Long Term Effects of Chronic Neurological Disability

Question 1

Describe the epidemiology of neurological conditions in the UK

Answer 1

COMMON

• 10 million in UK living with neurological condition impacting on their lives
• 1 million (2%) in UK disabled by their neurological condition
• Neurological conditions most common cause of serious disability

Question 2

Define Spina Bifida

Answer 2

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

Question 3

What are the proposed aetiologies of spina bifida?

Answer 3

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

Question 4

Name 2 minor defects assoicated with spina bifida

Answer 4

• spina bifida occulta
• sacral dimple

NO CLINICAL EFFECTS

Question 5

What form of spina bifida is compatible with life?

Answer 5

Anecephalus

Question 6

Describe a mild form of spina bifida?

Answer 6

• Ehen the cord remains in the neural canal
• Sac contains the meninges and CSF but not the spinal cord

Question 7

Describe a serious form of spina bifida

Answer 7

If the cord is displaced from the neural canal or has not developed

Question 8

What are the neurological consequences of severe spina bifida?

Answer 8

Paralysis, loss of sensation and reflexes distal to the abnormality

Question 9

What are the 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 circulation of CSF

Question 10

What can be installed to solve hydrocephalus and what is the assoicated risk

Answer 10

• shunts drain excess CSF from the ventricular system
• RISK = shunts can become infected

Question 11

What are the main categories of assoicated conditions with spina bifida?

Answer 11

• Renal anomalies
• Sphincter Function
• Intellectual impairments
• Musculoskeletal

Question 12

What are the issues assoicated with paralytic limb deformities in SB?

Answer 12

• Difficulties with shoe wear
• Plantar ulceration

Question 13

What causes scoliosis in SB patients?

Answer 13

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

Question 14

What is the prognosis for independent walking as an adult with SB?

Answer 14

Assumes no significant intellectual or psychological impairment to walking

Question 15

What effect does a thoracic and upper lumbar lesion have on walking in a patient with SB?

Answer 15

None

Question 16

What effect does a lower lumbar or sacral lesion have on walking in a patient with SB?

Answer 16

Can walk but will need orthotics to compensate for paralysed/weak muscles

Question 17

What does a patient with a flexed knee gait lesion in L4 rely on?

Answer 17

intact quadriceps muscles

Question 18

What are the results of a flexed knee gait L4 lesion?

Answer 18

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

Question 19

How does a flexed knee gait in L4 lesion affect an adult with SB?

Answer 19

• reliant on a wheelchair
• can still drive in an adapted vehicle

Question 20

Describe charcot joints

Answer 20

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

Question 21

What is the expectation of a normal lower urinary tract system?

Answer 21

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 22

Describe a neurogenic bladder

Answer 22

Affects S2-4

Incomplete bladder emptying leads to back pressure on ureters and eventual renal parenchymal damage and failure, if left untreated

Question 23

What is the aim of urinary continence of an individual with SB?

Answer 23

AIM - acheieve social continence in childhood

Clean intermittent cathiterisation is often the best option

Question 24

What are the stigmas assoicated with urianry incontience?

Answer 24

reduced QoL with social isolation and embarassment

urinary diversion may be necessary (stoma)

Question 25

What is the cause of faecal incontinence in patients with SB?

Answer 25

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

Question 26

How is the sexual function of a person with SB affected?

Answer 26

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

Question 27

What is the desired optimal outcome for a patient with SB in adulthood?

Answer 27

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

Question 28

What are the main challenges to a patient with SB living as an adult?

Answer 28

• 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