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PAEDS NEURO Flashcards

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1
Q

CEREBRAL PALSY
What is cerebral palsy?
How does it progress?

A
  • Permanent disorder of movement + posture due to a non-progressive lesion of motor pathways in the developing brain
  • Sx develop over time as the brain starts to develop
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2
Q

CEREBRAL PALSY
What are the causes of cerebral palsy?

A
  • Antenatal (80%) = genetics, congenital malformations or infections
  • Intrapartum (10%) = hypoxic-ischaemic injury
  • Postnatal (10%) = IV haemorrhage (prems), meningitis/encephalitis, trauma (NAI), hydrocephalus, kernicterus
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3
Q

CEREBRAL PALSY
What are some early features of cerebral palsy?

A
  • Abnormal limb/trunk tone + posture with delayed motor milestones
  • Feeding issues > oromotor incoordination, slow feeding, gagging + vomiting
  • Abnormal gait when walking achieved
  • Hand preference before 12m + primitive reflexes after 6m
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4
Q

CEREBRAL PALSY
What are some non-motor presentations of cerebral palsy?

A
  • LDs, epilepsy, squints + hearing impairment
  • Joint contractures, hip subluxation + scoliosis
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5
Q

CEREBRAL PALSY
What are the 4 broad types of cerebral palsy?

A
  • Spastic (pyramidal, 70%)
  • Ataxic (10%)
  • Dyskinetic (athetoid, 10%)
  • Mixed (10%)
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6
Q

CEREBRAL PALSY
What is affected in spastic cerebral palsy?

A
  • UMN pathways damaged (pyramidal or corticospinal) so UMN signs
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7
Q

CEREBRAL PALSY
What are the 3 subtypes of spastic cerebral palsy?

A
  • Hemiplegic
  • Quadriplegic
  • Diplegic
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8
Q

CEREBRAL PALSY
What is spastic hemiplegic cerebral palsy?

A
  • Unilateral involvement of arm + leg (arm worse)
  • Face spared, fisting of affected hand, flexed arm, tip-toe walking
  • Presents 4–12m, normal birth with no HIE
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9
Q

CEREBRAL PALSY
What is spastic diplegic cerebral palsy?

A
  • All 4 limbs (legs worse)
  • Hand function may be relatively normal but walking abnormal
  • Linked with preterm birth (periventricular brain damage)
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10
Q

CEREBRAL PALSY
What is ataxic cerebral palsy?

A
  • Cerebellum affected > cerebellar signs
  • Early trunk + limb hypotonia (symmetrical)
  • Poor balance + delayed motor development
  • Incoordination, ataxic gait + intention tremor
  • Mostly genetics, can be acquired brain injury
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11
Q

CEREBRAL PALSY
What is dyskinetic cerebral palsy?

A
  • Intellect unimpaired as basal ganglia affected (extra-pyramidal)
  • Associated with kernicterus + HIE
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12
Q

CEREBRAL PALSY
What is meant by chorea?

A

Irregular, sudden + brief non-repetitive movements

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13
Q

CEREBRAL PALSY
What are the investigations of cerebral palsy?

A
  • Clinical Dx (assess posture, pattern of tone, hand function + gait)
  • Functional ability judged by Gross Motor Function Classification System
  • MRI head to identify cause but not necessary for Dx
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14
Q

CEREBRAL PALSY
What are the stages of the Gross Motor Function Classification System?

A
  • I = walks without limitation
  • II = with limitation
  • III = handheld mobility device
  • IV = III with limitation
  • V = wheelchair
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15
Q

CEREBRAL PALSY
What is the MDT approach of cerebral palsy?

A
  • Drs
  • Physio (tone + posture issues)
  • SALT (swallowing issues)
  • OT (home adjustments, help with ADLs)
  • School (special educational needs)
  • Social workers (benefits)
  • Dietitians (?PEG feeding)
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16
Q

CEREBRAL PALSY
What is the management of spasticity in cerebral palsy?

A
  • PO or IT baclofen
  • PO diazepam
  • Botox injection
  • Orthopaedic surgery
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17
Q

VISION
What is vision like in children?

A
  • Visual acuity is poor in the newborn but increases to adult levels by age 4
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18
Q

VISION
What are some causes of severe visual impairment?

A

Genetic –

  • Congenital cataracts
  • Albinism
  • Retinal dystrophy
  • Retinoblastoma
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19
Q

VISION
How might visual impairments present in children?

A
  • Loss of red reflex (i.e. cataract)
  • White reflex in pupil (retinoblastoma, cataract, retinopathy of prematurity)
  • Not smiling responsively by 6w
  • Lack of eye contact with parents
  • Random eye movements
  • Failure to fix + follow
  • Nystagmus, squint, photophobia
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20
Q

VISION
When is vision screened in children?

A
  • Pre-school + school entry
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21
Q

STRABISMUS
What is strabismus?
Is it normal?

A
  • Misalignment of visual axis (squint)
    • Transient neonatal misalignments common in first few months when looking at near objects, reduce by 2m, gone by 12w
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22
Q

STRABISMUS
What are the 2 divisions of strabismus?

A
  • Concomitant (non-paralytic, common) = often refractive error, differences in control of extra-ocular muscles
  • Paralytic (rare) = paralysis in ≥1 of the extra-ocular muscles (if rapid onset may be sinister SOL)
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23
Q

STRABISMUS
What is the difference between a manifest and latent strabismus?

A
  • Manifest = present when views a target binocularly
  • Latent = binocular vision interrupted
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24
Q

STRABISMUS
What are the different types of manifest strabismus?
How does this compare to latent strabismus?

A
  • Esotropia = inward moving (cross-eyed)
  • Exotropia = outward moving
  • Hypertropia = upward moving
  • Hypotropia = downward moving
  • Latent is same but -phoria not -tropia (esophoria etc)
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25
STRABISMUS What is the pathophysiology of strabismus?
- When eyes not aligned the images on retina do not match + pt will experience diplopia - When this occurs in paeds, before the eyes have fully established their connections within the brain, the brain will cope by reducing the signal from the less dominant eye
26
STRABISMUS What is a complication of strabismus?
- One eye used to see (dominant) + one eye ignored (lazy) - If untreated lazy eye becomes progressively more disconnected from brain over time + problem worsens (amblyopia)
27
STRABISMUS What causes strabismus?
- Multifactorial (combination of hereditary + refractive errors) - Idiopathic - Secondary to vision loss - Higher incidence in cerebral palsy - SOL (retinoblastoma) rare but suspect if sudden onset + other neurology
28
STRABISMUS What are 3 types of refractive errors?
- Hypermetropia = long-sightedness (common, hard to see nearby objects) - Myopia = short-sightedness (uncommon, less likely to cause permanent visual damage) - Amblyopia = defective visual acuity that persists after correction of refractive error + removal of any pathology (lazy eye)
29
STRABISMUS How does amblyopia present?
- Often unilateral, potentially permanent loss of visual acuity in an eye that has not received a clear image
30
STRABISMUS What are some investigations for strabismus?
- (Single) cover test for manifest/tropias - Cover-uncover (alternate cover) test for latent/phorias - Corneal light reflex test (Hirschberg's test) - Important to assess visual acuity + ocular movements to exclude paralytic
31
STRABISMUS Explain the cover test
- Cover eye + observe the other eye for a shift in fixation - Direction of shift indicates the type of tropia - Close (33cm) + distant (6m) as some squints only present at one distance
32
STRABISMUS Explain the cover-uncover test
- Helps determine if misalignment is a tropia or phoria - Occlude eye + then quickly uncover, observing the occluded eye for refixation movement - A phoria will shift back to being straight, direction is opposite to the movement (shifts lateral = esophoria)
33
STRABISMUS Explain the corneal light reflex test
- Shine a pen torch in the eyes - Reflection of light should appear in the same position in both eyes
34
STRABISMUS What is the management of amblyopia?
- Early Tx = better to avoid damage, <7y ideal - Refractive adaptation = wear appropriate glasses for 16-18w - Occlusion of 'good' eye (part/full time) to force weaker eye to develop - Atropine drops in better eye causing vision in that eye to be blurred
35
STRABISMUS What are the aims of strabismus management?
- Restore comfortable binocular single vision - Eliminate diplopia - Restore good alignment of the eyes
36
STRABISMUS What are some conservative techniques for strabismus?
- Glasses/contact lenses - Prisms for diplopia - Orthoptic exercises to improve control over eye muscles
37
STRABISMUS What medical treatment can be given in strabismus?
- Botox injections = paralyse the muscle that is pulling the eye in a certain direction - Esotropia = MR, exotropia = LR - May need repeat injections as effects wear off, ketamine anaesthesia
38
STRABISMUS What surgery may be offered in strabismus?
- Strengthening procedure = resection of muscle on side eye does not face - Weakening procedure = recession of muscle on side the eye goes towards - Esotropia = bilateral MR recession or MR recession + LR resection - Exotropia = bilateral LR recession or LR recession + MR resection
39
HEARING What are the 3 types of hearing loss?
- Sensorineural = nerve damage (progressive, never reversible) - Conductive = obstruction in ear canal which prevents sound from getting through, bone conduction can still transmit sound (often reversible) - Mixed
40
HEARING What are some causes of conductive hearing loss?
- #1 = congestion behind eardrums (viral URTI) - Glue ear, ear wax, middle ear infection, perforated ear drum - Structural abnormality of the outer ear (syndromes)
41
HEARING What are some causes of sensorineural hearing loss?
- Genetic or syndromes - Perinatal (trauma, infection, hypoxia) - Congenital infections (rubella, CMV) - Meningitis (pneumococcus can cause ossification of cochlear)
42
HEARING What are some risk factors for conductive hearing loss?
- Down's syndrome, - craniofacial syndromes - cleft palate
43
HEARING How might hearing loss present?
- Parental concern - Incidental finding on screening - Problems with speech, behaviour or education (ignoring calls or sounds)
44
HEARING What are some behavioural changes that might occur in hearing loss?
- Sits near TV + volume loud - Misunderstands/slow in responding or answers incorrectly - Soft/fuzzy speech - Does not turn immediately when named called
45
HEARING What 2 types of hearing tests are part of the newborn hearing screening programme (NHSP)?
- Evoked otoacoustic emission (EOAE) - Auditory brainstem response (ABR) audiometry if EOAE fails
46
HEARING What is evoked otoacoustic emission? What are the pros? What are the cons?
- Earphone produces sound which evokes an echo from ear if cochlear function normal - Simple + quick - Misses auditory neuropathy, cochlear test not hearing, high false +ve in first 24h
47
HEARING What is auditory brainstem response audiometry? What are the pros? What are the cons?
- Computer analysis of EEG waveforms evoked in response to auditory stimuli - Screens hearing pathway ear>brainstem, low false +ve rate - Affected by movement (time consuming), electrodes on infant's head, complex computerised gear
48
HEARING What testing might be done in children 6–9m?
- Distraction testing - Relies on baby locating + turning appropriately to high + low frequency sounds out of field of vision - 2x trained staff
49
HEARING What testing might be done in children 10–18m?
- Visual reinforcement audiometry - Hearing thresholds are established using visual rewards (illumination of toys) to reinforce the child's head turn to stimuli of different frequencies - First test that does single ear measures
50
HEARING What hearing tests are done at... i) >2y? ii) >2.5y? iii) 4y?
i) Performance testing = child performs an action when hear a noise ii) Speech discrimination tests (McCormick toy test) iii) Pure tone audiometry at school entry = child responds to pure tone stimulus with headphones
51
HEARING What are 3 main investigations in hearing?
- Rinne's test (mastoid then external acoustic meatus) - Weber's (forehead in midline) - Audiograms
52
HEARING What does Rinne's test show you?
- Normal = louder at EAM - Conductive = louder on mastoid - Sensorineural = both decreased
53
HEARING What does Weber's test show you?
- Normal = vibrations equal in both ears - Conductive = louder in abnormal ear - Sensorineural = louder in normal ear
54
HEARING What do audiograms show you?
- Frequency (Hz, x) low>high + volume (dB, y) bottom = loud - Anything above 20dB line is normal - Sensorineural = both air + bone conduction impaired (>20dB) - Conductive = only air conduction impaired (>20dB), bone normal - Mixed = both impaired but air worse (>15dB difference)
55
HEARING What are some complications of hearing issues?
- Developmental delay (speech + language) - Social/behavioural problems (too loud or too quiet) - Impact on education, friendships/social life + psychologically
56
HEARING What is the management of conductive hearing loss?
- Most self-limiting so watch + wait - ENT referral for insertion of grommets to help drain excess fluid if necessary - Temporary hearing aids or if permanent cause
57
HEARING What is the management of sensorineural hearing loss?
- Hearing aids > aim to improve hearing so as much speech audible as possible - Cochlear implants reserved for profound hearing loss (>95dB), high frequency, bilateral hearing loss or meningitis related
58
HEARING What is the management of mixed hearing loss?
- Correct conductive problem first + then offer hearing aid
59
DEVELOPMENT MDT What is the child development service?
- MDT + multi-agency (health, social services, education) with aim of providing coordinated service with good inter-agency liaison to meet the functional needs of the child
60
DEVELOPMENT MDT What is the role of child developmental services?
- Liaise between home, school + the child's care needs - Assess child's functional ability + need - Provide therapy + psychosocial support where needed - Ensure health needs of child are met
61
DEVELOPMENT MDT Name some members of the child development services MDT and their role
- Paediatrician = Ax, Ix + Dx, continuing medical Mx, coordination - Physio = balance + mobility, prevent contractures, mobility aids - OT = ADLs, house adaptations - SALT = feeding, speech + language development - Dietician = advice on nutrition - Social worker = benefits - Psychologist = cognitive testing, behaviour management - Specialist health visitor + key workers = coordinate MDT + multi-agency care
62
SEIZURES What is a seizure?
- Sudden disturbance of neurological function due to excessive neuronal discharge
63
SEIZURES What are some causes of seizures?
- Epilepsy - Febrile convulsions - Metabolic (hypoglycaemia, hypocalcaemia) - Head trauma, infection (meningitis, encephalitis) - Toxins, iatrogenic (post-brain surgery)
64
FEBRILE CONVULSIONS What is a febrile convulsion?
- Seizures provoked by a fever in otherwise normal children
65
FEBRILE CONVULSIONS What is a simple febrile convulsion?
- Generalised (tonic-clonic) seizure - <15m - Typically no recurrence within 24h - Recovery within 1h
66
FEBRILE CONVULSIONS What is a complex febrile convulsion?
- Focal seizure - 15–30m - May recur within 24h
67
FEBRILE CONVULSIONS What are some complications of febrile convulsions?
- Usually no lasting damage - 1 in 3 will have another febrile convulsion - 2-7% will develop epilepsy after simple, 10–20% after complex
68
FEBRILE CONVULSIONS What is the management of febrile convulsions?
- Period of observation, paeds referral if first seizure or complex - Antipyretics have NOT shown to reduce risk of recurrence - Education = stay with them, ensure safe, nothing in mouth, call 999 if lasts >5m, teach how to use PR diazepam or buccal midazolam if Hx of prolonged seizures (>5m)
69
EPILEPSY What is epilepsy?
- Recurrent tendency to have unprovoked seizures
70
EPILEPSY What are some causes of epilepsy?
- 2/3rd idiopathic, FHx, alcohol/drugs (+ withdrawal) - Brain injury (hypoxia, trauma, surgery) - SOL (tumour, abscess) - CNS infection (meningitis, encephalitis)
71
EPILEPSY What are the two different types of seizures?
- Focal/partial seizures = arise from one area of cortex or part of one hemisphere - Generalised = discharge arises from both hemispheres
72
EPILEPSY What are the 3 types of partial seizures?
- Simple-partial = consciousness + awareness - Complex-partial = without consciousness + awareness - Secondary generalised = seizures start in one hemisphere but then spread to both (focal>general)
73
EPILEPSY How would a partial seizure present in... i) frontal lobe? ii) temporal lobe? iii) parietal lobe? iv) occipital lobe?
i) Strange smells, motor movements, Jacksonian march ii) Déjà/jamais-vu, automatisms (chewing, lip smacking), hallucinations, aura/sensations, amnesia iii) Contralateral altered sensations (tingling, electric-shock) iv) Flashing lights, eyelid fluttering, eye movements
74
EPILEPSY What is... i) Jacksonian march? ii) Todd's paresis?
i) Motor twitch starts on one side of body then "marches" over a few seconds to affect larger parts of both like entire hand, foot + may generalise ii) Focal weakness in a part or all of the body after a seizure
75
EPILEPSY What are the 4 main types of generalised seizures?
- Absence seizures - Tonic-clonic seizures - Myoclonic seizures - Atonic (akinetic) seizures "drop attacks"
76
EPILEPSY What are absence seizures?
Brief (<30s) pauses where activity stops (still, silent, stares), may have slight eyelid flickering, can be precipitated by hyperventilation
77
EPILEPSY What is a tonic-clonic seizure?
- Tonic = vague warning, rigidity, falls + may make sound, LOC + can stop breathing - Clonic = convulsions, bilateral rhythmic muscle jerks, irregular breathing, may tongue bite (lateral) or urinary incontinence - Post-ictal = drowsy, confused, irritable or depressed
78
EPILEPSY What is a myoclonic seizure?
- Brief, sudden muscle contractions like jerk of limb, face or trunk - Usually remains awake, can occur in juvenile myoclonic epilepsy - Can be physiological (hiccups, sleep myoclonus)
79
EPILEPSY What is an atonic (akinetic) seizure/drop attack?
- Brief, sudden loss of muscle tone causing a fall but no LOC - Often begin in childhood, ?indicate Lennox-Gastaut syndrome
80
EPILEPSY What are 4 epilepsy syndromes seen in children?
- Infantile spasms (West's syndrome) - Lennox-Gastaut syndrome - Juvenile myoclonic epilepsy - Benign Rolandic epilepsy = M>F, paraesthesia (unilateral face, tongue, twitching) during sleep, EEG shows centrotemporal focal spike waves
81
EPILEPSY Who is affected by infantile spasms?
- Early life (4-6m), M>F, often secondary to serious neuro abnormality (tuberous sclerosis, encephalitis, birth asphyxia)
82
EPILEPSY What are the 3 components to infantile spasms?
- Violent flexor spasms of head, trunk + limbs followed by extension of arms (salaam spasms) for 1-2s, can repeat up to 50 times - Progressive mental handicap - EEG shows hypsarrhythmia
83
EPILEPSY What is Lennox-Gastaut syndrome? How does it present? Management?
- Can be extension of infantile spasms, 1-5y - Atypical absences, falls, jerks + 90% have mod-severe mental handicap - EEG shows slow spike, ketogenic diet may help
84
EPILEPSY Who is juvenile myoclonic epilepsy more common in? How does it present? Management?
- Teens, F>M - Infrequent generalised seizures (often morning), daytime absences, sudden shock-like myoclonic seizures (can happen before seizures) - Good response to valproate
85
EPILEPSY What are some investigations for epilepsy?
- Mostly clinical Dx + witness Hx crucial - Exclude organic causes with FBC, U+E, LFTs, glucose, ECG - EEG, often sleep deprived or hyperventilate to provoke - ?Neuroimaging (CT/MRI head) if focal neurology + concerns of SOL
86
EPILEPSY What is the general management of epilepsy? What are the treatment principles?
- Ensure little harm, maintain airway, do not restrain - Aim for monotherapy, maximum tolerated dose before changing or adding drugs, avoid abrupt withdrawal
87
EPILEPSY What is the management of generalised seizures?
- 1st line = sodium valproate - 2nd line = lamotrigine, carbamazepine (TC), clonazepam (myoclonic)
88
EPILEPSY What is the management of focal seizures?
- 1st line = carbamazepine or lamotrigine - 2nd line = levetiracetam or sodium valproate
89
EPILEPSY What is the management of absence seizures?
- Ethosuximide or sodium valproate
90
EPILEPSY What is a note about treatment with carbamazepine?
- Can exacerbate absent + myoclonic seizures
91
'FUNNY TURNS' What are the triggers for reflex anoxic seizures?
- Pain or discomfort, - cold food, - fright (emotions)
92
'FUNNY TURNS' What are the triggers for breath holding attacks?
Upset, worked up or angry
93
'FUNNY TURNS' What are some other causes of 'funny turns'?
- Syncope - Migraine - Benign paroxysmal vertigo - Cardiac arrhythmias - NEAD - Fabricated by parent or child
94
'FUNNY TURNS' What is the presentation of syncope?
- Clonic movements may occur, - triggers like hot/stuffy, - standing long
95
DISORDERS Name 2 neurocutaneous disorders What is the mode of inheritance for these conditions?
- Neurofibromatosis (type 1 + 2) + tuberous sclerosis - AD
96
NEUROFIBROMATOSIS What is the clinical presentation of neurofibromatosis 1?
- No intellectual problems but lots of skin involvement - >5 café-au-lait spots - Axillary freckling in skin folds - Iris hamartomas, scoliosis + pheochromocytomas - Peripheral neurofibromas
97
NEUROFIBROMATOSIS What is the clinical presentation of neurofibromatosis 2?
- Hearing problems with no skin involvement - Bilateral vestibular schwannomas > sensorineural hearing loss then tinnitus + vertigo
98
TUBEROUS SCLEROSIS What are the cutaneous features of tuberous sclerosis?
- Hypopigmented 'ash-leaf' spots which fluoresce under UV light - Roughened (Shagreen) patches of skin over lumbar spine - Angiofibromas (butterfly distribution over nose) - Subungual fibromata
99
TUBEROUS SCLEROSIS What are some other features of tuberous sclerosis?
- Neuro = epilepsy (infantile spasms or partial), developmental delay + intellectual impairment - Retinal hamartomas, - polycystic kidneys, - rhabdomyomata of heart
100
NEURAL TUBE DEFECTS What are neural tube defects?
- Failure of normal fusion of the neural plate to form neural tube during first 28d pregnancy
101
NEURAL TUBE DEFECTS What are 5 different types of neural tube defects?
- Spina bifida occulta (#1) - Meningocele - Myelomeningocele (most severe) - Anencephaly - Encephalocele
102
NEURAL TUBE DEFECTS What is spina bifida occulta?
- Failure of fusion of the vertebral arch, often incidental XR finding
103
NEURAL TUBE DEFECTS What is meningocele?
- Sac of fluid protruding spinal canal (without neural tissue), not exposed
104
NEURAL TUBE DEFECTS What is a myelomeningocele?
- Sac of fluid protruding spinal canal (with neural tissue), open lesion
105
NEURAL TUBE DEFECTS How does Chiari malformation lead to hydrocephalus?
- Herniation of cerebellar tonsils + brainstem > foramen magnum = disrupt CSF flow
106
NEURAL TUBE DEFECTS What is anencephaly?
- Failure to develop cranium + brain, - stillborn or die rapidly
107
NEURAL TUBE DEFECTS What is encephalocele?
- Brain + meninges extrude through midline skull defect
108
NEURAL TUBE DEFECTS What can be used as prophylaxis for neural tube defects?
- Folic acid (0.4mg for normal pregnancies, 5mg if high risk
109
CEREBRAL PALSY What is spastic quadriplegic cerebral palsy?
- All 4 limbs, trunk involved with tendency to opisthotonus (extensor posturing), poor head control + low central tone - More severe > associated with seizures, microcephaly, low IQ - May have Hx of HIE
110
GLUE EAR What is glue ear/otitis media with effusion (OME)?
- Most common cause of conductive hearing loss in children
111
GLUE EAR What is the management?
- Insertion of ventilation tubes (grommets) to drain excess fluid - Adenoidectomy as adenoids can harbour organisms + obstruct Eustachian tube so poor ventilation + drainage
112
OTITIS MEDIA Who is most at risk?
Younger children as Eustachian tubes short, horizontal + function poorly
113
GLUE EAR What investigations would you do?
- Otoscopy (TM appears dull + retracted, often with visible fluid level) - Flat trace on tympanometry + evidence of conductive loss on pure tone audiometry (or reduced hearing on distraction test if younger)
114
GROMMETS what are grommets?
tiny tubes inserted into the tympanic membrane they allow fluid to drain from the middle ear into the ear canal
115
DEAFNESS what are the different types of deafness?
sensorineural conductive
116
DEAFNESS At what volume does deafness begin to cause problems with development?
hearing loss up to 20dB does not affect development loss over 40dB affects speech and language development
117
DEAFNESS what are the causes of sensorineural hearing loss?
Inherited/genetic - ushers syndrome, Waardenburg syndrome Acquired - perinatal - birth asphyxia, hyperbilirubinemia, congenital infection (rubella, CMV, syphilis) - postnatal - drugs, meningitis, head injury, labyrinthitis, acoustic neuroma
118
DEAFNESS what are the causes of conductive hearing loss?
External - ear canal atresia/stenosis Middle ear - acute/chronic otitis media, glue ear
119
PERIORBITAL CELLULITIS what is it?
an infection of the eyelid or skin around the eye
120
CEREBRAL PALSY What are the main features of spastic cerebral palsy?
- Limb tone persistently increased (spasticity, velocity-dependent) - Brisk deep tendon reflexes + extensor plantars (+ve Babinski) - Increased limb tone may suddenly yield under pressure (clasp knife)
121
CEREBRAL PALSY What are the features of dyskinetic cerebral palsy?
- Intellect unimpaired as basal ganglia affected (extra-pyramidal) - Chorea, athetosis, dystonia - Muscle tone is variable (floppiness), involuntary movements, poor trunk control - Associated with kernicterus + HIE
122
CEREBRAL PALSY What is meant by athetosis?
Slowly writhing movements distally like fanning fingers
123
CEREBRAL PALSY What is meant by dystonia?
Twisting appearance from simultaneous contraction of agonist + antagonist muscles
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STRABISMUS What are some causes of amblyopia?
Any interference with visual development > squint, refractive error, ptosis, cataracts
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HEARING What are some risk factors for sensorineural hearing loss?
- Premature, - FHx - consanguinity
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FEBRILE CONVULSIONS What are the 3 types?
- Simple - complex - febrile status epilepticus
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FEBRILE CONVULSIONS When does it happen?
Usually early in viral infections as temperature rises rapidly
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FEBRILE CONVULSIONS What is the epidemiology?
- 3% of children, - 6m–6y - often genetic predisposition
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FEBRILE CONVULSIONS What is a febrile status epilepticus?
A complex febrile convulsion that lasts for over 30 minutes
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EPILEPSY What are the 2 phases?
- Ictal = early phase w/ positive Sx such as excessive activity (jerky actions) - Post-ictal = later phase w/ negative Sx such as weakness, drowsiness
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EPILEPSY What is the epidemiology of absence seizures?
- Onset 4-8y, - F>M, - may progress tonic-clonic later
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EPILEPSY What are the investigations for absence seizures?
EEG = 3hz generalised spike, symmetrical
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EPILEPSY What is the prognosis for absence seizures?
Good, 90% seizure free in adolescence
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EPILEPSY What is the management of infantile spasms?
Vigabatrin or corticosteroids (poor prognosis)
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EPILEPSY What is the management of myoclonic seizures?
- 1st line = sodium valproate - 2nd line = clonazepam
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EPILEPSY What last line treatments may be used in epilepsy?
- Vagal stimulation, - surgery (hemispherectomy, non-dominant lobectomy)
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'FUNNY TURNS' What are the causes of reflex anoxic seizures?
Cardiac asystole from vagal inhibition to heart (syncopal episode)
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'FUNNY TURNS' What is the clinical presentation of reflex anoxic seizures?
- Child becomes pale + falls to floor, - hypoxia may induce generalised tonic-clonic seizure which is brief + child RAPIDLY recovers
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'FUNNY TURNS' What are the investigations for reflex anoxic seizures?
- Ocular compression under controlled conditions often lead to asystole - paroxysmal slow-wave discharge on EEG
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'FUNNY TURNS' What is the clinical presentation of breath holding attacks?
- Child cries, holds their breath + goes cyanotic, - sometimes brief LOC but RAPID recovery
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'FUNNY TURNS' What are breath holding attacks associated with?
Linked with Fe anaemia so treating as such may minimise further ones
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'FUNNY TURNS' What is the presentation of benign paroxysmal vertigo?
- Nystagmus, - unsteady, - headache
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'FUNNY TURNS' What is the presentation of migraine?
- Can happen ± headache, - unsteadiness/light headedness with other classic migraine Sx
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TUBEROUS SCLEROSIS What are the investigations?
CT/MRI will detect calcified subependymal nodules + tubers from 2nd year of life
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NEURAL TUBE DEFECTS What are the risk factors?
If you have a child with neural tube defect there is 10x increased risk of second foetus having similar problems
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NEURAL TUBE DEFECTS What are neural tube defects associated with?
- Insufficient folic acid - anti epileptic drugs
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NEURAL TUBE DEFECTS What is the presentation of spina bifida occulta?
Site may have identifiable birthmark, lipoma or hair patch (lumbar)
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NEURAL TUBE DEFECTS What is the management of spina bifida occulta?
Neurosurgery
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NEURAL TUBE DEFECTS How may a myelomeningocele present?
- Paralysis of legs, - dislocation of hip + talipes, - sensory loss, - neuropathic bladder + bowel, - scoliosis - hydrocephalus from Chiari malformation
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NEURAL TUBE DEFECTS What is the management of myelomeningocele?
- Close back lesion, - physio for paralysis, - ?indwelling catheter, - shunt
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NEURAL TUBE DEFECTS What is the management of anencephaly?
Antenatal USS Dx with TOP usually performed
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NEURAL TUBE DEFECTS What is the management of encephalocele?
- Surgical correction but often underlying cerebral malformations
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DEVELOPMENTAL DELAY what are the referral points?
- doesn't smile at 10 weeks - cannot sit unsupported at 12 months - cannot walk at 18 months
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DEVELOPMENTAL DELAY what problems can indicate fine motor problems?
hand preference before 12 months - can indicate cerebral palsy

MY REVISION - ALL PHASES (36 decks)

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