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37 Cards in this Set

  • Front
  • Back
Anencephaly
Failure of anterior neuropore development.
Brain not developed
Increased AFP
Dead
Spina Bifida
Occulta - mildest; vert fail to form around sc; No increased AFP; Asymptomatic

Meningocele - meninges through vert; High AFP

w/ meningomyelocele - mening and sc through vert defect; high afp

w/ meleschisis - most severe; sc seen externally; high afp
Arnold Chiari Malformation
Type 1- common, mostly asympto; downward displacement f cerebellar tonsils through foramen mag

Type 2- symptomatic; vermis and medulla through foramen mag; compression of 4th ventricle with obstructive hydrocephaly; frequent meningomyelocele; freq assoc with syringomyelia
Dandy Walker Malformation
failure of forameina luscka and magendie to open ->dilated 4th ventricle; agenesis of cerebellar vermis and slenium of corpus collosum
holoprosencephaly
with Trisomy 13 - Patau syndrome
incomplete sep of cereb hemispheres
one ventricle in telencephalon
Patau Syndrome
trisomy 13; holoprosencephaly; cleft palate; polydactyl; lowered ears; heart defect; neural devel defect
Horner Syndrome
decreased sympathetic activity
ipsilateral
partial ptosis, upside down ptosis; anhidrosis, miosis, enopthalmos, loss of ciliospinal reflex, bloodshot conjunctiva; possible flushing
craniopharyngioma
benign tumor of pituatary gland;
common in children
odontogenic epithelium w/ Ca deposits
aka adamantinoma, rathke pouch tumor
parkinsons
TARP - tremor, rigidity, akinesia, postural instability
lewy bodies with a-synuclein intracellular
loss of dopminergic neurons of sub nigra pars compacta - depigmentation
huntington
CAG repeat
caudate loses Ach and gaba
chorea, aggression, depressiondementia
neuronal death via nmda=r binding and glutamate toxicity; atrophy of straiatal nuclei
hemiballismus
sudden wild flailing of 1 arm +/- leg
contralateral subthalamic nucleus lesion -eg lacunar stroke; loss of inhibition of thalamus through globus pallidus
athetosis
slow, writhing movements, especially in fingers
like snake
basal ganglia lesion - eg huntington
dystonia
sustained involuntary muscle contraction
14 myoclonus
sudden brief muscle contract
Kluver bucy syndrome
bilateral amygdala lesion
hyperorality, hypersexuality, disinhibited behavior
assoc with HSV-1
Wernicke Korskoff Syndrome
mammillary body lesion
confusion, ophthalmoplegia, ataxia, memory loss, confabulations, personality change
can be due to low thiamine - eg alcoholism
central pontine myelenolysis
due to treatment for hyponatremia
acute paralysis, dysphagia, dysarthria
werdnig hoffman disease
infentile sma - lmn only
AR
smn1 gene
floppy baby syndrome
poliomyelitis
viral
motor neurons damaged - flaccid paralysis, muscle weakness
ALS
amyotropic lat scler
motor neuron disease
lou gherig
tabes dorsalis
assoc with neurosyphilis
dorsal column degeneration by macrophages
also with oral lesions due to macrophages of syphilis
friedreechs ataxia
AR
degener of nerves in sc
periph muscl weakenss, loss of coordination, dec vision and hearing, slurred speech, heart disorders
FXN gene
GAA repeat
brown sequard syndrome
ispilateral and below lesion- umn signs, loss of tactile, vibration, proprioception
contralateral and below lesion: pain and temp loss
Ipsi at level - all sensation
LMN signs at level
Bells Palsy
VII - facial nerve
idiopathic and usually unilateral
corticosteroid may help
hydrocephalus
communicating
noncommunicating
both
macular degeneration
dry
wet - neovascualr and exudative
not painful
gerstmann syndrome
lesion of inferior parietal, RL confustion, dyscalculia, finger anosia, contra L hemi/quadranopia
sheehan syndrome
post partam hemorrhagic infarct of pituatary -> no lactation
ademantinoma
craniopharyngioma
Picks disease
frontotemporal atrophy with dementia
dementia, aphasia, parkinsonian, pick bodies - Tau proteins;
spares parietal lobe and posterior 2/3 of superior temp gyrus
Gaucher disease
no glucocerebroside
lipid inclusion
krabbs disease
globoid cell leukodystrophy;
degenerative myeline
spastic paralysis
metachromatic leukodystrophy
arysulfate A deficiency
lys storage disease
flaccid paralysis
Nieman Pick disease
sphingomyelin accumulation
Tay-Sachs disease
ganglioside accumulation
Abnormal phosphorylation of Tau
flame shaped cells, neurofibrillary tangles
in Alzheimers
lewy body dementia
parkinsonism with dementia and hallucinations

a-synuclein defect