congenital

Question 1

what are some characteristics of Tubersclerosis?

Answer 1

mental retardation
seizures
sebaceous adenoma of malar rash on trunk and extremities
areas of hypomelatonin on body

Question 2

What does a CT of a Tubersclerosis patient reveal?

Answer 2

white small spots in cortex in subependymal area of lateral ventricle (tubers)
can have tubers in Lateral ventricle and ventriculomegaly

Question 3

What genes are mutated in tuberousclerosis?

Answer 3

harmartin and tuberin

Question 4

what is the onset of cerebral palsy?

Answer 4

in utero to 2 years

Question 5

What are some characteristics of cerebral palsy?

Answer 5

seizure (1/2 pt)
mental retardation (2/3 pt)
MC: muscle weakness spasticity 80% of cases
dyskinesia 20% of cases
failure to meet motor milestones
periventricular leucomalacia around lateral ventricles

Question 6

What are some complications of cerebral palsy?

Answer 6

osteopenia, osteomalacia, chronic MS pain

hip dislocate, difficult to eat and breathe

Question 7

As an adult a patient with cerebral palsy is more likely to have..

Answer 7

ceberbrovascular problems
cardiac problems
GI issues

Question 8

early fusion of bilateral coronal suture in a baby results in…

Answer 8

brachycephaly (short skull)

Question 9

Early unilateral fusion of coronal suture in a baby results in…

Answer 9

frontal plagiocephaly

Question 10

early lamboid fusion in a baby results in…

Answer 10

occipital plagiocephaly

Question 11

Early saggital suture fusion in a baby results in..

Answer 11

schizencephaly (boat skull)

Question 12

early metopic suture closure in a baby results in..

Answer 12

trigonocephaly, triangle skull

Question 13

when do the membranes for the cranium form?

Answer 13

2nd month…they then progress into bones by the 5th month

Question 14

what is the etiology of craniosynotosis?

Answer 14

primary due to defect in skull development

secondary due to altered brain growth or environment(positional plagiocephaly)

Question 15

What is the treatment for craniosynotosis?

Answer 15

surgery to decreased intracranial pressure

manipulation

Question 16

What are some causes of hydrocephalus in an infant?

Answer 16

dandy walker
obstruction of cerebral aqueduct
cerebral palsy

Question 17

How does hydrocephalus present in an infant?

Answer 17

enlarged head not other signs related to the hydrocephalus

Question 18

How does hydrocephalus present in a young child?

Answer 18

brisk DTR
spasticity
clonus

Question 19

how does hydrocephalus present in an older child?

Answer 19

irritability, headache, nausea, vomit, poor appetite

lethargy

Question 20

What are some complications that can occur with hydrocephalus?

Answer 20

developmental delay
memory problems
visual problems
behavior issues

Question 21

What is the etiology of Sturge-Weber?

Answer 21

rare neurocutaneous disorder when vascular supply forming too much supply to leptomeninges and not enough to the brain

Question 22

What are the consequences of Sturge Weber vascular deformities?

Answer 22

glaucoma in I/L eye
reddish stain around eye and V1 territory
seizure in 1st year
mental retardation (50% of patients)
learning disabilities (50% patients)
*brain atrophy and calcifications due to undervascularization

Question 23

what is the treatment of cerebral palsy?

Answer 23

anticonvulants
antispasmotic-botulinum toxin
rhizotomies for spastivity

Question 24

What is the etiology of cerebral palsy?

Answer 24

many possible
preterm birth
maternal or infant infection
(thyroid)

Question 25

What is the presentation of Chiari malformation Type I

Answer 25

STA
Syrinx in 14% of Chiari type I
Tonsil hernation
Adult onset (adolesent)
neck pain, headache
urinary frequency and apneic episodes as infants
progressive cerebellar ataxia and LE spasticity

Question 26

90% of syringomylia is cause by?

Answer 26

Type I chiari malformation

Question 27

What is the presentaion of Chiari malformation type II?

Answer 27

early in life onset (vermis herniates)
syncopal episodes, nystagmus
abducens palsy B/L
myleomeningiocele and hydrocephalus 
syrinx possible

Question 28

What is the etiology of Chiari type II

Answer 28

small posterior cranial fossa MC can be others

misshapen midline cerebellum (vermis herniates)

Question 29

What is a possible complication of Chiari?

Answer 29

compression of medulla-urinary frequency, apneic episode

Question 30

where do tonsils or vermis herniate through in Chiari malformations?

Answer 30

foramen magnum

Question 31

What is the etiology of Dandy walker?

Answer 31

dysraphism of metencephalon (pons and cerebellum)

causes: enlarged posterior cranial fossa, loss of vermis and lack of growth of cerebellum, large 4th ventricle

Question 32

what percentage of patients with Dandy walker have hydrocephalus?

Answer 32

1-4%

Question 33

what are some symptoms that present with Dandy Walker?

Answer 33

ventriculomegaly
child presentation
movement disorder
mental retardation

Question 34

what present on imaging for hydrocephalus ex vacuo?

Answer 34

1. low density area in cerebrum (spongy or no matter)
2. large ventricles
3. large posterior horn

Question 35

What is the etiology of noncommunicating hydrocephalus?

Answer 35

block cerebral aqueduct, can see cerebral aqueduct expand or see obstruction on imaging

Question 36

what is the presentation of noncommunicating hydrocephalus?

Answer 36

1. unremitting headache
2. deep sulci
3. expansion of CA

Question 37

Where is most CSF produced?

Answer 37

choracoid plexus in body of lateral ventricle 60%

surface of 3rd ventricle and lateral ventricle and surface of brain =40%