Myelodysplasia Flashcards Preview

Clinical Medicine (Fall 2016) > Myelodysplasia > Flashcards

Flashcards in Myelodysplasia Deck (100)
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1
Q

what is myelodyspasia

A

defective development of any part of the spinal cord

2
Q

what part of the spinal cord is most affected by myelodyspasia

A

lower segments (thoracic into lumbar)

3
Q

mechanism of myelodyspasia

A

congenital tube defect (NTD)

4
Q

for myelodyspasia, what is the degree of resulting dysfunction related to

A

anatomic level of the defect

5
Q

what accompanies myelodyspasia

A

loss of neurologic function (sensory and motor) below the level of the lesion

6
Q

what does multifactorial mean

A

possible environmental and genetic factors affecting

7
Q

what deficiency is present in myelodyspasia patients

A

folic acid

8
Q

what type of SES is present in myelodyspasia

A

lower SES

9
Q

what type of agents are present with myelodyspasia patients

A

teratogenic

10
Q

what vitamin deficiency is present in myelodyspasia patients

A

Vitamin A

11
Q

what factor is present in myelodyspasia patients

A

Rh factor

12
Q

what might be ingested in the womb of myelodyspasia patients

A

alcohol

13
Q

what is the genetic link of myelodyspasia patients who are prgenant

A

parents with one child with myelodyspasia have 50x higher chance of having another sibling

14
Q

what is the normal process of development from the embryo

A

embryo (20 days after conception)

–>

neural groove

–>

neural crest

–>

closure of neural tube

15
Q

when does the closure occur for the embryo

A

day 23

16
Q

typically, the embryo is completely closed except…

A

the hole at the top and hole at the bottom

17
Q

what does the hole at the top represent in the embryo

A

brain

18
Q

what does the hole at the bottom represent in the embryo

A

spinal cord

19
Q

where else might there be errors in development

A

vertebral architecture

20
Q

incidence of neural tube defects

A

1-2 in 1000

21
Q

are males or females more affected by neural tube defects

A

females

22
Q

what are two methods of pre-natal detection of neural tube defects

A
  1. ultrasonic scanning

2. serum AFP testing

23
Q

what birthing process should be planned for children with neural tube defects

A

c-section

24
Q

why is a c-section common in neural tube defect patients

A

to avoid trauma to the neural sac

25
Q

what is a method of post-natal diagnosis for neural tube defects

A

observation

26
Q

what are the main types of myelodyspasia

A

occulta and aperta

27
Q

what does occulta mean

A

hidden, not visible

28
Q

mechanism of occulta

A

failure of one or more vertebral arches to meet and fuse in 3rd month of development

29
Q

are spinal cord and meninges harmed in occulta

A

no.. they remain in the vertebral canal unharmed

30
Q

what happens to the bony defect and skin in occulta

A

it is covered by skin

31
Q

where is occulta most common

A

lumbosacral

32
Q

any neurologic or musculoskeletal issues in occulta

A

no.. both systems are fine

33
Q

what may be present in occulta patients (4)

A
  1. depression/dimple
  2. cafe au lait spot
  3. soft fatty deposit
  4. tuft of hair
34
Q

another word for aperta

A

cystica

35
Q

mechanism of aperta

A

neural tube and vertebral arches fail to close appropriately

36
Q

what is present in aperta

A

cystic protrusion of material through defective arches

37
Q

two types of aperta

A
  1. meningocele

2. myelomeningocele

38
Q

what happens to the meninges and CSF in meningocele

A

protrusion of meninges and CSF into cystic sac

39
Q

what happens to the spinal cord in meningocele

A

spinal cord remains within the vertebral column

40
Q

can abnormalities be present in meningocele

A

yes but less common

41
Q

mechanism of myelomeningocele

A

protrusion of both spinal cord and meninges into cystic sac

42
Q

generic types of myelomeningocele

A

open and closed

43
Q

how can closed myelomeningocele be described

A

covered with a combination of skin and membranes

44
Q

where is closed myelomeningocele most common

A

thoracic and lumbosacral areas

45
Q

what is an open myelomeningocele

A

nerve roots and spinal cord may be exposed with dura and skin at edge of lesion

46
Q

where does 2/3 of open myelomeningocele occur

A

thoracolumbar junction

47
Q

what imaging techniques can be used for myelomeningocele

A

MRI and CT

48
Q

three other types of myelodysplasia

A
  1. lipoma
  2. myelocystocele
  3. anencephaly
49
Q

what is lipoma

A

fatty tumor on spinal cord

50
Q

what is myelocystocele

A

cystic like tumor of spinal cord

51
Q

what is anencephaly

A

failure of closure of cranial end of neural tube

52
Q

what may be evident and absent in anencephaly

A

some brain tissue evident, forebrain usually absent

53
Q

does occulta usually cause neurologic dysfcuntion

A

no, usually no neurologic symptoms

54
Q

what are symptoms for occulta

A
  • occasional disturbances in bowel and bladder function

- foot weakness

55
Q

where is occulta most common

A

lumbosacral area

56
Q

what is motor function like in myelomeningocele

A

myotomal loss

57
Q

what is sensory function like in myelomeningocele

A

dermatomal loss

58
Q

what type of deformity may be present in myelomeningocele

A

skeletal deformity

59
Q

what do 90% of myelomeningocele patients develop

A

hyrdocephalus

60
Q

do alot of myelomeningocele patients have MR

A

no, low percentage

61
Q

do alot of myelomeningocele have a learning disability

A

yes, high percentage

62
Q

motor function of myelomeningocele patients

A

depends on level of lesion

63
Q

what type of patients do myelomeningocele patients have similar symptoms as

A

spinal cord injury

64
Q

when does post-natal closure of myelodysplasia have to occur

A

within 72 hours

65
Q

what is the surgical procedure of closure of myelodysplasia patients

A

place neural tissue into vertebral canal

66
Q

what is the goal of the thecal sac in surgical repair of myelodysplasia patients

A

achieve a flat, watertight closure of thecal sac

67
Q

percentage of myelodysplasia of recover after surgery and do not have further medical complications

A

10%

68
Q

what do 90% of myelodysplasia patients after surgery develop

A

hydrocephalus

69
Q

what else can myelodysplasia develop after surgery

A

Tethered cord and symptoms of Arnold Chiari

70
Q

what is hydrocephalus

A

abnormal accumulation of CSF in cranial vault

71
Q

what is hydrocephalus caused by (3)

A
  1. overproduction of CSF
  2. failure of absorption of CSF
  3. obstruction in normal flow of CSF through brain and spinal cord
72
Q

what does accumulation of CSF (hydrocephalus) cause

A

increased ICP

73
Q

what can hydrocephalus lead to

A

cerebral damage and cellular death

74
Q

what may be present with someone with hydropcephalus regarding their head

A

full, bulging, tense soft spot (fontanel) on top of the head

75
Q

what may be noted on the scalp of someone with hydropcephalus

A

large prominent veins on the scalp

76
Q

what sign might be present in someone with hydropcephalus

A

setting sun sign

77
Q

what is the setting sun sign

A
  • child appears to only look down

- the whites of the eyes are obvious above the colored portion of the eyes

78
Q

what changes may be noted in someone with hydropcephalus

A

behavioral changes (irritability, lethargy)

79
Q

what type of cry might be noted with someone with hydrocephalus

A

high pitched cry

80
Q

what medical condition might be noted in someone with hydropcephalus

A

seizures

81
Q

what may be noted in appetite regarding someone with hydrocephalus

A

vomiting or change in appetite

82
Q

what can be done medically done for someone with hydrocephalus

A

ventriculoperitoneal shunt

83
Q

what is a ventriculoperitoneal shunt

A

thin tube with a small pump attached which diverts CSF from lateral ventricles to a location where the fluid can be managed

84
Q

what is the most common type of shunt

A

ventriculoperitoneal

85
Q

what is an alternate to ventriculoperitoneal shunt

A

ventriculo-atrial shunt

86
Q

QUIZ MATERIAL ENDS HERE

A

….

87
Q

for shunt malfunction, what may the fontanels feel like

A

firm fontanels

88
Q

what is an arnold-chiari malformation

A

defect in formation of the brainstem

89
Q

mechanism of arnold-chiari malformation

A

hindbrain is displaced through the foramen magnum

90
Q

frequency of arnold-chairi malformation contributes to…

A

development of hydrocephalus

91
Q

arnold-chiari: ___ dysfunction and ____ involvement

A

hindbrain dysfunction and cranial nerve involvement

92
Q

for arnold-chiari malformations, how is feeding

A

difficult

93
Q

for arnold-chiari malformations, there is pooling of…

A

secretions

94
Q

for arnold-chiari malformations, there is ____ breathing

A

stridor

95
Q

what is stridor breathing

A

heavy breathing

96
Q

is there scoliosis for tethered cord?

A

yes

97
Q

what happens to spasticity for tethered cord

A

increased

98
Q

describe the postures and movements for tethered cord

A

asymetrical

99
Q

is the gait pattern altered for tethered cord

A

yup

100
Q

where is there pain present for tethered cord

A

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