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Neuro HR Flashcards

1
Q

12 month old boy presents with recurrent episodes of going pale, limp and having non-rhythmic or jerky limb movements. Episodes have occurred after a minor injury. He has had a normal EEG. What is the diagnosis?

  1. Infantile spasms
  2. Breath holding spells
  3. Benign myoclonus of infancy
  4. Self-gratification syndrome
A

B

Minor injury = trigger

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2
Q

Peak onset for breath holding spells? Occur in ___% of healthy children? Duration? Types?

A
  • Peak onset: 6-18 months old
  • Occur in 0.1-4.6% of healthy children
  • Duration: 10-60sec
  • Two types:
  • -Cyanotic - “the angry infant” - apnea, cyanosis after agitation, crying
  • -Pallid - “the injured infant” - limp & pallor after an injury
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3
Q

Etiology of breath holding spells?

A 
Multi-factorial
• Immature autonomic nervous system
 • Hyperventilation (i pCO2) – cyanotic
• Vagal nerve-mediated bradycardia – pallid
 • Iron deficiency anemia
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4
Q

Prognosis for breath holding spells? Management?

A
  • Usually disappear by 5 years old

- Reassure and consider iron supplementation

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5
Q

Infantile spasms - peak onset?

A

4-7mos old (~95% <12 mos)

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6
Q

Infantile spasms occur in ____ proportion of children.

A

Rare (1/10 000)

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7
Q

Infantile spasms - typical duration? Appearance?

A 
1 second (*clusters* key feature)
Neck flex, arm extends
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8
Q

Infantile spasms - etiology?

A
  • 2/3 – underlying cause: prior HIE, prior stroke, brain malformation, chromosome (tri 21) genetic (tuberous sclerosis complex, TSC)
  • 1/3 – no underlying cause (cryptogenic)
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9
Q

Infantile spasms - prognosis?

A

• Risk of long-term cognitive delay, other seizures

-Although infantile spasms will go away, other types of seizures will emerge

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10
Q

Infantile spasms EEG pattern and diagnosis?

A

Hypsarrhythmia

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11
Q

Treatment of infantile spasms and associated adverse effects?

A
  • Vigabatrin (retinal toxicity) *particularly effective in Tuberous Sclerosis
  • Prednisolone/ACTH (ACTH heightens excretion of endogenous steroids) (weight gain, hypertension, irritability, adrenal suppression)
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12
Q

What is a common infantile spasm mimic?

A

Sandifer Syndrome:
• Abnormal movements (axial stiffening) due to GERD
• Usually occur with or after feeds in a “spitty” baby

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13
Q

Peak of benign myoclonus of infancy?

A

<2 years old

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14
Q

Frequency of benign myoclonus of infancy?

A

Uncommon (perhaps under diagnosed)

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15
Q

Duration of benign myoclonus of infancy episodes?

A

Brief “shudder spells”

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16
Q

Description of benign myoclonus of infancy spells?

A

• Sudden brief symmetrical axial flexor spasms of trunk & head lasting 1-2 sec OR “vibratory” flexion of neck
**NO arm movement = impt distinguishing feature
and NOT in CLUSTERS **
-May be provoked by excitement / fear
• Normal exam.

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17
Q

EEG in benign myoclonus of infancy?

A

Normal

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18
Q

Prognosis for benign myoclonus of infancy?

A

Spontaneous remission by 5 years of life

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19
Q

Peak of benign sleep myoclonus of infancy?

A

Birth (term)-3 mos (<10 mos old)

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20
Q

Benign sleep myoclonus of infancy occurs in ____ proportion of infants?

A

~1/1000

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21
Q

Duration of episodes in benign sleep myoclonus of infancy?

A

Discreet limb jerks
-ONLY occur during sleep - if wake them, will not see the episodes. If feeding, will not see, If falling asleep or are asleep, that’s when they occur. Can not be this if happen when awake - would raise suspicion for actual seizure disorder

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22
Q

EEG in benign sleep myoclonus of infancy?

A

Normal

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23
Q

Prognosis in benign sleep myoclonus of infancy?

A

-Benign and resolves without sequelae

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24
Q

Onset of infantile self-gratification syndrome or “infantile masturbation”?

A
  • Onset < 12 months old 9 remits by ~3 y)

- More common in girls

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25
Q

Infantile self-gratification syndrome is characterized by_______.

A
  • Pelvic rocking. Pelvic pressure
  • Adduction of legs
  • May have associated flushing, diaphoresis
  • May be difficult to distract out of episode

(not unresponsive but can seem like in own little world, but don’t have complete behavioural arrest,
no post-ictal period )

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26
Q

A 6 year old girl presents with recurrent episodes of staring. She has recently started to have learning problems at school.
Her EEG shows generalized 3 Hz spike and wave discharges. What do you tell the family?
1. She has a high likelihood of developing GTC seizures
2. Treatment is not needed because of benign nature of condition
3. Treatment with carbazepine is recommended
4. She will likely outgrow these by adolescence

A 
4
Absence seizures (primary generalized epilepsy)

3 not best option - can exacerbate!

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27
Q

Peak onset of childhood absence epilepsy?

A

5-7 years (range 4-10 years)

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28
Q

Absence epilepsy occurs in what proportion of children?

A

1-5/100 000

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29
Q

Duration of episodes in childhood absence epilepsy?

A

Blank stare x 5-30 seconds

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30
Q

Characteristics of childhood absence seizures?

A

• May have hundreds per day
• May initially be misdiagnosed as “inattention” or
“learning disability”
• Children typically cognitively normal

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31
Q

Treatment for childhood absence epilsepsy?

A
  • Ethosuxamide - used most often, and ONLY effective for these seizures
  • Valproic acid
  • (or lamotrigine)
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32
Q

Prognosis for childhood absence epilepsy?

A

~75% complete remission by adolescence

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33
Q

A 6 year old boy presents with 3 episodes
of facial twitching and drooling at night. Each episode lasted 1-2 minutes. His EEG shows frequent epileptiform discharges in the central-temporal region What is the correct management?
1. Reassure family
2. Start ethosuxamide
3. Urgent MRI brain
4. Urgent Neurology referral

A

1

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34
Q

Peak onset of Benign Rolandic Epilepsy?

A

7-10 years (range 1-14 years)

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35
Q

Frequency of Benign Rolandic Epilepsy?

A

-Most common epilepsy syndrome (15-25% of all pediatric epilepsies)

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36
Q

Duration of episodes in Benign Rolandic Epilepsy?

A

Seizures usually 2-3 minutes

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37
Q

Characteristic of Benign Rolandic Epilepsy?

A
  • Nocturnal focal seizures
  • Rolandic area = corresponds to face, tongue
  • May awaken with inability to speak (“sleep walker”) • May have rhythmic facial twitching
  • May spread to be generalized tonic-clonic seizure
  • Seizures more common at night
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38
Q

Prognosis in Benign Rolandic Epilepsy?

A

-Most outgrow during puberty

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39
Q

EEG in Benign Rolandic Epilepsy?

A

Epileptiform discharges in central-temporal region

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40
Q

Treatment in Benign Rolandic Epilepsy?

A
  • Many do not require treatment at all

- If treatment required (depending on how disruptive or frequent they are): Levetiracetam, carbamazepine

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41
Q

Potential initial side effects of antiepileptics? When do they usually go away?

A
  • Fatigue/sedation
  • GI upset
  • Hyperactvity/Behaviour problems

-Usually go away ~1 week

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42
Q 
Potential idiosyncratic side effects of antiepileptics?
Levetiracetam
Carbamazepine
Topiramate
Lamotrigine
VPA
Vigabatrin
A
  • Levetiracetam - aggression/rage (20%), suicidality (rare - though there is a black box warning that could worsen in those with dep and passive SI)
  • Carbamazepine - rash, hepatitis, anemia
  • Topiramate - weight loss, kidney stones
  • Lamotrigine - rash (5%)
  • VPA - weight gain, hepatitis, pancreatitis, thrombocytopenia, rash, hair loss
  • Vigabatrin - retinal toxicity (5%)
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43
Q

Potential teratogenic side effects of antiepileptics?

A
  • Neural tube defects (folate!)
  • Fetal neurocogntive impairment
  • Increased risk for “old” drugs
  • -VPA>phenytoin>topiramate>carbamazepine
44
Q

Potential toxic (e.g. upper end of dosing or ingestion) side effects of antiepileptics?

A
  • Sedation –> CNS depression –> coma
  • Ataxia
  • Nausea/vomiting
45
Q

For children < 2 years old, which antiepileptics to use? Rationale? Which antiepileptics to avoid? Rationale?

A 
USE:
• Phenobarbitol 
• Levetiracetam 
• Topiramate
• Rationale:
• Solid &amp; growing clinical
experience
• Liquid formulation available

AVOID:
• Valproic acid
• Rationale:
If mitochondrial or metabolic disorder causing seizures, may trigger drug-induced liver failure
• Phenytoin, carbamazpine
• Rationale
Rapidly metabolized. Difficult to achieve therapeutic levels.

46
Q

Special considerations for phenytoin/fosphenytoin?

A

• May use for status epilepticus
• Not used as a routine anticonvulsant
–Gum hypertrophy (“dilantin gums”)
• Never give with dextrose (crystallizes)
• Never give as intramuscular injection (tissue necrosis, abscesses)

47
Q

Antiepileptics to use for primary generalized epilepsy? Do not use?

A 
PRIMARY GENERALIZED:
• Ethosuxamide (only absence) 
• Valproic acid (VPA)
• Lamotrigine (LTG)
• Levetiracetam (LEV)
• Topiramate (TPM)
  • DO NOT USE:
  • Carbamazepine (will worsen)
  • Phenytoin (caution – could worsen)
48
Q

Antiepileptics to use for secondarily generalized (focal onset) epilepsy? Do not use?

A

SECONDARILY GENERALIZED (FOCAL-ONSET):
•First line:
•Levetiracetam
•Carbamazepine / oxcarbamazpine

  • Second line:
  • Lacosamide
  • VPA, LTG, TMP
  • DO NOT USE:
  • Ethosuxamide (ineffective)
49
Q

You see a 7 yo boy with recurrent headaches. Which of the following would be most consistent with a migraine?

  1. Occipital location
  2. Thunderclap onset
  3. Nausea and vomiting
  4. New nocturnal enuresis
A

3

50
Q

How common is pediatric migraine?

A

Common - affects 10-30% of children

51
Q

Diagnostic criteria for pediatric migraine?

A 
Diagnostic Criteria
A. At least 5 attacks fulfilling B-D:
B. Headaches last 1-72 hrs
C. Headache has 2 or more of the following 
• Unilateral or bilateral (frontotemporal, not occipital)
• Pulsing quality
• Moderate-to-severe pain (miss school)
 • Worse with activity (climbing stairs)
D. During headache, at least 1 of:
• Nausea with or without vomiting
 • Photophobia and phonophobia
E. Cannot be explained by another disorder
52
Q

Types of auras? What is contraindicated because of these and why?

A
  • Visual
  • Somatosensory (“tingling”
  • Dysarthria

-Combined estrogen-progesterone OCP contraindicated because of 14 fold increased risk of stroke

53
Q

Management of pediatric migraine?

A

Prevention – life style :
• Food triggers - diary
• Ensure adequate sleep, hydration, exercise
• Avoid skipping meals. Counsel for stress
• Abortive agents:
• Ibuprofen +/- acetaminophen (children)
• Triptans (adolescents)
• Natural remedies: magnesium, vit B2, co-Q10
• Prescription: amitriptyline, topiramate, flunarazine sandomigraine

54
Q

Headache red flags?

A

• Sudden (thunderclap) headache or “worst headache of my life”
-Aneurysmal sentinel bleed

• Occipital location
-Chiari 1 malformation

• Pituitary symptoms?
-Short stature (growth failure)
\+/- obesity
-Bedwetting (diabetes insipidus)
-Vision loss

• Headache awakening from sleep

  • Papilledema
  • CN deficit
  • Focal deficit on neurological examination? (incl. papilledema)
  • Recent head injury and/or coagulopathy? (ie subdural, epidural bleeds)
  • New headache and risk of thrombosis (ie nephrotic syndrome, lupus)
55
Q

Diagnosis of medication overuse headache?

A 
• Daily (or near-daily) headache
-Present upon awakening
-Better (or relieved) with medication
-Recurs later in day
• Headaches >15 days per month (often daily)
• Regular use of:
-Ibuprofen or acetaminophen >15x/mos over >3 mos 
-Triptans >10x/mos over >3 mos
56
Q

Hypotonia vs. weakness, difference in strength vs tone?

A
  • Strength = maximum force muscles can exert with active movement (effort dependent!)
  • Tone = resistance of relaxed muscles to passive movement (state dependent!)
57
Q

Central vs peripheral hypotonia: location, likelihood of seizures, developmental characteristics, weakness, reflexes?

A

Central:

  • Location: Brain, spinal cord
  • Seizures: More likely
  • Development: GDD, persistent infantile reflexes, microcephaly/dysmorphism
  • Weakness: + (usually have antigravity movement)
  • Reflexes: Normal

Peripheral:

  • Location: Motor neuron, nerve, NMJ, muscle
  • Development: Isolated gross motor delay
  • Weakness: +++ (may not have antigravity movement)
  • Reflexes: Decreased/absent
58
Q

A 4 month old girl presents with hypotonia. She is weak with no anti-gravity movements and absent reflexes. She is alert and makes normal visual contact. What is the most likely diagnosis:

  1. Congenital myopathy
  2. Merosin negative muscular dystrophy
  3. Spinal muscular atrophy
  4. Congenital myotonic dystrophy
A

3

  • key is absent reflexes - would be preserved for muscle disorders unless profoundly weak
  • also paint picture of “bright”
59
Q

You clinically suspect spinal muscular atrophy. What is the most appropriate next text test to order?

  1. Muscle biopsy
  2. SMN1 gene deletion testing
  3. SMN1 gene sequencing
  4. SMN2 copy variant testing
A

2

60
Q

What structure affected in SMA? What chromosome and gene?

A
  • Lower alpha motor neurons with cell bodies in anterior horn
  • Deletion of SMN1 on Chromosome 5
61
Q

What structure affected in SMA? What chromosome and gene?

A
  • Lower alpha motor neurons with cell bodies in anterior horn
  • Homozygous deletion of SMN1 on Chromosome 5
62
Q

When a lot of muscle fibres affected by SMA, what other manifestation can see?

A
  • Fasiculations

- Decreased or absent DTRs

63
Q

Most severe type of SMA? Features?

A
  • Type 1a or Congenital SMA

- Starts before birth, so may notice decreased fetal movement

64
Q

What is the classic form of SMA? Features? Survival?

A
  • Type 1b or Infantile SMA or Werdnig Hoffman disease
  • Appear normal at birth
  • First few weeks of life, develop hypotonia
  • Progressive weakness, proximal > distal, more obvious in legs initially –> difficult to sit up
  • Weakness in sucking, chewing, swallowing muscles (bulbar weakness) –> aspiration
  • Weakness in chest wall and diaphragm –> eventually respiratory failure
  • Most survive a few years
65
Q

SMA Types 2, 3, and 4 are progressively ______ and have a _____ age of onset.

A

Milder, later

66
Q

In addition to muscle weakness, feeding problems and breathing difficulties, what are some chronic symptoms of SMA?

A
  • Scoliosis ( due to poor muscle support of spine)

- Extremely thin limbs (due to muscle wasting)

67
Q

Inheritance of SMA?

A

Autosomal recessive

68
Q

What protein is made by the SMN1 gene? Where is it expressed?

A
  • SMN protein

- All cells

69
Q

What is the SMN2 gene? What does it produce, what happens to the product?

A
  • A pseudogene (mutated copy that arose during evolution, less or non-functional version of counterpart) that sits next to SMN1 on chromosome 5
  • Produces SMN proteins that mostly get degraded, with couple full length, functional SMN proteins
70
Q

What genetic change determines severity of SMA?

A
  • Number of copies of SMN2 (since have no SMN1)

- More copies = more SMN protein –> milder phenotype

71
Q

Treatment for SMA?

A
  • Supportive e.g. nutrition, respiratory

- Nusinersin - an antisense oligonucleotide that binds to SMN2 pre-mRNA –> more expression, more normal protein

72
Q

Frequency of carriers for SMA? Frequency of SMA?

A
  • 1/50 people are carriers

- 1/10 000 born with SMA

73
Q

Most common cause of death in childhood due to a genetic cause?

A

-SMA (used to be CF)

74
Q

Which type of SMA can mimic DMD?

A
  • Type 3
  • Mean symptom onset 3-4years with proximal weakness, muscle wasting (vs. the large calves with DMD)
  • Normal CK
75
Q 
A 13 yo boy is referred for clumsiness. Neurodevelopment was entirely normal but he cannot ice skate. Exam shows distal weakness, areflexia, heel cord contractures and decreased sharp sensation to the feet.
Most likely diagnosis is:
1. Spinal muscular atrophy
2. Charcot-Marie-Tooth disease
3. Duchenne muscular dystrophy
4. Becker muscular dystrophy
A

B

76
Q

Clinical manifestations of Charcot-Marie-Tooth in infants and in children?

A

• Infant

  • Delayed gross motor milestones
  • Tight heel cords: “toe-walker”, clumsy gait
  • Hypotonia & Weakness
  • Self-mutilation

• Children

  • Abnormal gait & clumsiness.
  • Foot deformities: pes cavus, hammertoes
  • Weakness + foot drop

-Very slowly progressive - most will walk for entire life, require ambulatory aids when older

77
Q

Frequency of CMT?

A

-Common - 1/2500 people

78
Q

Investigations in CMT?

A
  • Nerve conduction studies/ electromyography

- If FH can go to genetic testing (panel)

79
Q

You see a 6 month old boy with gross motor delay. He can roll to one side, but not sit. He developed “failure to thrive” due to poor oral intake. He is babbling & alert. He has hypotonia, weakness but difficult to elicit reflexes (1+). His serum CK 80 U/L (normal < 175 U/L). What is the most likely diagnosis:

  1. Congenital myopathy
  2. Congenital muscular dystrophy
  3. Spinal muscular atrophy
  4. Congenital myotonic dystrophy
A

1

KEY factor is reflexes present but hard to get so NOT SMA
also CK is normal - why consistent with congenital myopathy and not a congenital muscular dystrophy

80
Q

Distinguishing muscular dystrophies from congenital myopathies?

A
  • Muscular dystrophies:
  • -Gene mutation impairing muscle fibre integrity / stability
  • -Muscle membrane/sarcolemma prone to tearing (HIGH CK)

-Congenital myopathies:
–Gene mutation impairing basic contractile unit (actin-myosin interaction) causing weakness
-Muscle membrane intact
(NORMAL CK)

81
Q

Characteristics of congenital myopathies? Diagnosis?

A
  • Cognitively normal
  • Extreme phenotypic variability
  • Muscle weakness is non-progressive
  • May show very slow gain in strength

• Diagnosis now by genetic testing (not muscle biopsy)

82
Q

You see a 6 month old boy with gross motor delay. He can roll to one side, but not sit. He developed “failure to thrive” due to poor oral intake. He is babbling & alert. He has hypotonia, weakness but difficult to elicit reflexes (1+). His serum CK 2,500 U/L (normal < 175 U/L). What is the most likely diagnosis:

  1. Congenital myopathy
  2. Congenital muscular dystrophy
  3. Spinal muscular atrophy
  4. Congenital myotonic dystrophy
A

2

Myotonic - have COGNITIVE impairment because affects brain as well. Key differentiating feature.

83
Q

What happ?

A

Muscle fibres are fragile and tear - CK leaks out

84
Q

Inheritance of Duchenne muscular dystrophy?

A

-X-linked (almost exclusively affects males)

85
Q

Clinical features of Duchenne muscular dystrophy? What is the milder phenotype?

A
  • Progressive, proximal muscle weakness
  • Symptom onset ~4-6 yo• Loss of ambulation ~10-13 yo
  • Life expectancy ~mid-to-late 20’s

-Milder phenotype: Beckers Muscular Dystrophy

86
Q

Pharmacotherapy for DMD? Benefit?

A

• Corticosteroids can be used (prednisone, deflazacort)

  • Prolong independent ambulation by 2-1/2 years
  • Preserve respiratory function; slow scoliosis onset & progression
87
Q

A 7 year old boy presents with increasing falls and difficulty getting up from the floor. He has a mild learning disability. He has proximal weakness and prominent calf muscles. His serum CK is 20,000 U/L. What is the most appropriate test:

  1. DMD duplication deletion analysis
  2. Sequence DMD gene
  3. Muscle biopsy
  4. Whole exome sequencing
A

1

2/3 of patients with dmd have deletion or duplication within gene
less common to have point mutation

88
Q

You perform a routine exam on a 10 year old boy. He has freckling in his armpits & skin lesions (cafe au laits). What is the most likely diagnosis?

  1. McCune-Albright syndrome
  2. Tuberous sclerosis
  3. Neurofibromatosis type 1
  4. Neurocutaneous melanosis
A

3

89
Q

NF-1 criteria? Inheritance?

A

-AD inheritance

2/7 of:

  • > /=6 cafe-au-lait macules
  • –>5mm prepubertal
  • –>15mm post-pubertal
  • Axillary or inguinal freckling
  • –Multiple hyperpigmented areas 2-3mm in diameter
  • > /=2 neurofibromas or 1 plexiform neurofibroma
  • > /=2 iris Lisch nodules
  • –Hamartomas within the iris
  • –Best identified with slit-lamp exam
  • Optic gliomas
  • -Mostly low-grade astrocytomas
  • Distinctive osseous lesion e.g. sphenoid dysplasia (may cause pulsating exophthalmos) OR cortical thinning of long bones +/- pseudoarthrosis (e.g. tibia)
  • FH - a first degree relative with NF-1 whose diagnosis was bsed on these criteria
90
Q

You see a 15 yo boy with learning difficulties & seizures He has 3 hypopigmented spots. What is the name of this facial lesion?

  1. Acne vulgaris
  2. Adenomatous sebaceum 3. Shagreen patch
  3. Molluscum fibrosum
A

2
Commonly misdiagnosed as acne

TS significant learning problems
NF mild cognition issue

91
Q

TS clinical criteria? Inheritance?

A
  • AD inheritance
  • Definite diagnosis: Two major features OR one major feature + >/= 2 minor

Major features:

  • Hypomelanotic macules (>/=3, at least 5mm diameter)
  • Angiofibromas (>/=3) or fibrous cephalic plaque
  • Ungual fibromas (>/=2)
  • Shagreen patch
  • Angiomyolipomas (>/=2)
  • Multiple retinal hamartomas
  • Subependymal nodules
  • Subependymal giant cell astrocytoma
  • Cardiac rhabdomyoma
  • Lymphangioleiomyomatosis (LAM)
  • Cortical dysplasia (includes tubers and cerebral white matter radial migration lines)

Minor features:

  • “Confetti” skin lesions
  • Dental enamel pits (>3)
  • Intraoral fibromas (>/=2)
  • Retinal achromic patch
92
Q

You see a newborn girl with seizures & vesicular lesions in a dermatomal distribution. HSV, VZV testing are negative. What is the most likely diagnosis?

  1. Congenital ichthyosis
  2. Hypomelanosis of ito
  3. Sturge Weber syndrome 4. Incontinenti pigmenti
A

4
dermatomal distribution
vesicular rash that can look a bit lioke shingles
NEMO gene ( jsut like the fish)
as older peg like teeth and higher incidence of learning problems

93
Q

What is the inheritance pattern for this disorder? (huge port wine stain)

  1. Autosomal dominant
  2. Autosomal recessive
  3. X-lined
  4. Sporadic
A

4

not all children with port wine in v1 have sturge weber 5-15%

94
Q

A 10 year old boy is seen for 3 week of discreet eye movements to the left. His examination is normal. What of the following is the most appropriate diagnosis:

  1. Sydenham chorea
  2. Transient tic disorder 3. Chronic tic disorder
  3. Tourette syndrome
A

2

description of event
discrete movements - not consistent with chorea

transient tic - discrete repetative mucle movements <12 months

> 12 months could be chronic tic

> 12 months and also 2 or more motor and vocal may meet criteria for tourette

95
Q

A 10 year old boy has a six week history of fidgeting and writhing movements of both arms. He reports joint pain without swelling or warmth. What is the most appropriate diagnosis for his movements?

  1. Neuroborreliosis (Lyme) chorea
  2. Huntington chorea
  3. Sydenham syndrome
  4. Somatization syndrome
A

3

96
Q

Cerebral palsy is most likely to arise following a hypotensive event at what time?

  1. 5-8 weeks GA
  2. 16-20 weeks GA
  3. 26-30 weeks GA
  4. 40-42 weeks GA
A

3
most typically seen in prems who are born during that time frame because cells starting to migrate out to cortex and if hypotensive event

97
Q

Spastic diplegia (CP) is associated with _______.

A

Periventricular leukomalacia

98
Q

PVL only occurs between _______ weeks gestation.

A

24-32

99
Q

Spastic hemiplegia often isn’t seen until after _____ of age, It presents with ______.

A

6 months, early hand preference

100
Q

Definition of BRUE?

A

An event occurring in an infant younger than 1 year when the observer reports a sudden, brief, and now resolved episode of >/= 1 of the following:

  • cyanosis or pallor
  • absent, decreased, or irregular breathing
  • marked change in tone (hyper- or hypotonia)
  • altered level of responsiveness
  • Only diagnosed after appropriate history and PE that produces NO EXPLANATION*
101
Q

In terms of BRUE, meaning of brief? Resolved? Unexplained?

A

Brief: <1 min, typically 20-30s
Resolved: Patient returned to his/her baseline state of health after the event, normal VS, normal appearance
Unexplained: Not explained by an identifiable medical condition

102
Q

Criteria to meet to be designated lower risk for BRUE?

A
  • Age >60 days
  • Prematurity: gestational age >/= 32 weeks and postconceptional age >/=45 weeks
  • First BRUE (No previous ever, and not in clusters)
  • Duration of event <1 minute
  • No CPR required by trained medical provider
  • No concerning historical features
  • No concerning physical examination findings
103
Q

When do immediately do MRI for sacral dimple?

A
  • Other associated cutaneous finding (e.g. hypertrichosis, hemangioma, atretic meningocele, subcutaneous mass, caudal appendage)
  • Abnormal neurologic exam findings
104
Q

Reasons to do an US OR MRI for sacral dimple?

A
  • Multiple dimples
  • Dimple diameter >5mm
  • Dimple >/=2.5cm above the anus
  • Dimple outside of the sacrococcygeal region
105
Q

Rick factors for recurrence of febrile seizures?

A
  • Younger age at first seizure
  • Short duration of fever before the onset of first febrile seizure
  • Low temperature at onset
  • Family history of febrile seizures
106
Q

Complex febrile seizures are characterized by?

A
  1. Focal clinical maniifestation
  2. Duration longer than 15 minutes
  3. More than one in a 24hour period

Neuro HR (1 decks)

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