Peds Neuro Flashcards
What is craniosynostosis?
premature fusion of a cranial suture
Most common suture affected by craniosynostosis?
sagittal suture = scaphocephaly
Causes of microcephaly
maternal alcohol use (leading cause)
maternal TORCH infections
chromosomal defect
metabolic disorder
Causes of macrocephaly
hydrocephalus (high CSF)
Aqueduct or ventricle obstruction
Chiari malformation
Toxoplasmosis infection
First signs of congenital hydrocephalus in infants
increased head circum, widening sutures, bulging fontanels
signs/symptoms of increased ICP in children
SUN-SETTING EYES
vomiting
lack of coordination
Cushing’s triad for increased ICP
bradycardia
HTN
irregular breathing
anencephalopy
no brain due to no closure of neural tube
encephalocele
protrusion of meninges /neural tissue through skull; may be surgically repaired
signs of spina bifida in newborns
hair tuft, dimples, fatty lump, hemangioma, or hypopigmentation on lower back
2 forms of spina bifida
oculta: defect in vertebrae only; often asx
cystica: myelomeningocele; protrusion of dural sac and neural element through defect in vertebrae
What are signs of spina bifida cystica?
lack of sensation and motor function below affected vertebrae
new incontinence, foot deformities, back pain, early scoliosis, tethered cord
How is hydrocephalus treated?
prompt VP shunting
Dx imaging for spina bifida
sacral U/S if < 2-3 mon old
XR to locate vertebrae
MRI: gold standard
Classifications of cerebral palsy
spastic vs non-spastic # of limb involved: mono-, hemi-, di-, quadplegia
Red flags of cerebral palsy
exaggerated primitive reflexes
hyper or hypotonia
clumsy motor skills
tremors
Disorders that commonly accompany cerebral palsy
seizure disorder, mental retardation
Cerebral palsy management
supportive; meds for spasticity and seizures
anti-epileptics
benzos
muscle relaxants
PT, OT, speech
Pathologies in pediatric patients that mimic seizure activity
Reflux (Sandifer syndrome)
Breathholding spells
Tics
Non-epileptic myoclonus
Signs of Sandifer syndrome
unusual dystonia posturing after feeds
choking, spitting up, vomiting, cyanosis, apnea, pallor
Breath holding spells most commonly seen in what age range?
15-36 months
Describe an absence seizure
- sudden cessation of motor activity or speech
- age usually over 5 yo
- last less than 30 seconds
- no post-ictal phase
Classic EEG of absence seizure
2.5-3 Hz spike-and-wave pattern
Absence seizure can be induced with __________.
hyperventilation
Tx of absence seizures
Ethosuximide
Valproic acid
How is daydreaming different than an absence seizure?
- episodes can be interrupted
- onset is slow
- lasts until something catches interest
- no associated automatisms
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
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
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
Risk factors of meningitis
head trauma, young age, splenectomy, facial infection, maternal pyrexia or infection upon delivery
Classic triad of meningitis
fever, nuchal rigidity, changes in mentation
In infants less than 3 mon, ____ may be only symptom of meningitis.
fever
but also look for poor feeding, lethargy, poor perfusion
Infection that most commonly causes meningitis?
VIRAL - enterovirus (>85%)
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)
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
Meningitis treatment
empirical abx to cover G+/staph: ampicillin (infant), vancomycin
empirical abx to cover G-: aminoglycoside
manage complications: sepsis, DIC, seizures, developmental delays
2 most common muscle dystrophies and how are they inherited?
Becker MD
Duchenne MD
both X-linked recessive with defect in dystrophin gene
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
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
Duchenne Muscular Dystrophy treatment
Prednisone
Supportive: ventilator dependent, cardiac insufficiency, GI dysmotility, PT/OT, genetic counseling, family support
Muscle biopsy findings for muscle dystrophy
variability in size, macrophagic activity, and increased fatty deposition into muscles
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)
Any boy who fails to walk by 18 months or loses ability to walk should have what checked?
CK levels
The “Big 3” neurocutaneous syndromes
Neurofibromatosis (NF-1 & NF-2)
Sturge Weber
Tuberous Sclerosis
What are NF-1 and NF-2 patients at high risk for?
CNS tumors; due to defects in tumor suppressor genes
Signs of NF-1
cafe au lait spots axillary freckling neurofibromas Lisch nodules (less likely in NF-2) short stature macrocephaly
Brain MRI results of neurofibromatosis
glial proliferation and fluid accumulation within myelin sheaths
NF-1 and NF-2 inheritance and etiologies
autosomal dominant
defects in tumor suppressor genes (NF-1 fibromin and NF-2 merlin)
NF-1 and NF-2 treatment
Annual ophthalmology exams
Correction of associated scoliosis and neuropathies
Surgery to remove tumors
NF-2: Auditory brainstem implant
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
Port-wine stain involving ophthalmic division of trigeminal nerve
Sturge Weber Syndrome
Associated symptoms of Sturge Weber syndrome
Seizures, TIA-like sx’s, developmental delays, buphthalmos (enlarged cornea), glaucoma
CT/MRI results of Sturge Weber syndrome
“tram track” calcifications
cerebral atrophy with angiomatosis
Sturge Weber syndrome treatment
Manage seizures (antiepileptic)
Glaucoma tx to prevent blindness: beta-blocker drops
Stroke prevention: aspirin for young adults
Cutaneous presentations of tuberous sclerosis
adenoma sebaceum
ash-leaf spots
Shagreen patch
Cardiac rhabdomyoma
acne + milar distribution + developmentally delayed, then search skin for ______.
Shagreen patch
CT/MRI of Tuberous Sclerosis
proliferation of glial cells (astrocytomas) in ventricles = tubers
intracranial calcifications
Tuberous Sclerosis treatment
manage seizures
chemo, resect tumors
Most common cause of death in tuberous sclerosis patients
renal failure
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
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
Define concussion (MTBI)
rapid short-lived (< 30 min) of mild impairment of neuro function (GCS >13)
confusion, change in mental status, +/- LOC
When should concussion be referred to specialist?
symptoms after 14 days
multiple risk factors
severe injury
When can return to play program be started?
when athlete has no symptoms for at least 24 hrs
