Neurology

Question 1

Definition of cerebral palsy

Answer 1

A dynamic/changing disorder of posture and movement caused by a non-progressive lesion to the developing brain
“CP describes a group of permanent disorders of the devlopment of movement and posture, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing foetal or infant brain. The motor disorders of cerebral palsy are often accompanied by distubrances of sensation, perception, congition, communication, and behaviour; by epilepsy and by secondary MSK problem.”

Question 2

Causes of cerebral palsy

Answer 2

Antenatal - toxins, teratogens, inutero infection
Perinatal - hypoxic insult, sepsis
Postnatal - meningitis, trauma

Question 3

Tetraplegic =

Answer 3

All four limmbs implies a lesion to both hemispheres, this may be causes by a global hypoxic ischaemic injury

Question 4

Hemiplegic =

Answer 4

One side of the body, a lesion of one hemisphere which may be caused by an antenatal stroke

Question 5

Diplegic =

Answer 5

Affecting lower limbs, implies a lesion of the white matter at the back of the brain (periventricular leukomalacia) typically associated with prematurity

Question 6

Co-morbidities in cerebral palsy

Answer 6

Epilepsy
Learning difficulties
Behaviour problems
Feeding problems/gastro-oesophageal reflux
Osteoporosis

Question 7

Management of cerebral palsy

Answer 7

Define the underlying aetiology
Promote/support participation in all aspects of society through practical measures, education and legislation
MDT approach - physician, phsyiotherapist, OT, SALT, dietician, nurse speciailist
Mx comorbidites e.g. epilepsy and feeding problems
Prevent deformity - PT, botulinum toxin, surgery in special situations

Question 8

What medication and dose can help prevent neural tube defects

Answer 8

Folic acid - 400 microg/day 1 month prior to conception

Question 9

Spina bifida - limitations of community ambulators

Answer 9

Community ambulators (L3 levels and lower) are able to walk indoors and outdoors for most activities, although a wheelchair may be used for longer trips outside their immediate vicinity

Question 10

Spina bifida - limitations of household ambulators

Answer 10

Household ambulators (L3 or mid lumbar) may be able to walk indoors and transfer to a wheelchair for community use and most outdoor activity

Question 11

Spina bifida - limitations of non-functional ambulators

Answer 11

Non-funcitonal ambulators (L1 to L3) may walk as part of a therapy session or in a gymnasium with orthotic devices, but use a wheelchair for any useful mobility needs

Question 12

Spina bifida - limitations of non-ambulators

Answer 12

Non-ambulators use a wheelchair for indoor and outdoor activities

Question 13

Definition of myelomeningiocele

Answer 13

Outpouching of the spinal cord and its coverings through a defect in posterior elements of vertebral arches
Cervical cord and brainstem are likely to be affected - the Chiari malformation

Question 14

Associated problems of myelomeningocele

Answer 14

Mobility, sensation, bowel and bladder function, hydrocephalus, specific learning problems

Question 15

What is seen when examining a child with spina bifida

Answer 15

Flaccid weakness of the lower limbs, reflexes will be absent and there will be a lack of sensation. Look for evidence of a ventriculo-peritoneal shunt and examine the back

Question 16

Aetiology of muscular dystrohyt

Answer 16

Duchenne muscular dystrophy and becker muscular dystrophy are due to mutations in the X-liked dystrohin genes. In Becker there is preserved weakness but a significatn amount of normal dystrophin is preserved.

Question 17

Clinical diagnosis of muscular dystrophy

Answer 17

Signs of muscle weakness, lumbar lordosis, calf muscle hypertrophy, learning (particularly speech) problems in some cases. Gowers sign

Question 18

Ix of muscular dystrophy

Answer 18

Elevated creatinine kinase, @DNA testing for mutations in the dystrophin gene. Later monitoring of respiratory and cardiac function and scoliosis

Question 19

Mx of muscular dystrophy

Answer 19

Genetic counselling, PT, aids for ambulation, resp support, scoliosis surgery, steroids, potential for gene therapy

Question 20

Definition of epileptic seizure

Answer 20

A transient occurrence of sings and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain

Question 21

Definition of epilepsy

Answer 21

Epilepsy is a disease f the brain defined by any of the following conditions:

1. At least 2 unprovoked seizures occurring more than 24 hours apart
2. One unprovoked seizure and a probability of further seizures similar similar to the general recurrence risk after 2 unprovoked seizures (approx 75% or more)
3. At least 2 seizures in a setting of reflex epilepsy

Question 22

Causes of non-epileptic seizures

Answer 22

Syncopes and anoxic seizures
Psychological/behaviour disorders
Derangements of the sleep process
Paroxysmal movement disorders
Migraine equivalents
Micellaneous neurological events

Question 23

Top 5 facts on positional plagiocephaly

Answer 23

Incidence increased following ‘Back to Sleep Campaign’.
Prevention/improvement can be achieved by encouraging ‘tummy time’ for playing, positioning toys/windows/people on the less preferred side and alternating lying direction in cot. Limit time in bouncy chair/car seat.
If baby’s head is always turned in one direction consider torticollis and refer for physio.
No firm evidence exists that moulded helmets or bands work.
Resolves over time. Usually by school age, no noticeable flattening remains. If there are concerns regarding early closure of suture lines
(over-riding sutures, abnormal head shape from birth) referral to paediatrics/neurosurgery is required to exclude craniosynostosis

Question 24

Facts on febrile convulsion

Answer 24

3% of children in the UK will have a febrile convulsion
Aged 6 months to 5 years (outwith this range warrants closer investigation)
No association underlying pathological abnormality.
Recur in 30% of kids
3% will progress to having epilepsy (more likely if complex febrile seizures)