22-09-23 - Long term effects of chronic neurological conditions Flashcards
Learning outcomes
- Explain the likely neurological disability in a patient whose last functioning spinal cord level is at the fourth lumbar level (L4)
- Explain the types of spina bifida and their possible complications
- Describe the four common types of incomplete spinal cord injuries e.g. Brown-Sequard syndrome, anterior cord syndrome, posterior cord syndrome and central cord syndrome
What is the Definition of a chronic neurological condition?
What are the 2 forms of brain or spinal cord injury?
What can cause these injuries?
- A chronic neurological condition is any condition affecting the brain, spinal cord or nerves that is chronic or recurrent
- Brain or spinal cord injury can be congenital (existing at birth) or acquired (anything occurring after birth)
- Acquired brain/ spinal cord injury is not just trauma.
- Can include any illnesses resulting in chronic injury e.g. stroke, motor neuron disease, cauda equina syndrome or meningitis to name a few
What is spina bifida?
What defect results in spina bifida?
How can spina bifida vary in severity?
Where does spina bifida occur?
When does the neural tube develop during pregnancy?
What is the cause of spina bifida? What is a risk factor?
What can be done to prevent this?
- Spina bifida is a congenital abnormality where there is incomplete closing of the spine and spinal cord membranes during foetal development
- Is a type of neural tube defect
- Can vary in severity from spina bifida occulta -> meningocele -> myelomeningocele -> myeloschisis (severe)
- Spina bifida usually occurs at the lower spine in the lumbosacral region
- The neural tube develops in the first trimester and usually closes by the 28th day after conception
- The cause of spina bifida is thought to be multifactorial: genetic, nutritional and environmental factors
- Low folic acid levels in early pregnancy is a risk factor
- All women recommended to take 400 micrograms folic acid daily if planning to conceive and during first trimester
When does the neural tube form during pregnancy?
How does this occur?
What is the neural tube the embryonic origin of?
- Neural tube formation begins in the third week after conception (around Day 21) and typically fuses by Day 28 of development
- The neural plate elongates and begins to fold in on itself to form a neural tube
- The neural tube is the embryonic origin for the brain and spinal cord
Describe the normal axial slice of spine (no spina bifida) (in picture)
Describe the 4 different severities of Spinal bifida
- 4 different severities of Spinal bifida:
1) Spina Bifida Occulta
* This is the mildest and most common form of spina bifida
* Estimated 10-20% of population affected in western countries
* Malformation of 1 or more vertebrae resulting in open neural arch
* Spinal cord and meninges intact
* Most patients asymptomatic
* May be picked up at birth as a sacral dimple (this is also sometime hairy)
* If you see a sacral dimple –always refer to paediatrics for imaging
2) Meningocele
* Protrusion of the meninges through the spine
* The spinal cord remains in normal position
* Can result in mild disability
* The spinal cord usually develops normally and surgery can usually be done without damaging the cord
3) Myelomeningocele
* Protrusion of the meninges and spinal cord through the spine
* All that protects the spinal cord from the external environment is the meninges
* More likely to result in permanent spinal cord injury, even after surgery
4) Myeloschisis
* The most severe form of spina bifida
* The meningeal sac is open, exposing CSF and spinal cord to the external environment
* The CSF and spinal cord are exposed to infection
* CSF will likely be continually leaking from the site
Spina Bifida Occulta (in picture)
Meningocele
Myelomeningocele
Myeloschisis
What are 8 potential complications of spina bifida?
- 8 potential complications of spina bifida:
1) Permanent spinal cord injury from level of lesion downwards
2) People with L1 lesions likely to be wheelchair-bound
3) People with sacral lesions likely to be able to walk
4) Sensory nerve damage – sensory loss or sometimes chronic pain
5) Bladder and bowel dysfunction
6) Congenital or acquired orthopaedic foot/ ankle deformities
7) Associated conditions include hydrocephalus + Chiari malformations, scoliosis, tethered cord, syringomyelia and anencephaly
8) Many people with spina bifida have normal IQ, some may have learning disabilities
What % of infants s born with myelomeningocele will have hydrocephalus?
What causes hydrocephalus?
What is hydrocephalus cause by in myelomeningocele?
- 80% of infants born with myelomeningocele will have hydrocephalus
- Hydrocephalus caused by disruption of CSF drainage, leading to increased amounts of CSF
- In myelomeningocele, hydrocephalus is caused by Chiari 2 Malformation, where parts of the brain are pushed into the top of the spinal canal, blocking normal flow of CSF and build-up of fluid on the brain
What shunting is commonly used in hydrocephalus?
How severe is a blocked VP shunt?
What are 8 signs/symptoms of VP shunt blockage?
- Ventriculoperitoneal (VP) shunts commonly used in hydrocephalus to drain CSF and keep normal intracranial pressure
- A blocked VP shunt is an emergency – needs discussed with neurosurgery
- 8 signs/symptoms of VP shunt blockage:
1) Headache
2) Drowsiness
3) Nausea/ Vomiting
4) Bulging fontanelle (in infants)
5) Seizures
6) Pain around shunt
7) Change in behaviour/ co-ordination
8) Associated fever may suggest shunt infection
What are 4 ways spina bifida can be detected?
When are spina bifida surgeries usually scheduled?
What type of surgery is changing this?
- 4 ways spina bifida can be detected:
1) 20-week antenatal anomaly scan has high sensitivity for detecting open spina bifida (meningocele/ myelomeningocele/ myeloschisis)
2) High maternal serum levels or amniotic fluid levels of AFP (alpha fetoprotein) may indicate spina bifida (not routinely tested in NHS)
3) Occasionally diagnosed at birth
4) Spina bifida occulta commonly picked up as incidental finding on spinal x-ray (pts often asymptomatic)
- Historically children usually scheduled for operation at birth but new developments in fetal surgery are changing this
- Fetal MMC surgery is allowing opportunities for spina bifida to be operated on in utero in order to lower the risk of long term neurological damage
How is function affected by the level of the lesion?
At what level of spinal cord injury will walking not be possible?
At what level of spinal cord injury will walking be possible with support?
- The loss of function is additive the higher the lesion is, e.g. you tend to lose everything downward of the lesion
- In complete thoracic or high lumbar cord injury walking not possible
- In complete lower lumbar/ sacral cord injury walking may be possible but may need support e.g. splints or walking stick to compensate for some paralysed muscle groups
Describe 4 common types of incomplete spinal cord injury (in picture)
What are 7 Consequences of an L4 spinal cord injury?
- 7 Consequences of an L4 spinal cord injury:
1) Loss of knee flexion (S1) (extension usually preserved as is L3)
2) Loss of hip extension and hip abduction (both L4/5)
3) Loss of ankle dorsiflexion (L4) and plantarflexion (S1)
4) Loss of ankle inversion (L4) and eversion (S1)
5) Loss of greater toe flexion (L5)
6) Loss of sensation to anteromedial aspects of lower legs + posterior thigh/ buttocks/ genitals
7) Other possible consequences discussed in next slides
Why do pressure sores develop?
What is a common cause of pressure sores?
How quickly can they develop? How are they graded?
In what groups is pressure sore prevention vital?
What are methods of pressure sore prevention?
- A pressure sore (ulcer) develops due to skin breakdown from poor blood supply
- They are commonly caused by staying in the same position for long periods of time with pressure on a certain area. Can also be caused by other trauma to skin
- Pressure sores can start to develop in as little as 20 minutes
- Graded 1 – 4, from very superficial skin irritation to full thickness ulceration and exposed tendon/bone
- Pressure sore prevention is vital in those who are less able to mobilise
- Regular repositioning and specialist mattresses/ cushions used for prevention
Autonomic Dysreflexia.
At what level of lesion is Autonomic Dysreflexia a potential problem?
How severe is this condition? How can it be triggered?
What are 5 symptoms of autonomic dysreflexia?
What are 4 steps in the management of autonomic dysreflexia?
- Autonomic Dysreflexia
- Autonomic dysreflexia is not an issue with lumbar SCI (spinal cord injury), but Tends to affect those with T6 and above injury
- It is a medical emergency, can be fatal
- Autonomic dysreflexia can be triggered by distended bowel/ bladder or by sex. Basically, anything with a large stimulus below the level of injury can trigger it
- 5 symptoms of autonomic dysreflexia:
1) Pounding headache
2) Flushed skin above the level of SCI
3) Hypertension
4) Low heart rate
5) Goosebumps below the level of SCI, anxiety - 4 steps in the management of autonomic dysreflexia:
1) Remove stimulus
2) Sit patient upright
3) Sublingual nifedipine
4) If symptoms persist call 999
