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Y5 Paediatrics > Neuro > Flashcards

Neuro Flashcards

1
Q

How can you classify headaches

A

Primary
Secondary
Trigeminal /other cranial neuralgia

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2
Q

WHat are primary headaches

A

Migraine
Tension
Cluster

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3
Q

What are the secondary headaches

A

intracranial haemorrhage
raised ICP
substance / its withdrawal
infection

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4
Q

Describe tension headacher

A

symmetrical
gradual onset
tightness

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5
Q

Describe migraine without aura

A 
Bilat /unilat 
pulsatile (frontal / temporal area) 
GI disturbance 
photophobia, phonophobia 
worse with activity, loud noises, light
better with sleep, dark
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6
Q

Describe migraine WITH aura

A

aura is usually visual disturbance

  • negative phenomenon: hemianopia, scotoma
  • positive phenomenon: zigzag
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7
Q

How do you manage migraine

A

Heacache diary

Nasal sumatriptan + NSAID/paracetamol

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8
Q

What are features of raised ICP

A 
worse lying down, straining
morning vomiting 
changes in mood/personality 
visual field defect 
abnormal gait
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9
Q

What is a seizure

A

paroxysmal abnoprmality of motor, sensory, autonomic, cognitive unction

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10
Q

What is an EPILEPTIC seizure

A

seizure caused by ABNORMAL underlying electrical activity

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11
Q

What are causes of seizures categorised as

A

epileptic

non-epilleptic

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12
Q

causes of epileptic seizure

A
  • idiopatic - cause UNKNOWN
  • cerebral vascular occlusion
  • cerebral dysgenesis/malformation
  • cerebral damage
  • cerebral tumour
  • neurodegenerativ disorder
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13
Q

What is causes of non epileptic seizures

A 
febrile seizure 
metabolic (hypoglycaemia, hypernatraemia, hyponat) 
head trauma 
meningitis 
encephalitis 
toxins, poisons
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14
Q

What is a febrile seizure

A

Seizure accompanied by FEVER without intracranial infection
without prior non febrile seizure
without infection of brain tissue

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15
Q

What are febrile seizures like

A

brief generalised tonic clonic

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16
Q

How do you manage a seizure in a child

A

protect from injury (cushion head)
remove harmful objects
Do NOT restrain / put anything in mouth
Once seizure stops, place in recovery position

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17
Q

What do you do if seizure is longer than 5 mins

A

STATUS EPILETTICUS
if IV access: Lorazepam

otherwise:
- rectal diazepam (repeated once after 5 mins)
- buccal midazolam

CALL FOR SENIOR HELP

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18
Q

What do you do if they are still seizing after the first round of medication > what about after that

A

if still seizing after 10 minutes: IV/IO Lorazepam (0.1mg/kg)

If still seizing after 10 mins: Senior Help (anaethetic/ICU)

  • phenytoin 20mg/kg IO/IV over 20 mins
  • if on phenytoin already: phenobarbitone 20mg/kg IV/IO over 20 mins
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19
Q

What are the types of epileptic seizures

A 
Tonic clonic 
atonic 
tonic 
myotonic 
absecnce
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20
Q

what is a myotonic seizure like

A

brief repetitive jerky movement of limbs

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21
Q

what is a tonic seizure like

A

increase in tone

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22
Q

whart is an atonic seizure like

A

loss of muscle tone (causes fall to floor / drop of head)

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23
Q

what are investigations for atonic seizure

A

ECG
EEG
MRI

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24
Q

Why do yu need to do an ECG in child with seizure

A

exclude arrhytmia causing convulsive syncope (e.g. long QT)

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25
Q

What is appropriate choice of antiepileptic

A

GENERALISED (tonic clonic, absence, myoclonic): VALPROATE

FOCAL: carbamazepine, lamotrigine

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26
Q

What are locations for neuromuscular disorders, and give examples of the disorders that arise

A

Anterior horn:

  • spinal muscular atrophy
  • polio

Peripheral nerve:

  • hereditary sensory myopathy = Charcot Marie Tooth Disease
  • GBS
  • Bell’s

NMJ
- MG

Muscle disorders

  • Duchenne/becker
  • Myositis, polymiositis, dermatomyositis
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27
Q

What is Gower’s sign

A

Need to turn prone to rise
Normal until 3yo
Then indicates weakness

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28
Q

What is spinal muscle atrophy

A

AR - mutation in survival motor neurone 1
causes progressive weakness and wasting of skeletal muscle
T1: Werndig-Hoffman Disease (reduced foetal movements, arthrogyposis, death from resp failure)

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29
Q

What is another name for Charcot Marie Tooth Disease

A

hereditary sensory myopathy

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30
Q

Explain Charcot Marie Tooth

A

mutation in myelin genes

causes symmetrical slow progressive distal muscle wasting

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31
Q

How does Charcot Marie Tooth present

A

during preschool

tripping from bilat foot drop

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32
Q

What does exam for Charcot Marie Tooth reveal

A

loss of ankle reflex
pes cavus
LL>UL

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33
Q

What are ix for Charcot Marie Tooth

A

nerve conduction studies

Nerve biopsy- onion bulb formation

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34
Q

What is GBS caused by

A

post infectious polyneuropathy

occurs 2-3 weeks post URTI/campylobacter infection

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35
Q

How does GBS present

A

ascending, progressive, symmetrical weakness over days / weeks
loss of tendon rteflexes

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36
Q

what is the risk of GBS

A

risk of aspiration (due to difficulty swallowing(

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37
Q

How do you Ix GBS

A

MRI
LP (high CSF protein, normal WCC)
Nerve conduction (reduced velocities)

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38
Q

What is bell’s palsy

A

LMN paresis of facial nerve (CN7)

39
Q

What can trigger bells palsy

A

HSV

Lyme disease

40
Q

What causes MG

A

antibody to nAch receptor in NMJ

41
Q

What is presentation of MG

A 
Opthalmoplegia 
Ptosis 
Facial droop 
Difficulty chewing 
Proximal weakness (also generalised)
42
Q

How do you investigate MG

A

Tensilon test

43
Q

How do you manage MG

A

Cholinesterase inhibitors e.g. pyridostigmine, neostigmine

44
Q

What do muscular dystrophies cause

A

progressive muscular degeneration

45
Q

What is duchenne muscular dystrophy (genetically)

A

x linked recessibe

deletion in dystrophin gene > myofibre necrosis

46
Q

How does duchenne muscular dystrophy present

A

waddling gait
language delay
Gower sign positive

47
Q

What is prognosis for duchenne muscular dystrophy (

A

not walking at 10yo

death at 20-30 yo from resp failure

48
Q

How do you manage duchenne muscular dystrophy (

A

physio
exercise
Surgery for scoliosis, tendoachilles lengthening
Weakness of intercostal muscles > nocturnal hypoxia > overnight CPAP

49
Q

Explain becker muscular dystrophy

A

milder, progresses slowly

50
Q

How does dermatomyositis present

A 
fever
mysery 
symmetrical muscle weakness 
purple rash on eyelids 
periorbital oedema 
HIGH CK
51
Q

What is Fredrich ataxi

A

AR > lack of frataxin protein

52
Q

How does Fredrich ataxia present

A

DAWN P

Dysarthria
Ataxia
Wasting of LL (distal)
No reflexes

Pes cavus

53
Q

What is anencephaly

A

failure to develop cranium and brain

stillborn /die after birth

54
Q

What is encephalocoele

A

extrusion of brain and meninges through midline skull defect

55
Q

What is spina bifida occulta

A

failuyre of fusion of vertebral arch

incidental finding on X reay

56
Q

How does spina bifida occulta present

A

tuft of hair, lipoma, birth mark

57
Q

what is meningocoele

A

sac protrudes from the body but only contains CSF

58
Q

What is myelomeningocoele

A

severe spina bifida

spinal corrd and nerves develop outside in CSF filled sac

59
Q

How do you manage neural tube defects

A

surgical closure of lesion
physio
walking aids, wheelchair
neuropathic bladder…

60
Q

what are the two types of hydrocephalus

A

communicating: failure to absorb CSF at arachnoid villi

non-communicating: obstruction in venricular system / aqueduct

61
Q

Give causes of communicating hydroceph

A

SAH

meningitis

62
Q

Give causes of non comm hydroceph

A

congenital malformation (aqueduct stenosis, fourth ventricle atrasia, Chiari)

63
Q

How do you investigate hydrocephalus

A

cranial USS
CT/MRI
head circumference monitored, plotted on centile chart

64
Q

What is management for hydrocphalus

A

insert ventriculoperioteal shunt

65
Q

What is NF1 pattern of inheritance

A

Autosomal DOMINANT mutation in NF1 gene

66
Q

What is criteria for NF1 dx

A

Two or more of following:

  • 6+ cafe au lait spots
  • 1+ neurofibroma
  • axillary freckling
  • optic glioma
  • 1 lisch nodule
  • first degree relative with NF
67
Q

What are features of NF2

A
  • Schwannoma
  • Meningioma
  • Ependymoma
68
Q

What is tuberous sclerosis presentation

A

CUTANEOUS FTS:

  • ash leaf shaped patches / amelanotic lesions, fluoresce under UV light
  • Shagreen patches (roughened patches of skin over lumbar region)
  • Adenoma sebaceum (angiofibromata over cheeks)

NEURO FTS:

  • Infantile spasms
  • Developmental delay
  • Epilepsy
  • intellectual disability
69
Q

What does Sturge-Weber syndrome present with

A

Port wine stain in trigeminal N distribution
With similar lesion intracranially
ALWAYS involves opthalmic division of trigeminal nerve

70
Q

what is the triad occurring in shaken baby syndrome

A

retinal haemorrhages
subdural haematoma
encephalopathy

71
Q

What is West syndrome also known as

A

infantile spasm w

72
Q

what is west syndrome

A

form of childhood epilepsy

73
Q

when does west syndrome present

A

4-8months of life

74
Q

what are features of west syndrome

A
  • Salaam attacks - flexion of head, trunk and arms, followed by extension of arms
  • lasts 1-2 seconds, repeated x50,
  • progressive mental handicap
75
Q

how do you investigate west syndrome

A

EEG (hypsarrhyrhmia)

CT (brain disease e.g. tuberous sclerosis)

76
Q

what is prognosis for west syndrome

A

POOR

77
Q

what do you need to explain to parents of children with febrile convulsions

A

NOT same as epilepsy
Risk of epilepsy in the future is only slightly higher than normal population
Short lasting seizures are NOT harmful to child
1/3 of children will have another febrile convulsion
Reducing the fever does not prevent recurrence

78
Q

what are features of cerebellar disortder

A

DANISH D

Dysdiadocokinesia 
Ataxia
Nystagmus 
Intention tremor 
Slurred staccato speech 
Hypotonia

Dysmetria

79
Q

What is dysdiadocokinesia

A

inability to perform rapid alternating movements (palm to palm-dorsum)

80
Q

what is dysmetria

A

past pointing

81
Q

what are differentials for a FLOPPY BABY

A

CoMe GeNe

CENTRAL

  • HIE
  • cortical malformation

METABOLIC:

  • hypothyroidism,
  • hypocalcaemia

GENETIC: Down’s, Prader WIlli

PERIPHERAL - Neuromuscular

  • spinal muscular atrophy
  • myopathy
  • myotonia
  • congenital myasthenia
82
Q

What mutations occur in tuberous sclerosis

A

AUTOSIOMAL DOMINANT

TSC1 and TSC2

83
Q

How do you manage Duchenne

A

FOR MOVEMENT:

  • Physio
  • Exercise, psychological support
  • Surgery: Tendochilles lengthening and scoliosis surgery if required

FOR RESP:

  • Consider overnight CPAP (if weak intercostal muscles)
  • glucocorticoids e.g. prednisolone

TO PREVENT WHEELCHAIR DEPENDENCE:
- Glucocorticoids

84
Q

what can you give in Duchenne to prevent wheelchair dependence

A

glucocorticoids

85
Q

How do you manage West syndrome

A

corticosteroids e.g prednisolone, vigabatrin

86
Q

What investigations can you get in seizure ?

A 
Obs (temperature for fever etc)
Glucose, urine dip, MSU 
Bloods if indicated (e.g. U&E if D&V) 
Consider LP 
Consider CT if suspecting raised ICP/neuro origin
87
Q

When do you admit a child with a suspected febrile seizure

A

first febrile seizure / priorly not assessed
diagnostic uncertainty
seizure lasted >15mins
focal features during the seizure
seizure recurred in same febrile illness
incomplete recovery

88
Q

What is an extradural hemorrhage due to

A

Extradural haemorrhage: due to head trauma & skull fracture (middle meningeal artery rupture )

89
Q

What are sx of extradural haemorrhage

A

lucid interval

then consciousness deteriorates

90
Q

What is a subdural haematoma due to

A

tearing of bridging veins across subdural space (slow bleed)

due to shaking injury in children

91
Q

What is a subarachnoid haematoma due to

A

Rupture of aneurysm / AV malformation

92
Q

What are symptoms of SAH

A 
Severe headache, rapid onset 
vomiting 
confusion 
reduced consciousness 
cioma
93
Q

What are the 3 features of shaken baby syndrome

A

Shake Every Registrar

Subdural haemorrhage
Encepalopathy
Retinal haemorrhage

94
Q

How do you manage traumatic head injury in children

A

Criteria:

  • LOC >5mins
  • Drowsy
  • Vomiting 3+ times
  • Dangerous mechanism of injury
  • Amnesia >5mins
  • On warfarin

If 0-1; observe for 4h
If 2+: Urgent CT

Criteria severe:

  • GCS <15
  • suspected open/depressed skull fraacture
  • seizuree
  • focal neurological deficit

Immediate CT + trauma call

Y5 Paediatrics (30 decks)

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