U11C1 Cerebral Palsy Flashcards
What are the stages of development?
Direct vs indirect impact
What are the cognitive developmental stages?
What is the lateral corticospinal tract?
Upper vs lower motor neuron lesions
What is the pathophysiology of cerebral palsy?
Non-progressive brain injury that can occur due to hypoxia, infection, complicated birth and is associated with premature births. This damage leads to:
- Incomplete development of the brain (hypoplasia)
- Polymicrogyria (more folds than normal)
- Periventricular leukomalacia
- Subcortical atrophy
This brain damage means that motor neurons do not receive innervation from the brain. As the motor neurons are not innervated, they die = muscle atrophy. This leads to the build up of laminin (an ECM protein that links with dystrophin). In hemiplegic spasticity CP the damage would occur to one side of the motor cortex
How does periventricular leukomalacia occur?
It is the softening of white matter surrounding the ventricles due to lack of blood and oxygen. It is a result of hypotension, ischemia and coagulation necrosis and the border of the deep penetrating arteries of the middle cerebral artery - anterolateral central arteries. These arteries are uniquely sensitive to ischemic injury. After the initial insult, immature oligodendrocytes are injured. This leads to a deficit of mature, myelin-producing oligodendrocytes, causing cerebral hypomyelination. Can be imaged using ultrasound and MRI
What are the signs and symptoms of cerebral palsy?
What are the types of cerebral palsy and risk factors?
What is spastic CP?
What is athetoid CP?
What is ataxic CP?
What is hypotonic CP?
What are the differential diagnoses for CP?
Spinal muscular atrophy - where there is muscle weakness and atrophy
- Floppiness at birth and exhibits progressive weakness but no spasticity
- DNA testing for most subtypes, muscle biopsy, MRI can reveal muscle loss in lower extremities and electromyography
Muscular dystrophy - DMD or Becker’s MD
- no spasticity but can develop contractures, children may be weak from birth or may have apparent normal development until age 3, followed by progressive loss of function and muscle weakness, positive Gowers’ sign
- Muscle biopsy shows degeneration of muscle cells, creatine kinase levels raised, electromyogram identifies muscle rather than nerve disease
Familial/primary dystonia - a neurological disorder characterised by disabling, abnormal, involuntary movements
- Onset of muscular deformity after several years of development, presents with sustained periods of muscle contraction and dystonia (abnormal muscle tone resulting in muscle spasm and abnormal posture) as well as a positive family history
- Molecular genetic testing is available
Myelodysplasia/spinal bifida - a common group of congenital disorders caused by various chromosome abnormalities that lead to failure to close the foetal spinal cord
- Usually not spastic, associated with a spinal defect and lack of sensation below a relatively specific spinal segment
- Ultrasound and X-rays to confirm spine/spinal cord defects
Familial (hereditary) spastic paraparesis – a group of disorders that involves weakness and spasticity/stiffness of the legs which gets worse over time
- Family history and is very progressive
Spinal stenosis/tethered cord - narrowing of the spinal canal in the back
- Progressive neurological disease with worsening of the underlying neurology over time
- MRI of the spinal canal reveals the abnormality
Brain tumour
- Initial development can be normal, presents acutely with headache, raised intracranial pressure, seizures and focal neurological defects which affect a specific location
What are the diagnostic tests for CP?
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Deep tendon reflex- involuntary muscle contractions that occur in response to the sudden stretch of a muscle
1. The muscle first stretches, (e.g. because of tapping the tendon)
2. Sensory input to spinal cord
3. Motor command cause muscle contraction (LMN)- local spinal reflex
4. This helps to resist the force that caused the stretch and helps to maintain muscle tone
5. UMN controls LMN by activating and inhibiting it
6. Upper motor neurone lesions in cp mean the LMN can function without UMN.
7. Causing hyper reflexia , spasticity
8. Hyperactive tendon reflexes in CP - Ultrasound or MRI- detect periventricular leukomalcia
- Babinski sign- sole of the foot is stroked and the toes should curl down in a normal response, in CP the big toe will curl up
- Asterognosis- sensory deficit, children can be hypo or hypersensitive to touch and sounds, it is a deficit in the ability to recognise objects by touch without seeing them
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Scissor gait- muscle tone in adductor muscle is increased more in the lower limb (hypertonic and hyper reflexia)which causes the knees and thighs to hit. Gait patterns seen in spastic cerebral palsy are 4 types based on the muscle groups effected
Type 1: drop foot , in the swing phase of gait cycle, inability to control ankle dorsi flexion.
Type 2: equinus where the upward bending of the ankle joint is limited . Because of the increased spasticity of the gastroc soles muscle
Type 3: Hemiplegia ; Stiff knee gait, the quadriceps are contracted,
Type 4: Hemiplegia unilateral involvement , marked asymmetry , equines and flexed hip
What is a CMFSC?
(gross motor function classification system)
It provides families and health care professionals with:
- a description of the child’s current motor function,
- an idea of assistive equipment or mobility aids that the child may need in the future (e.g., crutches, walking frames, wheelchairs).
- The focus is on determining which level best represents the present abilities and limitations in gross motor function. Emphasis is on usual performance in home, school, andcommunity settings (i.e., what they do), rather than what they are known to be able to do at their best (capability). It is therefore important to classify current performance in gross motor function and not to include judgments about the quality of movement or prognosis for improvement.
