Specific milestone developmental delay Flashcards
How will abnormal motor development present? 10 things
Delay in acquisition of motor skills e.g.
- head control
- rolling
- sitting
- standing
- walking
- problems with balance
- abnormal gait
- asymmetry of hand use
- involuntary movements
- (rarely0 loss of motor skills
Between what ages does concern about motor development usually present?
3 months - 2 years (when acquisition of motor skills usually occurs most rapidly)
What are 4 key causes of abnormal motor development?
- central motor deficit e.g. cerebral palsy (CP)
- congenital myopathy/ primary muscle disease
- spinal cord lesions e.g. spina bifida
- global developmental delay, as in many syndromes, or of unidentified cause
When is hand dominance usually acquired and when is it worrying?
1-2 years is normal; asymmetry of motor skills during first year is always abnormal, may suggest underlying hemiplegia
What 2 things should be considered with late walking (>18 months) in addition to a motor development problem?
Need to differentiate from children who
- display normal locomotor variants of bottom-shuffling or commando crawling where walking occurs later than with crawlers,
- and from children with joint hypermobility who may also achieve walking later than average
What should be done if there is concern about abnormal motor development?
Assessment by neurodevelopmental paediatrician and physiotherapist. Ongoing physio input and subsequent involvement of occupational therapist also likely to be needed
What is the definition of cerebral palsy?
Umbrella term for permanent disorder of movement and/or posture and of motor function due to a non-progressive abnormality in the developing brain
What is the most common cause of motor impairment in children?
Cerebral palsy - 2 per 1000 live births
How do the effects of cerebral palsy emerge with time?
Emerge gradually over time - despite being static lesion. due to balance between normal and abnormal cerebral maturation
When do the majority (80%) of causes of cerebral palsy occur?
antenatally
What are 6 antenatal causes of cerebral palsy?
- cerebrovascular haemorrhage
- cerebrovascular ischaemia
- cortical migration disorders
- structural maldevelopment of brain during gestation
- genetic syndromes
- congenital infection
What is the key perinatal cause of cerebral palsy and what proportion of cases does this account for?
Hypoxic-ischaemic injury - only 10% of cases
What is a key risk factor for developing cerebral palsy postnatally and why?
Prematurity - preterm infants especially vulnerable to brain damage from periventricular leukomalacia secondary to ishcaemia and/or severe intraventricular haemorrhage and venous infarction
In addition to prematurity what are 6 further postnatal causes of cerebral palsy?
- meningitis
- encephalitis/encephalopathy
- head trauma - accidental or non-accidental
- symptomatic hypoglycaemia
- hydrocephalus
- hyperbilirubinaemia
What investigations may aid the diagnosis of cerebral palsy?
MRI - may assist in identifying cause, directing further investigations and supporting explanations to parents
however not required for diagnosis
What are 6 early features of CP?
- Abnormal limb and/or trunk posture and tone in infancy with delayed motor milestones
- Slowing of head growth
- Feeding difficulties, with oromotor incoordination, slow feeding, gagging and vomiting
- Abnormal gait once walking is achieved
- Asymmetric hand function before 12 months of age
- Persistence of primitive reflexes
How is a diagnosis of cerebral palsy made?
Clinical examination - with particular attention to assessment of posture and pattern of tone in limbs and trunk, hand function and gait
What are the 5 levels of the gross motor function classification system (GMFCS)?
- I: walks without limitations
- II: walks with limitations
- III: walks using a handheld mobility device
- IV: self-mobility with limitatations, may use powered mobility
- V: transported in a manual wheelchair
How is cerebral palsy categorised and what are 4 examples of categories?
- Spastic: bilateral, unilateral, not otherwise specified
- Dyskinetic
- Ataxic
- Other
How is gross motor function described in cerebral palsy?
Using gross motor function classification system (GMFCS)
Why are some children assessed for cerebral palsy and who carries out the assessment?
Children with high-risk factors for brain damage e.g. significant prematurity or difficulties around time of birth
formal standardised assessment of general movements performed by trained therapist or clinician
What causes spastic cerebral palsy?
Damage to upper motor neurone (pyramidal or corticospinal tract) pathway
What are 4 features of spastic cerebral palsy?
- Limb tone persistently increased (spasticity)
- associated brisk deep tendon reflexes and extensor plantar responses
- tone is velocity dependent - faster muscle is stretched, the greater the resistance. elicits dynamic catch - hallmark of spasticity
- limb tone may suddenly yield under pressure ‘ ‘clasp knife’ fashion
How can spastic cerebral palsy be further classified? 3 groups
- Unilateral (hemiplegia) - unilateral involvement of arm and leg
- Bilateral (quadriplegia) - all 4 limbs affected
- Bilateral (diplegia) - all four limbs but legs affected to much greater degree
When can spastic cerebral palsy present?
Can present in neonatal period
When does unilateral spastic cerebral palsy usually present and what are the key features?
- 4-12 months
- Fisting of affected hand, flexed arm, pronated forearm, asymmetric reaching, hand function or toe pointing when lifting the chlid
- Tiptoe walk (toe-heel gait)
- Sometimes hemianopia on same side as affected limbs
What are 2 possible causes of unilateral (hemiplegic) spastic cerebral palsy?
- Silent prenatal cause
- Neonatal stroke - perinatal middle cerebral artery infarct
What are 4 key features of bilateral quadriplegic spastic cerebral palsy?
- all 4 limbs affected with trunk involvement (e.g. may see scissoring of legs)
- opisthotonus (extensor posturing)
- poor head control
- low central tone
What is the most severe form of spastic cerebral palsy?
Bilateral, quadriplegic form
What is the cause of quadriplegic bilteral spastic CP?
Hypoxic ischaemic encephalopathy - extensive damage to periventricular areas of devleoping brain, including cortex
What are 3 things that bilateral (quadriplegic) cerebral palsy may be associated with?
- Seizures
- Microcephaly
- Moderate to severe intellectual impairment
What are 2 key features of bilateral, diplegic spastic CP?
- Motor difficulties in arms most apparent with functional use of hands
- Walking abnormal
What is one of the key causes of bilateral, diplegic cerebral palsy?
Preterm birth due to periventricular brain damange (MRI shows periventricular leukomalacia)
What is dyskinetic cerebral palsy?
dyskinesia = involuntary, uncontrolled, stereotyped movements, more evident with active movement or stress
Muscle tone variable and primitive motor reflex patterns predominate
What are the 3 key things that can be used to describe dyskinetic cerebral palsy?
- chorea: irregular, sudden and brief non-repetitive movements
- athetosis: slow writhing movements occuring more distalling e.g. fanning of fingers
- dystonia: simultaneous contraction of agonist and antagonist muscles of trunk and proximal muscles - gives twisting appearance
What are 4 features of dyskinetic CP that patients may present with?
- unimpaired intellect
- floppiness
- poor trunk control
- delayed motor development in infancy
When may abnormal movements appear in dyskinetic cerebral palsy?
towards end of first year of life
What causes dyskinetic cerebral palsy?
damage or dysfunction in basal ganglia or extra-pyramidal pathways - most commonly due to hypoxic-ischaemic encephalopathy, due to perinatal asphyxia at term (previously due to kernicterus due to Rh incompatibility)
What is the cause of ataxic (hypotonic) cerebral palsy?
- mostly geneically determined
- also due to acquired brain injury to cerebellum or its connections
If ataxic CP is due to cerebellar injury, what side will signs occur on?
same side as injury (but usually relatively symmetrical)
What are 6 features of ataxic cerebral palsy?
- limb hypotonia
- poor balance
- delayed motor development
- intention tremor
- incoordinate movements
- ataxic gait
When is prognosis of CP best estimated?
when severity and pattern of evolving signs and child’s developmental progress have become clearer over several months or years of life
What are 4 available treatments for hypertonia in CP?
- botulinum toxin injections to muscles
- selective dorsal rhizotomy (proportion of nerve roots in spinal cord selectively cut to reduce spasticity)
- intrathecal baclofen (skeletal muscle relaxant)
- deep brain stimulation of basal ganglia
What is often the cause of unilateral spastic or dystonic CP?
perinatal middle cerebral artery infarct
What are 3 overall features of cerebral palsy?
- abnormal tone and posture
- delayed motor milestones
- feeding difficulties
What are 2 ways to categories disordered speech and language development?
- may be receptive or expressie deficit
- may be delay or disorder
What are 5 causes of speech and language delay?
- hearing loss
- global developmental delay
- difficulty in speech production from an anatomical deficit e.g. cleft palate, or oromotor incoordination, e.g. CP
- environmental deprivation/lack of opportunity for social interaction
- normal variant/familial pattern
What are 5 causes of speech and language disorders?
- Language comprehension
- Language expression - inability or difficulty in producing speech whilst knowing what is needing to be said
- Intelligibility and speech production such as stammering (dysfluency), dysarthria or verbal dyspraxia
- pragmatics (difference between sentence meaning and speaker’s meaning), construction of sentences, semantics, grammar
- social/communication skills (autism spectrum disorder)
What is usually the first step in assessment for speech and language problems?
Who should be involved thereafter?
Hearing test and assessment by speech and language therapist
Then involve neurodevelopmental paediatrician and paediatric audiological physician
What is the management of speech and language problems in children? 4 aspects
- Speech and language therapy: continuous, burst or review basis
- may involve alternative methods e.g. Makaton or Picture Exchange Communication System
- Special Schooling for very few
- Learning support at school entry
What are 2 examples of tests of language development?
- Symbolic toy test - assesses early language development
- Reynell test for receptive and expressive language, used for preschool children
What is the IQ range for children with borderline and mild learning difficulties?
70-80
What is the usual management for children with borderline and mild learning difficulties?
Supported by additionaal helpers in mainstream schools
What is the IQ range for moderate learning difficulty?
50-70
What is the IQ range for severe learning difficulty?
35-50
What is the IQ range for profound learning difficulty?
<35
What is usually needed as the management for children with moderate, severe or profound learning difficulties?
Resources of special schools
How will severe or profound learning difficulties present vs how moderate and mild may present?
Severe or profound = marked global developmental delay
Moderate = delay in speech and language
Mild = may only become apparent when starting school or much later
How will a child with profound learning difficulty present vs child with severe?
Profound = no significant language, completely dependent for all their needs
Severe = able to learn minimal self-care skills and acquire simple speech and language
How does the cause of severe learning difficulties contrast with that of moderate learning difficulty?
In severe, most have organic cause, but lower socioeconomic classes over-represented in moderate learning difficulty
What is meant by specific learning difficulty and what are 4 examples?
The skill described is more delayed than would be expected for chlid’s level of cognitive ability
- Dyspraxia (developmental coordination disorder)
- Dyslexia
- Dyscalculia (calculation)
- Dysgraphia (writing)
- Disorder of executive functions
What is developmental coordination disorder/ aka dyspraxia?
- Disorder of motor planning and/or execution with no signficant findings on standard neurological examinations
- Disorder of higher cortical processes
- problems with perception, language and putting thoughts together
What are 8 possible features of dyspraxia?
- handwriting is awkward, messy, slow, irregular, poorly spaced
- dressing difficulties with buttons, laces, clothes
- cutting up food
- poorly established laterality
- copying and drawing
- messy eating from poor coordination (oromotor dyspraxia), dribbling
- impact on educational progress - suggest greater academic difficulties than is the case
- poor self-esteem
What are 3 aspects of management of dyspraxia?
- Assessment and advice from occupational therapist
- Speech and language therapist for oromotor skills/speech if necessary
- Visual assessment may be helpful
Why might dyspraxia in a milder form go unnoticed in the first few years of life?
If mild and child acheives gross motor milestones at normal times
What are 4 things that therapy for dyslexia (with occupational therapist, SALT) may involve?
- Sensory integration
- Sequencing
- Executive planning
- Speech/language therapy if needed
What type of dyspraxia might raise more specific difficulties/
Verbal dyspraxia - more specific difficulties related to speech production in absence of muscle or nerve damage (still part of developmental dyspraxia)
What is the prognosis of dyspraxia?
With therapy and maturity, condition should improve
What is dyslexia?
Disorder of reading skills disproportionate to child’s IQ - when child’s reading age is more than 2 years behind chronological age
What does the management of dyslexia involve?
Assessment includes vision and hearing and involves educational psychologist
What is dyscalculia?
Disorder in development of calculation skills
What is dygraphia?
Disorder in development of writing skills
What are 5 types of specific learning difficulty?
- Developmental coordination disorder (dyspraxia)
- Dyslexia
- Dyscalculia
- Dysgraphia
- Disorder of executive functions