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Paediatrics > Neuro > Flashcards

Neuro Flashcards

1
Q

What are periodic syndromes?

A

Syndromes which affect children and are often precursors of migraine
Child is well between episodes

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2
Q

What is cyclical vomiting?

A

A periodic syndrome.
Recurrent stereotyped episodes of vomiting and intense nausea associated with pallor and lethargy
Child is well in between episodes

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3
Q

What is abdominal migraine?

A

Idiopathic recurrent disorder
Episodic midline abdominal pain in bouts lasting 1-72 hr
Pain moderate to severe in intensity
Associated with vasomotor symptoms, nausea and vomiting

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4
Q

What is benign paroxysmal vertigo of childhood?

A

Heterogenous disorder
Characterised by brief episodes of vertigo which occur without warning and resolve spontaneously
Children otherwise healthy with normal neuro, auditory and vestibular exam

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5
Q

When can sumatriptan be given in children with headaches? Route

A

Nasal preparation

Licensed for use in children over 12 years of age

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6
Q

What are febrile seizures?

A

Seizure accompanied by a fever in absence of intracranial infection due to meningitis or encephalitis

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7
Q

Incidence and age prevalence of febrile seizures?

A

3% of children between 6 months and 6 years

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8
Q

Inheritance of febrile seizures

A

Genetic predisposition with 10% chance if first degree relative had them

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9
Q

When during illness does seizure usually occur

A

Early on in viral infection when temperature is rising rapidly

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10
Q

What sort of seizures are febrile seizures?

A

Usually tonic-clonic seizures

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11
Q

What % of 1st febrile seizure will have further ones

A

30-40%

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12
Q

What increases likelihood of having more febrile seizures? x4

A

Earlier on in illness
Younger age of child
Lower temperature at time of seizure
Positive family history

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13
Q

When is there an increased risk of developing epilepsy with febrile seizures? x3

A

If complex febrile seizures

Aka, prolonged, repeated in same illness or focal seizures

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14
Q

If febrile seizure goes on longer than 5 mins

A

Buccal midazolam or rectal diazepam

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15
Q

What happens in breath-holding attacks?

A

Happens in toddlers when they are upset

Child cries, holds breath, goes blue. Will sometimes briefly lose consciousness but rapidly recover

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16
Q

What is reflex anoxic seizure

A

Occurs in infants or toddlers when scared or shocked (head trauma, cold food, fright or fever)
Becomes pale and falls to the floor
Hypoxia may induce tonic-clonic seizure
Due to cardiac asystole from vagal inhibition
Child recovers quickly after brief seizure

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17
Q

Pseudoseizures vs fabricated seizures

A

Pseudoseizures - child feigns seizure

Fabricated - by parents

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18
Q

When do you do metabolic investigations with seizures?

A

When there is developmental regression or when seizures are related to feeds or fasting

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19
Q

What is West syndrome?

A

Violent flexor spasms of head, trunk and limbs followed by extension of arms
Flexor spasms last 1-2s and usually 20-30 spasms
Often occur on waking
EEG signs
2/3 have underlying neuro cause

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20
Q

What age is West syndrome?

A

4-6 months

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21
Q

How do you treat West Syndrome? Response rate and future development

A

Vigabatrin or corticosteroids
Good response in 30-40%
Most lose skills and develop learning disability or epilepsy

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22
Q

What is Lennox-Gastaut syndrome?

A

Multiple seizure types - mostly drop attacks, tonic seizures and atypical absences
Also have neurodevelopmental arrest/regression and behavioural disorder

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23
Q

Age and prognosis of Lennox-Gastaut

A

1-3 years
Often other complex neurological problems and history of infantile spasms
Prognosis poor

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24
Q

First line for tonic-clonic seizures x2

A

Valproate and carbamazepine

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25
Q

First line for absence seizure x2

A

Valproate and ethosuximide

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26
Q

First line for myoclonic seizure x1

A

Valproate

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27
Q

First line for focal seizures x2

A

Carbamazepine and valproate

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28
Q

Signs of myopathy in children?

A

Waddling gait or positive Gowers sign (need to turn prone to rise to standing, use arms to stand up)

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29
Q

When is positive Gowers sign normal?

A

Before the age of 3 years

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30
Q

Investigative signs of myopathy

A

Serum creatine phosphokinase markedly elevated in muscular dystrophy and inflammatory myopathies

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31
Q

Presentation of anterior horn cell disorders x4

A

Weakness, wasting, absent reflexes and fasciculation

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32
Q

What is spinal muscular atrophy?

A

Autosomal recessive degeneration of the anterior horn cells

Progressive weakness and wasting of skeletal muscles

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33
Q

What gene is mutated in spinal muscular atrophy?

A

Survival motor neurone (SMN) gene

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34
Q

What is Werdnig-Hoffman disease

A

Spinal muscular atrophy type 1

Very severe and presents early at birth/in infancy

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35
Q

What is noticed in pregnancy with Werdnig-Hoffman disease

A

Diminished fetal movements

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36
Q

What is noticed at birth with Werdnig-Hoffman disease

A

There may be arthrogryposis (positional deformities of the limbs with contractures of at least two joints)

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37
Q

Other signs of Werdnig-Hoffman disease x5

A 
Lack of antigravity power in hip flexors 
Absent deep tendon reflexes
Intercostal recession 
Fasciculation of the tongue 
Never sit unaided
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38
Q

Prognosis of Werdnig-Hoffman disease

A

Death from respiratory failure at 12 months

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39
Q

Features of type 2 spinal muscular atrophy

A

Can sit but never walk independantly

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40
Q

Features of type 3 spinal muscular atrophy (Kugelberg-Welander)

A

Do walk and can present later in life

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41
Q

What are the hereditary motor sensory neuropathies

A

A peripheral neuropathy with slowly progressive muscular wasting (distal)
Type 1 = Charcot-Marie-Tooth disease
Nerves can be hypertrophic due to demyelination followed by attempted remyelination

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42
Q

What limb is more affected in hereditary motor sensory neuropathies. Age of onset

A

Legs more than arms

Usually onset in first decade

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43
Q

What is GBS? how does it arise?

A

Acute post-infectious polyneuropathy

2-3 weeks after URTI or campylobacter gastroenteritis

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44
Q

Features of GBS x7

A

Ascending symmetrical weakness
Loss of reflexes
Autonomic involvement
Sensory symptoms may also be present
Bulbar muscle involvement = difficulty with chewing and swallowing - risk aspiration
Respiratory depression may need ventilation

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45
Q

Recovery from GBS

A

95% fully recover

May take up to 2 years

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46
Q

Main complication of Bells Palsy

A

Conjunctival infection due to incomplete eye closure on blinking

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47
Q

Age of presentation of juvenile myasthenia gravis

A

Onset usually after 10 years

Presents with ophthalmoplegia and ptosis, loss of facial expression and difficulty chewing

48
Q

Treatment of MG

A

Anti-muscarinics (eg. atropine to reduce the muscarinic side effects of acetylcholinesterase inhibitors)
Neostigmine or pyridostigmine - prevent breakdown of acetylcholine
Longer term immunosuppression with prednisolone or azathioprine can help

49
Q

Presentation of Duchenne Muscular Dystrophy and gene

A 
Wadding gait and/or language delay 
Xp21 site (codes for dystrophin)
50
Q

Average age of diagnosis of Duchenne

A

5.5 years but symptoms will be present earlier

No longer ambulant by age 10-14

51
Q

What is used in Duchenne to preserve mobility and prevent scoliosis x4

A

Exercise, stretching to avoid contractures, brace for scoliosis
Prednisolone 10days per month
Mechanism of it helping is not known

52
Q

What is Becker muscular dystrophy

A

Milder form of Duchenne

Average onset 11 years and can walk into 20s (only 11-14 in Duchenne)

53
Q

Presentation of metabolic myopathies

A

Floppy infant or muscle weakness and cramps on exercise in older infants

54
Q

What is benign acute myositis?

A

Assumed to be post-viral
Follows UTRI and runs self-limiting course
Pain and weakness in affected muscles
CPK raised

55
Q

What is dermatomyositis?

A 
Systemic illness, symmetrical proximal muscle weakness
Characteristic violaceous (heliotrope) rash to eyelids and periorbital oedema
56
Q

Where can rash extend to in dermatomyositis?

A

Extensor surfaces of joints and can get subcutaneous calcification

57
Q

How and when does dermatomyositis present?

A

Presents usually between 5-10 years with fever, misery and eventually muscle weakness

58
Q

Treatment of dermatomyositis x3

A

Physiotherapy to prevent contractures
Corticosteroids are mainstay
Other immunosuppressants eg. methotrexate, ciclosporin, may be needed

59
Q

What do adults develop with dystrophia myotonica?

A

Cataracts (myotonia, delayed relaxation of muscles after contraction) and muscular dystrophy

60
Q

What do males develop with dystrophia myotonica

A

Baldness and testicular atrophy

61
Q

What is Friedreich ataxia?

A

Ataxia due to progressive damage of the nervous system - poor coordination and impairment of joint position and vibration sense - autosomal recessive
Eventually wheelchair is required for mobility

62
Q

How does Friedreich ataxia present x4

A

With worsening ataxia
Distal wasting in legs
Absent lower limb reflexes
Absent extensor plantar responses

63
Q

What happens in Freidreich ataxia due to pyramidal involvement? x2

A

Pes cavus and dysarthria

64
Q

Life expectancy with Freidreich ataxia

A

Death at 40-50 years due to developing kyphoscoliosis and cardiomyopathy - cardiorespiratory compromise

65
Q

Type of haemorhage in non-accidental injury or direct trauma in children

A

Subdural haematoma

66
Q

Most common causes of stroke in children x3

A

Cardiac or sickle cell disease

Varicella infection

67
Q

Mothers with neural tube defect child - chance of having another child with NTD

A

10x increased risk of second affected child

68
Q

What has reduced risk of NTD

A

Folic acid

69
Q

What is anencephaly?

A

Failure of development of most of the cranium and brain
Usually stillborn or die shortly after birth
Can detect antenatally and terminate pregnancy

70
Q

What is encephalocele?

A

Extrusion of brain and meninges through midline skull defect
Can be corrected surgically
Often underlying associated cerebral malformations

71
Q

What is spina bifida occulta?

A

Failure of fusion of vertebral arch
Can be overlying skin lesion
Often detected incidentally on x-ray

72
Q

What is diastematomyelia

A

Underlying tethering of cord to skin overlying spina bifida

As grows can cause neurological deficits of lower limbs and bladder

73
Q

What is meningocele?

A

Protrusion of dura and CSF through vertebral arch

74
Q

Prognosis of meningocele

A

Good prognosis following surgical repair

75
Q

What is myelomeningocele

A

Dura and spinal cord through unfused vertebral arch

76
Q

What can myelomeningocele be associated with x6

A 
Variable paralysis of legs 
Muscle imbalance with dislocations
Sensory loss
Bladder and bowel denervations (neuropathic bladder/bowel)
Scoliosis 
Hydrocephalus
77
Q

Management of myelomeningocele

A

Back lesion usually closed soon after birth - manage complications/resulting symptoms

78
Q

What is hydrocephalus?

A

Obstruction to flow of CSF therefore dilatation of ventricular system proximal to obstruction

79
Q

What is non-communicating/obstructive hydrocephalus

A

Obstruction within ventricular system or aqueduct

80
Q

What is communicating hydrocephalus

A

Obstruction at arachnoid villi (site of absorption)

81
Q

Features of hydrocephalus x4

A

Skull sutures have not fused therefore head circumference disproportionately large or excessive growth rate
Anterior fontanelle bulges
Sutures separate
Scalp veins distended

82
Q

Advanced sign of hydrocephalus

A

Fixed downward gaze or sun setting of the eyes

83
Q

Treatment of hydrocephalus

A

Insertion of ventriculoperitoneal shunt is mainstay

Endoscopic treatment to create ventriculostomy can now be performed

84
Q

How do neurocutaneous syndromes arise?

A

Nervous system and skin have common ectodermal origin

Therefore embryological disruption causes syndromes involving abnormalities of both systems

85
Q

Prevalence of neurofibromatosis 1

A

1 in 3000 live births

1/3 have new mutations

86
Q

What features are present in NF1 x7 (need 2 or more for diagnosis)

A

6 or more cafe au lait spots >5mm before puberty or >15mm afterwards
More than one neurofibroma
Axillary freckles
Optic glioma
Lisch nodule
Bony lesions from sphenoid dysplasia (can cause eye protrusion)
1st degree relative with NF1

87
Q

Where can neurofibromata appear in NF1

A

Anywhere on peripheral nerve - including visual/auditory nerves
Look unslightly and can cause neurological signs if where nerve passes through bone

88
Q

Features of NF2 x4

A

Presents in adolescence
Less common
Bilateral acoustic neuromata
Sometimes cerebellopontine angle syndrome (VII CN paresis and cerebellar ataxia)
(Also get cataracts and peripheral neuropathy and less defined skin features - possible schwannomas)

89
Q

What is tuberous sclerosis

A

Benign tumours grow on brain, other vital organs and skin

90
Q

Cutaneous features of tuberous sclerosis x4

A

Depigmented ‘ash leaf’ shaped patches - fluoresce under UV light
Roughened patches of skin - usually over lumbar spine = Shagreen patches
Adenoma sebaceum - butterfly distribution over nose and cheeks
Periungual fibroma

91
Q

Neurological features of tuberous sclerosis x3

A

Infantile spasms and developmental delay
Epilepsy (often focal)
Intellectual impairment

92
Q

Nail features of tuberous sclerosis

A

Fibromata beneath the nails - subungual fibromata

93
Q

What is Sturge-Weber syndrome?

A

Port-wine stains on one side of the face with tumours and CNS neurological defects

94
Q

Where is facial lesion in Sturge-Weber syndrome?

A

In distribution of trigeminal nerve - opthalmic division is always involved

95
Q

Neurological signs of Sturge-Weber syndrome x3

A

Epilepsy, Learning disability and hemiplegia - in most severe form

96
Q

What are neurodegenerative disorders

A

Deterioration in motor and intellectual function with abnormal neurological features, abnormal head circumference, visual/hearing loss and behavioural changes

97
Q

Causes of neurodegenerative disorders x5

A

Lysosomal storage diseases
Peroxisomal enzyme defects (involved in long chain fatty acid oxidation therefore LCFA accumulation)
Heredodegenerative disorders eg. HTT disease
Wilson disease
Subacute scelrosing panencephalitis (SSPE)

98
Q

Prophylactic agents for headache x3

A

Pizotifen (5-ht antagonist) - can cause weight gain and sleepiness
B-blockers - propanaolol - CI in asthma
Sodium channel blockers (valproate or topiramate)

99
Q

Management if epilepsy is suspected x4

A

EEG is always indicated (to add supportive evidence for diagnosis not to diagnose)
Can also do sleep or sleep-deprived EEG or 24 ambulatory EEG
MRI or CT is neurological signs are present between seizures or if they are focal
PET or SPECT (functional imaging to identify epileptogenic lesions)
Metabolic (see other flashcard)

100
Q

Typical age for childhood absence epilepsy

A

4-12 years

101
Q

What sort of diet might help epileptic children?

A

Ketogenic (fat-based) diet

102
Q

Valproate side effects x3

A

Weight gain, hair loss and rare idiosyncratic liver failure

103
Q

Carbamazepine/oxcarbezepine side effects x5

A

Rash, neutropenia, hyponatraemia, ataxia

Liver enzyme induction

104
Q

Vigabatrin side effects x2

A

Sedation

Restriction of visual fields

105
Q

Lamotrigine side effect

A

Rash

106
Q

Probable cause of Bells Palsy

A

Post-infectious

Association with herpes simplex virus in adults

107
Q

Management of Bells Palsy

A

Corticosteroids - reduce oedema in facial canal

108
Q

Important DDX of Bells Palsy

A

Cerebellopontine angle tumour

109
Q

Bilateral Bells Palsy DDX?

A

Sarcoidosis

Lyme disease

110
Q

DX of MG x3

A

Administration of IV edrophonium (anticholinesterase) leads to improvement
Ach receptor antibodies (present in 60-80% of cases)
More rarely - anti-muscle specific kinase (anti-MuSK) antibodies

111
Q

What are myotonic disorders?

A

Delayed relaxation after sustained muscle contraction - clinically and on EMG
eg. slow release of handshake

112
Q

Presentation of dystrophia myotonica in newborns

A

Hypotonia, feeding and respiratory difficulties due to muscle weakness

113
Q

What is ataxia telangiectasia?

A

Disorder of DNA repair - mild delay in motor development in infancy and oculomotor problems with incoordination

114
Q

What becomes evident at school age in ataxia telangiectasia x3

A

Telangiectasia in conjunctiva, neck and shoulders from about 4 years of age
Difficulty with balance and coordination
Wheelchair needed in early adoloscence

115
Q

Complications in ataxia telangiectasia x2

A

Increased susceptibility to infection

Develop malignant disorders, esp, acute lymphoblastic leukaemia

116
Q

What is raised in serum in ataxia telangiectasia

A

Alpha fetoprotein

117
Q

Complications of neurofibromatosis 1

A

60% develop learning difficulties
50% have ADHD
High blood pressure due to increased renin release from kidneys
Physical development abnormalities eg. large head, scoliosis, smaller weight and height than normal
Migraines, epilepsy and brain tumours

Paediatrics (16 decks)

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