Myelodysplasia (Spina Bifidia)

Question 1

Development of NS
- By 18 days of gestation

Answer 1

Cells of the neural plate differentiate to create the neural tube and neural crest

Question 2

Development of NS
- Neural Crest

Answer 2

Neural crest becomes the peripheral nervous systems (cranial and spinal nerves, autonomic nerves and ganglia)

Question 3

Development of NS
- Neural Tube

Answer 3

Neural tube becomes CNS: brain and spinal cord

Question 4

Development of NS
- 24th day of gestination

Answer 4

Cranial end of neural tube closes

Question 5

Development of NS
- If cranial end of neural tube does not close

Answer 5

Results in anencephaly a fatal condition

Question 6

Development of NS
- 26th day of gestination

Answer 6

Caudal end closes

Question 7

Development of NS
- If caudal end does not close

Answer 7

Failure to close at any point along the caudal border causes spina bifidia

Question 8

Development of NS
- Absence of motor and sensory function

Answer 8

Occurs below the level of defect

Question 9

Development of NS
- Level of defect

Answer 9

Loss of neural control of bowel and bladder
Functional deficits may be partial/complete but almost always permanent

Question 10

Determines the neuromuscular dysfunction

Answer 10

Type of neural tube defect

Question 11

Other names of neural tube defect

Answer 11

Spina bifida
Spina bifida aperta
Spinal dysraphism
Myelomeningocele
Meningomyelocele

Question 12

Etiology

Answer 12

Genetic predisposiiton
Env’t influences
Lower maternal folic acid levels
Maternal use of valproic acid (anticonvulsant)
Use of antidepresasnts
Highest accuracy in Irish and Celtic heritage

Question 13

Medical Diagnosis

Answer 13

Prenatal screening
Ultrasound
Amniocentesis

Question 14

Medical Diagnosis
- Prenatal screening

Answer 14

Maternal serum alpha-fetoprotein (AFP) levels

Question 15

Medical Diagnosis
- Prenatal screening
–> Alpha fetoprotein levels

Answer 15

AFP created by a developing baby
Lower levels indicate neural tube defects
Testing done between 15-20 wks of pregnancy
Depends on the trimester and the number of babies

Question 16

Common complications

Answer 16

Hydrocephalus
Arnold-Chiari Malformation II
Tethered Cord

Question 17

Hydrocephalus

Answer 17

Abnormal accumulation of CSF in the cranial vault

Question 18

Arnold-Chiari Malformation II

Answer 18

Deformity of cerebellum, medulla, cervical spinal cord
Posterior cerebellum and brainstem herniate caudally through foramen magnum and obstruct CSF released in the 4th ventricle

Question 19

Tethered Cord

Answer 19

As the child grows, the spinal cord gets tethered to the previous scar tissue, resulting in loss of motor/sensory function

Question 20

Surgical Managment

Answer 20

Fetoscopic surgery to close the neural tube defect in utero
Surgical closure of the site within 72 hours of birth
VP shunt to manage hydrocephalus
Subsequent surgery to manage tethered cord
Orthopedic surgeries to correct deformities

Question 21

Impairments

Answer 21

Sensory and motor paralysis
Msk deformities
Osteoporosis
Hydrocephalus
Cognitive dysfxn
Language dysfxn
Latex allergy
Cranial nerve palsies (auditory/vestibular, glossopharyngeal and vagus)
Spasticity
Seizures
Neurogenic bowel and bladder
Skin breakdown (pressure ulcers)
Obesity

Question 22

PT examination

Answer 22

Developmental testing
Muscle tone
ROM
Muscle extensibility
Joint alignment
Deformities
Sensation
Posture and gait

Question 23

PT examination Pt. 2

Answer 23

Scoliosis (congenital or acquired)
Muscle strength
Endurance
Mobility skills
ADLs
Equipment needs
Env’t accessibility

Question 24

Principles of PT Management

Answer 24

Maintain available motor and sensory fxn
Identify signs of worsening condition
Prevention of complications
Use available fxn to perform ADLs and improve participation
Orthotic prescription
Adaptive equipment
Pre and post op care in case of corrective orthopedic surgeries

Question 25

Principles of PT Management
- Prevention of complications

Answer 25

Pressure sores
Tightness/contractures/deformities

Question 26

Orthotic and Equipment Management

Answer 26

Foot and supramalleolar orthoses
AFO
KAFO
HKAFO
THKAFO
Parapodiums
Manual wheelchair
RGOs
Walkers
Crutches