Anterior fontanelle closes at how many months?
1-3 years!
Posterior fontanelle closes at how many months?
2-3 months
gag reflex
CN IX and X
Reflex for kid to extend back up when dropped
Moro reflex
brainstem
Run finger along spine and curve twds that or opposite (?) side is normal
Incurvation
kids gait is wide and and unsteady - closes
at age 6
inability to make vluntary smooth accurate movements
Ataxia
Cerebellum
slow and stiffness of volunt movement
bradykinesia
continuous involuntary random movements- occur at rest, dancing
chorea
abnormal muscle contractions that lead to twisting, jerking, spasms, or stiffening at rest or during movements
dystonia
very rapid and brief jerks
myoclonus
increase in muscle stiffness that worsens with rapid movement and may be ass’d with increased reflexes and weakness
(cerebral palsy)
spasticity
repetitive, stereotypical involuntary movements or sounds (may appear purposeful)
Tics
rhythmics involuntary back and forth sharking at rest or with movement
tremors
Tx for status epilepticus
Benzos (valium/ativan)
ABCs (what are those??)
Infantile spams is a seizure seen in
West Syndrome
Abnormal EEG in West SYndrome/Infantile spasms is called
Hypsarrhythmia
West Syndrome is characterized by
Hypsarrhythmia (chaotic brain waves in EEG) and infantile spasms
Onset between 4 and 8 mo
Infantile spasm
Sudden bending forward is a sign of
Infantile spasm.West syndrome
Occur just before sleep or upon awakening
Infantile spasm
To tx infantile spasm
ACHT, vigabatrin, KETOGENIC diet
Poor prognosis
most frequent permanently disabling defect
Spina bifida
prevented by Folic acid
spina bifida
what disorder has high incidence of LATEX allergy?
spina bifida
meningoMYEcele vx meningocele
mMyeCele is most severe: roots expose, CFS leakage, immediate surgical closure, paralysis, CHIARI malformations
meningocele: rare, only meninges exposed, surgical repair, better
4th ventricle enlarged hydrocephalus absent cerebral vermis slow motor devt, balance 50% normal IQ
Dandy walker
slowly evolving accum of CSF weeks to months
obstructed (ventricle is blocked) vs overproduction
ICP increase intracranial pressure
bulging anterior fontanelle
Tx; dueretics (temp), surgery, shunt drains fluid
Hydrocephalus
Gower’s sign
use hands to push up from floor to standing
in duchene and becker mucular dystrophy
sex linked recessive
around 3 yo
Dx: muscle biopsy m fiber and ST degeneration
absence of dystrophin Ig protein
high CPK serum level
wheelchair by age 10, dead by 20duchene muscular distrophy
duchenne tx
steroids, Ig ABs
emery dreyfuss musc distrphy
hip and shoulder
wheelchari by 30 yo
severe cardiomyopathy (CM) is in
Limb-Girdle musc dystrophy
onset 5-15 yo
Cafe au lait spots in
neurofibromatosis
Malignant peripheral nerve sheath tumors (15%)
Essential HTN
Usu not malignant but occupy space
Neurofibromatosis
inflammatory peripheral neuropathy
cause: post infection
Brds: CAMPYLOBACTER JEJUNI , Cmv, EBV,
rapid loss of motor strength - ascending, bilateral,
GUILLAIN BARRE SYDROME (acute idiopathis polyneuritis(
ABs form against Ach NICOTINIC RECEPTORS at neuromuscular jxns
(skel m and cardiac m)
Thymus involved?
Ptosis, diplopia, weakness of face, sx worse with exertion
NO MUSCLE PAIN, just weak
MYASTHENIA GRAVIS
progressive weakness, cant sit, cant stand, to facial weakness, death due to respiratory dysfx/infection
Spinal Muscular Atrophy Syndrome
Muscular distrophy
Duchenne and Becker
Which muscular dystrophy is less severe
Becker
