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Neurodegenerative/Demyelinating/Other CNS > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Max acceptable bladder pressure

A

40 cm H20

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2
Q

When to do surgery for scoli in spina bifida

A

50 degrees

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3
Q

Meningomyelocele

A

herniation of meninges and neural elements – most common
Associated with hydrocephalus and Chiari type 2
75% lumbosacral

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4
Q

Nerve we can feel related to bladder

A

Somatic nerves via pudendal, S2-S4 innervate the skeletal muscle component of the external urethral sphincter, contract during fill, relax during emptying.

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5
Q

Worst outcome of Chiari type II

A

Respiratory compromise
Impaired hand function
Lower cranial nerve function

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6
Q

Closed forms of Neural Tube Defects

A 
encephalocele
meningocele
iniencephaly
lipomeningocele
sacral genesis
occult spinal dysraphism
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7
Q

What level in spina bifida have most hip disloc

A

L3 based on muscle imbalance of hip flex and adduction present, other opposing do not

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8
Q

Environmental occupational increase in NTD

A

painters, agriculture, industrial process workers, healthcare, transportation (solvents)

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9
Q

How much does 4 mg/day help

A

Reduces risk by 70%

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10
Q

Folic acid supplementation

A

All women childbearing: 400 micrograms/day
Women w spina bifida or prior NTD preg 4 mg (day 1 to 3 months)
High risk pregame’s (valproic acid or diabetes) 4 mg/day

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11
Q

What is spina bifida occulta

A

bony defect with no herniation of meninges or nerves

CAN have tethered cord

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12
Q

Effect of folic acid supplementation in grains in USA

A

4,000 to 3,000 NTD per year

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13
Q

How many kids with Spina Bifida has tethered cord

A

Almost all (not all symptomatic)

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14
Q

Calcium supplement Spina bifida

A

500 mg age 1-3
800 mg 3-8
1,300 mg after

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15
Q

What does folate do

A

cofactor for the enzymatic process involved in purine and pyrimidine synthesis and facilitates the transfer of methyl groups during the metabolism of methionine and homocysteine

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16
Q

Are L4 and L5 spina bifida levels ambulators

A

Yes, by 4 years

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17
Q

CNS malformation associated with NTDs

A

Spinal Cord tethering
Arnold -Chiari malformations
Hydrocephalus
Forebrain abnormalities (that cause decreased cognition)

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18
Q

MTHFR

A

increased risk level 7.2 times

19
Q

Med most associated with NTD

A

Valproic acid

20
Q

Signs of hydrocephalus

A 
headache
vomiting
drowsiness
change in behavior
diplopia
papilledema
21
Q

Signs of tethered cord in Spina Bifida

A 
Increased lower extremity spasticity
Decline in lower extremity strength
Worsening scoliosis
Back pain
Worsened urologic function
22
Q

Kind of foot joint to watch for in spina bifida

A

Charcot joints

23
Q

Elevated homocysteine levels

A

known risk factor

24
Q

Effect of personal intake of folic acid in preg

A

50% decrease NTD

25
Q

Spinal level of parasympathetic innervation of bladder

A

S2 to S4, innervates the funds of the bladder and releases acetylcholine which causes emptying

26
Q

Other test for spinabifida

A

Amnio

Fetal MRI

27
Q

How common is Syrinx in Spina Bifida

28
Q

Meningocele

A

Bony defect with herniation of meninges

29
Q

Most important muscles in Spina bifida for gait

A

Hip abductors

30
Q

Goal of feet in spina bifida

A

Plantar-grade feet

31
Q

What is the classic lemon and banana sign on Ultrasound

A

How spina bifida looks spina bifida, splaying of pedicles plus shape of head and banana shaped foramen magnum

32
Q

Age of sacral level spina bifida ambulator

33
Q

Scoliosis risk in lumbosacral spinal bifida

34
Q

Other things to watch out for with Chiari type II malformations

A 
Worsened function to the point of tetraplegia
Ocular motility (saccadic eye, fix, and pursuit)
35
Q

Nonverbal learning disorders NVLD are common in spina bifida

A

Difficulty with
Motor
Visual-spatial
Social

36
Q

Signs of syrinx in spina bifida

A

Worsening function above level of lesion

37
Q

Scoliosis risk in Thoracic Spina Bifida

A

80 to 100%

38
Q

Prenatal screening

A

Quad screening:

  1. Alpha feta-protein (AFP)
  2. human chorionic gonadotropin (HCG)
  3. estriol
  4. inhibin A
39
Q

Genes involved in NTD

A

Sonic Hedgehog
PTC gene
SCRIB
PRICKLE

40
Q

Open forms of Neural tube defects

A

anencephaly

myelomeningocele

41
Q

Bladder capacity

A

Sum of age + 2 in oz
Babies is weight in kg x 7
Teans, 400-500

42
Q

Chiari Type II Malformation

A 
Very common (80-90%)
Medulla, pons, elongated 4th ventricle, cerebellar vermis into the cervical spine.  
Almost always hydrocephalus
43
Q

Spinal level of sympathetic innervation of bladder

A

T10-L1
The sympathetic control causes the detrusor to relax and bladder neck and posterior urethra to contract, effectively allowing storage of urine

44
Q

Which population has ongoing higher spina bifida

Neurodegenerative/Demyelinating/Other CNS (2 decks)

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