Long term effects of chronic neurological conditions

Question 1

Spina bifida (spine)(split)

Answer 1

congenital conditions where there is an incomplete development or covering of the brain and/or spinal cord, caused by a failure of the foetal spine to close normally in the first month of pregnancy

Question 2

Spina bifida – proposed aetiologies

Answer 2

• Multi-factorial inheritance.
• Potato blight.
• Vitamin deficiencies/folate.
• Maternal fever.
• Zinc deficiency.
• High sound intensity.
• Viral infection.
• Alcohol.
• Mineral deficiency.
• Medication – Phenytoin, Epilim, etc.

Question 3

what are minor neurological closure defects

Answer 3

• spina bifida occulta

this can be seen as an incomplete closure on a scan but should cause any issues

• sacral simple

again this should cause any issues

Question 4

Neural tube defects:

cranial and vertebral abnormalities

Answer 4

• anencephalus

- spina bifida

Question 5

if the cord remains in the neural canal

Answer 5

this means there should only be mild disability

the sac contains the meninges and CSf but not the spinal cord itself

Question 6

if the cord is outwit the neural canal

Answer 6

this results in a serious abnormallty

Question 7

Neurological consequences of severe spina bifida

Answer 7

Paralysis, loss of sensation and reflexes distal to the abnormality

Question 8

Neonatal consequences of severe spina bifida

Answer 8

High risk of meningitis in open lesions in the neonate

Hydrocephalus in 70-90% because of the interruption of the circulation of CSF

Question 9

what are the dangerous of shunting hydrocephalus

Answer 9

Shunts can become infected or blocked

Question 10

Antenatal diagnosis of abnormalities

Answer 10

• Ultrasound
spinal anomalies identifiable at 16-18 weeks
• α fetoprotein raised in neural tube defects maternal serum at 16-20 weeks amniocentesis

Question 11

associated conditions related to spina bifida

Answer 11

Renal anomalies
Sphincter function
Intellectual impairments Musculoskeletal

Question 12

after an antenatal diagnosis what are the challenges that are faced

Answer 12

• Antenatal counselling and possible termination of pregnancy
• Not everyone attends for antenatal care
• Some mothers may first attend after the legal gestational age for termination
• Culture/religion may prevent abortion

Question 13

Paralytic deformities of the feet

Answer 13

Difficulties with shoe wear Plantar ulceration

Question 14

Scoliosis

Answer 14

Scoliosis is due to a combination of
congenital abnormalities of the spine and spinal muscle weakness
If surgical correction is required,
this is usually done after the age of 10 years to allow sufficient spinal
growth beforehand

Question 15

Prognosis for independent walking as an adult

- Assumes no significant intellectual or psychological impairment to walking

Answer 15

• 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

Question 16

Flexed knee gait in a L4 lesion: patient relies on intact quadriceps

Answer 16

Paralysed calf muscles result in excess ankle dorsiflexion
• Muscle fatigue
Energy inefficient gait
• Knee pain – joint degeneration

Question 17

Challenges to mobility as an adult with a L4

Answer 17

Increasing height and weight as child grows but muscle strength does not change
Muscle fatigue and knee pain may worsen and adult becomes more reliant on a wheelchair
An L4 lesion does not preclude driving

Question 18

Challenges to mobility as an adult with a L4 lesion

Answer 18

If able to drive may need adapted vehicle
Likely to use a self propelling or electric wheelchair for shorter distances
Will require adapted housing if living independently

Question 19

why is there a problem with joints in neurological conditions

Answer 19

Loss of protective sensation (pain) and proprioception in a joint can result in joint destruction

resulting in Charcot joints

Question 20

Expectations of Lower Urinary Tracts

Answer 20

1. That bladder should fill to good capacity.
2. Should empty to completion.
3. Emptying should be under voluntary control.
4. That filling and emptying should not be detrimental to
   renal function.

Question 21

Urinary incontinence (S2-4): neurogenic bladder

Answer 21

Incomplete bladder emptying
leads to back pressure on ureters and eventual renal
parenchymal damage and failure, if left untreated
Spinal injury patients have kidneys scanned at least every two years

Question 22

Urinary incontinence: aim to

achieve social continence in childhood

Answer 22

Nappies (diapers) acceptable before primary school
Permanent bladder catheterisation is not a satisfactory long term solution because of a high risk of UTIs
Clean intermittent catheterisation often the best option - carer or patient self catherisation

Question 23

what is the social impacts of incontinence

Answer 23

Reduced quality of life - with social isolation and embarrassment
Urinary diversion may be necessary for physical or
social reasons

Question 24

Faecal incontinence

Answer 24

Faecal incontinence is often a barrier to social acceptance at any age
Paralysis of external anal sphincter and mechanism to indicate a full rectum
Some patients will have weak abdominal and perineal muscles

Question 25

what can help with faecal incontinence

Answer 25

Daily rectal enema

Constipation may require laxatives or manual evacuation

Surgical diversion may be
necessary for physical or social reasons

Question 26

4. Sexual potential

Answer 26

Libidomay be normal Sensation absent (S2-4)
Erections may be possible due to spinal reflex
Fertility females normal
males usually sterile
Menstruation may cause further social difficulty

Question 27

5: Education

Answer 27

Mainstream school possible, particularly where there is less hydocephalus
Special needs school: learning and physical disability
College to acquire life skills for those with learning difficulties

Question 28

Employment

Answer 28

‘Ability’ rather than ‘disability’

Extensive UK disability legislation to prevent discrimination
Patients whose disabilities preclude employment may attend a day centre – this is a dwindling facility

Question 29

Parents and caregivers

Answer 29

Emotional and physical support for child/young person
Need to provide assistance with daily physical tasks e.g. bathing, dressing, mobility
Don’t forget siblings and partner Ageing parents

Question 30

Independent living as an adult: optimal outcome

Answer 30

Normal intellect
Social continence stable
Independently mobile, can drive an adapted vehicle and use walking aids
Independent living - household adaptations, telecare and other technologies
Employed

Question 31

Disability paradox

Answer 31

Poor function but excellent quality of life

Question 32

Multiple sclerosis

Answer 32

• Multiple plaques of demyelination in the brain & spinal cord disseminated in time and space
• Most common sites – optic nerve, periventricular region, brain stem and cerebellar connections, cervical spine, posterior columns
• Clinical features – relapsing/remitting, chronic progressive •Investigations – MRI brain and spinal cord, CSF for oligoclonal bands