Paediatric Neuromuscular Conditions Flashcards
A child or young person is defined as having Complex Exceptional Needs if:
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
Cerebral Palsy
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.
How many children with complex needs are accounted for by cerebral palsy
7%
Epidemiology of Cerebral Palsy
Occurs in 2/1000 births.
Perinatal risk factors for cerebral palsy
Prematurity, anoxic injuries, infections, kernicterus (a rare but serious complication of untreated jaundice in babies), erythroblastosis fetalis (abnormal presence of erythroblasts in the blood)
Prenatal risk factors for cerebral palsy
Placental insufficiency, toxaemia, smoking, alcohol, drugs, infection (TORCH)
Postnatal risk factors for cerebral palsy
Infections (cytomegalovirus, rubella), head trauma.
Spastic
Effects the pyramidal system and or the motor cortex. There will be an increase of tone on passive stretch of muscle.
Athetoid (dyskinetic)
Effects the extrapyramidal system and or the basal ganglia. Combination of spastic and dystonic with involuntary movements.
Ataxia
Cerebellum and the brainstem. Poor balance, often with low trunk muscle tone.
Rigid or dystonic
Basal ganglia and motor cortex. An increase in tone on voluntary movement, change of position or posture.
Hemiballistic
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.
Monoplegia
One limb involved
Hemiplegia
One side of the body involved
Diplegia
Lower limbs
Quadriplegia
Total body involvement
Neurological examination of a child with cerebral palsy and spasticity will reveal sign of an upper motor neurone lesion:
Weakness Poor control of fine movements Increased tone Brisk deep tendon reflexes Lack of voluntary control Sensory impairment
Hemiplegic cerebral palsy is frequently associate with
Haemorrhage or ischameia in the territory of the middle cerebral artery.
Diplegic Cerebral Palsy is associated with
Damage to the periventricular white matter
Quadriplegic Cerebral Palsy is associated with
Wide-spread damage in watershed areas between vascular territories and in severe cases may result in severe atrophy of the brain with cystic changes.
Dynamic Contractures in Cerebral Palsy
selective spasticity of some muscles and weakness of others.
Fixed muscle contractures
Persistent spasticity resulting in contractures, shortened muscle tendon units and the deformity cannot be overcome.
Fixed contractures
joint subluxation/dislocation and secondary bone changes
Orthopaedic Priorities in Cerebral Palsy
Spine (posture), hip, feet, torsional lower limb problems (profound gait abnormalities over time due to rotation of the hip joint
Investigation of Cerebral palsy
Gait Analysis, Radiographys, MRI of Brain
Gait analysis in Cerebral Palsy
Check the nearness to normal gait
The spine in CP
Scolioisis is common. The severity parallels neurological involvement. Surgery conducted to maintain seating, respiration and to avoid rib and pelvic impingement.
Gross Motor Function Classification System
- Walks without limitation
- Walks with limitation
- Walking using hand held device
- Self-mobility with limitations
- Transported in a manual wheelchair.
The higher the score, the higher the risk of dislocation
Posture management CP
Physiotherapy, seating
Spasticity management CP
Ð General • Baclofen • Diazepam Ð Specific • Botulinum toxin • Surgery
Soft tissue release CP
Adductors, hamstrings
Bony realignment CP
Varus Derotation Osteotomy
Pelvic Osteotomy
Spina Bifida Prevelance
1:500
Spina Bifida
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.
Spina Bifida Occulta
Small defect in the neural arch of the vertebra with no underlying neural abnormality. Usually seen at the lumbosacral junction.
Spina Bifida Meningocele
Cystic swelling of the meninges that protrudes through the vertebral defect
Spina Bifiida Myelomeningocele
Meninges and the chord protrube through the defect
Encephalocele
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.
Anencephaly
Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development.
Antenatal Screening of Spina Bifida
US of spine and AFP form serum, if necessary, amniotic fluid at about 16-20 weeks.
Associated abnormalities of Spina Bifidia
Hydrocephalus
Abnormal structure of the corpus callosum resuling in learning difficulties.
Impaired walking
Sacral Anomalies
High arched feet and weak calf muscles. Lurching gait due to hip abductor weakness.
Lumbar Anomalies
Swing through gait.
Muscular Dystrophy
Progressive muscle weakness. There is FSH, DMD and BMD. Predominant in males. Diagnosed by muscle biopsy, DNA testing, blood enzymes, and EMG.
