Paediatric Neuromuscular Conditions

Question 1

A child or young person is defined as having Complex Exceptional Needs if:

Answer 1

There is severe impairment in at least 4 categories together with enteral/parenteral feeding
OR
Severe impairment in at least two categories and ventilation
AND
Impairments are sustained for more than 6 months and ongoing

Question 2

Cerebral Palsy

Answer 2

This is a permanent and non-progressive motor disorder due to brain damage before birth or during the first 2 years of life. It effects the cerebrum which disrupts the ability of the body to exhibit coordinated body control.

Question 3

How many children with complex needs are accounted for by cerebral palsy

Answer 3

7%

Question 4

Epidemiology of Cerebral Palsy

Answer 4

Occurs in 2/1000 births.

Question 5

Perinatal risk factors for cerebral palsy

Answer 5

Prematurity, anoxic injuries, infections, kernicterus (a rare but serious complication of untreated jaundice in babies), erythroblastosis fetalis (abnormal presence of erythroblasts in the blood)

Question 6

Prenatal risk factors for cerebral palsy

Answer 6

Placental insufficiency, toxaemia, smoking, alcohol, drugs, infection (TORCH)

Question 7

Postnatal risk factors for cerebral palsy

Answer 7

Infections (cytomegalovirus, rubella), head trauma.

Question 8

Spastic

Answer 8

Effects the pyramidal system and or the motor cortex. There will be an increase of tone on passive stretch of muscle.

Question 9

Athetoid (dyskinetic)

Answer 9

Effects the extrapyramidal system and or the basal ganglia. Combination of spastic and dystonic with involuntary movements.

Question 10

Ataxia

Answer 10

Cerebellum and the brainstem. Poor balance, often with low trunk muscle tone.

Question 11

Rigid or dystonic

Answer 11

Basal ganglia and motor cortex. An increase in tone on voluntary movement, change of position or posture.

Question 12

Hemiballistic

Answer 12

Ballismus or ballism (called hemiballismus or hemiballism in its unilateral form) is a very rare movement disorder. It is a type of chorea caused in most cases by a decrease in activity of the subthalamic nucleus of the basal ganglia, resulting in the appearance of flailing, ballistic, undesired movements of the limbs.

Question 13

Monoplegia

Answer 13

One limb involved

Question 14

Hemiplegia

Answer 14

One side of the body involved

Question 15

Diplegia

Answer 15

Lower limbs

Question 16

Quadriplegia

Answer 16

Total body involvement

Question 17

Neurological examination of a child with cerebral palsy and spasticity will reveal sign of an upper motor neurone lesion:

Answer 17

Weakness
Poor control of fine movements
Increased tone
Brisk deep tendon reflexes 
Lack of voluntary control
Sensory impairment

Question 18

Hemiplegic cerebral palsy is frequently associate with

Answer 18

Haemorrhage or ischameia in the territory of the middle cerebral artery.

Question 19

Diplegic Cerebral Palsy is associated with

Answer 19

Damage to the periventricular white matter

Question 20

Quadriplegic Cerebral Palsy is associated with

Answer 20

Wide-spread damage in watershed areas between vascular territories and in severe cases may result in severe atrophy of the brain with cystic changes.

Question 21

Dynamic Contractures in Cerebral Palsy

Answer 21

selective spasticity of some muscles and weakness of others.

Question 22

Fixed muscle contractures

Answer 22

Persistent spasticity resulting in contractures, shortened muscle tendon units and the deformity cannot be overcome.

Question 23

Fixed contractures

Answer 23

joint subluxation/dislocation and secondary bone changes

Question 24

Orthopaedic Priorities in Cerebral Palsy

Answer 24

Spine (posture), hip, feet, torsional lower limb problems (profound gait abnormalities over time due to rotation of the hip joint

Question 25

Investigation of Cerebral palsy

Answer 25

Gait Analysis, Radiographys, MRI of Brain

Question 26

Gait analysis in Cerebral Palsy

Answer 26

Check the nearness to normal gait

Question 27

The spine in CP

Answer 27

Scolioisis is common. The severity parallels neurological involvement. Surgery conducted to maintain seating, respiration and to avoid rib and pelvic impingement.

Question 28

Gross Motor Function Classification System

Answer 28

1. Walks without limitation 2. Walks with limitation 3. Walking using hand held device 4. Self-mobility with limitations 5. Transported in a manual wheelchair. The higher the score, the higher the risk of dislocation

Question 29

Posture management CP

Answer 29

Physiotherapy, seating

Question 30

Spasticity management CP

Answer 30

``` Ð General • Baclofen • Diazepam Ð Specific • Botulinum toxin • Surgery ```

Question 31

Soft tissue release CP

Answer 31

Adductors, hamstrings

Question 32

Bony realignment CP

Answer 32

Varus Derotation Osteotomy | Pelvic Osteotomy

Question 33

Spina Bifida Prevelance

Answer 33

1:500

Question 34

Spina Bifida

Answer 34

Congenital condition caused by failure of the neural tube to close. The lumbar and sacral parts of the spine are most commonly affected and the neural abnormality results in lower motor neurone lesion. Associated with a partially or completely absent posterior arch of the affected vertebrae.

Question 35

Spina Bifida Occulta

Answer 35

Small defect in the neural arch of the vertebra with no underlying neural abnormality. Usually seen at the lumbosacral junction.

Question 36

Spina Bifida Meningocele

Answer 36

Cystic swelling of the meninges that protrudes through the vertebral defect

Question 37

Spina Bifiida Myelomeningocele

Answer 37

Meninges and the chord protrube through the defect

Question 38

Encephalocele

Answer 38

Encephalocele, sometimes known as cranium bifidum, is a neural tube defect characterized by sac-like protrusions of the brain and the membranes that cover it through openings in the skull.

Question 39

Anencephaly

Answer 39

Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development.

Question 40

Antenatal Screening of Spina Bifida

Answer 40

US of spine and AFP form serum, if necessary, amniotic fluid at about 16-20 weeks.

Question 41

Associated abnormalities of Spina Bifidia

Answer 41

Hydrocephalus Abnormal structure of the corpus callosum resuling in learning difficulties. Impaired walking

Question 42

Sacral Anomalies

Answer 42

High arched feet and weak calf muscles. Lurching gait due to hip abductor weakness.

Question 43

Lumbar Anomalies

Answer 43

Swing through gait.

Question 44

Muscular Dystrophy

Answer 44

Progressive muscle weakness. There is FSH, DMD and BMD. Predominant in males. Diagnosed by muscle biopsy, DNA testing, blood enzymes, and EMG.