Cerebral palsy Flashcards
Define
Abnormality of movement and posture, causing activity limitation attributed to non-progressive disturbances that occurred in the developing foetal or infant brain
1 in 400 live births – most common cause of motor impairment
It may or may not be associated with cognitive imp
- If brain injury occurs after 2yo, it is diagnosed at acquired brain injury (and not CP)
Causes
Risk factors:
80 - Antenatal:
* preterm birth
* Multiple gestation
* Maternal infection e.g. chorioamnionitis
* Maternal respiratory tract or genito-urinary tract infection treated in hospital
* Other maternal illnesses (e.g. thyroid disease, iodine deficiency, TORCH infections)
* Maternal thrombotic disorders including Factor V Leiden mutations
* Teratogen exposure
* Foetal genetic and metabolic disorders
* Foetal brain malformations
* Placental abruption
10 - Perinatal:
- HIE, neonatal sepsis
- Preterm birth
- Low birth weight
- Respiratory distress
- Birth asphyxia
- Intraventricular haemorrhage
- Hyperbilirubinaemia
10 - Postnatal: meningitis
Presentation
Delayed milestones (i.e. not sitting by 8m, not walking by 18m) ± persistent primitive reflexes
CP is a non-progressive condition so there should be NO LOSS of previously attainted milestones
Early motor features
* Abnormal limb and/or trunk posture and tone in infancy
* Stiff legs, scissoring of legs
* Unable to lift head
* Unable to weight bear
* Rounded back when sitting
* Fisted hands
Abnormalities of tone- hypotonia, spasticity, dystonia
* Hand preference before 1 year old (esp. spastic unilateral CP)
* Abnormal motor development, late head control, rolling and crawling
* Ab gait once walking
* Feeding difficulties- oromotor incoordination, slow feeding, gagging, vomiting
Delayed motor milestones
* E.g., Not sitting by 8 months (corrected for GA)
* E.g., Not walking by 18 months (corrected for GA)
* E.g., Early asymmetry of hand function (hand preference) before 1 year (corrected for GA)
* Persistent toe-walking
* Primitive reflexes may persist or become obligatory
Absent or abnormal fidgety movements (exaggerated amplitude, speed or jerkiness) can be indicative of CP
Spastic cerebral palsy (90%)
Damage to UMN pathway -> ↑ tone (spasticity), brisk reflexes, extensor plantar, ‘clasp knife’ rigidity
- Damage to pyramidal (also known as corticospinal) tracts
- “Clasp knife” = increased tone suddenly gives under pressure
Dynamic catch → faster the muscle stretched, greater the resistance, “velocity dependent”
Presents early, even neonatally as hypotonia
3 main types of Spastic CP:
(1) Unilateral / Hemiplegia → unilateral arm and leg, face spared
Presents 4-12 months with [think of an Egyptian]
Fisting of affected hand and asymmetric hand function
Flexed pronated arm
With a tiptoe walk on affected side
Initially flaccid but then ↑↑ tone
Likely normal PMHx and unremarkable birth Hx (i.e. unknown antenatal cause)
(2) Bilateral / Quadriplegia – all 4 limbs, often severe
- Involving trunk, opisthotonos (extensor positioning)
- Poor head control
- Low central tone → associated seizures, microencephaly, moderate to severe learning disability, history of hypoxic-ischemic encephalopathy (HIE)
(3) Diplegia – legs affected to a greater degree (but all 4 limbs affected)
- Abnormal walking, difficulties with functional use of hands
- Associated with preterm birth damage and PVL
Dyskinetic Cerebral Palsy (6%)
6% of all
Dyskinesia refers to movements that are involuntary, uncontrolled, occasionally stereotyped and often more evident with active movement or stress
PRESENTATION
* Floppiness
* Poor trunk control
* Delayed motor development in infancy
* Abnormal movements may only become apparent after 1 year
* The signs usually result from damage or dysfunction in the basal ganglia and associated pathways (extra-pyramidal)
* Previously, the most common cause of dyskinetic cerebral palsy was kernicterus due to rhesus disease of the newborn
Now, the MOST COMMON cause is HIE at term
MRI will show bilateral changes predominantly in the basal ganglia
Ataxic (Hypotonic) Cerebral Palsy
Most are GENETICALLY determined
NOTE: When due to an acquired brain injury (cerebellum or its connections), the signs occur on the SAME side as the lesion but are usually relatively symmetrical
PRESENTATION:
- Early trunk and limb hypotonia
- Poor balance
- Delayed motor development
Later features include:
- Incoordinate movements
- Intension tremor
- Ataxic gait
Investigations
Thorough neurological examination- with attention to movement and muscle tone
Basic observations
Weight and height plotted
CLASSIFICATION (based on neurological features)
* Spastic (90%)
* Dyskinetic (6%)
* Ataxic (4%)
* Other (8%)
Gross Motor Functional Classification System (GMFCS) is used to describe the gross motor functional level (functional ability)
Level 1- walks without limitations
Level 2- walks with limitations
Level 3- walks using a handheld mobility device
Level 4- self-mobility with limitations, may use powered mobility
Level 5- transported in a manual wheelchair
MRI: offer MRI if the cause is not clear from history, developmental progress, clinical examination and cranial ultrasound (however, MRI cannot establish timing of injury) – GA or sedation is usually needed
Red flags for other neurological conditions:
1. Absence of risk factors
2. Family history of progressive neurological disorder
3. Loss of already attained cognitive or developmental abilities
4. Development of unexpected focal neurological signs
5. MRI findings suggestive of progressive (CP is classically non-progressive) neurological disorder
6. MRI findings not in keeping with CP
Management
very MDT orientated:
Information about prognosis to parents (and parental education):
Walking: children who can sit by age 2 years are likely to be able to walk unaided by 6 years old
Speech: 50% have difficulties with communication, 33% have difficulties with speech and language
Life Expectancy: the more severe the CP the greater the likelihood of reduced life expectancy
Support: · SCOPE disability charity · www.cerebralpalsy.org.uk
MDT Members:
Main Members: paediatrician, nurse, physiotherapist, OT, SALT, dietetics, child psychology
Supplementary Members: orthopaedics, orthotics, visual and hearing
Paediatrician – medical problems: 33% have epilepsy
Physiotherapy – encourage movement, improve strength and stop muscles from losing range of motion
Speech therapy – improve language abilities
50% have communication difficulties
33% have speech and language
Occupation therapy – identify everyday tasks that may be difficult and help make these tasks more accessible
Medication:
Stiffness – 1st: PO diazepam 2nd: PO or intrathecal baclofen
Sleeping – melatonin
Constipation – Movicol
Drooling – anticholinergic
Complications
- Feeding difficulties
- Sialorrhea/ drooling
- Aspiration, recurrent chest infection
- Vomiting, regurgitation, GORD
- Osteopaenia, osteoporosis
- Constipation, incontinence
- Visual impairment, hearing impairment, epilepsy
- Learning disability
- Communication difficulties
- Behavioural difficulties
- Mental/ psychological problems, neurodevelopmental disorders (ASD, ADHD)
- Pain
- Sleep disturbance
- Impaired social interaction and participation
- Reduced QoL
- Reduced life expectancy
PACES
Explain the diagnosis (damage to the brain that would have occurred early in development)
‘A condition where the brain is affected causing movement, posture difficulties and can affect learning’
The damage to the brain doesn’t get worse, but the way it manifests will change as the child gets older
Refer to MDT and especially a paediatrician specialising in developmental disorders
Long-term management will include physiotherapy, speech and language therapy and special educational needs
Medications can also be given to help with symptoms
Information about prognosis to parents (and parental education):
Walking: children who can sit by age 2 years are likely to be able to walk unaided by 6 years old
Speech: 50% have difficulties with communication, 33% have difficulties with speech and language
Life Expectancy: the more severe the CP the greater the likelihood of reduced life expectancy
Support:
SCOPE disability charity
www.cerebralpalsy.org.uk
