Peds Neuro Flashcards

1
Q

What is craniosynostosis?

A

premature fusion of a cranial suture

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2
Q

Most common suture affected by craniosynostosis?

A

sagittal suture = scaphocephaly

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3
Q

Causes of microcephaly

A

maternal alcohol use (leading cause)
maternal TORCH infections
chromosomal defect
metabolic disorder

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4
Q

Causes of macrocephaly

A

hydrocephalus (high CSF)
Aqueduct or ventricle obstruction
Chiari malformation
Toxoplasmosis infection

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5
Q

First signs of congenital hydrocephalus in infants

A

increased head circum, widening sutures, bulging fontanels

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6
Q

signs/symptoms of increased ICP in children

A

SUN-SETTING EYES
vomiting
lack of coordination

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7
Q

Cushing’s triad for increased ICP

A

bradycardia
HTN
irregular breathing

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8
Q

anencephalopy

A

no brain due to no closure of neural tube

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9
Q

encephalocele

A

protrusion of meninges /neural tissue through skull; may be surgically repaired

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10
Q

signs of spina bifida in newborns

A

hair tuft, dimples, fatty lump, hemangioma, or hypopigmentation on lower back

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11
Q

2 forms of spina bifida

A

oculta: defect in vertebrae only; often asx
cystica: myelomeningocele; protrusion of dural sac and neural element through defect in vertebrae

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12
Q

What are signs of spina bifida cystica?

A

lack of sensation and motor function below affected vertebrae

new incontinence, foot deformities, back pain, early scoliosis, tethered cord

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13
Q

How is hydrocephalus treated?

A

prompt VP shunting

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14
Q

Dx imaging for spina bifida

A

sacral U/S if < 2-3 mon old
XR to locate vertebrae
MRI: gold standard

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15
Q

Classifications of cerebral palsy

A
spastic vs non-spastic
# of limb involved: mono-, hemi-, di-, quadplegia
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16
Q

Red flags of cerebral palsy

A

exaggerated primitive reflexes
hyper or hypotonia
clumsy motor skills
tremors

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17
Q

Disorders that commonly accompany cerebral palsy

A

seizure disorder, mental retardation

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18
Q

Cerebral palsy management

A

supportive; meds for spasticity and seizures

anti-epileptics
benzos
muscle relaxants
PT, OT, speech

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19
Q

Pathologies in pediatric patients that mimic seizure activity

A

Reflux (Sandifer syndrome)
Breathholding spells
Tics
Non-epileptic myoclonus

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20
Q

Signs of Sandifer syndrome

A

unusual dystonia posturing after feeds

choking, spitting up, vomiting, cyanosis, apnea, pallor

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21
Q

Breath holding spells most commonly seen in what age range?

A

15-36 months

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22
Q

Describe an absence seizure

A
  • sudden cessation of motor activity or speech
  • age usually over 5 yo
  • last less than 30 seconds
  • no post-ictal phase
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23
Q

Classic EEG of absence seizure

A

2.5-3 Hz spike-and-wave pattern

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24
Q

Absence seizure can be induced with __________.

A

hyperventilation

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25
Q

Tx of absence seizures

A

Ethosuximide

Valproic acid

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26
Q

How is daydreaming different than an absence seizure?

A
  • episodes can be interrupted
  • onset is slow
  • lasts until something catches interest
  • no associated automatisms
27
Q

Criteria of febrile seizure

A
  • Simple Generalized seizure with fever (100.4F)
  • Last < 15 mins
  • Doesn’t recur within 24 hrs
  • Age 6 months-6 years (Peak 2 yo)
  • No signs of CNS infection, hx of non-febrile seizures, fhx of epilepsy, focal neural deficits
28
Q

When should LP be done for patient with febrile seizure?

A

< 2-3 mon or meningeal signs: ALWAYS
< 12 mon: highly consider, esp. if unknown immunization status
> 12 mon or recent abx use: consider per exam

29
Q

Febrile seizure treatment

A

Reassurance!

  • very low risk of progressing to epilepsy
  • no evidence that anti-epileptics or anti-pyretics work

Tell parent there is high recurrence rate through age 6

30
Q

Risk factors of meningitis

A

head trauma, young age, splenectomy, facial infection, maternal pyrexia or infection upon delivery

31
Q

Classic triad of meningitis

A

fever, nuchal rigidity, changes in mentation

32
Q

In infants less than 3 mon, ____ may be only symptom of meningitis.

A

fever

but also look for poor feeding, lethargy, poor perfusion

33
Q

Infection that most commonly causes meningitis?

A

VIRAL - enterovirus (>85%)

34
Q

Bacterial that cause meningitis in neonates, infants, and children

A

neonate: Group B strep
infants: Listeria, Group B strep, E. coli
children: strep pneumo, H-flu, Neisseria (older kids)

35
Q

Results of spinal tap for meningitis: bacterial vs viral

A

Bacterial: elevated pressure, high WBC, PMN dominate, high protein, decreased glucose; then gram stain

Viral: normal pressure, lymphocyte dominate, normal protein and glucose

36
Q

Meningitis treatment

A

empirical abx to cover G+/staph: ampicillin (infant), vancomycin

empirical abx to cover G-: aminoglycoside

manage complications: sepsis, DIC, seizures, developmental delays

37
Q

2 most common muscle dystrophies and how are they inherited?

A

Becker MD
Duchenne MD

both X-linked recessive with defect in dystrophin gene

38
Q

Duchenne Muscular Dystrophy signs

A
hip flexors/extensors first sign
progressive weakness of all muscles
waddling gait
pseudo-hypertrophy of calves
"Gower Sign" = can't stand w/o arms
39
Q

Duchenne Muscular Dystrophy labs and dx

A

elevated muscle enzymes: CK, LDH, aldolase
chromosomal microarrays: dystrophin gene mutation
EMG: low amplitude contractions
Muscle biopsy: gold standard

40
Q

Duchenne Muscular Dystrophy treatment

A

Prednisone

Supportive: ventilator dependent, cardiac insufficiency, GI dysmotility, PT/OT, genetic counseling, family support

41
Q

Muscle biopsy findings for muscle dystrophy

A

variability in size, macrophagic activity, and increased fatty deposition into muscles

42
Q

Difference btwn Duchenne and Becker muscular dystrophy

A

Becker is incomplete loss of dystrophin fxtn while Duchenne is complete

Many Becker patients live to adulthood and Duchenne don’t live past teens (cardiac or respiratory failure)

43
Q

Any boy who fails to walk by 18 months or loses ability to walk should have what checked?

A

CK levels

44
Q

The “Big 3” neurocutaneous syndromes

A

Neurofibromatosis (NF-1 & NF-2)
Sturge Weber
Tuberous Sclerosis

45
Q

What are NF-1 and NF-2 patients at high risk for?

A

CNS tumors; due to defects in tumor suppressor genes

46
Q

Signs of NF-1

A
cafe au lait spots
axillary freckling
neurofibromas
Lisch nodules (less likely in NF-2)
short stature
macrocephaly
47
Q

Brain MRI results of neurofibromatosis

A

glial proliferation and fluid accumulation within myelin sheaths

48
Q

NF-1 and NF-2 inheritance and etiologies

A

autosomal dominant

defects in tumor suppressor genes (NF-1 fibromin and NF-2 merlin)

49
Q

NF-1 and NF-2 treatment

A

Annual ophthalmology exams
Correction of associated scoliosis and neuropathies
Surgery to remove tumors

NF-2: Auditory brainstem implant

50
Q

Dx criteria for NF-2

A

bilateral acoustic neuroma
OR
Fhx of NF-2 (1st deg) with unilateral acoustic neuroma and 2 of following: Meningioma
Glioma, Schwannoma, juvenile cataracts

51
Q

Port-wine stain involving ophthalmic division of trigeminal nerve

A

Sturge Weber Syndrome

52
Q

Associated symptoms of Sturge Weber syndrome

A

Seizures, TIA-like sx’s, developmental delays, buphthalmos (enlarged cornea), glaucoma

53
Q

CT/MRI results of Sturge Weber syndrome

A

“tram track” calcifications

cerebral atrophy with angiomatosis

54
Q

Sturge Weber syndrome treatment

A

Manage seizures (antiepileptic)

Glaucoma tx to prevent blindness: beta-blocker drops

Stroke prevention: aspirin for young adults

55
Q

Cutaneous presentations of tuberous sclerosis

A

adenoma sebaceum
ash-leaf spots
Shagreen patch
Cardiac rhabdomyoma

56
Q

acne + milar distribution + developmentally delayed, then search skin for ______.

A

Shagreen patch

57
Q

CT/MRI of Tuberous Sclerosis

A

proliferation of glial cells (astrocytomas) in ventricles = tubers

intracranial calcifications

58
Q

Tuberous Sclerosis treatment

A

manage seizures

chemo, resect tumors

59
Q

Most common cause of death in tuberous sclerosis patients

A

renal failure

60
Q

When to get CT for head trauma?

A
initial GCS < 13
2 hrs after injury GCS < 15
LOC > 1 min
Suspect skull fracture
Signs of basilar skull fracture
Vision changes
2 or more vomiting episodes
amnesia at least 30 min before impact
high velocity injury
61
Q

When to get CT for head trauma in infant < 2yo?

A
High force or hard surface
Scalp hematoma
Unwitnessed trauma
Suspected abuse
Freq vomiting
Seizures
LOC > 30 sec
Age < 3 mon
Behavior changes
62
Q

Define concussion (MTBI)

A

rapid short-lived (< 30 min) of mild impairment of neuro function (GCS >13)

confusion, change in mental status, +/- LOC

63
Q

When should concussion be referred to specialist?

A

symptoms after 14 days
multiple risk factors
severe injury

64
Q

When can return to play program be started?

A

when athlete has no symptoms for at least 24 hrs