U11W1: Cerebral Palsy Flashcards

1
Q

What is cerebral palsy?

A

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.

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2
Q

What are the risk factors of cerebral palsy?

A

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

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3
Q

What is the genetic link behind cerebral palsy?**

A

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.

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4
Q

What are the common signs and symptoms of cerebral palsy?

A

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.

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5
Q

What are some common complications of cerebral palsy?

A

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

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6
Q

What is the normal gait?

A

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%)

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7
Q

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

A

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

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8
Q

What are some common gait abnormalities?

A

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

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9
Q

What is a trendelenburg gait?

A

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

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10
Q

What is an antalgic gait?

A

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

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11
Q

What is an ataxic gait?

A

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.

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12
Q

What is a parkinsonian gait?

A

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

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13
Q

What are the features of a hemiplegic gait?

A

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)

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14
Q

What is a steppage gait?

A

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

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15
Q

What is a diplegic gait?

A

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.

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16
Q

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

A

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.

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17
Q

What are the different types of cerebral palsy?

A

Spastic (most common)
Dyskinetic
Ataxic
Mixed
Hypotinic

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18
Q

What is spastic cerebral palsy?

A

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.

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19
Q

What is dyskinetic cerebral palsy?

A

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)

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20
Q

What is ataxic cerebral palsy?

A

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.

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21
Q

What is hypotonic cerebral palsy?

A

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

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22
Q

What are the features of an upper motor neuron lesion?

A

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

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23
Q

What are the features of a lower motor neuron lesion?

A

Muscle weakness
Muscle atrophy
Fasciculations
Decreased reflexes
Decreased muscle tone

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23
Q

Describe the structure of tendons.

A

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.

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23
Q

What is the function of the corticospinal tracts?

A

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

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24
Q

What are some important ligaments/tendons around the knee?

A

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

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25
Q

What is a deep tendon reflex?

A

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.

26
Q

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

A

Knee jerk
Ankle jerk
Plantar reflex

27
Q

What is the knee jerk reflex in the lower limb?

A

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

28
Q

What is the ankle jerk reflex?

A

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

29
Q

What is the plantar reflex in the lower limb?

A

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

30
Q

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

A
  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
31
Q

Describe how a tendon can be broken down into smaller sections

A

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).

32
Q

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

A

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.

33
Q

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

A

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

34
Q

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

A

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

35
Q

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

A

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.

36
Q

What is the role of Botox in cerebral palsy?

A

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

37
Q

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

A

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.

38
Q

What is the purpose of casting in cerebral palsy treatment?

A

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.

39
Q

What is the mechanism behind casting in cerebral palsy?

A

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.

40
Q

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

A

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.

41
Q

What is the role of baclofen in cerebral palsy?

A

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

42
Q

What is the role of diazepam in cerebral palsy?

A

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.

43
Q

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

A

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.

44
Q

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

A

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.

45
Q

Why do tendons take a long time to heal?

A

Avascular - decreases rate of rehabilitation and healing.
Stable tissue

46
Q

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

A

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.

47
Q

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

A

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.

48
Q

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

A

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

49
Q

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

A

Selective Dorsal Rhizotomy
Calf muscle lengthening
Tendon lengthening
Osteotomy

50
Q

What is a selective dorsal rhizotomy?

A

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

51
Q

What happens in calf muscle lengthening?

A

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.

52
Q

What happens in tendon lengthening?

A

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.

53
Q

What happens in an osteotomy?

A

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.

54
Q

What is the role of paediatric care and clinics?

A

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.

55
Q

What is the role of occupational therapy in cerebral palsy?

A

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.

56
Q

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

A

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

57
Q

What is the psychosocial impact of disability on the child?

A

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.

58
Q

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

A

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.

59
Q

What is an EHCP?

A

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

60
Q

What is the application process for an EHCP?

A

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.

61
Q

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

A

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.

62
Q

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

A

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

63
Q

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

A

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

64
Q

What are some support groups for cerebral palsy?

A

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.