Neurology + Development (2s + 3) Flashcards

1
Q

What are children with special needs?

A
  • if they have developmental problems and disabilities
  • these can affect their:
    • behaviour or ability to socialsie
    • reading + writing, eg. dyslexia
    • ability to understand things
    • concentration levels, eg. ADHD
    • physical ability
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2
Q

What is the child development service?

A
  • secondary care service available for children w/ special needs till they are of school-leaving age
  • child has regular assessment + management that aims to meet all functional needs
  • it involves multidisciplinary teams consisting of paeditricians, physios, OTs, SALT, clin psychologist, dietician, special health visitors, social services
  • many agencies are involved: health, social, educational, voluntary, parent support groups
  • each child has key worker who makes sure they get access to correct care
  • community or hospital based
  • service records all of children they provide for Special Needs register
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3
Q

Several education acts ensure that all children have educational inputs appropriate to their requirements and that everyone can attend mainstream schools if at all possible.

How are learning difficulties classified in terms of IQ and how are the moderate/mild met in terms of education needs?

A
  • mild → 70-90
  • moderate → 50-70
  • severe → 20-50
  • profound → <20

Those w/ mild and some with moderate learning difficulties are able to attend mainstream school if supported by additional helpers. Each child w/ special needs has a special educational needs coordinator from the teaching staff allocated to them. They formulate individual care plans.

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4
Q

For those who require high levels of support, what is provided?

A
  • Local Educations Authority may need to do a Statement of Special Education Needs which is reviewed annually
  • those w/ profound, severe and some of those w/ moderate learning difficulties need to attend special schools
  • it is also necessary for some of those with severe physical, sensory, communication or behavioural problems to attend them as well
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5
Q

What is squint (strabismus) and what are the different types?

A
  • failure of the 2 eyes to maintain proper alignment and work together as a team
  • if you have stabismus, 1 eye looks directly at the object you are viewing while the other eye is misaligned:
    • inward (esotropia, “cross-eyed”)
    • outward (exotropia, “wall eyes”)
    • upward (hypertropia)
    • downward (hypotropia)

Can be divided into concomitant (common) and paralytic (rare)

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6
Q

What are the causes of a squint?

A

Exact causes of a squint isn’t always known, some people are born with a squint and others develop one later in life. In children, a squint is often caused by the eye attempting to overcome a vision problem, such as:

  • short-sightedness
  • long-sightedness
  • astigmatism
  • rarer causes ⇒ infections (measles), Down’s, developmental delays, CP
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7
Q

How are squints picked up in children, in terms of investigations?

A
  • cover testfor older children - child shown object which their eyes fixate on, a squint is present if when the fixating eye is covered with a piece of cardboard, the other eye has to move to fixate on the object
  • Hirshberg testlight shone on eyes from arm’s length, light should be symmetrically reflected + centred in each eye - if not, then stabismus present
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8
Q

Amblyopia is more likely in children with squint. What is amblyopia?

A
  • in strabismus there will be normal vision in the preferred eye
  • but due to the discrepancy between 2 images projecting to the brain from 2 eyes, the brain may ignore it from the deviated eye
  • amblyopia describes abnormal brain development due to lack of stimulation of the brain from visual system
  • this abnormal brain development is completed at 7 years of age
  • so if strabismus not corrected by then, there will be permanent loss of vision in deviated eye
  • occlusion of the good eye with a patch for a period of time each day will encourage the use of the deviated eye
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9
Q

What is the management of squint?

A
  • glasses → can help if cause is eye-sight related
  • eye exercises
  • surgery → involves moving the muscles that control eye movement so the eyes line up correctly, recommended if glasses aren’t fully effective on their own
  • injections to eye muscles → weaken them, which can help eyes line up better
  • eye patches → may help prevent amblyopia
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10
Q

What would be your differentials for seizures in children?

A
  • febrile convulsion → most common cause in kids, present as a single episode w/out a prior hx of seizures, but can be recurrent
  • non-epileptic seizures → psychological, hard to diagnose, appear externally as seizures, features: flapping limbs (not tonic or clonic), tightly shut eyes which resist forced opening + distractability out of episodes
  • breath-holding spells → precipitated by anger/frustration
  • long QT syndrome → triggered by pain/fear/exercise
  • syncope
  • paroxysmal movement disorders → tics, ataxias, dyskinesias
  • G-O reflux
  • epilepsy
  • panic disorder
  • self-gratification behaviour
  • reflex anoxic seizures → ECG shows asystole
  • infection → sepsis, cerebral abscess, subdural empyemas can all lead to seizures
  • tumour → focal mass may present w/ focal signs or signs of inc ICP
  • toxins → drugs
  • metablic → electrolyte imbalances or hypoglycaemia
  • head injury → trauma or NAI
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11
Q

How are seizures classified?

A
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12
Q

What are infantile spasms?

A
  • typically begin in infants aged 4-8months
  • consist of clusters of myoclonic spasms
  • typically upon awakening or falling asleep
  • presentations can be more subtle
  • include slight eye flutter or head drop
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13
Q

What is absence epilepsy?

A
  • aka petit mal epilepsy
  • frequent (can be 100+ / day) episodes
  • brief staring spells, fluttering of eyelids
  • last only a few seconds (typically up to 15s)
  • patients return immediately to baseline mental status
  • primarily generalised in onset
  • diagnosis can be assisted by classical EEG features + hyperventilation trial, which often provokes seizures
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14
Q

What investigations can be done for suspected seizures in children?

A
  • EEG → may show abnormal rhythms specific to epilepsy syndrome
  • blood glucose
  • basic metabolic panel
  • FBC
  • ECG
  • MRI brain
  • lumbar puncture
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15
Q

Language delay is when there is delay in understanding or expressing speech and/or language. An ‘alarm bell’ is not making clear single words by 2 years of age.

What are the red flags for developmental language disorder?

A
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16
Q

What are the causes of language delay?

A
  • normal variant or familial
  • global developmental delay
  • learning difficulties
  • hearing impairment
  • anatomical defect causing abnormal speech production
  • autism
  • environmental or social deprivation
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17
Q

A child should be saying 6 words by 18months, joining 2 words by 2years and most children can say 50 words by the 3rd year.

In a child presenting with language delay, what features would you like to assess?

A
  • vocalised? → cooing/babbling/laughing/ + words - joining/repeating
  • siblings? → compare
  • assess hearing: been tested? newborn screening? can they copy sounds?
  • failure comprehension → respond to voice? follow command? pointing objects?
  • failure producing sounds → can you understand any noises?
  • does child use non-verbal communication - pointing, gestures, facial expression?
  • socially responsive → how do they act in social situations? temper tantrums? playing w/ other children?
  • think autism spectrum disorder features
  • rest of development milestones + Hx
  • pre/peri/postnatal hx → preg infections, birth trauma, resus, sepsis, cranial USS, seizures
  • PMHx → infections esp meningitis, recurrent ear infections, exposure to ototoxic drugs (eg. gentamicin), immunisations, screening
  • co-morbidities → seizures, visual problems, squint, feeding probs, joint contractures, abnormal posturing/gait?
  • FHx → learning disability, speech delay, hearing problems
  • social hx → nursery? plays w other kids? any fam stresses?
18
Q

What investigations can be done for language delay?

A
  • audiological, neurodevelopmental and SALT assessment
19
Q

What are types of language and speech disorders?

A
  • language:
    • receptive dysphagia → unable to comprehend language
    • expressive dysphagia → unable to express what they want to say
    • problems w/ grammar, semantics + construction of sentences
  • speech:
    • dysarthria → unclear pronunciation
    • dysfluency → stammering
    • abnormal phonation
  • social or communication skills → autism spectrum disorder
20
Q

What are the 3 themes that characterise ADHD?

A
  • hyperactivity → fidgety, constantly on the move, reduced sleep requirement
  • inattention → easily distracted, poor concentration
  • impulsiveness → acts without reflection, impatient
21
Q

How might a parent or teacher describe a child who is presenting with suspected ADHD?

A
  • failure to give close attention to details
  • making careless mistakes in school work + activities
  • difficulty sustaining attention in play activities
  • does not seem to listen when spoken to directly
  • doesn’t follow instructions + fails to finish school work/chores
  • avoids/dislikes tasks that require sustained mental effort
  • often loses things necessary for tasks or activities
  • easily distracted by extraneous stimuli
  • forgetful in daily activities
  • fidgets w/ hands or feet or squirms in seat
  • leaves seat in classroom in which remaining seated is expected
  • runs about/cimbs excessively during inappt situations
  • difficulty playing or engaging in leisure activities quietly
  • often ‘on the go’ or acts as if ‘driven by a motor
  • often talks excessively
  • blurts out answers before questions have been completed
  • often has difficulty awaiting turn
  • often butts into conversations or games
22
Q

Genetic and environmental factors have been implicated in the aetiology of ADHD.

There are common co-existing and not mutually exclusive diagnoses. What are these?

A
  • mood disorder
  • conduct disorder
  • learning difficulties
  • communication disorders
  • anxiety disorders
23
Q

How is ADHD investigated?

A
  • ADHD diagnosed clinically
  • by child psychiatrist or community paediatrician
  • without any biochemical tests unless alternate diagnosis considered
  • for diagnosis, condition must:
    • have early onset
    • have moderate impairment to emotional, educational + social functioning
    • demonstrate chronicity

Features should occur in 2 or more settings to demonstrate the behaviour is not specific to one environment. Diagnosis is supported w/ scoring charts, assessing for traits in the three themes described.

24
Q

What is the management of ADHD?

A
  • involves MDT approach
  • w/ education, behavioural intervention, medication + ensuring a healthy, nutritionally balanced diet
  • 1st line medication is usually methylphenidate (a dopamine reuptake inhibitor)
25
Q

What are the differential diagnoses for headaches in children?

A
  • cluster headache
  • tension headache
  • post-traumatic headache
  • dental abscess
  • otitis media
  • meningitis
  • sinusitis
  • CNS pathology/tumour
  • refraction error
  • hypertension
  • headache of low ICP
  • toxin-related headache
  • temporomandibular joint dysfunction
  • obstructive sleep apnoea
  • analgesic headache
26
Q

Headache is one of the most common reasons for adolescents and older children to seek medical advice.

What is the difference between primary and secondary headaches?

A
  • primary headaches eg. migraines, tension type headaches + cluster headaches
  • secondary headaches are a symptom of an underlying cause eg. intracranial mass, intracranial bleed, infection, benign intracranial hypertension
27
Q

Migraine has a high prevalence in children (10%) and is a significant source of morbidity. Careful consideration of the broad differential diagnosis is important when evaluating a child with headache.

What are the clinical features of migraine?

A
  • +/- aura
  • lasts for 2-48hrs
  • unilateral + gradual onset (15-30mins)
  • pulsating quality
  • aggrevated by routine physical activity
  • nausea + vomiting
  • photophobia + phonophobia
  • more common in >2yr olds than under
28
Q

What are the risk factors (+ triggers) for migraine?

A
  • FHx → 90% of children have a first or second degree family member w/ recurrent headaches
  • food → chocolate, cheese + citrus fruits
  • stress → 25% of children (school + relationships)
  • hormonal changes → gonadal fluctuations, menses
  • alcohol + caffeine
  • sleep disturbance
  • bright or flickering lights
29
Q

What is the International Headache Society (IHS) criteria for paediatric migraine without aura?

A
30
Q

What is the management for migraine?

A
  • conservative → includes avoiding triggers + selecting strategies for dealing w/ an attack
  • drugs:
    • acute episodes → combo therapy of triptans (eg. sumatriptan) + NSAIDs/paracetamol
      • sumatriptan = 5HT agonist
      • NICE recommends that triptans may be used in children >12yrs but follow-up is required
    • prophylaxis → beta blockers, topiramate or pizotifen
      • evidence base is limited + no clear concensus guidelines exist
31
Q

What are red flags/indications for considering CT or MRI brain scans for headaches?

A
  • focal neurological features
  • signs of raised ICP
  • headaches which are worse on lying down
  • confusion
  • altered consciousness
  • change in personality
  • abnormal gait
  • diplopia
  • growth failure
  • visual field defects
  • seizures
  • thunderclap headaches
32
Q

Tension type headache is the second most common cause of headache in children. What are the clinical features?

A
  • symmetrical/bilateral
  • last from 30 mins to 7 days
  • have a pressing or tightening quality
  • unaffected by physical activity
  • management → paracetamol or NSAIDs for acute
33
Q

Describe characteristics of a cluster headache

A
  • severe, unilateral, orbital, supra-orbital and/or temporal
  • last from 15-180 mins
  • pain tends to be relieved by physical activity
  • autonomic features often accompany the headache (eg. nasal congestion, watering/redness of eye)
  • management → providing oxygen + subcut/nasal triptan may be benefician in acute attacks, verapamil can be effective prophylactically
34
Q

Autism spectrum disorder (ASD) is characterised by persistent impairments in social communication, and restricted, repetitive, and stereotyped patterns of behaviours, interests, or activities. ASD is caused by genetic and non-genetic factors; other factors are also likely to have a role in causing ASD. Boys are affected more (4:1).

What are the clinical features of autism spectrum disorder?

A
  • SOCIAL functioning + interaction ⇒
    • lack theory of mind: do not appreciate that others have thoughts + feelings
    • prefers own company
    • does not share pleasure from relationships + seek comfort
    • gaze avoidance
    • does not understand social cues, inappropriate behaviour
    • no make-believe or social play
  • OBSESSIONAL or ritualistic tendencies:
    • unusual stereotypical movements eg. tiptoe walking
    • intense resistance to change, insist on routine
    • peculiar interests
    • some have an exceptional skill in one area eg. calculation or music
  • LANGUAGE + communication:
    • delayed development
    • echolalia
    • limited use of gestures + facial expressions
    • over-literal interpretation of speech
35
Q

What is the management for ASD?

A
  • applied behavioural analysis (ABA) is the most widely accepted treatment
  • requires 25-40hrs a week of individual therapy making it costly + time consuming
  • appropriate educational centre which may incorporate the ABA approach
36
Q

Muscular dystrophy is a set of muscle diseases characterised by weakness and wasting of muscles. They all have a recognisable pattern of inheritance. Diagnosis can be made by muscle biopsy.

What is Duchenne Muscular Dystrophy (DMD)?

A
  • an inherited primary myopathy
  • X-linked recessive disease
  • causes lack of dystrophin ⇒ progressive muscle necrosis + weakness
  • most common inherited neuromuscular disease, affecting roughly 1 in 3,600 male children
37
Q

What are the clinical features of a muscular dystrophy?

A

Children first present w/

  • delayed walking
  • frequent falls
  • developmental delay (eg. lang delay)
  • weakness ⇒ waddling gait or Trendelenburg gait → both due to proximal muscle weakness
  • muscle pseudohypertrophy (particularly in calves) is also seen, where muscle enlarges due to fat deposition rather than muscle
  • muscle weakness in the back ⇒ kyphoscoliosis
  • Gower’s sign typically starts as early as the age of 3 + develops by 5-6yrs of age, it refers to motion of using hands to climb over their body in order to stand
38
Q

What investigations can be done for muscular dystrophy?

A
  • elevated serum creatine kinase (CK)
  • genetic testing ⇒ Xp21 mutation
  • muscle biopsy ⇒ absence of dystrophin
39
Q

What is Becker’s Muscular Dystrophy (BMD)?

A
  • BMD similar disease to DMD
  • caused by similar genetic defect in same locus X chromosome
  • clinical presentation milder bc dystrophin is still produced in small amounts
  • age of onset is delayed, typically at around 10yrs old
  • patients are usually able to walk until they are 20 to 30 years old
  • life expectance is far greater, at around 40 years old
40
Q

What is the 3-stage physical management of muscuar dystrophies?

A
  • stage 1 (ambulatory) ⇒ goals include early diagnosis, psychological support, prevention or reduction of musculotendinous contractures of extremities, role of exercise + preservation of muscle strength w/ use of glucocorticoids
  • stage 2 (non-ambulant) ⇒ goals include maintenance of optimal nutrition + activities of daily living as well as prevention of scoliosis
  • stage 3 (ventilator-supported) ⇒ goals include inspiratory and expiratory muscle rest and support

Common goals of all 3 stages are prevention or reduction of soft-tissue contractures of the chest, prevention of cardiac complications + application of the respiratory protocol