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Neuromuscular Pathology (Midterm) > Congenital Spinal Cord Injury > Flashcards

Congenital Spinal Cord Injury Flashcards

Spina Bifida Chiari II Malformation

1
Q

2 main categories of spinal cord injury:

A
  1. Congenital SCI
  2. Traumatic SCI
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2
Q

Where does congenital SCI occurs?

A

in utero

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3
Q

Defect in neural tube closure & overlying posterior vertebral arches

A

Spina Bifida

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4
Q

Two types of spina bifida:

A
  1. Spina bifida occulta
  2. Spina bifida cystica
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5
Q

In what type of congenital SCI there is a failure of one or more of the vertebral arches to fuse in the 3rd month of gestation, there no SC and meninges involved, and is covered by skin with a patch of hair or dimple ?

A

Spina Bifida Oculta

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6
Q

Are there usually any resultant neurological or musculoskeletal impairments seen in pt with spina bifida oculta?

A

no

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7
Q

Cystic protrusions of meninges alone or both meninges & spinal cord through defective vertebral arches

A

Spina Bifida Cystica

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8
Q

What are the types of spina bifida cystica?

Which is the most common type?

A
  1. Meningocele
  2. Myelocele (myelocystocele)
  3. Lipomeningocele
  4. Myelomeningocele (most common)
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9
Q
A

Meningocele

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10
Q
A

myelomeningocele

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11
Q
A
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12
Q
A

spina bifida oculta

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13
Q

Which is the milder form of spina bifida?

A

meningocele

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14
Q

Neural tube closes normally, but central canal of spinal cord distends & produces large, skin covered cyst:

A

myelocele (myelocystocele)

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15
Q

Protrusion of meninges & CSF into cystic sac, SC remains within vertebral canal, and is relatively uncommon form of spina bifida

A

meningocele

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16
Q
  • Rare form of spina bifida
  • Prompt medical intervention is required
  • Neural tube closes normally, but is distended due to cystic swelling
A

Myelocele (myelocystocele)

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17
Q

Due to vertebral defect associated with superficial fatty mass (lipoma) that merges with lower level of spinal cord

A

lipomeningocele

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18
Q

Most common & severe form of spina bifida cystica where both spinal cord & meninges protrude into cystic sac, and results in abnormal transmission of neural impulses

A

myelomeningocele (spina bifida)

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19
Q

Incidence of spina bifida has decreased worldwide since implementation of ____ ______ fortification programs

A

folic acid

20
Q

Detection of spina bifida via _______
has decreased the incidence of spina bifida

A

amniocentesis:

  • which a small amount of amniotic fluid, which contains fetal tissues, is sampled from the amniotic sac
  • Significant levels of alpha-fetoprotein in
    amniotic fluid aids detection
21
Q

etiology of spina bifida

A
  • cause is unknown
  • Enviromental and genetic factors
  • Folic acid and vitamin A deficiencies
22
Q

80% of neural tube defects can be detected
via:

A

maternal serum alpha-fetoprotein levels

23
Q

Clinical manifestation of spina bifida (mayelomeningocele):

A
  • Sensory: all types of sensory loss
  • MSK: weakness/paralysis, deformities
  • Neurological: hydrocephalus
  • Integumentary
24
Q

Early detection of spina bifida (mayelomeningocele) can afford possibility for:

A
  • newer in utero treatments of hydrocephalus
  • in utero surgical repair of myelomeningocele
25
Q

In spina bifida, how is the weakness/paralysis presented if SC is involved below level of lesion?

A
  • flaccid paralysis
  • absent reflexes
26
Q

In spina bifida, how is the weakness/paralysis presented if SC remains intact below level of lesion?

A
  • Flaccid paralysis immediately below lesion
  • Hyperactive reflexes more distally
27
Q

In spina bifida, sensory loss below level of lesion includes:

A

all types

  • Kinesthetic
  • Proprioceptive
  • Somatosensory
28
Q

Osteoporosis fx may occur in pt with spina bifida due to:

A

no stress on the bones

29
Q

Spina bifida orthopedic deformities

A
  • lumbar khyphosis
  • hip flexion, knee extension & ankle DF contractures
  • equinovarus (clubfoot)
  • hip flex/add/IR = hip dislocation
30
Q

What is hydrocephalus?

A

blockage of normal flow of CSF between ventricles & spinal cord

31
Q

Hydrocephalus occurs occurs in approx. in how many cases of spina bifida?

A

80 – 90% of cases

32
Q

If hydrocephalus not treated:

A

brain damage and death

33
Q

What is Chiari II Malformation?

A
  • Congenital anomaly of hindbrain involving
    herniation of medulla into upper Cx canal
  • Children with myelomeningocele have a 99% chance of having this malformation
  • Occurs usually between C1 to C4
34
Q

What is Hydromyelia:

A
  • Dilation of center canal of spinal cord due to
    accumulation of CSF
35
Q

What is Tethered Cord?

A
  • Closely associated with spina bifida oculta
  • Pathological fixation of the spinal cord in an
    abnormal caudal location
36
Q

Presenting symptoms in tethered cord:

A
  • Decreased strength
  • LE spasticity
  • Back pain at site of sac closure
  • Scoliosis development
37
Q

Common symptoms of hydromyelia:

A
  • Rapidly progressive scoliosis (from mm imbalance)
  • UE weakness
  • Spasticity
  • Ascending motor loss in LE’s
38
Q

Symptoms of Chiari II Malformation:

A
  • May be symptomatic or asymptomatic
  • Symptomatic: see respiratory & bulbar function impairments, sometimes UE weakness
39
Q

how is tethered cord treated?

A

cord release

40
Q

Neurological impairments in myelomeningocele (spina bifida)

A
  • Bowel & bladder dysfunction
  • Cognitive impairment & Learning issues:
    • impaired intellectual function
    • Moderate – severe visual-motor perceptual
      deficits
41
Q

Integumentary impairments in myelomeningocele:

A
  • Latex allergy
  • Risks of pressure sores
42
Q

Medical mgmt of myelomeningocele (spina bifida):

A
  • surgical sac closure 24-48 h after birth
  • in utero surgery
  • hydrocephalus mgmt:
    • ventricular shunt placed
43
Q

what is the main casue of death for myelomeningocele

A
  • Increased intracranial pressure
  • infections of CNS
44
Q

Survival rate for myelomeningocele is

45
Q

Multiple episodes of P.T needed throughout
the lifespan in pt living with spina bifida.

What are the pediatric and adult goals?

A
  • Pediatric Goals:
    • Achievement of developmental motor milestones
    • Enhance independent mobility and transfers
    • Maximize stability to facilitate play, social
      development, learning, language
  • Adolescent/Adult Goals:
    • Address changing needs as growth
      continues/aging occurs
    • Post-surgical rehab needs

Neuromuscular Pathology (Midterm) (9 decks)

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