Neuro Flashcards Preview

Paediatrics > Neuro > Flashcards

Flashcards in Neuro Deck (117):
1

What are periodic syndromes?

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

2

What is cyclical vomiting?

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

3

What is abdominal migraine?

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

4

What is benign paroxysmal vertigo of childhood?

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

5

When can sumatriptan be given in children with headaches? Route

Nasal preparation
Licensed for use in children over 12 years of age

6

What are febrile seizures?

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

7

Incidence and age prevalence of febrile seizures?

3% of children between 6 months and 6 years

8

Inheritance of febrile seizures

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

9

When during illness does seizure usually occur

Early on in viral infection when temperature is rising rapidly

10

What sort of seizures are febrile seizures?

Usually tonic-clonic seizures

11

What % of 1st febrile seizure will have further ones

30-40%

12

What increases likelihood of having more febrile seizures? x4

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

13

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

If complex febrile seizures
Aka, prolonged, repeated in same illness or focal seizures

14

If febrile seizure goes on longer than 5 mins

Buccal midazolam or rectal diazepam

15

What happens in breath-holding attacks?

Happens in toddlers when they are upset
Child cries, holds breath, goes blue. Will sometimes briefly lose consciousness but rapidly recover

16

What is reflex anoxic seizure

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

17

Pseudoseizures vs fabricated seizures

Pseudoseizures - child feigns seizure
Fabricated - by parents

18

When do you do metabolic investigations with seizures?

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

19

What is West syndrome?

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

20

What age is West syndrome?

4-6 months

21

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

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

22

What is Lennox-Gastaut syndrome?

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

23

Age and prognosis of Lennox-Gastaut

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

24

First line for tonic-clonic seizures x2

Valproate and carbamazepine

25

First line for absence seizure x2

Valproate and ethosuximide

26

First line for myoclonic seizure x1

Valproate

27

First line for focal seizures x2

Carbamazepine and valproate

28

Signs of myopathy in children?

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

29

When is positive Gowers sign normal?

Before the age of 3 years

30

Investigative signs of myopathy

Serum creatine phosphokinase markedly elevated in muscular dystrophy and inflammatory myopathies

31

Presentation of anterior horn cell disorders x4

Weakness, wasting, absent reflexes and fasciculation

32

What is spinal muscular atrophy?

Autosomal recessive degeneration of the anterior horn cells
Progressive weakness and wasting of skeletal muscles

33

What gene is mutated in spinal muscular atrophy?

Survival motor neurone (SMN) gene

34

What is Werdnig-Hoffman disease

Spinal muscular atrophy type 1
Very severe and presents early at birth/in infancy

35

What is noticed in pregnancy with Werdnig-Hoffman disease

Diminished fetal movements

36

What is noticed at birth with Werdnig-Hoffman disease

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

37

Other signs of Werdnig-Hoffman disease x5

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

38

Prognosis of Werdnig-Hoffman disease

Death from respiratory failure at 12 months

39

Features of type 2 spinal muscular atrophy

Can sit but never walk independantly

40

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

Do walk and can present later in life

41

What are the hereditary motor sensory neuropathies

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

42

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

Legs more than arms
Usually onset in first decade

43

What is GBS? how does it arise?

Acute post-infectious polyneuropathy
2-3 weeks after URTI or campylobacter gastroenteritis

44

Features of GBS x7

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

45

Recovery from GBS

95% fully recover
May take up to 2 years

46

Main complication of Bells Palsy

Conjunctival infection due to incomplete eye closure on blinking

47

Age of presentation of juvenile myasthenia gravis

Onset usually after 10 years
Presents with ophthalmoplegia and ptosis, loss of facial expression and difficulty chewing

48

Treatment of MG

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

Presentation of Duchenne Muscular Dystrophy and gene

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

50

Average age of diagnosis of Duchenne

5.5 years but symptoms will be present earlier
No longer ambulant by age 10-14

51

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

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

52

What is Becker muscular dystrophy

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

53

Presentation of metabolic myopathies

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

54

What is benign acute myositis?

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

55

What is dermatomyositis?

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

56

Where can rash extend to in dermatomyositis?

Extensor surfaces of joints and can get subcutaneous calcification

57

How and when does dermatomyositis present?

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

58

Treatment of dermatomyositis x3

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

59

What do adults develop with dystrophia myotonica?

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

60

What do males develop with dystrophia myotonica

Baldness and testicular atrophy

61

What is Friedreich ataxia?

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

How does Friedreich ataxia present x4

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

63

What happens in Freidreich ataxia due to pyramidal involvement? x2

Pes cavus and dysarthria

64

Life expectancy with Freidreich ataxia

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

65

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

Subdural haematoma

66

Most common causes of stroke in children x3

Cardiac or sickle cell disease
Varicella infection

67

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

10x increased risk of second affected child

68

What has reduced risk of NTD

Folic acid

69

What is anencephaly?

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

70

What is encephalocele?

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

71

What is spina bifida occulta?

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

72

What is diastematomyelia

Underlying tethering of cord to skin overlying spina bifida
As grows can cause neurological deficits of lower limbs and bladder

73

What is meningocele?

Protrusion of dura and CSF through vertebral arch

74

Prognosis of meningocele

Good prognosis following surgical repair

75

What is myelomeningocele

Dura and spinal cord through unfused vertebral arch

76

What can myelomeningocele be associated with x6

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

77

Management of myelomeningocele

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

78

What is hydrocephalus?

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

79

What is non-communicating/obstructive hydrocephalus

Obstruction within ventricular system or aqueduct

80

What is communicating hydrocephalus

Obstruction at arachnoid villi (site of absorption)

81

Features of hydrocephalus x4

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

82

Advanced sign of hydrocephalus

Fixed downward gaze or sun setting of the eyes

83

Treatment of hydrocephalus

Insertion of ventriculoperitoneal shunt is mainstay
Endoscopic treatment to create ventriculostomy can now be performed

84

How do neurocutaneous syndromes arise?

Nervous system and skin have common ectodermal origin
Therefore embryological disruption causes syndromes involving abnormalities of both systems

85

Prevalence of neurofibromatosis 1

1 in 3000 live births
1/3 have new mutations

86

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

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

Where can neurofibromata appear in NF1

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

88

Features of NF2 x4

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

What is tuberous sclerosis

Benign tumours grow on brain, other vital organs and skin

90

Cutaneous features of tuberous sclerosis x4

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

Neurological features of tuberous sclerosis x3

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

92

Nail features of tuberous sclerosis

Fibromata beneath the nails - subungual fibromata

93

What is Sturge-Weber syndrome?

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

94

Where is facial lesion in Sturge-Weber syndrome?

In distribution of trigeminal nerve - opthalmic division is always involved

95

Neurological signs of Sturge-Weber syndrome x3

Epilepsy, Learning disability and hemiplegia - in most severe form

96

What are neurodegenerative disorders

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

97

Causes of neurodegenerative disorders x5

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

Prophylactic agents for headache x3

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

99

Management if epilepsy is suspected x4

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

Typical age for childhood absence epilepsy

4-12 years

101

What sort of diet might help epileptic children?

Ketogenic (fat-based) diet

102

Valproate side effects x3

Weight gain, hair loss and rare idiosyncratic liver failure

103

Carbamazepine/oxcarbezepine side effects x5

Rash, neutropenia, hyponatraemia, ataxia
Liver enzyme induction

104

Vigabatrin side effects x2

Sedation
Restriction of visual fields

105

Lamotrigine side effect

Rash

106

Probable cause of Bells Palsy

Post-infectious
Association with herpes simplex virus in adults

107

Management of Bells Palsy

Corticosteroids - reduce oedema in facial canal

108

Important DDX of Bells Palsy

Cerebellopontine angle tumour

109

Bilateral Bells Palsy DDX?

Sarcoidosis
Lyme disease

110

DX of MG x3

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

What are myotonic disorders?

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

112

Presentation of dystrophia myotonica in newborns

Hypotonia, feeding and respiratory difficulties due to muscle weakness

113

What is ataxia telangiectasia?

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

114

What becomes evident at school age in ataxia telangiectasia x3

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

115

Complications in ataxia telangiectasia x2

Increased susceptibility to infection
Develop malignant disorders, esp, acute lymphoblastic leukaemia

116

What is raised in serum in ataxia telangiectasia

Alpha fetoprotein

117

Complications of neurofibromatosis 1

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