Long term effects of chronic neurological conditions

Question 1

What s Spina Bifida?

Answer 1

Spina (spine) Bifida (split);

Describes a group of congenital neural tube malformation conditions

• There is an incomplete development or covering of the brain and/or spinal cord
• Caused by a failure of the fatal spine to close normally in the first month of pregnancy (usually caudal end there’s an issue with)

Question 2

What general happens in spinal cord embryology?

Answer 2

Image

Question 3

Who first described spinal spina bifida, what year and who liked it to another condition?

Answer 3

First described by Nicolaes Tulp in 1593

Giovanni Morgagni linked Hydrocephalus with Spina Bifida in 1761

Question 4

How common is spina bifida?

Answer 4

Occurs 1-1.5 in 2,000 births

Question 5

What does a normal spine look like?

Answer 5

Image

Question 6

What minor defects can occur in the spine forming?

Answer 6

Spina bifida occulta - poorly formed spinous process

Sacral dimple - open neural arch (no spinous process) usually find hair grows onto of the region

These are the most common forms of Spina bifida and have the mildest effects on the patient - aren’t seen as dangerous

Question 7

What is Meningocele?

Answer 7

Meningocele is when the sac contains the meninges and CSF but not the spinal cord

Mild disability but least common form

Question 8

What is Myelomeningocele ?

Answer 8

Much more serious condition where the spinal cord is displaced and exposed to the external environment, protected only by meningeal layers

Can even get a lot of damage during natural birth to these so c-section

Question 9

What is Myeloschisis ?

Answer 9

Very serious condition often with exposed neurons and continual leakage of CFS out of the lesion

Infection worry!

Question 10

Name each of these types of spina bifida?

Answer 10

A). Normal lumbosacral anatomy at lumber 3 vertebra,

B). Spina bifida occulta result of partial agenesis of posterior vertebral arch and little pit on the covering
skin,

C). Tuft of hair over complete defect of posterior arch in spina bifida occulta,

D). Meningocele,

E). Myelomeningocele

F). Myeloschisis (rachischisis).

Myelo = spinal cord displaced - dangerous !

Question 11

What are the neural consequences of serious forms of Spina Bifida ?

Answer 11

Neural consequences of serious forms of Spina Bifida;
- Paralysis
- Loss of sensation distal to abnormality
- Loss of reflexes distal to the abnormality
- CSF circulation disruption causing hydrocephalus in up to 80% of patients
- High risk of foetal meningitis where spinal cord is not covered

Question 12

How do we close the defect?

Answer 12

Prenatal Surgery;
This is thought to protect the baby’s spinal cord from ongoing damage in the uterus. The Management of Myelomeningocele Study (MOMS) showed that prenatal surgery to close the defect in the spinal cord improved outcomes compared to children who had postnatal surgery for spina bifida.

Patients with hydrocephalus can have a drain inserted into the left ventricle

Question 13

What does shunting in hydrocephalus do?

Answer 13

During shunt surgery, a thin tube called a shunt is implanted in your brain. The excess cerebrospinal fluid (CSF) in the brain flows through the shunt to another part of your body, usually your tummy. From here, it’s absorbed into your blood. Reduces neurocraniopressure and reduces CSF content

Principal drawback is that shunt valves and tubes can become blocked or infected (Usually ok tho - but would see headaches, confusion, nausea if not)

Question 14

What other conditions are associated with Spina Bifida?

Answer 14

Other conditions associated with Spina Bifida;
- Renal anomalies
- Sphincter function
- Intelectual impairments
- Musculoskeletal issues

Question 15

What orthopaedic issues can be seen in the feet of Spina Bifida patients?

Answer 15

Can have;
- Rigid bilateral clubfoot
- Vertical talus
- Rotated talus

Even after surgical correction, weak leg muscles cause by spinal cord developmental and ankle valgus deformity problems, which can cause shortening of the fibula and lateral wedging of the tibia

Resulting in skin abrasions and callus

Question 16

What causes Scoliosis in Spina Bifida patients?

Answer 16

Scoliosis is due to a combination of congenital abnormalities of the spine and spinal muscle weakness

Found in about 50% of patients with Spina Bifida

If surgical correction is required this is usually done after the age of 10 years to allow sufficient spinal growth beforehand

Surgical treatment of Scoliosis results in fusion of the vertebra, giving joint stability and often a reduction in pain

Question 17

How can function be affected be Spina Bifida?

Answer 17

The loss of function is additive the higher the lesion is

Those with cervical lesions often loose much more function than those with low sacral lesions of the same type

Question 18

How may a lesion from Spina Bifida affect an adults walk?

Answer 18

Prognosis for independent walking as an adult
(Assuming no significant intellectual or psychological impairment to walking)

• Thoracic and upper lumbar lesions walking not possible
• Lower lumbar and sacral— can walk but will need orthotics (splints) to compensate for
  paralysed/weak muscles

Mobility is not the same as walking

Question 19

How may mobility be challenged with an L4 lesion?

Answer 19

Mobility challenges with an L4 lesion;

• Increasing height and weight as child grows but muscle strength does not
  change as lack of innervation
• Muscle fatigue and knee pain may worsen, and adult becomes more reliant
  on a wheelchair
• An L4 lesion does not preclude driving— but may need an adapted vehicle
• Likely to use a self propelling or electric wheelchair for shorter distances
• Will require adapted housing if living independently

Question 20

What might you expect to see in a Spina Bifida patient with an L4 lesion in their gait?

Answer 20

L4 lesion in gait will cause a flexed knee gait

Weak or paralysed calf muscles result in excess ankle dorsiflexion

Energy inefficient gait

Muscle fatigue

Knee pain and joint deterioration

In adults this may cause “Charcot Joints” - General description of degeneration of joints

The loss of protective pain sensation and proprioception leads in time to knee joint destruction

Question 21

What urinary tract issues should we monitor in patients with Spina Bifida?

Answer 21

Key factors in patient survival;
- That bladder should fill to good capacity
- Should empty to completion (if partially empty risk of infection)
- Emptying should be under voluntary control
- That filling and emptying should not be detrimental to renal function

Question 22

What may an S2-S4 lesion cause in the Urinary tract?

Answer 22

S2-S4 lesion may cause Urinary incontinence - or neurogenic bladder

If left untreated leads to incomplete bladder emptying which can cause;
- back pressure on ureters
- Eventual renal parenchymal damage and failure

Spinal injury patients have kidneys scanned at least every 2 years as preventative measure

Therapeutic goal is to have social continence in childhood

Some of these patients may need to use;

• Nappies acceptable before primary school
• Permanent bladder catheterisation is not a satisfactory long term solution because high risk of UTI’s
• Clean intermittently catheterisation often best option - carer or patient self catheterisation

Urinary incontinence causes social isolation, embarrassment and reduction in quality of life

In some cases urinary diversion can be performed to help this - the passage of urine is diverted from uriters and drains directly into a stoma bag

Question 23

How may Spina Bifida affect bowel function?

Answer 23

Facal incontinence can be a barrier to social acceptance

Paralysis of external anal sphincter and mechanism to indicate a full rectum

Some patients will have weak abdominal and perineal muscles

Treatments;
- Daily rectal enema
- Constipation relief using laxatives or manual evacuation
- Surgical diversion through a stoma into a bag can be applied fo broths social or physical reasons

Question 24

How may Spina Bifida affect sexual potential ?

Answer 24

• Libido may be normal
• Sensation may be absent at (S2-4)
• For males erections may still be possible due to spinal reflex
• Fertility in females is normal however for sone females mensuration may cause social difficulty
• Males are usually infertile but this is often linked to incontinence and ejaculatory dysfunction rather than a lowered sperm count (would use IVF)

Question 25

How may Spina Bifida affect someones education ?

Answer 25

Mainstream school is possible, particularly where there is less hydrocephalus

Special needs school: to overcome learning and physical disability

Collage to acquire life skills for those with learning difficulties

Question 26

How may Spina Bifida affect someones employment?

Answer 26

Ability rather than disability - i.e what the person can and cannot do

Extensive UK disability legislation to prevent discrimination

Patients whose disabilities preclude employment may attend a day centre - this is a dwindling facility

Question 27

How may Spina Bifida be a challenge for parents/caregivers ?

Answer 27

Challenges for parents & caregivers
- emotional and physical support for child/young person
- Need to provide assistance with daily physical tasks (bathing, dressing, mobility)
- Don’t forget siblings and partner
- Ageing parents

Question 28

How may Spina Bifida affect someones independence as an adult ?

Answer 28

Independent living as an adult: optimal outcome
- Normal intellect
- Social continence stable
- Independently mobil, can drive and adapted vehicles and use walking aids
- Independent living - household adaptations, tele care and other
- Employed

Challenges to independent living as an adult:
- Learning difficulties
- Behavioural difficulties
- Social continence not achieved
- Cannot transfer independently
- Ageing parents
- Some patients will live in a sheltered/residential facility and be looked after by a team of carers - options for community living are limited tough in reality

Disability paradox - Poor function but excellent quality of life !