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85 Cards in this Set

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NTD: risk factors
1.maternal malnutrition
2.drugs
3.radiation
4.genetics
5.congenital infection
NTD: closes when?
week 3-4 gestation
drugs associated w/NTDs
1.valproic acid
2.carbamazepine

antiepileptics
NTD screen when?
wk 16-18
Cushing triad
HTN
bradycardia
Cheyne-Stokes respiration
upward gaze paralysis
"setting sun sign"

dx?
hydrocephalus
clonus
(+) Babinski
diplopia
setting sun sign
papilledema

dx?
hydrocephalus
T/F: if increased intracranial pressure, then it's ok to LP
false! herniation thru foramen magnum
hydrocephalus: tx
shunts
IV abx
acetazolimide
indwelling shunt: most common bug
staph epidermidis
late indicator of hydrocephalus
Cushing triad
most common movement disorder
CP
microcephaly / macrocephaly
2 s.d. below / above mean
premature fusion of cranial sutures
craniosynostosis
craniosynostosis: causes
1.idiopathic
2.syndrome component
tuberous sclerosis: inheritance pattern
autosomal dominant
tuberous sclerosis: sporadic / inherited cases more common?
sporadic
ash-leaf spots

what is it?
dx?
flat, hypopigmented macules

tuberous sclerosis
areas of abnl skin thickening
shagreen patches
tuberous sclerosis: skin sx
1.ash-leaf spots (earliest)
2.shagreen patches
3.sebaceous adenomas
4.uncal fibromas
tuberous sclerosis: neuro sx
1.mental retardation
2.seizures
3.periventricular knoblike areas ("tubers")
4.subependymal nodules
5.giant cell astrocytomas
tuberous sclerosis: tumors where?
1.kidney
2.heart (rhabdomyomas)
3.retina
tuberous sclerosis: tx
1.antiepileptics
2.surgical removal
macrocephaly: causes
1.large brain
2.cranioskeletal dysplasias
3.storage disease
4.hydrocephalus
microcephaly: causes
1.genetic (tri 21, Prader-willi)
2.congenital insults
craniosynostosis: sagittal suture closure -> ?
long head, narrow face = scaphocephaly (most common)
craniosynostosis: coronal suture closure -> ?
wide face, boxlike skull
craniosynostosis: tx
surgery = controversial

if done, age < 2
headaches: important to first do what?
r/o life-threatening conditions
-tumors
-intracranial bleeds
-acute hydrocephalus
-meningitis
headaches: differential
1.vascular
2.tension
3.increased intracranial pressure
4.systemic illness (infxn)
5.sinusitis
6.dental abscess
7.poor vision
8.TMJ
migraines: possible etiology
vasodilation of intracranial vessels in response to vascular/neuronal stimulus
tension headaches: possible etiology
prolonged low-grade muscle contraction
headache that wakes pt fr/sleep

dx?
increased intracranial pressure
headache: worse w...
-lying flat
-bending,sneezing,straining
-n/v
-personality changes, gait disturbances, vision abnlities
increased intracranial pressure
headaches: what to ask in HPI?
-history (acute/chronic)
-onset
-progression
-severity
-location
-duration
-timing
-alleviating/exacerbating
-medication response
-precipitants
-stress
-life changes
papilledema + 6th nerve palsy

dx?
increased intracranial pressure
headache + carotid bruit

dx?
AVMs
complicated migraine
accompanied/followed by...
-weakness,paralysis
-sensory loss
-difficulty speaking
-alteration in vision/mental status
headaches: severe, recurrent, pounding, focal, relieved by sleep

dx?
migraine
headaches: diffuse, constant, symmetric, "bandlike", under stress / fatigued, not interfere w/ADL

dx?
tension headaches
headaches: overwt adolescent female, tetracycline / corticosteroid use

dx?
pseudotumor cerebri
(idiopathic intracranial HTN)
impaired CSF resorption
papilledema
nl CT

dx?
pseudotumor cerebri
pseudotumor cerebri: tx
-repeat LPs
-acetazolamide
-surgery (optic nerve sheath fenestration)
migraine: tx
1.acetaminophen
2.ibuprofen
3.metoclopramide
4.sumatriptan
5.ergotamine
tension headaches: tx
1.nonprescription analgesics
2.rest
3.stress management
4.biofeedback training
most common cause of intracranial hemorrhage in children
AVMSs

(abnl collection of arteries & veins)
encephalopathy: definition
generalized cerebral dysfunction
recent/concurrent febrile illness
focal findings (hemiparesis, ataxia, CN deficits)
seizures

dx?
HSV encephalitis
Reye's syndrome
mitochondrial disorder
acute-onset encephalopathy
degenerative liver dz
s/p viral illness + ASA use
n/v
delirium
stupor
hypoglycemia
elevated transaminases & ammonia

dx?
reye's syndrome
EEG: focal mediotemporal spikes superimposed on diffuse slow wave pattern

CT: temporal lobe abnlities

dx?
HSV encephalitis
status epilepticus: tx
IV/rectal lorazepam/diazepam
motor & vocal tics daily for > 1 yr
OCD
ADHD

dx?
Tourette's syndrome
tics
-sudden
-involuntary
-repetitive behavior
simple febrile seizure
< 5-10 minutes
generalized
not recurring during preciptating illness
complex febrile seizure
>10-15 minutes
recur w/in 24 hrs
focal features
goal of supportive care in severe brain injury?
optimizing cerebral perfusion pressure
most common cause of pediatric death & disability in developed world?
acute head trauma (MVAs)
EEG: hypsarrhythmia

dx?
infantile spasms
infantile spasms
-recurrent
-age 2-7 months
-mixed flex-ex spasms (seconds) x 100
-associated w/neurodevelopmental diseases
-dx confirm by EEG (hypsarrhythmia)
infantile spasms -> ?
Lennox-Gastaut's syndrome

(frequent occurences of mixed, generalized seizures refractory to meds)
acute, progressive, ascending paralysis/weakness, no DTRs, increased CSF protein

dx?
GBS
Guillan-Barre: ascending/descending weakness
ascending
GBS: pathophysiology
demyelination of peripheral nerves s/p viral illness
GBS: tx
IVIG / plasmapheresis may hasten resolution
resembles GBS, but ocular palsie + pupillary abnlity

dx?
tick paralysis
tick paralysis: tx
remove tick
MG: pathophysiology
autoimmune disase of NMJ
poliomyelitis: pathophysiology
viral illness
anterior horn cells (spine)
T/F: oral polio vaccine provides herd immunity
true!
Clostridium botulinum: paralysis via...
toxin which irreversibly blocks ACh release at motor endplate
spinal muscle atrophy: pathophysiology
inherited
degeneration of anterior horn cells & CN motor nuclei
Werdnig-Hoffman's disease type I
generalized hypotonia, weakness
evident in early infancy
leukodystrophies
white matter disorders
leukodystrophies: pathophysiology
-abnl formed myeline
-impaired conduction
-rapid myelin breakdown
-spasticity
-developmental milestone loss
-visual disturbances
-personality changes
-poor school performance
leukodystrophies
girl w/nl development until age 1, after which developed microcephaly & milestone regression

dx?
Rett's syndrome
-mental retardation
-seizures
-autistic
-hand wringing, ataxia
Rett's syndrome
ataxia
inability to coordinate movement and control balance
most common causes of ataxia in children
1.drugs (phenytoin, carbamazepine, sedatives, hypnotics, phencyclidine)

2.acute posinfectious cerebellar ataxia
retinal angiomas
cerebellar hemangioblastoma
renal cell CA
pheochromocytoma

dx?
Von Hippel-Lindau's disease
Von Hippel-Lindau's disease: presents at...
adolescence or older
port-wine stain (nevus flammeus)
progressive mental retardation
seizures
hemiparesis
visual impairment
glaucoma

dx?
Sturge-Weber's disease
iris hamartomas (Lisch nodules)

dx?
nf-1
bilateral acoustic neuromas

dx?
nf-2