Spina Bifida, Syringomyelia, Cerebral Palsy (Exam 2)

Question 1

T/F: neural tube defects can occur anywhere along the neural tube

Answer 1

true

Question 2

What is the most common region for a neural tube defect?

Answer 2

distal aspect of the spinal cord

Question 3

What accounts for most CNS malformations?

Answer 3

neural tube defects

Question 4

What are the 4 types of neural tube defects?

Answer 4

1) anencephaly
2) myelomeningocele
3) meningocele
4) spina bifida occulta

Question 5

What is anencephaly?

Answer 5

-malformation of anterior end of neural tube
-failure of the rostral neuropore
-partial absence of brain and calvarium
-forebrain does not develop
-stillborn baby

Question 6

Generally speaking, what is spina bifida?

Answer 6

-neural tube does not close properly
-can occur anywhere in the spine
-vertebral structures do not form
-often results in damage to spinal cord and/or nerves
-may cause physical and intellectual disabilities

Question 7

What is a myelomeningocele?

Answer 7

-a fluid filled sac containing part of the spinal cord and nerves
-involves both the spinal cord and meninges
-causes moderate to severe disabilities

Question 8

What is the most serious type of spina bifida?

Answer 8

myelomingocele

Question 9

What is a meningocele?

Answer 9

-a fluid filled sac protrudes through an opening in the baby’s spine
-does not contain the cord in the sac
-can cause minor disabilities

Question 10

What is the mildest form of spina bifida?

Answer 10

spina bifida occulta

Question 11

What is spina bifida occulta?

Answer 11

-small gap at the posterior aspect of the vertebral arch
-often asymptomatic
-does not cause any disabilities
-often presents with a tuft of hair over the defect area

Question 12

What is syringomyelia?

Boards q

Answer 12

-development of a fluid filled cyst called a syrinx within the spinal cord
-the cyst is filled with CSF and can block CSF flow
-most commonly occurs in the cervical region, which affects the brachial plexus nerve roots
-the cyst may enlarge and damage the cord
-impeded CSF flow would increase intracranial pressure
-can be due to congenital defect (Arnold-Chiari Malformation), trauma, tumors, infections
-may be asymptomatic until patient hyperflexes or hyperextends
-destruction of the gray and white matter around the syrinx, specifically sensory fibers crossing the anterior spinal commissure to join the spinothalamic tract
-isolated loss of pain and temp sensation across the shoulders and upper extremities
-patient experiences a cape like or shawl like distribution where they do not feel pain and or temp in areas usually covered by a cape or shawl

Question 13

What is an Arnold-Chiari Malformation?

Answer 13

-brain tissue extends into the spinal canal
-can be asymptomatic, or symptoms can be triggered by a traumatic event like a car accident
-there are 3 types

Question 14

What are the 4 complications of Arnold-Chiari Malformation?

Answer 14

1) hydrocephalus
2) spina bifida
3) tethered cord syndrome
4) syringomyelia

Question 15

What is the most common type of Arnold-Chiari Malformation?

Answer 15

type 1

Question 16

What is the mildest form of Arnold-Chiari Malformation?

Answer 16

type 1

Question 17

What is type 1 Arnold-Chiari Malformation?

Answer 17

-develops as the skull and brain are growing
-cerebellar tonsils extend into the foramen magnum
-herniation of the cerebellar tonsils, medulla, and 4th ventricle into the spinal canal

Question 18

What is type 2 Arnold-Chiari Malformation?

Answer 18

-observed via ultrasound in utero
-small shallow posterior fossa
-greater amount of brain tissue extends through the foramen magnum
-often associated with myelomeningocele spina bifida

Question 19

What is type 3 Arnold-Chiari Malformation?

Answer 19

-observed via ultrasound in utero
-cerebellum and brainstem protrude into the foramen magnum

Question 20

What is the most severe type of Arnold-Chiari Malformation?

Answer 20

type III

Question 21

What is cerebral palsy?

Answer 21

-non-progressive neurological motor deficit associated with injury during the prenatal and perinatal periods
-brain injury occurring in the perinatal period
-probable causes of brain injury include: cerebral anoxia (baby loses oxygen, most common cause, can be cord wrapped around neck), hemorrhage (this happens when mom is Rh- and baby is Rh+ the mom develops Abs against baby’s blood), stroke with mom or baby, CNS infection of mom or baby, or encephalopathy
-4 types of cerebral palsy

Question 22

What is the most common motor disability in childhood?

Answer 22

cerebral palsy

Question 23

What are the 4 types of cerebral palsy?

Answer 23

1) spastic
2) ataxic
3) athetoid or dyskinetic
4) mixed

Question 24

What is spastic cerebral palsy?

Answer 24

-muscles appear stiff and tight
-increased muscle tone
-arises from motor cortex damage
-damages UMNs
-dystonia
-hyper-reflexive DTR
-has 3 types of gait: spastic diplegic gait (scissor walk), spastic hemiplegic gait, spastic quadriplegia (cannot walk)

Question 25

What is the most common type of cerebral palsy?

Answer 25

spastic

Question 26

What is ataxic cerebral palsy?

Answer 26

-characterized by shaky movements
-affects balance and sense of position in space
-arises from cerebellum damage or its pathways
-hypotonia (abnormally low muscle tone)
-hyporeflexia (decreased DTR)
-ataxic wide based gait

Question 27

What is athetoid or dyskinetic cerebral palsy?

Answer 27

-characterized by obvious abnormal involuntary movements like chorea, athetosis, choreoathetosis, involuntary facial expressions
-arises from basal ganglia damage
-non-ambulatory
-can become more severe with stress, decrease with relaxation and disappear entirely with sleep

Question 28

What is mixed cerebral palsy?

Answer 28

-combination damage of both spastic and athetoid/dyskinetic
-significantly impaired function
delayed growth and development
-wheelchair bound
-dysphagia (difficulty eating)
-dysarthria (impaired speech)
-intellectual impairment
-seizures

Question 29

What is the most severe cerebral palsy?

Answer 29

mixed