Nervous System Flashcards
Definition of CP:
- Permanent disorder of motor and posture development
- Caused by non-progressive insult to developing brain (occurring in the developing brain)
- Can be associated with other developmental difficulties and epilepsy.
When does the damage in CP occur?
For 50-75% of children with cerebral palsy, the causative lesion occurs between 24 weeks of postmenstrual age and term. It can occur during the birth and postnatal period also though.
Antenatal risk factors for CP
preterm birth, with risk increasing with decreasing gestational age (cerebrovascular haemorrhages/ ischaemia)
chorioamnionitis
maternal respiratory tract or genito-urinary infection treated in hospital
Intrauterine infections- TORCH
Congenital syndroms/ malformations
Teratogen consumption e.g. smoking, alcohol , drugs
Multiple births
Periventricular leukomalacia
Name some perinatal risk factors for CP:
low birth weight
chorioamnionitis
neonatal encephalopathy
neonatal sepsis (particularly with a birth weight below 1.5 kg)
maternal respiratory tract or genito-urinary infection treated in hospital
Name a postnatal risk factor for CP:
meningitis 20% other infections head injury hypoglycaemia hyperbilirubinaemia
Name the four types of CP:
Spastic type - there may be intermittently increased tone and pathological reflexes. 70%
Athetoid - this is characterised by increased activity (hyperkinesia). This has been described as ‘stormy movement’.
Ataxic type - there may be a loss of orderly muscular co-ordination so that movements are performed with abnormal force, rhythm or accuracy.
Mixed - there may be a combination of several forms.
Presentations of CP:
- unusual fidgety movements or other abnormalities of movement, including asymmetry or paucity of movement
- abnormalities of tone, including hypotonia (floppiness), spasticity (stiffness) or dyskinesia (fluctuating tone)
- abnormal motor development, including late head control, rolling, and crawling
- feeding difficulties.
What are the most common developmental delays in children with CP?
- not sitting by 8 months (corrected for gestational age)
- not walking by 18 months (corrected for gestational age)
- early asymmetry of hand function (hand preference) before 1 year (corrected for gestational age).
What is the most common to least common types of CP?
Bilateral spastic cerebral palsy is the most prevalent, followed by unilateral or hemiplegic cerebral palsy. Bilateral dystonic cerebral palsy is the least common.
Associated potential problems with CP:
- Learning disability (IQ below 70) occurs in around 1 in 2. Severe learning disability (IQ below 50) occurs in around 1 in 4.
- Communication difficulties occur in around 1 in 2 children and young people with cerebral palsy. At least 1 in 10 need augmentative and alternative communication because of cognitive and sensory impairments and communication difficulties.
- Emotional and behavioural difficulties (eg, low self-esteem) are reported in up to 1 in 4 children and young people with cerebral palsy. Including depression, anxiety and conduct disorders, challenging behaviours, problems with peer relationships, neurodevelopmental disorders, including autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD).
- Around 1 in 2 children will have some form of visual impairment. Hearing impairment occurs in around 1 in 10.
- Pain is common in people with cerebral palsy, especially those with more severe motor impairment. Common condition-specific causes of pain, discomfort and distress include: MSK problems (eg, scoliosis, hip subluxation and dislocation), Increased muscle tone (including dystonia and spasticity), Muscle fatigue and immobility.
- Vomiting, regurgitation and gastro-oesophageal reflux are common in children and young people with cerebral palsy. Around 3 in 5 children and young people with cerebral palsy have chronic constipation.
Epilepsy occurs in around 1 in 3 children with cerebral palsy. It is reported in around 1 in 2 children with dyskinetic cerebral palsy.
- Eating, drinking and swallowing difficulties and poor nutrition: failure to thrive and malnourishment.
- Bladder problems: incontinence and infections are common.
- Sleep disturbances: for example, fragmented sleep, which can occur in up to 50% of children.
- Drooling associated with poor saliva control.
-Orthopaedic problems: eg, progressive joint contractures, shortened muscles, hip or foot deformities and scoliosis.
Increased risk of low bone mineral density and therefore fractures due to osteomalacia or osteoporosis.
Red flag indicators for neurological disorders other than cerebral palsy include:
- absence of known risk factors (see risk factors)
- family history of a progressive neurological disorder
- loss of already attained cognitive or developmental abilities
- development of unexpected focal neurological signs
- MRI findings suggestive of a progressive neurological -disorder
- MRI findings not in keeping with clinical signs of cerebral palsy.
How is a diagnosis of CP made?
The diagnosis of cerebral palsy is based on clinical examination and parental observation. However, investigations occasionally required to exclude other diagnoses might include:
- Thyroid studies.
- Chromosomal analysis.
- Pyruvate and lactate levels to exclude mitochondrial cytopathies
- Organic and amino acid levels to exclude inborn errors of metabolism presenting with neurological symptoms
- Cerebrospinal fluid - protein, lactate and pyruvate levels may be helpful in determining whether there has been any asphyxia in the neonatal period.
Out line the GrossMotor Function Classification System level I:
GMFCS Level I – walks without limitations.
Out line the GrossMotor Function Classification System level II:
GMFCS Level II – walks with limitations. Limitations include walking long distances and balancing, but not as able as Level I to run or jump; may require use of mobility devices when first learning to walk, usually prior to age 4; and may rely on wheeled mobility equipment when outside of home for traveling long distances.
Out line the GrossMotor Function Classification System level III:
GMFCS Level III – walks with adaptive equipment assistance. Requires hand-held mobility assistance to walk indoors, while utilizing wheeled mobility outdoors, in the community and at school; can sit on own or with limited external support; and has some independence in standing transfers.
Out line the GrossMotor Function Classification System level IV:
GMFCS Level IV – self-mobility with use of powered mobility assistance. Usually supported when sitting; self-mobility is limited; and likely to be transported in manual wheelchair or powered mobility.
Out line the GrossMotor Function Classification System level V:
GMFCS Level V – severe head and trunk control limitations. Requires extensive use of assisted technology and physical assistance; and transported in a manual wheelchair, unless self-mobility can be achieved by learning to operate a powered wheelchair.
What type of gait would a patient with hemiplegic cerebral palsy have?
Circumduction gait- Due to excessive hip abduction as the leg swings forward creating a semi-circular movement of the leg.
What type of gait would a patient with di/ quadriplegic cerebral palsy have?
spastic gait
Stiff walking and the foot is seen to be inverted and dragged along. Often accompanied by flexion of upper limbs.
What type of gait would a patient with ataxic cerebral palsy have?
Ataxic gait
Instability and alternating between a narrow to wide base of gait.
What type of gait would a patient with diplegic cerebral palsy have?
Toe-walking gait (“equinus”). Can get scissoring of legs
Walking on tip toe with lack of heel contact (also seen in Duchenne’s)
Pathology of spastic CP:
In this type, there is damage to the upper motor neurone (pyramidal or corticospinal tract) pathway. Limb tone is persistently increased (spasticity) with associated brisk deep tendon reflexes and extensor plantar responses. The tone in spasticity is velocity dependent.
- Limb involvement is increasingly described as unilateral or bilateral to acknowledge asymmetrical signs.
- Spasticity tends to present early and may even be seen in the neonatal period. Sometimes there is initial hypotonia, particularly of the head and trunk.
-Developmental problems and the child with special needs
increased tone soon emerges as the predominant sign.
What are the three main types of spastic CP?:
- unilateral (hemiplegia) – unilateral involvement of the arm and leg. The arm is usually affected more than the leg, with the face spared. Affected children often present at 4–12 months of age with fisting of the affected hand, a flexed arm, a pronated forearm, asymmetric reaching, hand function or toe pointing when lifting the child. Subsequently, a tiptoe walk (toe–heel gait) on the affected side may become evident. Affected limbs may initially be flaccid and hypotonic, but increased tone soon emerges as the predominant sign. Often no history of ischaemic event and unrecognised antenatal cause may be assumed.
- bilateral(quadriplegia)–all four limbs are affected, often severely. The trunk is involved with a tendency to opisthotonus (extensor posturing), poor head control and low central tone. This more severe form of CP is often associated with seizures, microcephaly and moderate or severe intellectual impairment. There may have been a history of perinatal hypoxic-ischaemic encephalopathy.
- bilateral(diplegia)–all four limbs,but the legs are affected to a much greater degree than the arms, so that hand function may appear to be relatively normal. Motor difficulties in the arms are most apparent with functional use of the hands. Diplegia is one of the patterns associated with preterm birth due to periventricular brain damage. The MRI brain scan may show periventricular leukomalacia.
Pathology of dyskinetic CP:
Perinatal asphyxia – particularly affecting the basal ganglia. Also kernicterus, but this is now rare.
Dyskinesia refers to movements that are involuntary, uncontrolled, occasionally stereotyped and often more evident with active movement or stress. Muscle tone is variable and primitive motor reflex patterns predominate. May be described as:
• chorea–irregular, sudden and brief non-repetitive movements
• athetosis–slow writhing movements occurring more distally such as fanning of the fingers
• dystonia–simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles often giving a twisting appearance.
Intellect may be relatively unimpaired. Affected children often present with floppiness, poor trunk control and delayed motor development in infancy. Abnormal movements may only appear towards the end of the first year of life.
