U11W1: Cerebral Palsy

Question 1

What is cerebral palsy?

Answer 1

Is a clinical description of children who share features of a non-progressive brain injury or legion acquired during the antenatal, perinatal or early postnatal period.
Often presents as difficulty in motor function, also high risk of problems with other senses, communication and intellectual disability.

Question 2

What are the risk factors of cerebral palsy?

Answer 2

Majority of the causes are congential meaning developed before birth, many of the specific reasons are unknown.
Preterm birth
Low birth weight
Perinatal ischaemic stroke
Multiple gestations
Placental abnormalities
Fetal growth retardation
Hyperbilurubinaemia - often from reactivity between maternal and infant blood e.g rhesus factor.
Birth asphyxia - often due to complicated vaginal delivery.
Infections during pregnanacy of early infancy
Injury to babies head
Hypoglycemia of baby

Question 3

What is the genetic link behind cerebral palsy?**

Answer 3

FBX031- regulating cell cycle and neuronal migration
RHOB - signals for cell proliferation, mutation cause cause apoptosis in neuron - normally spontaneous.
DHX32 -regulates proliferation through beta-catenin, increases invasive potential
ALK - nervous system development

Increases risk or predisposition not causative.

Question 4

What are the common signs and symptoms of cerebral palsy?

Answer 4

Most CP cases are spastic
Delays in reaching developmental milestones
Difficulties with muscle and coordination of fine and gross movement - including muscle tone, spasticity, ataxia, tremors
Difficulty in speech and eating
Intellectual disabilities
Impaired senses, sight, hearing etc.
Delayed social development or behavioural problems.
Bladder control problems
Pain sensations.

Question 5

What are some common complications of cerebral palsy?

Answer 5

Contracture - muscle shortening due to severe muscle tension, this can affect bone growth and joint structure
Malnutrition - due to difficulty swallowing and feeding, impair growth and infant health, some may need a feeding tube
Mental health conditions - social isolation, challenges of coping, behavioural issues
Heart and lung disease - difficulty swallowing increases risk of aspiration pneumonia.
Osteoarthiritis - from pressure or misalignement of joints

Question 6

What is the normal gait?

Answer 6

Consits of a stance phase (foot on floor) and a swing phase (foot off floor)
Stance (60%): heel strike, flat foot, midstance, heel off, toe off
Swing: middle swing (split into acceleration up until middle and desceleration down towards the ground), heel strike. (40%)

Question 7

What are the different terms used in the new gait cycle?

Answer 7

Intial contact (heel strike)
Loading response (shifting weight from one foot to another from heel strike to other foot off ground)
Mid stance - other foot off and up
Terminal stance - other foot coming down
Preswing - from heel off to toe off
Intial swing - foot off and up
midwing - foot coming down
Terminal swing - toe down to heel stike

Question 8

What are some common gait abnormalities?

Answer 8

Trendelenburg gait
Antalgic gait
Ataxis gait
Parkinsonian gait
Hemiplegic gait (foot drop)
Steppage gait (foot drop)
Diplegic gait (spasticity, tiptoeing and toe dragging)

Question 9

What is a trendelenburg gait?

Answer 9

When standing on the leg with the effected gluteus medius/minimus muscle the contralteral side of the pelvis will drop
Defective hip abductor.
Contrlateral Toe will drag when not compensated, or lean to side of affected muscle to compensate

Question 10

What is an antalgic gait?

Answer 10

Abnormal pattern of walking secondary to pain - causes a limp.
Stance phase is shortend on affected leg relative to swing phase

Question 11

What is an ataxic gait?

Answer 11

Poor cordination of the lower limbs
Changing step length
Foot often crosses midline
Stumbling
Wide stance
High risk of falls
May sway from side to side when standing still - this is known as titubation.

Question 12

What is a parkinsonian gait?

Answer 12

Small shuffling steps
Struggle to pick up feet
Usually hunched posture
Slow to initiate movement

Question 13

What are the features of a hemiplegic gait?

Answer 13

Effects the upper and lower limbs on only one side of the body
Unilateral weakness of the affected side, arm flexed, adductoed and internally roated.
When walking tends to hold arm close to body and drags affected lef in a semicircle, due to weakness of distal muscles (often also has foot drop)

Question 14

What is a steppage gait?

Answer 14

Inability to lift the foot while walking due to the weakness of muscles that cause dorsiflexion of the ankle joint (anterior leg)

Question 15

What is a diplegic gait?

Answer 15

Patient have involvement on both sides with spasticity in lower extremities worse than upper extremities
Patiesn walk with an abrnoamlly wide base, dorgging both legs and scrapping the toes.
Flexion of hip and knees, tight adduction of the knee, normally internally rotates feet so circular swining of legs and midgard position of upper extremetiies.

Question 16

What are the key brain changes that may be seen on cerebral palsy?

Answer 16

Identified by an MRI
Hypoplasia - incomplete developement of the coretx, low cell division
Polymicrogyria - to many gyri which are abnormally small
Periventricular leukomalacia - white matter surrounding the ventricles is deprived of blood or oxygen, can be associated with ischemia, inflammation and tissue vulnerability, causes death or sfotening of the white matter, causes loss of motor neuron tracts. Appears on MRI with an abnormally high signal intensity.

Question 17

What are the different types of cerebral palsy?

Answer 17

Spastic (most common)
Dyskinetic
Ataxic
Mixed
Hypotinic

Question 18

What is spastic cerebral palsy?

Answer 18

Spastic (primary motor cortex is affected so damaged UMNL) can be hemiplegic, diplegic or quadriplegia
Is the most common type and the patient normally presents with
Hyperreflexia, increased deep tendon reflexes, clonus, increased muscle tone and muscle weakness.

Question 19

What is dyskinetic cerebral palsy?

Answer 19

Primarily injury of the basal ganglia
Unable to initiate movement or prevent unwanted movmenet
Chorea - jerky, rhytmic movement
Athetosis - involutnary writing movement
Dystonia - sustain involuntary muscle contractions - presents as slow reptitive movment or abnormal posture (painful)

Question 20

What is ataxic cerebral palsy?

Answer 20

INjury to the cerebellum
Loss of tone, blance, coordination and posture
Can present with wide based giat and loss of balance (ataxia), dysdiadochokinesia (inability to perform rapidly alternative muscle movements)
Dysmetria - abnormal finger to nose test. (no direction or range control over movement)
Hypotonia and muscle weakness.

Question 21

What is hypotonic cerebral palsy?

Answer 21

Low muscle tone
Difficult to generate movement
Require more support surface contact.
Can be precuros for spastic or dyskinietic CP

Question 22

What are the features of an upper motor neuron lesion?

Answer 22

Muscle weakness
Midl atrophy if any
No fasciculations
Positive Babinski sign
Increased reflexes
Increased muscle tone/spasticity

Question 23

What are the features of a lower motor neuron lesion?

Answer 23

Muscle weakness
Muscle atrophy
Fasciculations
Decreased reflexes
Decreased muscle tone

Question 23

Describe the structure of tendons.

Answer 23

Dense Regular Fibrous Connective tissue that joins muscle to bones.
Consists of mainly type 1 collagen and parallel fibroblasts (tenoblasts or tenocytes) which continuously produce ECM, alongside elastin and type 3 collagen.
Relatively well organised and can withstand greater tensile force
Surrounded by a loose connective tissue sheath the epitenon
Endotenon - subdivides the tendon internally into fascicles that act as connective tissue septae.

Question 23

What is the function of the corticospinal tracts?

Answer 23

Anterior - motor innervation to the axis (trunk)
Lateral - motor innervation to the limbs

Question 24

What are some important ligaments/tendons around the knee?

Answer 24

Patella tendon
Quadriceps tendon
Medial collateral ligament
Lateral collateral ligament
Anterior Cruciate ligament
Posterior Cruciate ligament
Medial and lateral meniscus ligament
Transverse ligament of the knee

Question 25

What is a deep tendon reflex?

Answer 25

A rapid stretch of muscle stimulates the muscle spindles’ intrafusal muscle fibre to project into the spinal cord and synapse onto a motor neuron causing contraction of the muscle.
Combined with reciprocal inhibition of antagonist.
Can become faulty if there is damage to the lower motor neuron or the the sensory afferent.

Question 26

What are the different deep tendon reflexes in the lower limb?

Answer 26

Knee jerk
Ankle jerk
Plantar reflex

Question 27

What is the knee jerk reflex in the lower limb?

Answer 27

Tap the patella tendon - test the quadriceps femoris muscles
Nerve route L2,3,4 Femoral nerve
Expected result is extension of the knee joint

Question 28

What is the ankle jerk reflex?

Answer 28

Tests the gastrocnemius and soleus muscle - Tibial nerve S1,2
Tap the achilles tendon behind ankle joint
Expected reflex is plantar flexion of the ankle

Question 29

What is the plantar reflex in the lower limb?

Answer 29

Use the end of a tendon hammer to scrape the lateral plantar edge of the foot from the heel to the fifth toe (curve-like pattern)
Look for flexion of the toes
Abnormal extension of the hallucis and fanning out of the toes indicates a pathological positive Babinski sign.
Tests L5,S1,S2

Question 30

What are the key signs of cerebral palsy on an MRI?

Answer 30

1. Hypoplasia - incomplete development of cortex (smaller or parts missing)
2. Polymicrogyria - too many gyri that are unusually small
3. Periventricular leukomalacia - bleeding into white matter tracts, appears white and bright on an MRI

Question 31

Describe how a tendon can be broken down into smaller sections

Answer 31

Tendon - surrounded by a fibroblastic paratenon - elastic sleeve to aid free gliding movement.
Underneath, tendon is surrounded by thin epitenon (connective tissue)
The epitenon and paratenon are sometimes referred to as the peritendon.
Split into tertiary fibre bundles
Split into a fascicle
Split into a fiber
Split into a fibril
Endotendon is connective tissue throughout the inside structure of the tendon (continuous with the epitenon).

Question 32

How are Upper motor lesions detected on an MRI?**

Answer 32

Can identify potential causative factors of an UMNL
Brain and spinal cord tumours
Inflammation/Infection
Vascular irregularities (risk of stroke)
Structural changes in the brain such as necrosis or periventricular leukomalacia.

Question 33

How does a lower motor neuron lesion present on an EMG?**

Answer 33

Rapdily firing reduced polyphasic motor unit action potentials.
Overall decreases electrical activity
Increased nerve conduction time.
Decreased electrical activity during a reflex

Question 34

How does an Upper motor neuron lesion present on an EMG?**

Answer 34

Increased electrical activity during a deep tendon reflex.
Nerve conduction will be normal.
Slower firing reduced polyphasic motor unit action potentials.

Question 35

How is a lower motor neuron lesion present on an MRI?**

Answer 35

Lack of abnormalities in central nervous system that could explain symptoms.
Damage to nerves in the periphery, particularly where they leave the spinal cord.
Identify compression on nerves from tumours etc.

Question 36

What is the role of Botox in cerebral palsy?

Answer 36

Treatment for spasticity - can reduce muscle tone around the elbow, wrist and thumb areas - allows joints to straighten
This aids mobility and reduces the risk of fixed contractures, bony abnormalities and joint instability.
Is a muscle relaxant

Question 37

What is the mechanism of action of botox in cerebral palsy?

Answer 37

Chemistry: is a muscle relaxant derived from the bacterium clostridium botulinum.

Pharmacology: heavy chain binds to receptors on motor nerve terminals, forms a toxin receptor complex that is taken up by endocytosis.
Light chain is cleaved from toxin and interacts with SNAP-25 components of the SNARE complex responsible for fusion of acetylcholine vesicles
inhibits acetylcholine release.

Physiology: inhibits neural signals that cause muscle contraction, ACh is unable to bind to nicotinic receptors so muscle contraction is not initiated.

Clinical: acts as a muscle relaxant, overcomes spasticity in cerebral palsy. is temporary as is overcome by regeneration of proximal axons and neuromuscular junctions so treatment should be repeated every few months.

Question 38

What is the purpose of casting in cerebral palsy treatment?

Answer 38

Is a non-invasive treatment that aims to gently stretch contracted muscles over a period of time - prevent tightening of muscles and increases motion.
Applies soft plaster cast for 7 - 14 days.
Repeated process up to 8 weeks
Accompanied by physiotherapy
Increases range of motion by holding in a position of stretch for a prolonged period of time.

Question 39

What is the mechanism behind casting in cerebral palsy?

Answer 39

A soft cast holds the muscle in a prolonged stretch
This increases muscle fibre tension and strength - leading to an increase in serial sarcomeres
Realignment of muscle and collagen fibres in connective tissue Stimulates the growth of the muscle
Stretch must be maintained or the muscle will re-shorten.

Question 40

What is the role of an ankle-foot orthosis in cerebral palsy?

Answer 40

Is a custom-made or prefabricated brace designed to control the range of movement of a joint (ankle joint) and hold within an optimum position.
Hard and fixed structure
Commonly addresses foot drop, incorrect joint position, ankle instability and gait abnormalities.
Are removable and can be worn inside or of shoes.

Question 41

What is the role of baclofen in cerebral palsy?

Answer 41

Is a muscle relaxant use in chronic severe conditions.
Relive muscle spasticity, cramps or spasms and indirectly reduce the pain associated with this.

Question 42

What is the role of diazepam in cerebral palsy?

Answer 42

Used to treat muscle spasm of varied aetiology, including acute muscle spasm, tetanus, spastic cerebral palsy or in anxiety.
Relief of muscle spasms can increase mobility and reduce pain in children with cerebral palsy.

Question 43

What is meant by single-event multiple level orthopaedic surgery?

Answer 43

Procedure on the lower extermities where multiple corrections for soft tissue and bone problems are done in one orthopaedic surgery.
This leads to fewer phases of rehabilitation, fewer hospital admissions, less procedures requiring anaesthetic - which tends to reduce secondary complications.

Question 44

What is the role of a tendon and how is it suited to this function?

Answer 44

The role is to transmit high tensile force from muscle to bone.
Adapted as approximately 70% longitudinally arranged collagen which provides strength to the tissue, some elastin - this means are relatively inextensible to can withstand high tensile forces.
This is suited to their function to be exposed to strong unidrectional forces.

Question 45

Why do tendons take a long time to heal?

Answer 45

Avascular - decreases rate of rehabilitation and healing.
Stable tissue

Question 46

What is the mechanism of action of baclofen in cerebral palsy treatment?

Answer 46

Chemistry: GABA agonist - anti-spasmodic
Pharmacology: agonist of GABA B receptors on pre and post synaptic neurons in the CNS.
Post synpatic - Causes a rapid opening of K+ channels (K+ efflux) causing hyperpolarisation of the neuronal membrane,
Presynaptic - Inhibits Ca2+ mobilisation reduces excitatory NT release.
Inhibits the transmission of electrical activity between the upper and lower motor neuron in the ventral horn
Physiology: reduces the electrical activity of the alpha motor neuron ( less activation from UMN), resulting in decreased contraction of the extrafusal muscle fibres reducing spasticity.
Clinical: indicated to reduced chronic and severe spasticity or muscle stiffness, indirectly reduces the pain associated with this.
In children with CP - reduce muscle tone and often improves mobility.

Question 47

What is the mechanism of action of diazepam in cerebral palsy?

Answer 47

Chemistry: Is a benzodiazepine, long-acting with rapid onset.
Pharmacology: Allosteric regulation binding to the gamma subunit of GABA A receptor increases the activity of GABA receptors as more likely activated by binding of GABA - increases the duration of Cl- channel opening - hyperpolarisation - decreases neuronal transmission as less likley to fire an action potential.
This effect is in the CNS.
Physiology: less muscle contraction, and reduced spasticity as electrical activity is not passed from the upper motor neuron lesion to the lower motor neuron lesion.
Clinical: adults with cerebral palsy - to reduce spastic symptoms.

Question 48

What is the guidance regarding administering diazepam and/or baclofen to a patient with cerebral palsy?

Answer 48

Adults: baclofen is the first line, diazepam should only be given in acute situations when spasticity is causing severe pain or anxiety.

Question 49

What surgeries normally take place in Single-Event Multilevel Surgery?

Answer 49

Selective Dorsal Rhizotomy
Calf muscle lengthening
Tendon lengthening
Osteotomy

Question 50

What is a selective dorsal rhizotomy?

Answer 50

Cutting some of the sensory nerve roots at the level of the spinal cord, dorsal nerve roots are split into rootlets which are stimulated by electromyography and tested for spasticity the most abnormal are cut, some remain to keep other sensory and motor functions, reducing input from sensory fibres - reduces spasticity and stiffness
Irreversible

Question 51

What happens in calf muscle lengthening?

Answer 51

A cut is made on the back of he calf and part of the gristle (aponeurosis or fibrous tissue surrounding the gastrocnemius) is cut, this allows the muscle to lengthen which is promoted by stretching and physio this reduces tightness and improves motion.

Question 52

What happens in tendon lengthening?

Answer 52

Purpose: reduce painful contractures and increase motion
Achilles tendon - inacreases ability to walk
1) Percutaneous method - small incisions in tendon, cut areas move apart elongating the tendon
2)Z-plasty - Z shaped incision in tendon, lengthened and rejoined back together.

Question 53

What happens in an osteotomy?

Answer 53

Realign joints for better posture and mobility
Moves bones to angles more conducive to healthy alignments better for posture and mobility, commonly corrects hip dislocations.

Question 54

What is the role of paediatric care and clinics?

Answer 54

Paediatricians are specifically trained to provide medical care to children.
Normally until age 16yrs, then up to 18yrs in specialist conditions.
Offer inpatient, outpatient and emergency services.
Will contain specialities within paediatrics such as cardiologist, gastroenetrologist, immunologist.
May overlap with role of neonatologist - who cares for newborns up to one year old.

Question 55

What is the role of occupational therapy in cerebral palsy?

Answer 55

Role is slightly different as work with children (so focus on school, home and life goals rather than work)
Aid with independence - teach about adaptations and strategies for everyday tasks such as getting dressed, cooking and bathing.
May also look at family life and social aspexts such as techniques to cope in socially overwhelming situations.
THis also benefits parents/ care givers - by reducing demand, provide sense of security and hope for normality for their child.

Question 56

What are some techniques that may be used by an occupational therapise for a child with cerebral palsy?

Answer 56

Padiatric constrina induced movement therapy - restrain strong arm to encourage moving weaker
Sensory integration therapy - increase sensory experiences such as carpet, sand and water
Adapation tools - button hooks, zipper pulls
Assistive devices - computer softwater, pencil grips, bathing aids, games and toys that help with motor and cognitive development
All developmental branches will be assessed by the occupational therapist, interview parent and child to find out goals. strengths and weaknesses - reviewed every 6-9 months

Question 57

What is the psychosocial impact of disability on the child?

Answer 57

Social isolation - physically unable to join in activities or not being welcomes due to discrimination
Central to self-identity - struggle to familiarise with peers, struggle to think of self outside of illness, perceive self as having a low self value
More likely victim of hate crime or bullying - poor self-esteem and social anxiety
Embarrassed or scared - reduce help-seeking behaviour, quiet and reserved in characteristics.

Question 58

What are the impacts of childhood disability on the parent of the child?

Answer 58

Fear for child - bullying and barriers they will face, blame themselves for conditions (genetic etc)
Grief for future the child should have had - loss of perfect child
Socially isolating - loss of contact with friends, find it difficult to be understood by others
Socially - harder to find child support, economic and time burden.

Question 59

What is an EHCP?

Answer 59

Is an Education, Health and Care Plan
Legal document describing child/young persons individualised special educations needs and disbailities and used this to describe the support that is needed to meet these needs.
Meet needs in school, preparing for adulthood

Question 60

What is the application process for an EHCP?

Answer 60

Applied for is child has a long term special educational need or disability that will not be met by ordinary provisions.
Assessment is completed by SEND panel and reviewed annually.
Application can be submitted by young person (over 16yrs), carer, parent or school on their behalf.
Can take up to twenty weeks to be approved and start to put in place - must be repeated in any new council district.

Question 61

What can be put in place to help a child/young person with a disability?

Answer 61

EHC - by local council
IEP - done by the school, allows the school to assess if they can fulfil the child’s needs themselves, often managed by the school’s special educational needs co-ordinator.
Disabled Student Allowance - for higher education students with a health problem, disability or learning difficulty - support student-related costs e.g technology to aid learning, travel costs, BSL interpreter, financial aid, requires a needs assessment - separate to student finance application.

Question 62

What help is required of schools to help children with disabilities?

Answer 62

Equality act means all schools are required to make all reasonable adjustments such as a ramp, changes to assessments, specialist teachers or equipment.
Schools often have a special education needs co-ordinator - help identify and support children with special needs and liase with parents and council

Question 63

What are the features of education tailored to children with special needs?

Answer 63

Alternative provision education - education outside of school arranged by local authorities or schools, is full time. Often has smaller pupil numbers and can be in specialised enviornments such as community centres

Special needs school - additional equipment, smaller class sizes and teachers trained to deal with health conditions, adapted curriculum

Question 64

What are some support groups for cerebral palsy?

Answer 64

NCL United Foundation - football sessions specially adapted with trained staff for children with disabilities.
CureCP - provides access to information leaflets and fundraisers to support research into treatments of cerebral palsy.
March of Dimes - supports health and wellness of new mothers and babies, and provides cummunity support for mothers who are experiencing raising children with special needs.
The brain charity - provide emotional support such as counselling, phone befirending, group therapy and social activities.