Neurology - Updated 2019! 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
c) Syringomyelelia
d) Tethered cord
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)
d. dislocated hip in spina bifida occulta
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, normal nasalabial folds. Identify abnormality.
Asymmetric Crying Faceies (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
1. Abnormal blood vessel in brain (ipsilateral leptomeningeal angioma aka leptomeningeal capillary-venous malformation)
- Abnormal blood vessel of eye (leading to glaucoma)
- visual field deficits
- focal seizures
- hemiparesis
- developmental delay
- stroke like episodes
- Intellectual disability
- Growth hormone deficiency
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)
TS Dx can be bade on genetics or clinically (Definite 2 major or 1 major and 2 minor)
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): > 5mm prepubertal and >15mm post pubertal
- 2+ lisch nodules (Irish hamartomas)
- axillary/inguinal freckling
- 2+ neurofibromas
- optic gliomas
- bone changes - sphenoid dysplasia, cortical thinning of long bones, ± pseudoarthrosis
- 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
Lysosomal storage disorder. 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.
Progressive dysfunction of adrenal cortex, central and peripheral nervous centre white matter. ADHD -> vegetative state.
MELAS (mitochondrial encephalopathy. lactic acidosis stroke like episodes) - maternally inherited, stunted growth, episodic vomiting, seizures, recurring cerebral insults. Degenerative.
TS - shouldn’t cause regression
List 4 features of Rett syndrome.
- acquired microcephaly
- hand wringing
- plateau and then regression of developmental milestones
- seizures
- scoliosis
- sighing respirations
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?
- By Mouth : use least invasive method if possible
- By the clock: scheduled to avoid break through
- For the child: right medications tailored to ensure adequate pain relief
- Anticipate side effects
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 at 2-3 years.
*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
Absence: Onset 5-8y, Offset 10-12y
▪ <4y should evaluate for glucose transporter defect (low CSF glucose) gene- GLUT-1 def
30-50% will have GTCs, often years later
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 (with fewer SE than VPA)
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
c. familial myoclonus
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, previous afebrile seizure, age <6mos for first febrile seizure
- Simple = <15 mins, 1sz in 24h, generalized; risk of recurrence of febrile sz 30-50%, risk of epilepsy 1-2% (general population 1%)
- Risk of epilepsy if has risk factors: 20-50%
If just complex febrile Sz= 5-10%
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 and drooling. Seizure pattern? A. Centro-temporal b. 3 hz spike and wave c. hypsarrhythmia d. poly spike wave
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 phenytoin
a) Decrease phenytoin
b) Stop valproic
c) Decease valproic
a) decrease phenytoin. Can reduce risk with folic acid + dental hygiene. May need to change drugs but probably don’t want cold turkey w/o another plan.
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
benign focal epilepsy of childhood - focal motor seizures of mouth, tongue, face or upper extremity. Occur during sleep-wake transition.
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
*** we said Ibuprofen! UTD references one study showing ibuprofen was superior
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)
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
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
Describes a child with headaches, emesis, acne, being teased at school. Exam shows papilledema. Diagnosis?
Idiopathic intracranial hypertension (pseudotumour cerebri) secondary to isotretinoin
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”
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)
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
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
(Pimozide is a high potent antipsychotic)
Can also use VPA but has lots of reactions
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
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
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
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
A 1 month old has a droopy lower left lip. The forehead moves normally. What is the problem:
- centrally mediated facial nerve palsy
- peripherally mediated facial nerve palsy
- congenital absence of the mouth angle depressor muscle
- congenital absence of the mouth angle depressor muscle
Congenital Absence of Depressor Angularis Oris Muscle: Facial asymmetry, especially when infant cries.
Often a/w other anomalies - especially cardiac
Is NOT a facial nerve lesion, does not interfere with feeding
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
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
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)
Criteria for JD: Classic rash (heliotrope, gottron papule) PLUS 3 of: - weakness (symmetric, proximal) - Elevated CK, AST, LD or aldolase -EMG changes -Muscle Bx w/ necrosis/inflammation
3 yo with proximal muscle weakness. You suspect Duchenne. How do you confirm the dx?
a. Dystrophin assay
b. Biopsy
c. Molecular testing
d. EMG
c. Molecular testing
- blood PCR for dystrophin gene - if positive can defer biopsy
Molecular genetic testing is indicated for patients with an elevated serum CK level and clinical findings suggestive of a dystrophinopathy. The diagnosis is established if a disease-causing mutation of the DMD gene is identified
Child with proximal muscle weakness and decreased DTR
a. Congenital myopathy
b. Peripheral neuropathy
c. Spinal cord
d. Neuromuscular junction problem
a. Congenital myopathy
Decreased DTR = LMN
Proximal muscle weakness - a muscle problem
Spinal cord = UMN, expect increased DTR
Peripheral neuropathy and NMJ would expect distal>proximal weakness
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
Dx: Hx/PE, serum autoantibodies, EMG
Tx: Acetylcholinesterase inhibitors, PLEX, IVG
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?
- Absent dystrophin in myofibrils, myofiber necrosis, fibroblast infiltration
- X-linked, ⅔ inherited
- review 3 generation family tree
- 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
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
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
- Feeding difficulties
- 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
Most specific finding in duchenne’s
a. pelvic girdle weakness
b. fasciculations
c. distal muscle weakness
a. pelvic girdle weakness
= proximal muscle weakness
eg. lordotic posture to compensate for gluteal weakness, gower’s sign, trendelenburg gait
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
also see HA
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
c. molecular genetics
UTD: In most cases, genetic testing can confirm the Dx. A small proportion will not be picked up with genetics and will require a muscle biopsy.
EMG is non specific
Serum Dystrophin has many false negatives
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
Newborn with an Erb’s palsy. Which is true?
a. extension at the wrist
b. preserved grasp
c. symmetric moro
b. preserved grasp
Erb - C5/6 palsy, hand and wrist movement preserved
Klumpke- C8/T1, can have isolated hand paralysis and Horner syndrome
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)
Miosis, Ptosis, Anhidrosis
*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
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.
- the child likely has spastic diplegia which is often associated with prematurity and intraventricular hemorrhage
- The history is unusual in that children with CP usually have a history suggestive of birth asphyxia
- IVH would cause PVL which would result in hemiplegia
- there will be progressive decline in his development
- the child likely has spastic diplegia which is often associated with prematurity and intraventricular hemorrhage
Spastic Diplegia - these days is usually due to PVL, but IVH can also be a cause
IVH - usually develops spontaneously especially in PREMS and is less commonly due to trauma or asphyxia. 30% of poems <1500g have IVH
PVL - involves both intrauterine and post natal events - hypoxia-ischemia, venous obstruction from IVH, undetected fetal stress may all contribute to periventricualr hemorrhage and necrosis.
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
d. normal infant
c. Spastic cerebral palsy
Werdnig Hoffman = SMA
Tonic neck should go way by 6-7 mos at the latest
What is the most common cause of spastic diplegia:
a. Prenatal brain injury
b. Periventricular leukomalacia
c. Birth asphyxia
b. Periventricular leukomalacia
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?
- Likely spastic diplegia secondary to IVH/PVL which occurred as a complication of prematurity
- CT: loss of periventricular white matter (MRI), cystic changes, ventricular dilation, thin corpus callosum
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
- Increased tone
- Toe walking
- Flexion contracture
- Delayed walking / delayed motor milestones
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?
- Hemiplegic spastic cerebral palsy
- 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
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
In general
-> Spastic diplegia associated with PVL/ prematurity, or damage between 24 and 32 weeks GA
- > Quadriplegia associated with intrauterine complications (e.g. infection, cerebral dysgenesis)
- > Hemiplegia: isolated antenatal stroke
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
<10% of children with CP had evidence of intrapartum asphyxia
Toddler with “description of breath-holding spells”. Your presumptive diagnosis is breath-holding spells. What treatment, if any, would you recommend to mom?
- 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 out iron deficiency
- CPR training for parents
- Prepare child for unpleasant experiences, do not surprise the kid
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
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
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
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
oral isotretinoin also a/w IICH
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
- posterior fossa brain tumour
2. medulloblastoma, cerebellar astrocytoma
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
A child has developed ataxia, a head tilt and diploplia. What is most likely?
a. Craniopharyngioma
b. Brainstem glioma
c. Posterior fossa hematoma
d. Cerebellar astrocytoma
b. Brainstem glioma
or
cerebellar astrocytoma!
-> Similar Q in onc - was HA, ataxia, diplopia and we said cerebellar astrocytoma b/c it is more common
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
or cerebellar astrocytoma
- 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
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
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
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
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
6 y/o boy with 2 weeks of sudden onset of OCD behaviours. Which infectious agent would you be concerned about:
- Strep pneumonia
- Group A Strep
- E. Coli
- H. Flu
- Echovirus
- 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
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
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
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 and
c. ADHD (up to 60%)
*a. tics are present for 6 months ( No, need to be present for >1yr to make dx )
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.
- Huntington’s Chorea
- Sydenham’s Chorea
- Lupus
- Sydenham’s Chorea
SLE is a cause of chorea, though not the typical presenting complaint
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
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
Picture of a CT scan of the head with intracranial calcifications. What is the diagnosis?
prenatal CMV infection; also could be HIV or toxo (toxo has macrocephaly)
Kid with hearing loss. Test most likely to help with etiology?
a. + CMV in urine
b. MRI
a. + CMV in urine (prior to 3 weeks of age)
- 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
List 4 absolute contraindications to LP
- thrombocytopenia, DIC
- space occupying lesion in brain
- signs of increased ICP (papilledema, CN VI palsy, etc)
- cellulitis over LP site
- cardioresp instability
- spinal anomalies
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 normal (no pleocytosis)
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
d. Refer to physio
- Use integrated multi-d rehab
- treatment that emphasizes return to normal adaptive functioning
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?
- Rectal diazepam
- Nothing
- Diphenhydramine
- Phenobarbitol
- Diphenhydramine
Acute dystonic reactions - common with dopamine blocking agents (including antipsychotics, antiemetics and atypical antipsychotics) - usually involve face and neck.
Side effects of hepatotoxicity, pancreatitis and low platelets. Which drug?
a. Phenytoin
b. Carbamazepine
c. Phenobarb
d. Valproic Acid
d. Valproic Acid
(the only one that has pancreatitis)
Phenytoin: liver dysfunction, SJS
Phb: liver dysfunction, SJS
Carbamazepine: leukopenia, aplastic anemia, thrombocytopenia, liver dysfunction
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
Midaz is hydroxylated by CYP3A4/CYP3A5 - the activity of these enzymes in liver are lower at birth, reach adult levels at 3-12 mos.
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
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 inhibitor)
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: ?
b. CPS - Infants delivered <29 weeks outside teritary center should be considered for immediate intubation and surfactant after stabilization
c. No evidence for giving more than 3 doses of surfactant
d. No evidence for use > 72 h
Abortive Medications for Migraine
Ibuprofen (± acetaminophen)
- your go-to drug, especially if <12 years old
Triptans (5-HT agonist): technically not approved from children/adolescents
Ergotamines
Prophylactic medications for Migraine
Cyproheptadine Anticonvulsants (topamax, VPA, gabapentin) TCAs: amitriptyline Verapamil Propanolol Flunarizine (CCB) Coenzyme Q10, riboflavin, Mg, buterrbur
Child with optic neuritis (bilat) treated with IVIG :
At risk for MS
No risk for recurrent given treatment with IVIG
At risk for visual impairment
At risk for MS
At risk for visual impairment - 20% will have functional visual impairment.
Risk of MS may be lower if optic neuritis occurs in childhood (overall risk 35-45%)
Child with 12 hour hx of esotropia. What do you think of first?
MS
Lyme disease
atypical GBS
increased ICP
increased ICP
*CN6: often first nerve compressed with increased ICP
presents w/ esotropia - dysfunction of lateral rectus
A 7year old male presents with increasing difficulties at school. On exam he has a pale optic nerve and evidence of slight ataxia with subtle pyramidal signs. What is the cause of his problems?
a) Mucopolysaccaridosis
b) Adrenoleukodystrophy
b) Adrenoleukodystrophy
X-Linked - starts with hyperactivity, worsening school performance. Auditory discrimination impaired.
- Vision disturbance -> blind
- Ataxia
- Seizures
- Strabismus
- Adrenal insufficiency
Kid with CP presents with difficulties swallowing. She is tolerating her GT feeds but has difficulty managing her oral secretions. Of note, she got Botox injections to her legs last week. On exam, she has oral secretions and her legs feel less hypertonic. What do you do?
pH probe
do a swallow study
observe in hospital
Observe in hospital
Systemic effect from botox injection - reports of severe swelling problems, breathing difficulty.
Kid vomiting recurrently, missing lots of school, mom has headaches. Most likely cause?
a) abdominal migraines (or cyclic vomiting)
b) benign paroxysmal vertigo
c) separation anxiety
a. Abdominal migraine
Most common cause of seizures in adolescents?
Head trauma
Genetic disorders
Substance abuse
Cortical dysplasia
Depends on who you ask.
UTD: Head Trauma
Most common epilepsy is Juvenile myoclonic Epilepsy
AAP:
The causes of epilepsy are varied. The most common causes of acute seizures are fevers, infections, and head injury, which are detected through history and laboratory testing.
5-day old baby with hypotonia, tongue fasciculation, poor feeding. Absent DTRs. Dad wants to know about risk for future pregnancies?
25%
50%
50% for males only
No increased risk above general popular
25% - AR; SMN1 gene
1-month old routine health visit. What should be part of routine health supervision at diagnosis? Kid has achondroplasia (in addition to anticipatory guidance).
Echo
ECG
EEG
MRI head
MRI head
At risk of spinal stenosis as infants
Also at risk of central apnea due to this so needs polysomnography.
Febrile Seizure X 3 in an otherwise healthy child; how to manage?
Reassurance
Start antiepileptic
Image
Reassure
Recurrence risk:
- 30% risk after first episode
- 50% risk after 2 or more
- 50% in infants <1 year
- increases if duration of fever was <24 hours
- FHx
- Daycare
- Male
- Low Na+ at presentation
2-7% proceed to develop epilepsy
Large congenital birth mark on face; what is associated?
a) Brain atrophy
b) Glaucoma
c) leptomeningeal melanocytes
d) developmental delay
b) Glaucoma - Sturge-Weber associated with glaucoma and capillary malformation
MRI w/ contrast for Dx shows leptomeningeal angiomatosis, can see atrophy on ipsilateral side
Seizures, stroke like episodes, developmental delay later in life.
Erb’s palsy. What will they have?
a) biceps reflex
b) intact wrist and finger flexion
c) grasp
d) symmetric morrow
c) grasp : present in Erb’s but not Klumpke
Baby with congenital giant cell pigmented nevus. What else is this child at risk for other than malignant transformation?
a) leptomeningeal melanocytosis
b) brain calcifications
c) cataracts
d) brain atrophy
a) leptomeningeal melanocytosis
(neurocutaneous melanocytosis)
Giant Congenital Pigmented Nevus:
- predisposed to malignant melanoma
A child on carbamazepine. Mom calls and tells you he has a rash. What do you do?
stop carbamazepine and switch AED
see him in the office today
refer to Derm
Reassure
see him in the office today
risk of SJS/TEN
A 8 year old girl has had a history of episodic, frontal / bitemporal headaches. She has 4-5 episodes per month, lasting 3-4 hours, sometimes associated with pallor and nausea. Detailed examination, including neurological examination, is normal. What is your management?
Consult neurology
MRI head
Ibuprofen with start of episodes
Propranolol daily
Ibuprofen with start of episodes
A baby was found to have rhabdomyoma. On examination, they have multiple hypopigmented lesions on their skin. What is the syndrome?
Tuberous sclerosis
NF-1
Beckwith-Wiedeman
Tuberous sclerosis
Associated with cardiac rhabdomyoma + hypomelanotic macule
A 5 month old baby comes in with episodes of vomiting and flexion movements of his limbs associated with grimacing and pallor, after which he is more drowsy. On examination, his abdomen is slightly distended, but his neurological exam, other than being a bit drowsy, is normal. Which investigation is likely to give you the diagnosis?
a) EEG
b) MRI head
c) ultrasound abdomen
c) Ultrasound abdomen
Sounds like intussusception
A newborn is found to have decreased tone and tongue fasciculations with decreased reflexes. The child is otherwise alert and bright. What do you do to establish diagnosis?
TSH
Genetic testing
Metabolic screen
MRI head and spine
Genetic testing
SMA
A girl presents with a new onset left-sided esotropia for 24 hour. What is the most likely diagnosis?
a. Bacterial meningitis
b. Increased ICP
c. Myasthenia gravis
d. Vitamin B12 deficiency
Increased ICP
6th cranial nerve palsy
Girl teased at school because of acne, headaches, diplopia, normal CN, papilledema, MRI/CT normal. Which medication is a cause
a. Minocycline
b. OCP
c. topical retinoids
d. clindamycin
Minocycline
Pseudotumor cerebri
6 months. Cant sit. Has lateral propping to L but not right. Atonic neck reflex on L. R hand fisted. Only bringing L hand to midline.
a. Trauma to R arm
b. Hemiplegia R side- spastic
c. Spastic Diplegia
b. hemiplegia R side-spastic
R side weakness
R hand fisted (increased tone)
Fits UMN on Left side
Diplegia - lower extremities affected, upper extremities not
Hemiplegia - one half of the body (R or L)
Child Migraines. Interfering with function. Also has mild asthma, controlled. What to give for prophylaxis?
a) Ergotamines
b) Triptan
c) Amitriptyline
d) Propanolol
b) Amitriptyline
Not propranolol due to asthma
Ergot / Triptans are abortive
Seizing kid, gets 0.5mg/kg diazepam. What is likely SE?
a. Respiratory depression
b. Ataxia
c. Paradoxical reaction
a. Respiratory depression
Child wakes in night with seizures, inside of face feels numb, aware, can’t speak what is the diagnosis:
a. Benign rolandic epilepsy
b. Juvenile myoclonic epilepsy
c. Juvenile absence
Benign rolandic epilepsy
aka. benign childhood epilepsy with centrotemporal spikes
7-8 YO
Focal seizure of face, vocalization, drooling
¾ happen at night or upon wakening
- can have secondary GTC
Remit at 12-13 years
Carbamazepine and side effects in utero:
a. cleft palate
b. neural tube
c. heart defect
d. omphalocele
Neural tube
- Spina bifida
- Neurodev delay
Which is most concerning in a 5 month old
a. Fisting of right hand
b. Not always smiling at caregivers
c. Only sitting with pelvis supported
d. Not consistently turning to name
a. fisting of right hand
A parent calls your office. Her 12 year-old adolescent is lying in bed and has not woken up in the last 2 hours. She has had 2 similar episodes in the past 6 months. When her mother exerts a painful stimulus, the girl turns over in her bed. Her pupils are equally round and reactive to light. The most likely diagnosis is:
A confusional migraine
A brain tumour
Meningitis
A seizure
Confusional migraine
A woman presents to the emergency department with her child for suspected seizures. The child has tonic, involuntary contractions of his limbs. His mouth is “stuck” and he cannot close it. His mother notes that he has had two days of gastroenteritis symptoms and she has been giving him anti-nausea suppositories, but cannot recall their name. Administration of which of the following medications will you order first?
a. Diphenhydramine
b. Flumazenil
c. n-Acetylcysteine
d. Naloxone
a. diphenhydramine
Sounds like acute dystonia, treat with antihistamine.
You are called to the postpartum ward to assess a newborn who seems weak. On examination, the baby has marked hypotonia of the lower limbs with limited anti-gravity movements of the arms. You are unable to elicit deep tendon reflexes. What is the most likely diagnosis?
a. Spinal muscular atrophy
b. Myotonic dystrophy
c. Congenital muscular dystrophy
d. Duchenne’s muscular dystrophy
a. Spinal muscular atrophy
A child presents to the emergency department with difficulty walking. He describes progressive motor impairment affected the lower limbs, which seems to be involving more proximal muscle groups over time. Consent is obtained for a diagnostic lumbar puncture. On the chemistry and hematology profiles of the CSF of a non-traumatic tap, you expect to find:
a. 2.5g/L of protein and 5 WBCs
b. 0.7g/L of protein and 100 WBCs
c. 2.5g/L of protein and 100 WBCs
d. 0.7g/L of protein and 5 WBCs
a. 2.5g/L of protein and 5 WBCs
GBS - high protein, low WBC
MRI - thickening of caudal equine
A 2month old is brought to your attention for intermittent spastic movements of his upper limbs. These movements do not seem to be precipitated by specific events. An EEG is performed and reveals large amplitude waves in a chaotic background. What is the underlying diagnosis?
a. Infantile spasms
b. Benign sleep myoclonus of infancy
c. Physiologic variation in a vigorous baby
d. Focal seizures
a. Infantile spasms
Hypsarrhythmia - high voltage, slow chaotic backgrounders with multifocal spikes
10 year old male presents with ataxia and ADHD features. Maternal uncle died at age 10. What is the most likely diagnosis?
fredrichs ataxia
X- linked adrenleukodystrophy
Ataxia telangectasia
DMD
x- linked adrenoleukodystrophy
Friedrich Ataxia- autosomal recessive. Slowly progressive ataxia. Explosive dysarthria speech, nystagmus, normal intelligence. Pes vagus, hammertoes, kyphoscoliosis. Hypertrophic cardiomyopathy.
AT is autosomal recessive
3 month old term baby has one episode of limpness, colour change. The episode lasts for 30 seconds, no precipitating factors. No previous episodes. Exam is normal afterwards. What is the BEST management?
Reassure and no further investigations
Admit for polysomnography study
EEG
Head ultrasound
Reassure and no further investigations
Low risk BRUE:
- age > 60 days
- Born >32 weeks with corrected age >45 weeks
- No CPR by HCP
- Lasted < 1 minute
- first event
16 yo M presents with headache for the past 2 years, occuring once every 2 weeks. Have not changed in frequency or severity. Normal physical exam. Cough and bowel movements make headache worse. Best management step?
Amitriptyline
MRI Brain
Headache Diary
Flunarizine
MRI Brain
An infant has a sacral dimple. What would make you more concerned about spinal dysraphism?
Slate-gray nevus over dimple
Located 2 cm from anal verge
3 mm in diameter
Located above the gluteal cleft
Located about the gluteal cleft
Dimples that require further evaluation:
- 2.5cm above the anal verge
- Multiple dimples
- Dimple with other cutaneous marker
- Dimple diameter larger than 5mm
18 month old boy with first episode febrile seizure. What factor influences likelihood of repeat febrile seizure?
a. MRI result
b. EEG result
c. Family history of febrile seizures
d. Etiology of fever
c. Family history of febrile seizures
Major Criteria of TS
list as many as you can
- Hypomelanotic macules (≥ 3 at least 5mm)
- Angiofibromas
- Ungual fibromas
- Shagreen patch
- Multiple retinal hamartomas
- Cortical dysplasias (tubers and cerebral white matter radial migration lines)
- Subependymal nodules
- Subependymal giant cell astrocytoma
- Cardiac rhabdomyoma
- Lymphangioleiomyomatosis
- Angiomylipomas
Minor:
- Confetti skin lesions
- Dental enamel pits
- Intraoral fibromas
- Retinal achromatic patch
- Multiple renal cysts
- Nonrenal hamartomas
Differentiate between infantile spasms and benign myoclonus of infancy
Benign myoclonus of infancy:
- clusters, often around feeding
- typically during sleep
- normal EEG
- Normal Neuro exam
Infantile Spasms
- awake events, not suppressible
- hypsarrhythmia on EEG
- Can be a/w developmental regression (West Syndrome)
Erb’s palsy : what is prognosis and what do you do if not improved by 1 mo
Prognosis:
- spontaneous recovery in 1-3 mos but up to 30% will have functional impairment
If not better at 1 month:
- Refer to multi-d team at 1 month
(incl OT/PT, splinting)
- Surgical consideration at 2-4 mos
Rett syndrome on valproic acid, vomiting on and off x several weeks, with lethargy.
3 things to order
VPA level Ammonia Lipase Liver enzymes Head CT
Concern of VPA toxicity:
- VPA hyperammonemic encephalopathy
- Acute hepatocellular injury
- Pancreatitis
Clinical signs of increased ICP
Headache Vomiting Altered LOC Papilledema Pupillary changes Cushing Triad (HTN, bradycardia, respiratory depression) CN6 palsy (estropea)
In a patient admitted with head injury what are 4 things that would cause increased risk of adverse outcomes?
- GCS ≤ 5 at presentation
- Raised ICP at presentation
- Other injuries
- Pre-injury ADHD
- Low Socioeconomic status
Picture baby Scaphocephaly (elongated narrow head in AP direction), ears that are level.
Give the Dx
What on PE is specific to this Dx
Craniosynostosis (sagittal)
- Prominent occiput, broad forehead
- Normal neuro exam
- No increased ICP
- Palpable ridge
14yr girl in ER after waking with right sided H/A. Normal exam. Hx of right sided H/A. Mom with hx of migraines, who thinks daughter has migraines too.
How would you manage her?
If stem sounds like they’re giving you red flags for a mass then do an MRI.
If not:
- IV Fluids
- IV Ketorolac (Toradol)
- Metoclopramide
- Dexamethasone
- Sumatriptan
Dark + Quiet room
A boy has been diagnosed with Duchenne’s Muscular Dystrophy. Other than proximal weakness in the musculoskeletal system, name FOUR organ systems involved and for each system name ONE problem directed related to dystrophin deficiency
- Respiratory: nocturnal hypoventilation, ineffective cough
- Cardiac: dilated cardiomyopathy
- Brain: Intellectual disability
- HEENT: swallow dysfunction
- GI: constipation
- MSK: scoliosis
Name 4 features of an atypical febrile seizure
- Lasts > 15 minutes
- More than one in 24 hours
- Focality
- Postictal paralysis (Todd Paresis)
- < 6 months of > 6 years
- Abnormal development or neurological exam
Teenage girl with concussion from hockey. Having headaches and cognitive impairment.
a. What 2 things would you suggest for cognitive rest?
b. What steps should she take to return to school? (2 lines)
c. What criteria must be met before can she return to hockey? (2 lines)
CPS:
a. “Decrease and limit cognitive tasks and screen time at home. No School”
- Quiet environment
- As symptoms improve, slowly increase cognitive tasks at home in 15-20 minute increments.
b. Graded return - start with part time, light activities, in class modifications (breaks). Increase attendance as symptoms allow.
c. Back at school (ideally 7-10 day rest). Symptom free gradual return from light aerobic exercise to non-contact to drills to contact. Each stage 24 hours symptom free.
Child with suspected Guillain Barre Syndrome. What are three possible severe complications to be aware of?
- Respiratory muscle weakness
(may require assisted ventilation) - Oropharyngeal weakness (aspiration risk)
- Dysautonomia (arrhythmia, hypotension, hypertension)
Picture of child with angiofibroma on face, history of seizure, CT scan with calcifications.
- What is the Dx
- How is it inherited
- Tuberous sclerosis
2. Autosomal dominant (or sporadic mutation)
Child comes to you with headache list 5 features on history that would make you think this is an infratentorial brain tumor
- Ataxia
- Diploplia
- Nystagmus
- Torticollus
- Headache
- Nausea
- Vomiting
- Blurred vision
Child with headache, list 5 features that you make you think this was a basilar migraine
Now called Migraine with brainstem aura.
- Tinnitus
- Vertigo
- Diplopia
- Ataxia
- Dysarthria
- Confusion
- Obtundation
- Blurred vision
- paresthesias
- Hypacusis
- Dilated pupils, ptosis
Child who is seizing IN hospital with IV access in place list the steps in managing the seizure
- Evaluate ABCs
- Check chemstrip
- Place on monitors
- Supplemental O2
- Lorazepam 0.1 mg/kg IV (max 4mg) at 5 mins - can give x 2 q5min
- Phenytoin 20mg/kg over 20 mins (max 1000)
- (Still seizing after 5 mins) Phenobarbitol 20 mg/kg over 20 mins (max 1000)
- Call PICU
- (Status)
- RSI
- Midazolam infusion
- Thiopental/Pentobarbitol
9 year old. GTC. Returned to baseline
a. What was the recurrence risk of a first non-febrile seizure?
b. How would you counsel the family regarding management ( you were told you are not going to start antiepileptics)
c. Child with single afebrile GTC seizure. Unprovoked. What tests would you do and why (two lines). What would you do/tell this family prior to discharge
a. 40% (AAP - 60% who experience single unprovoked seizure will NOT have another)
b. safety – lateral decubitus positioning, assure no fall risk, don’t put anything in the mouth, time the seizure, call 911 if >5min
c. Careful Hx and PE with investigations guided by this. Consider Brain imaging by CT or MRI. ECG to rule out long Qt. EEG.
Where in the CNS is related to spasticity?
Corticospinal tracts
(white matter motor pathway starting at cerebral cortex, terminating an lower motor neurons and interneurons in spinal cord)
A 13 year old previously well boy presents to the ED after having a prolonged generalized tonic-clonic seizure.
- What is the one thing you should test now?
- List sequentially the medications you would use to treat seizures (five points)
- C/S (but also electrolytes, tox screen, evaluate for infection, CT head)
- Lorazepam or Midazolam x2
Fosphenytoin or Phenytoin
Phenobarbitol
Midazolam infusion after RSI
Teen with acute onset facial asymmetry x 24 hours.
A. What is the Dx
B. Two steps in treatment
C. Prognosis
A. Bell’s palsy
B. Oral prednisone (1 mg/kg/day for 1 week - then 1 week taper)
Oral acyclovir
C. 85% have no residual weakness
Common Causes: HSV, Lyme, Ramsay Hunt
3 year old developing ascending weakness in inferior limbs 1 week after a URTI. You suspect Guillain-Barre Syndrome.
A. List 2 investigations and list what results are expected.
B. List 2 treatments for this condition.
A.
LP - Protein elevated 2x normal, normal glucose, no pleocytosis
MRI - thickening of caudex equine and intrathecal nerve roots with gadolinium enhancement
B. IVIG 0.4 g/kg/day x 5 days or 1 g/kg/day x 2 days or Plasmapheresis
