Paediatric Neurological Conditions

Question 1

Neurological and neuromuscular paediatric disorders are usually due to

Answer 1

Insult on developing CNS pre/peri/postnatally
Genetic or congenital conditions
Trauma

Question 2

Main conditions in paediatrics include

Answer 2

Cerebral Palsy (CP)
Spina bifida (SB)
(Duchenne) Muscular dystrophy (DMD)
Acquired head injury (HI)
Learning disabilities (LD) e.g. Downe’s syndrome
Chromosomal defects

Question 3

Define Cerebral palsy

Answer 3

A permanent impairment of movement and posture resulting from a non-progressive brain disorder due to hereditary factors or events during pregnancy, delivery, neonatal period and the first 2-3/5 years of life

Question 4

Aetiology of CP

Answer 4

Prevalence 1:400 births, 1800 children diagnosed every year.
>30,000 children have CP in UK
Insult can occur pre/peri/post natally
Causes can be bleeding in brain, reduced O2, infection, meningitis, head injuries

Question 5

3 Descriptions of CP dependent on the area of the body affected?

Answer 5

1. Hemiplegia
2. Diplegia
3. Quadriplegia

Question 6

Can you label the different types of CP:

Answer 6

Hypertonic:

1. Spastic
2. Dystonic
3. Chorea

Hypotonic

1. Athetoid
2. Ataxic

Question 7

The most common form of CP is:

Answer 7

Spastic (80%) - presents as high tone and exaggerated reflexes

Question 8

The second most common form of CP is:

Answer 8

Dyskinetic cerebral palsy - It is marked by abnormal movements in the arms, hands, and legs, making it difficult to control body movements and coordination.
The several types of movement disorders seen in dyskinetic cerebral palsy are: dystonic, athetoid and chorea

Question 9

Dystonic movement present as:

Answer 9

twisting, repetitive unplanned and involuntary movements (dystonia)

Question 10

Chorea movement presents as:

Answer 10

abrupt, fidgety unpredictable movements that make the child appear uncoordinated and clumsy

Question 11

Athetoid movement presents as:

Answer 11

slow, writhing movements with extreme fluctuations (athetosis)

Question 12

Ataxic movement presents as:

Answer 12

incoordinated, jerky movements which may result in decreased balance and coordination

Question 13

Windswept is seen in what condition and what does it present as:

Answer 13

CP with high tone. Child will lie on back and face up however legs will be flexed and rotated to one side, i.e. ‘windswept’

Question 14

Aetiology of adult CP

Answer 14

100,000 CP in the UK (100,000 MS patients) – 2/3 are adults
CP is non-progressive but 25% decrease esp. >30 yrs
Similar life expectancy to non-disabled people

Question 15

Define Spina Bifida (SB)

Answer 15

Congenital abnormality in formation of spinal vertebrae.

Question 16

What are the 3 types, number them in order of severity (1 being the most severe):

Answer 16

1. cystica myelomeningocele
2. cystica meningocele
3. occulta

Question 17

Cystica myelomeningocele is when:

Answer 17

Both membrane and spinal cord push out through the spine (most severe)

Question 18

Cystic meningocele is when:

Answer 18

The membranes around spinal cord push out through the spine

Question 19

Occulta is when:

Answer 19

Usually where 1 or more vertebrae do not form properly therefore the gap is small (most common, mildest form of SB)

Question 20

Aetiology of SB

Answer 20

Incidence is decreasing.
Cause is commonly lack of folic acid

Question 21

What is hydrocephalus?

Answer 21

Increased/build-up of CSF in ventricles of brain

Question 22

Aetiology of hydrocephalus

Answer 22

75- 80% S.B. myelomeningocele have hydrocephalus
Can be congenital cause, i.e. infection, or can be acquired by injury or disease, i.e meningitis
Signs of blockage - Headaches, Change in arousal/ temperament
Two types: communicating, obstructive

Question 23

Aetiology of adults SB

Answer 23

Approx half of the people living with Spina Bifida are adults.

Transition to adulthood is difficult due to lack of services as with CP

Question 24

How is hydrocephalus treated?

Answer 24

Treated by shunt/stent surgery
PT usually assess brain damage due to pressure post-op

Question 25

What is muscular dystrophy

Answer 25

The muscular dystrophies (MD) are a group of inherited genetic conditions that gradually cause the muscles to weaken, leading to an increasing level of disability.

Question 26

What causes MD

Answer 26

MD is caused by mutations in the genes responsible for the structure and functioning of a person’s muscles

Question 27

What is Duchenne MD

Answer 27

Duchenne MD is one of the most common and severe forms of MD, it usually affects boys in early childhood; people with the condition will usually only live into their 20s or 30s

Question 28

Name types of muscular dystrophy:

Answer 28

• Duchenne MD
• Myotonic dystrophy
• Facioscapulohumeral MD
• Becker MD
• Limb-girdle MD
• Oculopharyngeal MD
• Emery-Dreifuss MD

Question 29

Life expectancy of DMD

Answer 29

With medical care, most people with Duchenne MD die from heart or respiratory failure before or during their 30s.

Question 30

Gower’s manoeuvre describes:

Answer 30

A patient that has to use his hands and arms to walk up his body in order to achieve an erect position. This is due to weakness of the quadriceps and gluteus muscles. The patient begins in the squatting position. This is followed by slowly lifting the hip and assuming a tripod position. The patient then places his hands on the knees as he slowly walks up his body.

Question 31

Adult CP can present with:

Answer 31

Pain, Fatigue, Falls 80% fallen

Question 32

NICE guidelines for Adult CP recommends:

Answer 32

• Annual review
• Encourage independent living
• Encourage work
• Communication difficulties – refer to SALT