Neurology Flashcards
Recovery time for facial nerve palsy
2-3 weeks
Tearing, photophobia, enlarged globe
Glaucoma
Also have corneal edema and clouding causing dull red reflex and dampened pupillary reflex
Sturge Weber eye association
Congenital glaucoma in 30-70%
Syndromes associated with glaucoma
NF, Retinoblastoma, homocystinuria, trisomy 21, congenital rubella (also cataracts), Stickler syndrome, Sturge Weber syndrome, corticosteroids
CMV eye association
Chorioretinitis
Toxoplasmosis eye association
Chorioretinitis
MC outcome in infant with Cystic PVL
Spastic diplegia (Damages deeper, more medial fibers that control lower extremity function)
Lack of babbling by which age is concern
11 months
Echolalia begins
Between 15-18 months
Repetition of sounds
First line treatment for moderate or severe hearing loss
Amplification combined with intensive speech and language
Normal EEG of extremely preterm infant
Discontinuous
When does EEG become continuous?
31-33 weeks
Hypotonia, severe mental deficiency, ataxia
Angelman syndrome
Maternal deletion
Hypotonia, weakness, contractors, absent DTRs, tongue fasiculations
Spinal muscular atrophy or Werdnig Hoffman disease (degeneration of anterior horn cell)
First manifestation of NF-1
Cafe au lait macules (smooth borders)
When are pupils reactive to light
30-32 weeks
What age is optic nerve myelination complete?
24 months
MC etiology of congenital hearing loss
Genetic (MC genetic cause is connexin 26 gene mutation)
Abnormal hearing screen threshold
35 db
Palmar grasp reflex appears and disappears. Persistence signifies:
Appears 28 weeks
Established by 32 weeks
Disappears by 2-4 months
Persistence - athetoid CP
Moro reflex appears and disappears
Appears 28-32 weeks
Established by 37 weeks
Disappears by 6 months
Crossed extensor reflex appears and disappears
Leg extended and sole rubbed → opposite leg will first have flexion and then extension with fanning of toes
Appears 26 weeks
Established by 34 weeks
Disappears by 2 months
Rooting reflex appears and disappears
Appears 30 weeks
Established by 34 weeks
Disappears by 4 months
Tonic reflex appears and disappears. Persistence means?
Appears 35 weeks
Established by 1-2 months
Disappears by 6 months
Persistence: Focal cerebral abnormalities
Only medication to decrease IVH
ACS (24-48h before delivery)
Also maternal transfer before imminent preterm birth
Prader Willi genetic cause
Paternal deletion in chromosome 15q11-13
Both copies come from mother (maternal uniparental disomy)
Angelman genetic cause
Maternal deletion in chromosome 15q11-13
Both copies come from father (MANs copies) (paternal uniparental disomy)
Myotonic dystrophy cause
Trinucleotide repeat
Effects of Kernicterus
TEAM Teeth - dental enamel dysplasia Eye abnormalities (paralysis of upward gaze) Auditory dysfunction Movement dysfunction - Athetoid CP
Leading cause of vision impairment in preterm infants
ROP
Threshold disease
ROP in Zones I or II in at least 5 contiguous or 8 total clock hours + plus disease
50% progress to stage 5
Treatment for threshold disease
Laser within 2-3 days
Most common area involved in ROP
Zone III
Where is Zone I of eye
Most posterior part of retina
Vessels closest to optic disc
Most immature zone
ROP Stage “Distinct demarcation Line”
Stage 1
ROP Stage “demarcation line is thickened and elevated, ridge”
Stage 2
ROP Stage “ridge with extra retinal neovascularization
Stage 3
ROP Stage with partial retinal detachment
Stage 4
ROP Stage with complete retinal detachment
Stage 5
What refractive errors do premature infants get?
Hyperopia and myopia
Myopia strongly associated with hx of significant ROP
Up vs down slanting palpebral fissures
Line away from nose
Up in Downs
Enlargement of the 4th ventricle, complete or partial absence of the cerebellar vermis, cysts
Dandy Walker syndrome
Can clonus be a normal finding in newborns?
Yes, up to 3 months of age
Area of brain injury in HIE
White matter
EEG pattern in HIE
depression or burst suppression
Acquired maternal antibodies that block Ach receptors at the NMJ
Transient neonatal MG
Measures brainwaves generated in response to an auditory stimulus
ABR
Measures the acoustic feedback from the cochlea in response to an auditory stimulus
OAE
Which test takes longer? ABR or OAE?
ABR
Which test may be unable to detect some forms of sensorineural hearing loss?
OAE
ELBW population has disproportionate risk of which disability?
Non-verbal, math related
MC cranial nerve to be injured at birth
Facial nerve
Weakness of upper and lower facial muscles
Facial nerve palsy (L more likely)
Ocular dysplasia, hydrocephalus, cerebral malformations
Walker Warburg syndrome
Nerves for Erb Duchenne vs Klumpke’s palsy
C5-7 vs C8-T1
Conductive hearing loss on bone and air conduction
Good bone conduction, poor air conduction
Sensorineural hearing loss on bone and air conduction
poor air and bone conduction
Are NTD preventable by folic acid?
Most but 1/3 are not preventable d/t other factors such as diabetes, inc BMI, antiepileptics (Valproic acid), Trisomy 13 and 18
Hypomyeliniation with PVL is associated with which stage of oligodendrocyte?
Pre-oligodendrocyte (90% of oligodendrocyte population at 28 weeks)
Space that subgaleal occurs
Subaponeurotic space
Bleeding of emissary veins between scalp and dural sinuses
Space that cephalohematoma occurs
Subperiosteum
Benefit of therapeutic hypothermia
25% reduction in combined death or major NDI in moderate or severe HIE
Adverse effects of cooling
bradycardia, thrombocytopenia, subcutaneous fat necrosis
MC condition misdiagnosed as neonatal seizures
Benign sleep myoclonus
Why is aEEG less sensitive at picking up focal seizures?
Reduced number of channels
Use of a two channel aEEG with simultaneous raw EEG is more sensitive
MCC neonatal seizures
Global cerebral hypoxic ischemia ie HIE
Disability and mortality with newborn seizures
54% surviving children with disability
10% mortality (higher in HIE, cerebral dysgenesis and infection)
What causes inflammation in HIE?
Microglia (macrophages of the brain) release cytokines, chemokine and proteases
Monocytes and neutrophils infiltrate the injured brain
Initial treatment for spina bifida
Prone, sterile saline soaked dressing, plastic wrap (not bag)
Normal resting posture at 28 weeks
Limbs extended
Normal resting posture at 32 weeks
Slight flexion of the knee then hips
Normal resting posture at 36 weeks
Flexion of all 4 extremities
Structural or functional abnormality at an organ level
Impairment ie. damage to vision
Restriction at the personal level that results from an impairment
Disability ie. worsening eye sight
Disadvantage at the societal level that prevents fulfillment of the individual’s role
Handicap ie. inability to drive
Testing to get with ear tags or skin pits
Audiologic evaluation + Renal ultrasound (especially if family history, can be Goldenhar syndrome)
Sniffling in infant due to
Mucosal edema 2/2 estrogen exposure
Negatives of Phenobarbital for seizures
Only 1/3 patients respond to initial bolus
Increased neuronal apoptosis and impaired ND outcomes
How is hereditary retinoblastoma treated?
Chemotherapy
Hereditary vs nonhereditary retinoblastoma
Hereditary in 40% RB, AD; inactivation both alleles of RB1 gene; usually bilateral
Nonhered usually unilateral
When can fetus respond to sound?
20-25 weeks
MC lesion in neonates - supratentorial vs infratentorial
Supratentorial
MC presentation of congenital intracranial tumor:
Increasing head circumference with bulging fontanelle (50%)
