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1 - Paeds AE > Developmental problems and the child with special needs > Flashcards

Developmental problems and the child with special needs Flashcards

1
Q

What are some clinical signs that may aid a diagnosis of developmental problems?

A

Growth: height, weight, head circumference
Dysmorphic features: face, limbs, body, cardiac
Skin: injuries, cleanliness
CNS: posture, wasting, power/tone, reflexes
Visual function
Hearing: ask parents
General: mobility, dexterity, communication
Cognition

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2
Q

What is ‘delay’?

A

slow acquisition of all skills (global delay) or of one particular field or area of skill (specific delay)

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3
Q

What is ‘learning difficulty’?

A

Used in relation to children of school age and may be cognitive, physical, both or relate to specific functional skills

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4
Q

What is ‘disorder’?

A

Maldevelopment of a skill

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5
Q

What is ‘impairment’?

A

Loss or abnormality of physiological function or anatomical structure

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6
Q

What is ‘disability’?

A

Any restriction or lack of ability due to the impairment

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7
Q

What is ‘disadvantage’?

A

This results from the disability, and limits or prevents fulfilment of a normal role. It is situationally specific.

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8
Q

How can the pattern of abnormal development be categorised?

A

Slow but steady
Plateau effect
Showing regression

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9
Q

When does global developmental delay usually present?

A

In the first 2 years of life

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10
Q

When do concerns about motor developmental delay usually present?

A

Between 3 months and 2 years

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11
Q

What are some causes of abnormal motor development?

A

Central motor deficit (cerebral palsy)
Congenital myopathy/primary muscle disease
Spinal cord lesions (spina bifida)
Global developmental delay

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12
Q

When is asymmetry of motor skills always abnormal?

A

During the first year of life, because hand dominance is not acquired until 1-2 years or later.

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13
Q

What is cerebral palsy?

A

It may be defined as an abnormalities of movement and posture, causing activity limitation attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.

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14
Q

What are the non-motor symptoms of cerebral palsy?

A

It is often accompanied by disturbances of cognition, communication, perception, sensation, behaviour and seizure disorder and secondary musculoskeletal problems

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15
Q

What are the causes of cerebral palsy?

A

80% are antenatal in origin: vascular occlusion, cortical migration disorders or structural maldevelopment of the brain.
Hypoxic-ischaemic injury during delivery
Postnatal causes: meningitis, head trauma NAI, hydrocephalus

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16
Q

What are the early clinical features of cerebral palsy?

A

Abnormal limb and/or trunk posture and tone in infancy with delayed motor milestones, slowing of head growth
Feeding difficulties
Abnormal gait
Asymmetric hand function before 12 months

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17
Q

What happens to the primitive reflexes in cerebral palsy?

A

Primitive reflexes may persist and become obligatory

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18
Q

What are the three main clinical subtypes of cerebral palsy?

A

Spastic (90%)
Dyskinetic (6%)
Ataxic (4%)

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19
Q

What neurones are affected in spastic cerebral palsy?

A

The upper motor neurone pathway is damaged

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20
Q

What are the clinical features of spastic cerebral palsy?

A

Limb tone is persistently increased (spasticity) with associated brisk deep tendon reflexes and extensor plantar responses.

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21
Q

What are the features of spasticity?

A

The tone in spasticity is velocity dependent, so the faster the muscle is stretched the greater the resistance it will have. This elicits a dynamic catch which is the hallmark of spasticity. The increased limb tone may suddenly yield under pressure in a ‘clasp knife’ fashion.

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22
Q

What are the clinical features of hemiplegia in cerebral palsy?

A

Unilateral involvement of the arm and leg. Fisting of the affected hand, a flexed arm, a pronated forearm, asymmetric reaching or hand function. Subsequently a tiptoe walk on the affected side.

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23
Q

With hemiplegic cerebral palsy, are the arms or legs usually more effected?

A

The arm is usually affected more than the leg, with the face spared.

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24
Q

What are the clinical features of quadriplegic cerebral palsy?

A

All four limbs are affected, often severely. The trunk is involved with a tendency to opisothonus (extensor posturing), poor head control and low central tone.

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25
Q

What is quadriplegic cerebral palsy commonly associated with?

A

Seizures, microcephaly and moderate or severe cognitive impairment

26
Q

What are the clinical features of Dyskinetic cerebral palsy?

A

Movements with are involuntary, uncontrolled, occasionally stereotyped, and often more evident with active movement or stress. Muscle tone is variable and primitive motor reflex patterns predominate

27
Q

How can Dyskinetic cerebral palsy be described?

A

Chorea - irregular, sudden and brief non-repetitive movements
Athetosis - slow writhing movements occurring more distally (fanning of the fingers)
Dystonia - simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles often giving a twisted appearance

28
Q

What are the causes of speech and language DELAY?

A 
Hearing loss
Global developmental delay
Anatomical deficits (cleft palate, cerebral palsy)
Environmental deprivation/lack of opportunity for social interaction
Normal variant/familial pattern
29
Q

What are the causes of speech and language DISORDERS?

A

Language comprehension
Language expression
Phonation and speech production (stammer, dysarthria, verbal dyspraxia)
Pragmatics (difference between sentence meaning and speaker meaning)
Social/communication skills (ASD)

30
Q

What are the tests used to test language development?

A

The Symbolic Toy test

The Reynell Test

31
Q

Is autism more common in girls or boys?

A

Boys

32
Q

When does ASD normally present?

A

Between 2 and 4 years of age when language and social skills normally rapidly expand.

33
Q

What is Asperger syndrome?

A

A child with the social impairments of an autistic spectrum disorder but at the milder end, and near-normal speech development.

34
Q

What are the three subgroups of the features of ASD?

A

Impaired social interaction
Speech and language disorder
Imposition of routines with ritualistic and repetitive behaviour

35
Q

What are the clinical features of the impaired social interaction in ASD?

A

Does not seek comfort, share pleasure, form close friendships
Prefers own company, no interest or ability in interacting with peers
Gaze avoidance
Lack of joint attention
Socially and emotionally inappropriate behaviour
Does not appreciate that others have thoughts and feelings
Lack of appreciation of social cues

36
Q

What are the clinical features of the speech and language disorders in ASD?

A

Delayed development, may be severe
Limited use of gestures and facial expression
Formal pedantic language, monotonous voice
Impaired comprehension with over-literal interpretation of speech
Echoes questions, repeats instructions, refers to self as ‘you’
Can have superficially good expressive speech

37
Q

What are the clinical features of the imposition of routines with ritualistic and repetitive behaviour?

A

On self and others, with violent temper tantrums if disrupted
Unusual stereotypical movements such as hand flapping and tiptoe gait
Concrete play
Poverty of imagination in play and general activities
Peculiar interests and repetitive adherence
Restriction in behaviour repertoire

38
Q

How do you treat ASD?

A

Parental support
Applied behavioural analysis
Appropriate educational placement

39
Q

What is developmental coordination disorder (DCD) or dyspraxia?

A

A disorder of motor planning and/or execution with no significant findings on standard neurological examination. A disorder of the higher cortical processes and there may be problems of perception. use of language and putting thoughts together

40
Q

What are the things in which patients may struggle with is they have dyspraxia?

A

Handwriting (awkward, messy, slow, irregular and poorly spaced)
Dressing (buttons, laces and clothes)
Cutting up food
Poorly established laterality
Copying and drawing
Messy eating (difficulty coordinating biting, chewing and swallowing)

41
Q

What are some associated co-morbidities of specific learning disorders?

A

ADD
Hyperactivity
Poor sensory integration skills (touch, balance)
Depression, conduct disorders

42
Q

What are the types of hearing loss?

A

Sensorineural - a lesion in the cochlea or auditory nerve and is usually present at birth
Conductive - abnormalities of the ear canal or the middle ear, most often from otitis media with effusion

43
Q

What are the causes of sensorineural hearing loss?

A

Genetic (majority)

Ante- and perinatal: congenital infection, preterm, meningitis, head injury, neurodegenerative disorders

44
Q

What are the causes of conductive hearing loss?

A

Otitis media with effusion
Eustachian tube dysfunction: down syndrome, cleft palate, mid-facial hypoplasia
Wax

45
Q

What are the natural histories of sensorineural and conductive hearing loss?

A

Sensorineural - does not improve and may progress

Conductive - intermittent or resolves

46
Q

What are the managements of sensorineural and conductive hearing loss?

A

Sensorineural - amplification or cochlear implant if necessary
Conductive - conservative, amplification or surgery (insertion of grommets may be considered)

47
Q

What investigation is used to determine middle ear function?

A

Impedance auditory tests, which measure the air pressure within the middle ear and the compliance of the tympanic membrane

48
Q

What are the different ways in which visual impairment may present in infancy?

A 
Loss of red reflex from cataract
A white reflex in the pupil
Not smiling responsively by 6 weeks
Lack of eye contact with parents
Visual inattention
Random eye movements
Nystagmus
Squint 
Photophobia
49
Q

What is a squint (strabismus)?

A

Misalignment of the visual axes

50
Q

Are squints normal at any age?

A

Newborn babies usually have transient misalignments up to 3 months of age.

51
Q

What usually causes a squint?

A

Failure to develop binocular vision due to refractive errors, but cataracts, retinoblastoma and other intraocular causes must be excluded.

52
Q

What are the two common divisions of squint?

A

Concomitant (non-paralytic, common)

Paralytic (rare)

53
Q

What causes a concomitant squint and how do you treat it?

A

Usually due to a refractive error in one or both eyes, which is often treated by correction with glasses but may require surgery

54
Q

Does the squinting eye normally turn inwards or outwards?

A

Most often turns inwards (convergent), but there can be outward (divergent) or, rarely, vertical deviation

55
Q

How does the corneal light reflex test show a squint?

A

Hold a light at a distance. The light reflection should appear in the same position in the two pupils. If it does not, a squint is present.

56
Q

What is the cover test when looking for a squint?

A

When a squint is present an the fixing eye is covered, the squinting eye moves to take up fixation.

57
Q

Is long sight (hypermetropia) or short sight (myopia) more common in young children?

A

Long sight (hypermetropia)

58
Q

How do you treat astigmatism?

A

Relieving deprivation and correction of any refractive error with glasses, together with patching for the good eye for specific periods of the day to force the lazy eye to work and therefore develop better vision.

59
Q

When should you treat squints?

A

Early treatment is essential, as after 7 years of age improvement is unlikely

60
Q

What are the causes of visual impairment?

A

Genetic: cataract, albinism, retinal dystrophy, retinoblastoma
Antenatal and perinatal: congenital infection, retinopathy of prematurity, optic nerve hypoplasia
Postnatal: trauma, infection, juvenile idiopathic arthritis

61
Q

What is a paralytic squint?

A

Rare, due to paralysis of the motor nerves; if rapid onset, consider space-occupying lesion

1 - Paeds AE (24 decks)

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