Long Term Effects of Chronic Neurological Conditions

Question 1

How many people in the UK live with neurological condition impacting on their lives ? How many are disabled by their condition ?

Answer 1

10Million
1 Million (2% of population)

Question 2

How many in the UK are diagnosed with neurological condition each year ?

Answer 2

600,000 (1%) newly diagnosed each year with neurological condition

Question 3

What proportion of hospital admissions are for neurological problem requiring treatment from a neurologist or neurosurgeon ?

Answer 3

19% of hospital admissions are for neurological problem requiring treatment from a neurologist or neurosurgeon

Question 4

What is spina bifida ?

Answer 4

Describes a group of 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. Often linked with Hydrocephalus.

Question 5

Identify proposed aetiologies of spina bifida.

Answer 5

• 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 6

What determines the severity of spina bifida ?

Answer 6

Mild disability when the cord remains in the neural canal (sac contains the meninges and CSF but not the spinal cord)

Serious disability if the cord is displaced from the neural canal or has not developed

Question 7

What are the neurological consequences of severe spina bifida ?

Answer 7

Paralysis, loss of sensation and reflexes distal to the abnormality

Question 8

How is spina bifida treated ?

Answer 8

Surgery to close the defect (reduce exposure, and risk of meningitis)

Question 9

Identify neonatal consequences of severe spina bifida.

Answer 9

• High risk of meningitis in open lesions in the neonate

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

Question 10

How is a hydrocephalus treated ? What is the main issue with hydrocephalus ?

Answer 10

Shunting, in order to drain the hydrocephalus from ventricular system
Shunts can become infected or blocked.

Question 11

How is antenatal diagnosis of spina bifida performed ?

Answer 11

• Ultrasound
spinal anomalies identifiable at 16-18 weeks

• α fetoprotein raised in neural tube defects maternal serum at 16-20 weeks amniocentesis

Question 12

Identify conditions associated with spina bifida.

Answer 12

• Renal anomalies
• Sphincter function
• Intellectual impairments
• Musculoskeletal

Question 13

What can be done if a spina bifida is found antenatally ? What is the problem with this ?

Answer 13

• Antenatal counselling and possible termination of pregnancy

BUT not everyone attends for antenatal care, and some mothers may first attend after the legal gestational age for termination

Question 14

Identify examples of musculoskeletal conditions associated with spina bifida.

Answer 14

Paralytic deformities of the feet

• Difficulties with shoe wear
• Plantar ulceration

Scoliosis
-Due to combo of congenital abnormalities of the spine and spinal muscle weakness

Abnormalities of gait/mobility (depending where lesion is)

Question 15

When is surgery performed for scoliosis ?

Answer 15

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

Question 16

Describe prognosis for independent walking as a adult, for a child diagnosed with spina bifida.

Answer 16

Assumes no significant intellectual or psychological impairment to walking:

-Thoracic and upper lumbar lesions – no walking ability
-Lower lumbar and sacral– can walk but will need
orthotics (splints) to compensate for paralysed/weak muscles

Question 17

Describe gait, and mobility in a L4 lesion.

Answer 17

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

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

MOBILITY

• 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
• 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 18

Define Charcot Joint.

Answer 18

Loss of protective sensation and proprioception in a joint, can result in joint destruction

Question 19

Identify a lesion which can cause Urinary Tract consequences.

Answer 19

S2-4 lesion: urinary incontinence (neurogenic bladder), so incomplete bladder emptying leads to back pressure on ureters and eventual renal
parenchymal damage and failure, if left untreated

Question 20

What are the treatment options for urinary incontinence at childhood ?

Answer 20

Urinary incontinence: aim to
achieve social continence in childhood

• 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 21

What are the social consequences of urinary incontinence ?

Answer 21

Reduced quality of life - with social isolation and embarrassment

Urinary diversion may be necessary for physical or
social reasons (urine drains continuously into a stoma bag)

Question 22

Identify possible neurological causes of faecal incontinence.

Answer 22

• Paralysis of external anal sphincter and mechanism to indicate a full rectum
• Some patients will have weak abdominal and perineal muscles

Question 23

What is the treatment for faecal incontinence ?

Answer 23

-Daily rectal enema
-Constipation may require laxatives or manual evacuation
-Surgical diversion may be
necessary for physical or social reasons

Question 24

Identify a lesion which can cause fecal incontinence.

Answer 24

S2-S4 lesion

Question 25

Describe the sexual potential after an S2-S4 lesion.

Answer 25

-Libido may 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 26

Describe the implications on education of a chronic neurological condition.

Answer 26

• Mainstream school
• Special needs school: learning and physical disability
• College to acquire life skills for those with learning difficulties

Question 27

Describe the implications on employment of a chronic neurological condition.

Answer 27

• Ability’ rather than ‘disability’ i.e. what the young person or adult can do and not what they cannot do
• Extensive UK disability legislation
• Patients whose disabilities preclude employment may attend a day centre

Question 28

Describe the implications on parents and caregivers of chronic neurological conditions.

Answer 28

• Emotional and physical support for child/young person
• Need to provide assistance with daily physical tasks e.g. bathing, dressing, mobility
• Also need not to forget siblings and partner
• Difficult for ageing parents

Question 29

Identify the optimal outcome of independent living as an adult with a chronic neurological condition.

Answer 29

• 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 30

Identify challenges to independent living as an adult with a chronic neurological condition.

Answer 30

• 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 though in reality

Question 31

Describe disability paradox.

Answer 31

Many people with serious and persistent disabilities report that they experience a good or excellent quality of life when to most external observers these individuals seem to live an undesirable daily existence

Question 32

What is cerebral palsy ?

Answer 32

• Spectrum of motor disorders affecting posture, movement and co-ordination
• Caused by a brain lesion resulting in abnormal development of CNS

Question 33

Identify risk factors for cerebral palsy.

Answer 33

Low birth weight or preterm birth
Multiple gestations
Infertility treatments

Question 34

What proportion of live births in the UK are affected by cerebral palsy ?

Answer 34

0.2-0.3%

Question 35

Do younger people with cerebral palsy have a different perception of their social integration compared to older people with cerebral palsy ?

Answer 35

Older people with CP feel less socially integrated than younger people with CP due to:

• Vocational Status
• Living Situation
• Musculoskeletal Pain

Question 36

MS

• Underlying cause
• Most common sites
• Clinical features
• Investigations

Answer 36

MS

• Underlying cause: Multiple plaques of demyelination in the brain and 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