Neurology Flashcards
What is the most common cause of childhood headaches.
a. Migraine
b. Myopia
c. Sleep disorder
Migraine
14 year old girl with asthma has throbbing headaches associated with nausea, photophobia. Her mother has a history of migraines. What treatment do you offer for prophylaxis? A. Amitriptyline B. Propranolol C. Phenytoin D. Sumatriptan E. Ergotamine
Amitriptylline
No propranolol!!
Flunarazine would also be an option
7 year old migraine, 3-4 x/month. Management: A. Ibuprofen as needed B. Propranalol prophylaxis C. Referral to neurologist D. Brain MRI
Ibuprofen PRN
Consider preventative Tx if >1 H/A per week or >1 disabling H/A per month
To stop H/A: NSAIDs and triptans. Limit use
No obvious red flags to warrant neuroimaging
If had >1 H/A/wk then prophylaxis: flunarazine,
amitriptylline, topiramate, propranolol
A 14 year old girl presents to the ER with abdominal pain rated 8/10. Soft abdomen on exam, no rebound, tender but not worse with palpation. She is also complaining of a headache. She states she’s had 4 similar episodes in the past which resolved over hours. Likely diagnosis?
a. abdominal migraine
b. appendicitis
c. PID
- ————–
58) Kid vomiting recurrently, missing lots of school, mom has headaches. Most likely cause?
a. abdominal migraines
b. benign paroxysmal vertigo
c. separation anxiety
Abdominal migraine
arome IV
- > =2 episodes in 6mo
- midline, periumbilical or diffuse abdo pain. Abdo pain is most severe + distressing Sx. Lasts >=1H. Interferes with normal activities
- at time of abdo pain, >=2 of:
1) N
2) V
3) anorexia
4) pallor
5) H/A
6) Photophobia - Personal/Fhx of migraines
Tx A. nonpharm - explain + reassure - avoid triggers - modified diet (high fibre, probiotics) - psychotherapy B. Pharm 1. Abortive (INH sumitriptan, IV VPA) 2. PPx: 1) propranolol, 2) cypraheptadine, flunarizine
Prognosis
- predicts cephalic migraines later in life
A girl presents with severe abdominal pain 8/10 in the periumbilical area. She is also complaining of a severe frontal headache. She has had five of the episodes previously and they have self-resolved in two to three days. Her mother suffers from migraines. Which of the following is the underlying diagnosis?
a. Familial Mediterranean fever
b. Abdominal migraine
Abdominal migraine
Rome Criteria
- > =2 in 6mo
- midline, periumbilical or diffuse abdo pain. Abdo pain is most severe + distressing Sx. Lasts >=1H. Interferes with normal activities
- at time of abdo pain, has >=2
1. N
2. V
3. anorexia
4. pallor
5. H/A
6. Photophobia - Personal/FHx of migraines
Mgmts A. Nonpharm - reassure - avoid triggers - improve diet (high fibre, probiotics) - psychotherapy
B. Pharm
- Abortive: INH sumatriptan, IV VPA
- Ppx: flunarazine, propranolol, cypraheptadine
Predicts future cephalic migraines
A picture of child showing the parachute reflex is shown. What is true?
a. This is a primitive reflex that disappears by 4 months
b. This is a voluntary reflex which disappears when child starts walking
c. This is an involuntary reflex that appears at 7-9 months and does not disappear
Involuntary reflex that appears at 7-9mo and does not disappear
Myelomeningocele – what associated abnormality is most likely to need surgical intervention? a. Chiari malformation b. Diastematomyelia c. Hydrocephalus -------- Most common need for surgery in a patient with myelomeningocele? a. Syrinx b. Tethered cord c. Hydrocephalus
Hydrocephalus (though most are in association with Chiari II malformation)
Child with brachial plexus injury. How long before if no change in exam is prognosis poor?
a. 1 mo
b. 3 mo
c. 6 mo
d. 12 mo
1 month (CPS)
Brachial plexus injury-extended hand, pronated, but can grip. Which nerve roots
a) C3-C4
b) C5-C6
c) C7-C8
d) C8-T1
C5-C6, Erb’s
- likely asymmetric Moro +ATNR
- No brachial reflexes
A 1 month old has a droopy lower left lip. The forehead moves normally. What is the problem:
a. centrally mediated facial nerve palsy
b. peripherally mediated facial nerve palsy
c. congenital absence of the mouth angle depressor muscle
d. Mobius syndrome
—————
Baby with facial droop. Normal frontal muscle, can close eyes and normal nasolabial fold:
a. absence of depressor angularis oris
—————
Drooping mouth but eyes were able to close, normal nasolabial folds. Diagnosis?
a. Mobius syndrome
b. congenital absence of the depressor anguli oris muscle
c. CN VII lesion
d. upper lobe lesion
Congenital absence of the mouth depressor anguli oris muscle
- Kid presents with 3rd febrile seizure in the past month. Otherwise well, no post-ictal. What do you do?
a) Reassure
b) EEG
c) Admit
d) MRI brain
- ———— - An 18 month-old female presents to the emergency department with a febrile seizure secondary to an acute otitis media. This is her third febrile seizure in three months. What is the next step in management?
a. EEG
b. CT head
c. Neurology consult
d. Reassure parents, and no further investigations
Reassure
- 30% recur if 1st episode
- 50% recur if >2 episodes
- 50% recur if <1yo
- no routine investigations
- LP if <12mo to r/o meningitis. consider in 12-18mo. Only if SSx if 18mo.
- EEG not routinely indicated
RFs of epilepsy
- simple febrile sz (1%)
- complex febrile sz (6%)
- recurrent febrile sz (4%)
- focal complex feb sz
- neurodvptal abN
- FHx of epilepsy
- fever <1H before feb sz
18-month-old boy presents with fever, AOM, and febrile seizure. This is his third recurrent febrile seizure in the past few months. What is your next step?
a) EEG
b) CT head
c) Neuro consult
d) Reassure parents
Reassure parents
A child with epilepsy presents unwell with findings of hepatotoxicity and pancreatitis and thrombocytopenia. Which medication is this likely related to? A. Valproic acid B. Carbamazepine C. Topiramate D. Keppra
VPA
Serious: hepatic + pancreatic toxicity
Nuisance: wt gain, alopecia, menstrual irregularities, hyperammonemia tremor
9y boy w episodic r side face twitching and drooling at night with preserved consciousness. What is the most likely diagnosis?
a. Rolandic epilepsy
b. Juvenile myoclonic epilepsy
c. Tourette syndrome
Rolandic epilepsy
- rolling in bed awake
- AD, 3-13yo (usually stop after puberty)
- Typical sz: wake from sleep, mouth twisted, IL twitching of mouth/face/pharynx, speech arrest or dysarthria, drooling preserved consciousness
- lasts 1-2min
Normal neuro exam
EEG: centrotemporal spikes
Mgmt: keppra, oxcarbazepine. Most AED effective
9 year old has woke 3 times now….each time the same. Right sided facial twitching, ? limb involvement, but after few minutes well. What is the diagnosis?
a. rolandic epilepsy
b. absence seizures
c. sleep-walking (ambusomnulence or something)
d. juvenile myoclonus
Rolandic epilepsy
10 year old boy previously healthy has been waking up the parents at night with symptoms of mouth twitching, and drooling. He has a normal neurological exam and a normal MRI. Which of the following is most likely on his EEG?
a. Centrotemporal Spikes
b. 3 Hz spike and wave
c. high amplitude waves and a background of irregular spikes
Centrotemporal spikes
A 7 year old girl has had episodes over the past couple months where she awakes from sleep, has twitching of her right lip and is unable to verbalize. The episodes last 1-2 minutes. You suspect seizure activity and order and EEG. What is it likely to show?
a. spikes in the centrotemporal (rolandic) region
b. Generalized slow waves
c. 3 Hz generalized spikes over a normal background
d. normal
- ————–
55) 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. hypsarrhymia
Centrotemporal spikes
Benign rolandic epilepsy
- rolling in bed awake
- AD
- 3-13yo (disappear by puberty)
- Typical sz: awake from sleep, pulling of face on one side, speech arrest or dysarthria, drooling, consciousness preserved
Normal neuro
EEG centrotemporal spike
No routine MRI
Tx: keppra, oxcarbamazepine. Most AED work
Kid with facial twitch in the middle of the night. Seizure pattern?
a. Centro-temporal
b. 3 hz spike and wave
- ———–
45) 5 yo child wakes up to tell mom that he has been drooling, and facial movements in the middle of the night. Aware of events. What likely finding on EEG?
a. Centrotemporal spikes
Centro-temporal spikes
11 month old baby boy is admitted because yesterday morning before breakfast he had two episodes where he “flopped back” and seemed hypotonic. This resolved. This morning after breakfast, he had another episode where he flopped back, became hypotonic, and had irregular breathing. His blood glucose is normal, as was a CBC and electrolytes. Which of the following would be next step?
a) EEG
b) Lumbar puncture
c) Pyruvate, lactate, fasting blood glucose
EEG
- repeated loss of tone, which suggests atonic Sz
- no fever or infectious Sx to warrant LP
- no red flags for metabolic disease based on initial normal BW
5 month old boy with episodes of head flexion and limb extension lasting a few seconds. His EEG shows a disorganized background with multifocal spikes. What is the most likely diagnosis?
a) Benign familial neonatal convulsions
b) Infantile spasms
c) GERD
d) Benign myoclonus
Infantile spasms
- first year of life
- Cause: Cryptogenic vs symptomatic (tuberous sclerosis!)
- clusters of brief body spasms, Q2-10s x 2-3min
- may have DD +/ regression
EEG: hypsarrhythmia
MRI to look for brain injury + abN
Mgmt
- Vigabatrin for Tub Scler (SE: tunnel vision!)
- ACTH IM for cryptogenic. (SE: high BP, glycosuria, irritability, facial edema, immunosuppression)
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
- —————
38) 5 month old baby who has intermittent flexion of arms/legs for several seconds and in between that, has normal behavior. EEG – abnormal background with polyspikes. What is diagnosis?
a. benign neonatal myoclonus
b. infantile spasms
c. benign sleep myoclonus
- —————–
39) Kid with myoclonic spasms. Well in between spasms. No regression. They also describe seizure pattern in questions. I think they did describe hypsarrythmia. Ask for dx?
a. Infantile spasms
b. Benign myoclonic epilepsy
Infantile spasms
Infant w hypopigmented macules and repeated flexion movements of the entire body. EEG shows a disorganized pattern. What is the diagnosis?
a. Infantile spasms
b. Benign myoclonus of infancy
c. Benign sleep myoclonus
Infantile spasms
50) Infant with hypopigmented lesions over body and brief flexion/extension of upper and lower body periodically throughout the day. Baby is mildly developmentally delayed. How would you confirm the underlying diagnosis:
a. EEG
b. Urine OA
c. Head MRI
EEG to look for hypsarrhythmia in context of infantile spasm
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
Breath holding spells
1) cyanotic (most common): d/t expiratory apnea, can have brief LOC + very brief tonic-clonic sz
2) pallid: d/t reflex vagal bradycardia + asystole
- can be mixed
- assoc’d with IRON DEFICIENCY ANEMIA
- Tx: reassurance + education. Will outgrow
Teenage girl with asthma has frontal headaches associated with nausea, photophobia. Her mom has a history of migraines. What treatment do you offer for prophylaxis?
a. sumatriptan
b. propranolol
c. amitriptyline
d. phenytoin
Amitriptyline
- Prophylactic treatment for migraines in child with asthma
a. Propranolol
b. Amitriptyline
c. Sumatriptan
- ———- - Teenager with a history of asthma presents with migraine headaches interfering with daily life. Which medication for prophylaxis?
a. Propanolol
b. Amitryptiline
Amitryptyline
A 14 year old girl presents to the ER with abdominal pain rated 8/10. Soft abdomen on exam, no rebound, tender but not worse with palpation. She is also complaining of a headache. She states she’s had 4 similar episodes in the past which resolved over hours. Likely diagnosis?
a. abdominal migraine
b. appendicitis
c. PID
- ————–
58) Kid vomiting recurrently, missing lots of school, mom has headaches. Most likely cause?
a. abdominal migraines
b. benign paroxysmal vertigo
c. separation anxiety
Abdominal migraine
A girl presents with severe abdominal pain 8/10 in the periumbilical area. She is also complaining of a severe frontal headache. She has had five of the episodes previously and they have self-resolved in two to three days. Her mother suffers from migraines. Which of the following is the underlying diagnosis?
a. Familial Mediterranean fever
b. Abdominal migraine
Abdominal migraine
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
Refer to PT
15 yo obese girl lives in group home, and was sexually assaulted by her father 3 years ago. Acute onset headache, and syncope but neurological exam detailing fundoscopy, DTR, tone, cerebellar reflexes are normal. Complains of difficulty walking, and difficulty urinating. Manages to urinate in bedpan. Yesterday had sensation to thigh, and today sensation at ankles. Absent plantar reflex. Walks requiring assistance, leans heavily on you. Feet are 8cm apart. Management:
a) confront her with the fact that physical findings do not correlate with symptoms
b) MRI - ?absent plantar reflex
Confront her?
Leans heavily = good strength
Feet 8cm apart = not ataxic gait
?absent plantar reflex = babinski not present?
Conversion disorder
- > =1 Sx/deficit affect voluntary motor or sensory fxn
- Sx not compatible with recognized neuro or medical conditions
- Not better explained by another medical or mental d/o
- Causes clinically significant distress or impairment in social, occupation, or other important area of functioning or warrants medical evaluation
43) . CT head with a bleed (can’t remember what the image showed). Choices were
a. Epidural
b. subdural
c. intraventricular
d. subgaleal
Epidural = rounded, meningeal artery, lucid period then decompensate rapidly Subdural = linear, ruptured bridging veins. Immediately unconscious. Inflicted trauma!
Player experiences brief, transient loss of consciousness (<5min) during a sporting event accident; what do you advise the coach to do?
a. Player must sit out for at least one week
b. May return to game after 15 minutes if asymptomatic
c. Go home with parents with head injury instructions
d. Out for the season
—————
A child playing a sports game has a head injury with transient loss of consciousness. What to do:
a. Have him do mental tasks. If he succeeds, have him return to game
b. Sit out for 1 week
c. Sit out for 15 minutes
Go home with parents with head injury instructions
Concussions
- in adol, Sx can resolves in 7-10d
- in children, can take weeks to months
10) An 11 year old boy presents with unilateral facial droop. The entire half of his face is paralyzed. Taste seems to be in tact. There are vesicles inside his ear. Most appropriate treatment?
1) Acyclovir alone
2) Steroids alone
3) Acyclovir and steroids
4) Wait for it to self-resolve
Acyclovir + steroids
Ramsay Hunt
- Herpes zoster
6 mo baby with hand preference, moving less on right side noted over last 2 months
a. hemiparesis
b. brachial plexus paralysis
Hemiparesis
CP
- hand dominance before 18mo is abnormal
Young child with hypotonia, only some facial movements, no deep tendon reflexes. +Fasciculations on tongue. Parents are asking about chance of recurrence if they have more children. What do you tell them?
a) 25%
b) 50%
c) 50% if the child is male
d) Sporadic
25%
No DTR + fasciculations
= SMA
AR
- Child with description of spinal muscular atrophy. Parents want to know risk of recurrence?
a) 25%
b) 50%
c) 50% if male
same risk of general population
——————- - Myotonic infant with fasiculations, low reflexes. Parents wonder chance of recurrence?
a) 25% recur
b) 50%
c) 50% males
d) Sporadic
25% recur
SMA: AR, fasciculations, absent reflexes
Myotonic dystrophy: AD, no fasciculations, preserved reflexes, myotonia
6 month old baby with decreased reflexes, fasciculation’s, weak muscles. Best diagnosis?
a. MRI
b. Thyroid test
c. Genetic testing
d. Metabolic testing
Genetic testing
SMA
6 wk old male presents with poor feeding and hypotonia. on physical exam you note that he has difficulties sucking and swallowing and has fasciculations and decreased deep tendon reflexes. what would be the most diagnostic test?
a) MRI
b) thyroid function
c) metabolic screen
d) genetic testing
genetic testing for SMA
Previously healthy 2 yo presents with 2 weeks proximal muscle weakness. Most likely disorder:
a) congenital myopathy
b) neuromuscular junction
c) peripheral neuropathy
d) Spinal cord injury
Congenital myopathy
Proximal => muscle problem
Congenital myopathy: problem with muscle contraction, normal CK
Weakness => not central cause, r/o spinal cord injury
Peripheral neuropathy would have sensory + motor issue. Distal to proximal weakness. Decreased reflexes
NMJ would expect fatiguable weakness + oculobulbar Sx
- A 2 month old baby is floppy. He smiles and looks around, but he does not make many voluntary movements. On exam, he has no deep tendon reflexes. What is the most likely diagnosis?
a) Congenital muscular dystrophy
b) Congenital myotonic dystrophy
c) SMA
SMA
IQ intact, no DTR
3mo girl with hypotonia, hypotonic facies, 1+ symmetric DTR. What is the most likely diagnosis?
a. Congenital muscular dystrophy
b. Myotonic dystrophy
c. SMA1
d. Nemaline rod
Myotonic dystrophy
b/c hypotonic facies, reflexes still present
SMA: no DTR, IQ intact, fasciculations
Congenital muscular dystrophy: problem with muscle contraction, normal CK, prox weakness. Won’t have same degree of facial involvement, will have brain involvement.
Nemaline rod: generalized hypotonia = weakness, muscles fo jaw too weak to hold closed
Child with proximal muscle weakness and decreased DTR
a. Congenital myopathy
b. Peripheral neuropathy
c. Spinal cord
d. Neuromuscular junction problem
Congenital myopathy
Baby with symptoms of myotonic dystrophy described. Give diagnosis.
AD
Problem with relaxation
(nursemaid grip)
Normal CK
DISTAL weakness -> proximal
Hypotonic facies (upturned V-shape of upper lip, scalloped concave temporalis, narrow head, high arched palate)
Myotonia; grip, percussion on thumb, tongue
Heart block/arrhythmias. No cardiomyopathies
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 complain of? a. Headache early in morning b. Headaches in the afternoon c. Tingling of his fingers d. Dyspnea with exertion ----------------- 17yo male with Duchenne’s muscular dystrophy. Wheelchair bound. FEV1 decreased from 30% --> 21% in your assessment. What is the most likely problem he will have? a. early AM headache b. late PM headache c. dyspnea on exertion
Early AM H/A
Restrictive lung disease
A teenager with Duchenne muscular dystrophy is in a wheelchair. His FEV1 has progressively declined from 30% to 21% in the last year. What will he likely complain of?
a. dyspnea on exertion
b. morning headaches
c. early afternoon fatigue
Morning H/A
Child with proximal weakness, calf hypertrophy. Suspect Duchenne’s. What is the best test?
a. molecular studies
b. serum dystrophin
c. biopsy
d. EMG
Molecular studies
If not Dx, then get Bx and look for limb girlde dystrophy
Do Bx initially if autoimmune disease (dermatomyositis)
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
Molecular testing
PCR for dystrophin gene mutation
Child with suspicion of Duchenne’s muscular dystrophy. Best test to confirm diagnosis: a. Molecular studies b. Muscle biopsy c. Muscle Enzyme studies d. Serum dystrophin assay --------------- 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
Molecular studies
- PCR for dystrophin gene mutation
DMD is XR mutation of dystrophin (expressed in brain, retinal, striated muscle, cardiac muscle)
- 10 year old girl diagnosed with optic neuritis 3 months ago. She is currently asymptomatic. How do you counsel her mother:
a) Chance of recurrence is low
b) The gamma globulin she received protects against recurrence
c) Risk of macular degeneration
d) She is at significant risk of developing MS
She is at significant risk fo developing MS
10 year female with optic neuritis 3 months ago. No symptoms, mom worried about recurrence long-term. Your advice?
a. increased risk MS
b. no risk recurrence as got IVIG
c. increased risk macular degeneration
—————–
An 8 year old girl has an episode of optic neuritis. What would you tell her mother about her prognosis?
a. She was given medication which will prevent recurrences
b. She is at risk for future eye problems
c. She is a significantly increased risk for multiple sclerosis
—————–
Child with optic neuritis (bilat) treated with IVIG
a. At risk for MS
b. No risk for recurrent given treatment with IVIG
c. At risk for visual impairment
Increased risk of MS
- cumulative risk of 50% at 15yo
- Have risk for visual impairment, but no macular involvement
Optic neuritis
- inflammation or demyelination of optic nerve with imparied fucntion
- acute rapidly progressive loss of vision
- pain on EOM or palpation of globe
- Decrease visual acuity, decrease colour vision, RAPD
Tx
1. High dose methylpred
2. Eculizumab
- improve vision within 1-3wk
A child has symptoms suggestive of Guillain Barre syndrome and has a lumbar puncture. What are you most likely to find on the CSF?
a) High WBCs, low protein
b) High WBC, high protein
c) Low WBC, low protein
d) Low WBC, high protein
low WBC (<10), high protein (>2X ULN), normal glucose
Child previously well has ascending hyporeflexia, paralysis. Cause?
a) Campylobacter
Campylobacter related to GBS #2: H pylori #3: Mycoplasma Less common: EBV, CMV, Lyme, H influ Post vaccines: rabies, influenza, polio (oral form), conjugated meningococcal
GBS = acute demylinating disease of peripheral nervous system
- Girl walked and tripped on a curb now has cold extremity painful to touch
a) CRPS
b) osteomyelitis
CRPS
Child with episodes of clutching father and screaming then well. Frightened. Alert during episode and back to normal after. Normal exam. Diagnosis?
a. Cerebellar ataxia
b. Benign paroxysmal vertigo
c. Seizure
d. Meniere’s disease
Benign paroxysmal vertigo
- recurrent episodes of brief dysequilibrium
- occurs w/o warning, maximal at onset, resolve spontaneously <1min
- during attack: frightened, off balance, reach out to steady self
- NO LOC
- can have nystagmus, diaphoresis, N/V, pallor
- usually resolve by 5yo. Many develop migraines
Unlikely sz b/c alert during and no post-ictal
Cerebellar ataxia: expect more persistent poor gait, and abnormal exam
Menierre’s: 1) episodic vertigo, 2) tinnitus, 3) SNHL
9mo girl who preferentially uses left hand. Right hand is fisted. When try to sit her up, she keels over to the right. What is the explanation for the findings?
a. Erb’s palsy
b. Trauma to right arm
c. Hemiplegia
d. Spastic diplegia
Hemiplegia
9 year old child with spastic quadriplegia cerebral palsy presents with a three day history of not swallowing her secretions. On baclofen, fluticasone for asthma. Looks well. Has G-tube and has been fed with thickened feeds orally. Last week received botox for lower leg contractures. Continues to tolerate po feeds and abdo exam is normal. On assessment today he is afebrile, with drooling and clear chest, no stridor. Remaining vital signs are normal. Improved movement of lower legs. Your next step is:
a. pH probe
b. Admit for observation
c. Outpatient follow up regarding dysphagia
d. Cine video swallowing fluoroscopic study
- ————–
49) 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:
a. pH probe
b. do a swallow study
c. observe in hospital
Admit for observation b/c concern for adverse reaction to botox
If botox injected IV, new drooling would be concerning + botox could progress and would be risk of aspiration pneumonia
Botox could last for wks to mo (only repeat injection Q3mo)
No antidote to botox
9 month old infant with asymmetric tonic neck reflex. What does this go with?
a. Werdnig Hoffman
b. cerebral palsy
c. normal infant
CP
- ATNR starts at 35wk GA, well-established by 1mo postnatal. Disappears by 4mo
Note: Werdnigg Hoffman disease = SMA Type 1 (infantile form)
Persisting asymmetric tonic neck reflex at 9 months is most likely associated with: a. Cerebral palsy b. Normal infant c. Werndig-Hoffman (SMA) d. hypothyriodism ---------- An 11-month old child presents with persistent ATNR reflex. What are you worried about? a. cerebral palsy b. normal child
CP
- ATNR disappears by 3-4mo
Ex-24 week prem now 18 months old. Increased tone, increased DTR, spastic. What age can you make the dx? a. 18 months b. 24 months c. 36 months d. 48 months ------------------ An ex-25 week premature infant is now 18 months old. She has bilateral increased reflexes in the lower limbs. What can you make the definitive diagnosis? a. 18m b. 24m c. 36m d. 40m ----------------- 18 m.o. with spasticity. When can diagnosis of CP be made a. 18 months b. 24 months c. 36 months d. 48 months
18mo
CP
- group of permanent disorders of movement + posture causing activity limitation secondary to non-progressive disturbance in fetal or infant brain
A child with cerebral palsy comes to your office with the complaint of poor school performance. Your exam is normal. You do a CT head and there are no problems with his shunt. What do you do next? a. EEG b. thyroid? c. test for meningitis? --------------- Kid with cerebral palsy + shunt who is doing worse in school (some other details given.) Normal exam, CT just like last one. Management? a. refer to psych --------------- 10 years of age. Hydrocephalus in past with spina bifida. Shunted. Sudden recent decrease in school performance. CT head normal. What do next? a. Neurosurg referral b. EEG c. Psych assessment
EEG r/o seizures
Sick Kids consensus
Dr. McMillan
- If CP, would suspect sz (e.g. not obvious complex partial sz) or psych. Nonsurgical.
- If have hydrocephalus with shunt, ventricles can become rigid. Therefore, CT is not most helpful to r/o pressure problem. So if it’s just hydrocephalus with shunt, then would consider neurosurgery referral (for EVD to monitor). Would especially do if high ICP or obtunded
A baby is diagnosed with spastic diplegia. What do you tell the parents is the most likely cause?
a. periventricular leukomalacia
b. intraventricular hemorrhage
c. prenatal brain insult
d. perinatal asphyxia
PVL
- 14y girl presents with blurry vision, morning headaches and vomiting for 2 weeks. She has bilateral papilledema. No mention of meds. What’s the most likely dx?
a. Posterior fossa tumor
b. Pseudotumour cerebri
Pseudotumour cerebri
Child with 12 hour hx of esotropia. What do you think of first? a. MS b. Lyme disease c. atypical GBS d. increased ICP ------------------ A 8 year girl presents with a right eye esotropia for the past three days. Which of the following is the likely cause? a. Increased ICP b. Atypical GBS c. Lyme disease ---------------- Girl with new onset esotropia a. increased ICP b. GBC c. Lyme disease
Increased ICP
14 year old girl presents with persistent esotropia for 24 hours (that’s all they tell you!). What is the FIRST thing you would think of?
a. Guillan Barre
b. Lyme disease
c. Increased intracranial pressure
d. Multiple sclerosis
Increased ICP
12-year old girl presents with esotropia. What diagnosis should you rule out first?
a. multiple sclerosis
b. brain tumour
c. atypical Guillain-Barre syndrome
d. meningitis
Brain tumour
- 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
————–
Boy with known recurrent strep pharyngitis. Has had 6 weeks of spasmodic twitching of neck and head. Dx?
a. Tourette syndrome ?PANDAS
b. Transient tic syndrome
c. Rheumatic fever
———–
10 yr old boy with 6 wk history of repetitive movements of head and shoulders. Has history of recurrent strep pharyngitis. What is the most likely diagnosis?
a. Tourettes
b. Transient tic disorder
c. partial seizures
d. acute rheumatic fever
Transient tic disorder
-?PANDAS
Tourette’s requires >1y duration
Sydenham chorea:
1) chorea - involuntary, random, quick, jerking movements, flow from one limb to another
- NONREPETITIVE and arrhythmic
2) hypotonia
3) emotional lability
A kid is having involuntary facial tics. What would strengthen a diagnosis of Tourette’s?
a. Positive family history of OCD
b. Hyperactivity (ADHD)
c. Tics lasting for 6 months
d. Male
—————
A 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
ADHD
10 yo female with long history of handwashing 10-12 times per day. Now handwashing 100 times per day. She also has new onset eye blinking and throat clearing. She had a sore throat 2 weeks ago. What should she be treated with?
a. Risperadol
b. Clonidine
c. Penicillin
d. Dexedrine
Penicillin
A 7 yo boy has become ataxic over the last few months, gradually worsening. What is the probable diagnosis?
a) Adrenoleukodystrophy
b) Encephalitis
Adrenoleukodystrophy
- X linked VLCFA accumulation
Emotional lability and choreiform movements
a. Tourettes
b. SLE
c. Huntingtons
d. Sydenhams
- ————–
41) Writing difficulties and moody after a URTI
a. Tourettes
b. Sydenham’s chorea ???
c. Lupus
d. Huntingtons
Sydenham’s chorea
Clinical hallmarks
1) chorea - rapid chaotic mvmts that flow from one part of body to another (darting tongue, milkmaid grip)
2) hypotonia - pronator sign (palms turn outward when arms held overhead), spooning of extended hand by flexion of wrist + extension of fingers
3) emotional lability
56) An 8 year old girl with subtle choreoathetotic movements. Handwriting has worsened and she is emotionally labile. Evaluations including a throat culture are negative. What is the most likely diagnosis?
a. Huntington’s chorea
b. Syndenham’s chorea
c. SLE
—————
Child presenting with writing difficulties and seems moody. What does she have?
a. Lupus
b. Sydenham’s chorea
c. Tourettes
d. Huntington’s disease
Sydenham’s chorea
1) chorea
2) hypotonia
3) emotional lability
Kid with hx of chorea and emotional lability. Throat culture –ve. Most likely dx?
a. RF
b. TIC d/o
Rheumatic fever
Child with sudden movements of head, neck and shoulders. Has a history of recurrent GAS pharyngitis. Dx?
a. Rheumatic fever (could represent Syndenham chorea)
b. Tourette’s
c. Transient tic disorder
- —————-
61) A 15 year old male with strep throat presents two weeks later with unusual movements of his head and shoulders. What of the following is the likely cause?
a. Rheumatic fever
b. Transient tics
c. Tourette syndrome
d. Complex partial seizures
Rheumatic fever
2 year old with chorea and personality changes, likely cause?
a. Huntington’s
b. SLE
c. Sydenham’s
d. Tourette’s
- ——————-
71) 8y female with subtle choreoathetotic movements. Handwriting has worsened and she is emotionally labile. Evaluations including a throat culture are negative. What is the most likely diagnosis?
a. Huntigton’s chorea
b. Syndenham’s chorea
c. SLE
Sydenham chorea
1) chorea
2) hypotonia
3) emotional lability
A 2 month old baby has an abnormally shaped head. What would make you pursue further investigations?
a) Posterior displacement of the ipsilateral ear
b) Frontal bossing of the ipsilateral forehead
Posterior displacement of ipsilateral ear
Craniosynostosis
A 1 month old has a droopy lower left lip. The forehead moves normally. What is the problem:
a. centrally mediated facial nerve palsy
b. peripherally mediated facial nerve palsy
c. congenital absence of the mouth angle depressor muscle
d. Mobius syndrome
—————
Baby with facial droop. Normal frontal muscle, can close eyes and normal nasolabial fold:
a. absence of depressor angularis oris
—————
Drooping mouth but eyes were able to close, normal nasolabial folds. Diagnosis?
a. Mobius syndrome
b. congenital absence of the depressor anguli oris muscle
c. CN VII lesion
d. upper lobe lesion
Congenital absence of depressor angularis oris muscle
Forehead spared, so not peripheral nerve lesion
Brachial plexus injury-extended hand, pronated, but can grip. Which nerve roots
a) C3-C4
b) C5-C6
c) C7-C8
d) C8-T1
C5-C6
Erb’s
Child with brachial plexus injury. How long before if no change in exam is prognosis poor?
a. 1 mo
b. 3 mo
c. 6 mo
d. 12 mo
1 mo as per CPS statement
- 75% of infants recover completely in first mo
- 25% with permanent impairment or disability
- If no complete recovery on PEx by 1 mo, unlikely for full recovery -> refer to multi D team
Most common need for surgery in a patient with myelomeningocele? a. Syrinx b. Tethered cord c. Hydrocephalus ----------------- Myelomeningocele – what associated abnormality is most likely to need surgical intervention? a. Chiari malformation b. Diastematomyelia c. Hydrocephalus
Hydrocephalus
Hydrocephalus in assoc’n with type II Chiari malformation develops in at least 80% of pts with myelomeningocele
A picture of child showing the parachute reflex is shown. What is true?
a. This is a primitive reflex that disappears by 4 months
b. This is a voluntary reflex which disappears when child starts walking
c. This is an involuntary reflex that appears at 7-9 months and does not disappear
This is an involuntary reflex that appears at 7-9 months and does not disappear
