in her legs. Exam is normal, plantar reflexes normal, DTR normal. Initial loss of sensation to L4, the next day she has sensation to her ankles. Able to
walk without ataxia leaning heavily on your hands, feet spaced 8 cm apart. What is your next step in management?
a. EEG
b. MRI head and spine
c. Confrontation and explanation that her symptoms are not organic
d. Refer to PT
c. Confrontation and explanation that her symptoms are not organic
- not "confrontation" but need to explain that presentation is more on the psychosocial than biologic spectrum of disease
- mgmt is psychological - CBT, relaxation, biofeedback
105
Child at hospital with tonic movements. Consciousness is preserved. He also has ataxia, vomiting, and inability to close mouth. He has been vomiting for several days and mom has been giving him a suppository but can't remember its name. What do you do?
1. Rectal diazepam
2. Nothing
3. Diphenhydramine
4. Phenobarbitol
3. Diphenhydramine (treating presumed EPS from something like rectal anti-emetic)
106
Side effects of hepatotoxicity, pancreatitis and low platelets. Which drug?
a. Phenytoin
b. Carbamazepine
c. Phenobarb
d. Valproic Acid
d. Valproic Acid
107
Which of the following is the main reason that diazepam is not commonly used as an anticonvulsant in neonates:
a. Increased volume of liquid distribution
b. Decreased volume of fat distribution
c. Decreased liver metabolism
c. Decreased liver metabolism
108
Which of the following is higher in neonates vs. adolescents:
a. Volume of liquid distribution
b. Volume of fat distribution
c. Speed of liver clearance
d. Speed of renal clearance
ANSWER: a. Volume of liquid distribution
* b. Volume of fat distribution → lower
c. Speed of liver clearance → slower
d. Speed of renal clearance → slower
109
A child on dilantin is given septra for an infection. She returns ataxic with abnormal speech. What is the mechanism of this interaction?
a. Septra increases absorption of Dilantin
b. Septra displaces dilatin from protein binding sites
c. Septa causes decreased metabolism of Dilatin
d. Septra causes decreased excretion of Dilantin
c. Septa causes decreased metabolism of Dilatin (CYP2)
110
Which of the following newborns requires surfactant administration according to recent guidelines:
a. Any newborn being transferred between centres
b. A 29 week infant with no symptoms being transferred between centres
c. A critically ill infant who has required 3 doses of surfactant in the first 18 hours
d. An infant with RDS who is intubated and requiring more than 50% O2 after 72 hours
?BEST ANSWER: d. An infant with RDS who is intubated and requiring more than 50% O2 after 72 hours - no evidence for use >72h
No evidence for giving more than 3 doses of surfactant
