Lecture 3 - Spina Bifida and Hydrocephalus

Question 1

What is Hydrocephalus?

Answer 1

Occurs when there is an imbalance between the production and absorption of cerebrospinal fluid (CSF)

Question 2

What is the broad consequences of hydrocephalus?

Answer 2

If the flow of CSF is obstructed this leads to a build up of pressure on the brain, which can impact on the structure and function of the brain.

If left untreated this results in damage to the brain cells and ultimately death.

Question 3

What are the three ways hydrocephalus can be classified?

Answer 3

1. Type: obstructive or communicating
2. Aetiology: congenital or acquired
3. Co-morbidities: present or absent

Question 4

What are some common neuropathological consequences of hydrocephalus?

Answer 4

• Axonal destruction and white matter atrophy
• Disruption to myelination
• Thinning/stretching of callosal and projection fibres
• Atrophy of olfactory nerves and optic tract

Question 5

What is the main surgical treatment for hydrocephalus?

Answer 5

Treatment
•Usually involves insertion of a shunt
•The shunt is inserted via a burr hole through the skull and into the ventricle
•Accumulated CSF drains through the shunt to the extracranial body cavity

Question 6

What are the consequences of shunt malfunctions? (hydrocephalus)

Answer 6

Blocked, malfunctioning or infected shunts give rise to physical and cognitive changes
•Signs may include:
–Gradual deterioration in functioning
–More severe malfunctions include: persistent headaches, vomiting, lowered arousal
•If left untreated may lead to acute illness and coma

Question 7

What is an Arnold Chiari Malformation?

what is a possible consequence?

Answer 7

Malformation of the brainstem and cerebellum
•Structures displaced into the spinal canal
•Can obstruct flow of CSF and cause hydrocephalus

Question 8

What is Spina Bifida?

Answer 8

A defect of neural tube closure (bottom) in early embriogenesis, occurring around the end of the first month of gestation during the neurolationphase
•It manifests as a malformation along the spinal cord
Malformations on the spinal cord can occur at any point between the cervical and lower sacral regions

Question 9

What are the three forms of Spina Bifida?

Answer 9

–Spina bifida occulta
–Spina bifida cystica (2 forms):
–1. Meningocele
–2. Myelomeningocele

Question 10

What is Spina bifida occulta?

Answer 10

A mild form of spina bifida,
•Asymptomatic
•Vertebral arches incomplete, CNS develops normally
•May be apparent because of abnormalities in skin and tissue at the site of the malformation

Outer layer of vertebrae not completely joined. Spinal cord and covering (meninges) undamaged. Hair often at the site of defect.

Question 11

What is Spina bifida Meningocele?

Answer 11

• Protrusion of the meninges and CSF through abnormal vertebral arches
• This protrusion forms a skin covered sac, usually in the region of the lumbosacral spine
• CNS usually otherwise intact

Question 12

What is Spina bifida Myelomeningocele?

Answer 12

• Most common and severe form of spina bifida
• At birth, appears as an open cyst/external sac on the back
• This sac contains damaged spinal cord and nerves
• The level of the lesion is related to the severity of the impairments

Question 13

90% of children with Spina bifida Myelomeningocele will have what four issues?

Answer 13

–Hydrocephalus
–Arnold Chiari Malformation
–Lower Limb Paralysis
–Bowel and Bladder Incontinence

Question 14

What are the physical issues associated with Spina Bifida

Answer 14

•Usually weakness and paralysis of lower limbs (varies in severity)
•Child may require:
–Special braces to support muscles
–Crutches
–Wheelchairs

Question 15

What continence issues are associated with spina bifida?

Answer 15

•Incontinence may result from impairments in ability of nerve signals to move from brain to bowel/bladder
•Management usually involves intermittent cathedarisation
•Bowel management strategies:
–Toilet timing
–Diet monitoring

Question 16

Broadly, what are the main neuropsychological deficits associated with Spina Bifida and Hydrocephalus?

Answer 16

• Majority of children with SB and hydrocephalus demonstrate “average” intellectual functioning
• However, most experience specific cognitive impairments

•Common deficits include:
–Attention & concentration
–Language
–Memory and Learning
–Visuo-perceptual and motor integration
–Executive skills

Question 17

Why are their neuropsychological deficits in spina bifida? (neuropathology)

Answer 17

Cognitive deficits are directly caused by underlying neurological problems associated with SB:
–Hydrocephalus
–Arnold Chiari Malformation
–Cortical Dysplasias

Question 18

What are some common Attention & Concentration symptoms of SB and Hydroceph?

Answer 18

• Poor sustained attention
• Difficulties with selective attention
• Poor focused attention & distractibility
• Reduced arousal, motivation and initiation

Question 19

What are some common language symptoms of SB and Hydroceph?

Answer 19

• Good vocabularies, talkative
• Disorder of language usage rather than content
• Deficits in fluency, comprehension and inferential language
• Poor monitoring of logic, relevance or appropriateness
• Slow and reduced comprehension

Question 20

What are some common memory & learning symptoms of SB and Hydroceph?

Answer 20

• Difficulty with encoding, rate of learning, delayed recall and spontaneous retrieval of information
• Recognition memory and cued recall stronger
• Encoding and retrieval deficits on visual memory tasks
• Struggle with learning abstract concepts (like mathematics)

Question 21

What are some common perceptual & motor-integration symptoms of SB and Hydroceph?

Answer 21

• Poor at interpreting visuo-spatial information
• Impaired fine motor skills
• Clumsiness
• Slow and untidy handwriting

Question 22

What are some common executive function symptoms of SB and Hydroceph?

Answer 22

• Poor planning and organisation
• Problem solving and reasoning difficulties
• Difficulty maintaining goal directed behaviour
• Inflexible thinking
• Concrete thinking

Question 23

What are some common visuo-academic symptoms of SB and Hydroceph?

Answer 23

• Impaired reading comprehension
• Poor spelling
• Slow and messy handwriting
• Poor copying skills
• Difficulties with mathematical problem solving