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Peadiatrics > Neuro > Flashcards

Neuro Flashcards

1
Q

What is hydrocephalus

A

Enlargement of the cerebral ventricles due to excessive accumulation of CSF

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2
Q

What are the 2 types of hydrocephalus and where are the blockages

A

Non communicating - obstruction in ventricles / aqueduct

Communicating - arachnoid villi (where CSF absorbed)

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3
Q

Causes of non - communicating hydrocephalus

A

Brain tumour, inter ventricular haemorrhage, congenital malformation, dandy walker syndrome, Arnold - chiari malformation

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4
Q

Causes of communicating hydrocephalus

A

sub arachnoid haemorrhage, tuberculous meningitis, duct stenosis

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5
Q

Features of hydrocephalus

A

Head circumference - disproportionally large & rapid growing
Increased pressure on ant fontanelle - sutures separate, scalp veins prominent

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6
Q

What can increased pressure in hydrocephalus lead to if untreated ? What’s this sign called

A

Eyes deviate downward - setting sun sign

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7
Q

Features of hydrocephalus in older children

A

features of raised ICP
Headache, vomiting, respiratory abnormalities, pupil size / reaction, papilloedema, decerebrate (extended) / decorticate (flexed arms)

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8
Q

Diagnosis of hydrocephalus

A

Uss - if sutures still open can assess level of ventricular dilation
CT/MRI - establish diagnosis and cause

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9
Q

Treatment of hydrocephalus

A

Ventriculoperitoneal (VP) shunt

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10
Q

Complications of vp shunts

A

Obstruction, infection

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11
Q

What is craniosynostosis? Cause?

A

Premature fusion of skull sutures.

Part of syndrome in 30% - rest idiopathic

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12
Q

Features of craniosynostosis

A

Abnormal head shape - fused sutures cause over expansion of other sutures to compensate

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13
Q

Most common craniosynostosis and shape?

A

Sagittal -> long thin head

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14
Q

Raised ICP in craniosynostosis treated with?

A

Cranioectomy

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15
Q

Egs. Of neural tube defects

A 
Spina bifida 
Meningocele 
Myelomeningocele
Encephalocele 
Anencephaly
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16
Q

What causes spina bifida occulta ? Sign?

A

Vertebral arch fails to fuse

Hairy patch on skin over birth

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17
Q

What happens in meningocele

A

Meninges herniate through a vertebral defect -> outpouch

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18
Q

Difference between myelomeningocele and menigocele

A

Myelo - meninges & spinal cord herniate

Most severe form of spinal dysplasia

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19
Q

What is encephalocele

A

Extrusion of brain and meninges though a mid line skull defect

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20
Q

What is anencephaly

A

Brain and cranium fail to develop (detected an antenatal USS & TOP usually performed )

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21
Q

What is cerebral palsy

A

Disorder of motor function (movement +/- posture) due to a non-progressive static lesion of the developing brain

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22
Q

Why does cerebral palsy evolve with time even though the lesson is static

A

Manifestations evolve as CNS develops - due to CNS plasticity in young children

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23
Q

Does cerebral palsy just affect movement

A

No, often have other problems reflecting more widespread Brain damage

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24
Q

When is most cerebral palsy caused? Egs?

A

Antenatal 80% - cerebral dysgeneis, congenital infections (rubella, cmv, toxoplasmosis)

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25
Q

Intrapartum cause of cerebral palsy

A

Birth asphyxia / hypoxia -> ischemic brain injury

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26
Q

Post natal causes of cerebral palsy

A 
Preterm birth - interventricular haemorrhage, hypoxic-ischemic encephalopathy, periventricular leukomalacia 
Hyper bilirubinaemia
Hypoglycaemia
Intracranial infections 
Head trauma
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27
Q

Features of CP

A

Delayed motor milestones
Abnormal tone and posture
Asymmetric hand function before 1 year old
Feeding difficulties (decreased oromoter coordination)
Speech and language delay

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28
Q

Diagnosis of cp

A

Examination - tone 👆/👇, power (eg. Hemiparesis), reflexes (brisk -> UMN)(prolonged primitive reflexes) abnormal movements / posture / gait

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29
Q

When should primitive reflexes disappear

A

4-6/12

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30
Q

Classes of cp

A

Spastic -70%
Ataxic - 10%
Dyskinetic 10%
Mixed 10%

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31
Q

Where is damage for spastic cp? Associated with?

A

UMN (pyramidal / corticospinal)

Brisk deep tendon reflexes & extensor plantar response

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32
Q

Why might hypotonia still be spastic cp

A

Turns spastic with age

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33
Q

What type of spastic cp is associated with birth asphyxia

A

Quadriplegic

Other types are hemi/para

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34
Q

What does spasticity of bulbar muscles cause

A

Dysphasia and dribbling

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35
Q

What is the clasp knife response

A

Increased limb tone suddenly gives way under increasing pressure (UMN)

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36
Q

What causes ataxic cp

A

Damage to cerebellumans pathways

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37
Q

Early / late signs of ataxic cp

A

Early - uncoordinated movements, trunk and limb hypotonia, delayed motor development, poor balance

Late - intention tremor and ataxic gait

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38
Q

What causes dyskinetic cp? Other name?

A

Damage to basal ganglia / Extrapyramidal pathways (eg by kernicterus)
Choreoathetoid

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39
Q

What is causes kernicterus

A

Bilirubin

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40
Q

Presentation of dyskinetic cp

A

Hypotonia & delayed motor development

Abnormal movements often don’t appear until 12 months

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41
Q

Abnormal movements in dyskinetic cp

A

Chorea - abrupt, jerky
Athetosis - slow, writhing, continuous
Dystonia - sustained abnormal posture

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42
Q

Intellect impairment in dyskinetic cp

A

Completely / relatively unimpaired

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43
Q

Problems associated with cp

A

mental retardation, opthalmic & auditory abnormalities, seizure, GOR, recurrent LRTI/pneumonia
Feeding problems / failure to thrive

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44
Q

Members of MDT for cp

A

Paediatrician, carers / parents, gp, health visitor, SALT, physio, OT

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45
Q

Management of cp

A

Motor function - physio, muscle relaxants (hypertonia), Botox injections to specific muscles
Treat associated features eg. Epilepsy, constipation, malnutrition, behavioural

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46
Q

Eg of muscle relaxants for cp

A

Diazepam, baclofen

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47
Q

Definition of epilepsy

A

A chronic disorder of the brain characterised by recurrent, unprovoked seizures

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48
Q

Egs of epilepsy mimics (funny turns)

A

Breath holding attacks
Reflex anoxic seizures
Syncope

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49
Q

What is a breath holding attack? Demographic?

A

Provoked by temper / frustration in 6/12 - 6years

Child holds breath, goes blue, then limb, then makes rapid recovery

50
Q

What are triggers for reflex anoxic seizures?
Physiology?
What does it look like ? Possible ->?
Prognosis ?

A

Pain (usually mild head injury), fear, cold food.
Increased Vagal tone -> cardiac asystole.
Child becomes pale & falls to floor - hypoxia may cause GTC seizure.
Brief and child rapidly recovers

51
Q

Usual triggers for syncope?
Prodromal Sx?
What happens?
Recovery?

A

Emotion / hot environment.
Nausea & dizziness
Loss of consciousness & posture
Rapid recovery

52
Q

Egs of generalised seizures

A

Absence, myoclonic, clonic, tonic clonic, atonic

53
Q

Difference between simple and complex partial seizures

A

Consciousness is retained in simple

54
Q

Lobe in motor seizure

A

Frontal

55
Q

Somatosensory / special sensory Sx seizure lobe?

A

Temporal (smells/ taste), parietal (sensations)

56
Q

Usual lobe for complex partial seizure

A

Temporal

57
Q

Usual cause of epilepsy

A

Idiopathic 75%

58
Q

Egs of secondary causes of epilepsy

A

Cortical dysgenesis / malformations / tumours, neurocutaneous syndromes, downs, fragile X, inborn errors of metabolism (eg PKU), cerebral damage

59
Q

Eg of neurocutaneous syndromes

A

Neurofibromatosis, tuberous sclerosis

60
Q

Eg of causes od cerebral damage

A

Non accidental injury, birth asphyxia, hypoxia-ischemia following meningitis / encephalitis

61
Q

Egs of generalised epilepsy syndromes

A

Infantile spasms (west syndrome), Lennox-gastaut syndrome, childhood absence epilepsy, juvenile myoclonic epilepsy

62
Q

When are infantile spasms (west syndrome) most common? What happens in the seizures ?

A 
4-6/12 
Myoclonic seizures (salaam attacks) - violent flexion of head, trunk and limbs followed by extension of arms
63
Q

What are infantile spasms often misdiagnosed as ?

A

Colic

64
Q

Seen on EEG for infantile spasms ?

A

hypsarrhythmia - chaotic, large-amplitude slow waves with spikes & sharp waves

65
Q

Usual cause of infantile spasms

A

Secondary - tuberous sclerosis / hypoxic-ischemic encephalopathy

66
Q

Treatment for infantile spasms

A

ACTH

Vigabatrin (TS)

67
Q

Important side effect of vigabatrin

A

Permanent visual field defects

68
Q

Usual age for Lennox-gastraut syndrome?
Usual seizure and frequency ?
Other key feature?

A

2-6 years.
Daily seizures - nocturnal tonic most common (90%), myoclonic drop attacks next.
Slowed / arrested psycho-motor development

69
Q

Usual age for childhood absence epilepsy?
What are the seizures like?
How common are the seizures ?

A

4-12 years (peak at 6-7).
Transient 5-15s, - unaware blank spells without loss of body tone.
Can be up to several hundred / day

70
Q

How can absence seizures be induced ? What is this useful for? What’s seen ?

A

Hyperventilation for EEG.

*Generalised bilaterally synchronous 3Hz (3/sec) spike and wave *

71
Q

Juvenile myoclonic epilepsy presentation

A

After a GTC seizure, often with Hx of *clumsiness in morning * (“cornflake / toothbrush epilepsy”)

72
Q

What causes clumsiness in morning in juvenile myoclonic epilepsy

A

Brief myoclonic seizures usually after waking up

73
Q

What can often progress into juvenile myoclonic epilepsy

A

Childhood absence epilepsy

74
Q

Prognosis of Juvenile myoclonic epilepsy

A

Persist into adulthood - need to advise girls re pregnancy and medication

75
Q

Non pharmacological Management of epilepsy

A

Supervision during baths , don’t lock doors, wear cycle helmets, avoid climbing rocks / trees, supervision when swimming

76
Q

Pharmacological management of epilepsy ? Generalised ? Partial?

A

Aim for monotherapy.
G- sodium valproate
P- carbamazepine

77
Q

What should be used for girls of child bearing age with generalised epilepsy

A

Lamotrigine

78
Q

What is a febrile seizure

A

Seizure associated with fever in a child (6/12 - 6 years) in the absence of intracranial infection or an identifiable neurological disorder

79
Q

Features of febrile seizure

A

Rapid increase in temp -> brief (1-2 minute) GTC seizure

80
Q

What is the usual cause of fever -> febrile seizures ? What is another cause and needs to be ruled out? How?

A

Viral infection
Bacterial meningitis
LP

81
Q

When are febrile seizures more worrying

A

In the presence of developmental delay

82
Q

Prognosis of febrile seizures

A

30% will have a recurrence - more likely if first seizure before 18/12

83
Q

Management of febrile seizures

A

Septic screen - identify source

Keep patient cool - antipyretics (paracetamol / ibuprofen), tepid sponging

84
Q

What to be done in prolonged febrile seizure (>10mins)

A

Terminate using rectal diazepam

85
Q

Which is more common , NF type 1 or 2

A 
Type 1 (1/4000)
2 - 1/40000
86
Q

Genetics of type 1

A

Autosomal dominant - 50% have no family Hx

87
Q

Features of NF1 ? When seen?

A

>6 cafe-au-lait spots >5mm (before puberty

88
Q

What characterises NF2 ? What does this lead to?

A

Bilateral acoustic neuroma -> deafness & sometimes cerebellopontine angle syndrome (CN VII palsy & Cerebellar ataxia)

89
Q

Mutations for NF type 1 / 2

A

1- chromosome 17

2- chromosome 22

90
Q

Genetics of tuberous sclerosis

A

Autosomal dominant - 75% new mutations

1/7000 births

91
Q

Cutaneous features of TS

A

De-pigmented “ash leaf” shaped patches which fluoresce under woods light
Roughened patches of skin (shagreen patches) usually over lumbar spine
Facial *angiofibromas in butterfly distribution *

92
Q

Neurological features of TS

A

West syndrome & developmental delay
Focal epilepsy
Intellectual impairment

93
Q

What else is affected in TS

A

Multi system - heart, lungs, kidneys also

94
Q

What type of syndrome is sturge - Weber syndrome

A

Neurocutaneous (like TS / NF)

95
Q

Characteristic of sturge Weber syndrome

A

Unilateral facial naevus (port wine stain) in the distribution of the opthalmic division of CN V
Angiomas involving the leptomeningeal vessles in the brain (->seizures)
Haemangiomas in the spinal cord

96
Q

Where are there abnormal blood vessles in sturge Weber syndrome and what do they cause?

A

Surface of the brain -> seizures, hemiplegia & learning difficulties.
Ocular involvement -> glaucoma

97
Q

What’s seen in CT head in sturge Weber syndrome

A

Calcification of gyri in characteristic rail road track pattern

98
Q

Egs of muscular dystrophies

A

Duchenne, Becker, myotonic

99
Q

Genetics of duchenne? How many new mutations? Gene codes for what?

A

X linked recessive (1/4000), 1/3 new mutations.

Gene encodes dystrophin - sarcolemmal membrane protein

100
Q

When does duchenne present? Do they ever walk ?

A

Sx at 2-4 years

Independent walking tends to be delayed and these children never run

101
Q

Features of duchenne

A

pseudohypertrophy of calf muscles & proximal muscle weakness
positive Gowlers sign
Scoliosis
Dilated cardiomyopathy
Mild learning difficulties

102
Q

What is Gowlers sign

A

3-5 years - hands used to push on legs to achieve upright posture (weakness of lower back and pelvic griddle muscles)

103
Q

*Diagnosis of duchennes *

A

Serum creatinine kinase (10-20x increased)
Muscle biopsy and EMG
DNA analysis to identify dystrophin gene mutations (+ve in 65%)

104
Q

Management of duchennes ?

A

Supportive
Walking prolonged using orthoses
Scoliosis helped by truncal brace
Genetic counciling for female carriers

105
Q

Prognosis of duchennes

A

Cardiomyopathy worsens with age -> death

106
Q

What is Becker muscular dystrophy ? How does it compare to duchenne

A

Milder than duchenne but caused by a mutation in same gene.

Onset is later (second decade) with prolonged survival

107
Q

Genetics of myotonic dystrophy ? What does it affect?

A

Acquired autosomal dominant .

Muscle, endocrine, cardiac function, CNS & immunity

108
Q

Features of myotonic dystrophy

A

Hypotonia
Progressive muscle wasting
Typical facial features
Myotonia

109
Q

Typical facial features in myotonic dystrophy

A

Inverted v shaped upper lip, thin cheeks, high arched palate

110
Q

What is myotonia ? When is it seen?

A

Seen beyond 5 years

Slow relaxation of muscle after contraction

111
Q

How can you test myotonia

A

Ask to make tight fists then quickly open hands

112
Q

Diagnosis of myotonic dystrophy

A

DNA -> CTG repeat

Serum Creatine kinase usually normal (unlike duchennes)

113
Q

Treatment of myotonic dystrophy

A

Supportive

114
Q

What is spinal muscular atrophy ? Genetics?

A

Autosomal recessive.

Degeneration of anterior horn cells -> progressive weakness & wasting of skeletal muscles

115
Q

What’s seen in spinal muscular atrophy

A

Wasting & weakness
Hypotonia and weak tendon reflexes
Fasiculations in tongue, deltoids / biceps -> characteristic & sign of denervation

116
Q

What is ataxia ? Cause?

A

Incoordination of movement, speech and posture.

Cerebellar / posterior sensory pathway problems

117
Q

Egs of cause of Cerebellar ataxia

A

Acute - drugs / medications (alcohol and solvent abuse)
Post viral - usually after varicella infection
Posterior fossa lesions - medulloblastoma
Genetic / degenerative disorders - ataxic CP, freidrich ataxia, ataxia- telangiectasia

118
Q

Cause of freidrichs ataxia ? How does it present ? Sensory impairment ?

A

Autosomal resseive mutation in frataxin gene .
worsening ataxia, distal wasting in legs, absent lower limb reflexes pes cavus & dysarthria.
Impairment of proprioception and vibrosence

119
Q

What is ataxia-telangiectasia ? How does it present ?

A

Autosomal recessive disorder of DNA repair.

Ataxia with developmental delay. Telangiectasia develop in the conjunctiva, neck and shoulders from 4 years.

120
Q

What must be ? In acute strabismus

A

Raised ICP

121
Q

What does no red light reflex indicate ? What needs to happen ?

A

Cataracts / retinoblastoma . Repair to avoid ambylopia in affected eye

Peadiatrics (14 decks)

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