Long term effects of chronic neurological conditions Flashcards
What s Spina Bifida?
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)
What general happens in spinal cord embryology?
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Who first described spinal spina bifida, what year and who liked it to another condition?
First described by Nicolaes Tulp in 1593
Giovanni Morgagni linked Hydrocephalus with Spina Bifida in 1761
How common is spina bifida?
Occurs 1-1.5 in 2,000 births
What does a normal spine look like?
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What minor defects can occur in the spine forming?
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
What is Meningocele?
Meningocele is when the sac contains the meninges and CSF but not the spinal cord
Mild disability but least common form
What is Myelomeningocele ?
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
What is Myeloschisis ?
Very serious condition often with exposed neurons and continual leakage of CFS out of the lesion
Infection worry!
Name each of these types of spina bifida?
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 !
What are the neural consequences of serious forms of Spina Bifida ?
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
How do we close the defect?
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
What does shunting in hydrocephalus do?
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)
What other conditions are associated with Spina Bifida?
Other conditions associated with Spina Bifida;
- Renal anomalies
- Sphincter function
- Intelectual impairments
- Musculoskeletal issues
What orthopaedic issues can be seen in the feet of Spina Bifida patients?
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
What causes Scoliosis in Spina Bifida patients?
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
How can function be affected be Spina Bifida?
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
How may a lesion from Spina Bifida affect an adults walk?
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
How may mobility be challenged with an L4 lesion?
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
What might you expect to see in a Spina Bifida patient with an L4 lesion in their gait?
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
What urinary tract issues should we monitor in patients with Spina Bifida?
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
What may an S2-S4 lesion cause in the Urinary tract?
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
How may Spina Bifida affect bowel function?
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
How may Spina Bifida affect sexual potential ?
- 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)
How may Spina Bifida affect someones education ?
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
How may Spina Bifida affect someones employment?
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
How may Spina Bifida be a challenge for parents/caregivers ?
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
How may Spina Bifida affect someones independence as an adult ?
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 !
