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
Tx of absence seizures
Ethosuximide | Valproic acid
26
How is daydreaming different than an absence seizure?
- episodes can be interrupted - onset is slow - lasts until something catches interest - no associated automatisms
27
Criteria of febrile seizure
- 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
When should LP be done for patient with febrile seizure?
< 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
Febrile seizure treatment
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
Risk factors of meningitis
head trauma, young age, splenectomy, facial infection, maternal pyrexia or infection upon delivery
31
Classic triad of meningitis
fever, nuchal rigidity, changes in mentation
32
In infants less than 3 mon, ____ may be only symptom of meningitis.
fever | but also look for poor feeding, lethargy, poor perfusion
33
Infection that most commonly causes meningitis?
VIRAL - enterovirus (>85%)
34
Bacterial that cause meningitis in neonates, infants, and children
neonate: Group B strep infants: Listeria, Group B strep, E. coli children: strep pneumo, H-flu, Neisseria (older kids)
35
Results of spinal tap for meningitis: bacterial vs viral
Bacterial: elevated pressure, high WBC, PMN dominate, high protein, decreased glucose; then gram stain Viral: normal pressure, lymphocyte dominate, normal protein and glucose
36
Meningitis treatment
empirical abx to cover G+/staph: ampicillin (infant), vancomycin empirical abx to cover G-: aminoglycoside manage complications: sepsis, DIC, seizures, developmental delays
37
2 most common muscle dystrophies and how are they inherited?
Becker MD Duchenne MD both X-linked recessive with defect in dystrophin gene
38
Duchenne Muscular Dystrophy signs
``` 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
Duchenne Muscular Dystrophy labs and dx
elevated muscle enzymes: CK, LDH, aldolase chromosomal microarrays: dystrophin gene mutation EMG: low amplitude contractions Muscle biopsy: gold standard
40
Duchenne Muscular Dystrophy treatment
Prednisone Supportive: ventilator dependent, cardiac insufficiency, GI dysmotility, PT/OT, genetic counseling, family support
41
Muscle biopsy findings for muscle dystrophy
variability in size, macrophagic activity, and increased fatty deposition into muscles
42
Difference btwn Duchenne and Becker muscular dystrophy
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
Any boy who fails to walk by 18 months or loses ability to walk should have what checked?
CK levels
44
The "Big 3" neurocutaneous syndromes
Neurofibromatosis (NF-1 & NF-2) Sturge Weber Tuberous Sclerosis
45
What are NF-1 and NF-2 patients at high risk for?
CNS tumors; due to defects in tumor suppressor genes
46
Signs of NF-1
``` cafe au lait spots axillary freckling neurofibromas Lisch nodules (less likely in NF-2) short stature macrocephaly ```
47
Brain MRI results of neurofibromatosis
glial proliferation and fluid accumulation within myelin sheaths
48
NF-1 and NF-2 inheritance and etiologies
autosomal dominant defects in tumor suppressor genes (NF-1 fibromin and NF-2 merlin)
49
NF-1 and NF-2 treatment
Annual ophthalmology exams Correction of associated scoliosis and neuropathies Surgery to remove tumors NF-2: Auditory brainstem implant
50
Dx criteria for NF-2
bilateral acoustic neuroma OR Fhx of NF-2 (1st deg) with unilateral acoustic neuroma and 2 of following: Meningioma Glioma, Schwannoma, juvenile cataracts
51
Port-wine stain involving ophthalmic division of trigeminal nerve
Sturge Weber Syndrome
52
Associated symptoms of Sturge Weber syndrome
Seizures, TIA-like sx's, developmental delays, buphthalmos (enlarged cornea), glaucoma
53
CT/MRI results of Sturge Weber syndrome
"tram track" calcifications | cerebral atrophy with angiomatosis
54
Sturge Weber syndrome treatment
Manage seizures (antiepileptic) Glaucoma tx to prevent blindness: beta-blocker drops Stroke prevention: aspirin for young adults
55
Cutaneous presentations of tuberous sclerosis
adenoma sebaceum ash-leaf spots Shagreen patch Cardiac rhabdomyoma
56
acne + milar distribution + developmentally delayed, then search skin for ______.
Shagreen patch
57
CT/MRI of Tuberous Sclerosis
proliferation of glial cells (astrocytomas) in ventricles = tubers intracranial calcifications
58
Tuberous Sclerosis treatment
manage seizures | chemo, resect tumors
59
Most common cause of death in tuberous sclerosis patients
renal failure
60
When to get CT for head trauma?
``` 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
When to get CT for head trauma in infant < 2yo?
``` High force or hard surface Scalp hematoma Unwitnessed trauma Suspected abuse Freq vomiting Seizures LOC > 30 sec Age < 3 mon Behavior changes ```
62
Define concussion (MTBI)
rapid short-lived (< 30 min) of mild impairment of neuro function (GCS >13) confusion, change in mental status, +/- LOC
63
When should concussion be referred to specialist?
symptoms after 14 days multiple risk factors severe injury
64
When can return to play program be started?
when athlete has no symptoms for at least 24 hrs