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Sarah Paeds > Neuro > Flashcards

Neuro Flashcards

1
Q

West syndrome

A 
Infantile spasms 
Intellectual disability (deteriorating social interaction)
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2
Q

5 types generalised epilepsy

A

Absence
Atonic - loss of muscle tone and sudden fall to floor
Tonic - increased tone all over
Myotonic - repetitive jerky movements of limbs, head, trunk
GTC - tonic, followed by rhythmical contraction of muscle groups

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3
Q

Ix for hydrocephalus

A

Measuring HC
CrUSS
CT/MRI

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4
Q

Features of a migraine with aura

A

Lasts a few hours
Children go to lie down in a dark room and sleep

Genetic predisposition

Triggers - reduced stress, certain foods

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5
Q

NF1 features

A 
Cafe au lait spots (6+)
2+ neurofibroma 
Axillary freckles 
Optic glioma 
Lisch nodule (harmatoma of iris)
Bony lesions 
Fhx (first degree)
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6
Q

Origin of neurocutaneous syndromes

A

Nervous system and skin have common ectodermal origin. Embryological disruption = problems in both systems

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7
Q

Non-AED treatment for epilepsy

A

Surgery - hemispherotomy
Ketogenic diets
Vagal nerve stimulation

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8
Q

Muscular dystrophies

A

Inherited disorders with muscular degeneration. Often progressive

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9
Q

Features of a generalised tonic clonic seizure

A

Loss of tone followed by rhythmical contraction of muscle groups.

Lasts seconds to minutes

Can become cyanotic (hold breath)

Drooling saliva,
Bitten tongue
Incontinence

Post ictal deep sleep

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10
Q

Medical mx epilepsy

A

AED - monotherapy starting at the lowest effective dose

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11
Q

Side effects carbamazepine

A

Rash, neutropenia, hyponatraemia, interference with other medication

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12
Q

Mx of breath holding attacks

A

Distraction and behavioural techniques

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13
Q

Ddx seizures

A

Epilepsy

Non-epileptic:

  • Febrile convulsion
  • Breath holding attack
  • Reflex anoxic
  • West Syndrome
  • Hypoglycaemia
  • Hypocalcaemia
  • Meningitis
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14
Q

Features of tuberous sclerosis

A

Ash leaf patches - depigmented macules
Shargreen patches - rough areas over lumbar spine
Adenoma sebaceum - angiofibromata in butterfly distribution

Infantile spasms, developmental delay often with autistic features, focal epilepsy, intellectual impairment.

Subungual fibromata
Rhabdomyomara
Polycytic kidneys

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15
Q

Diagnosis myasthaenia gravis

A

Anti-AChR Abs

Anti-MuSK

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16
Q

NF2 features

A

Bilateral acoustic neuroma

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17
Q

Is cerebral palsy progressive?

A

No (although things appear to change as child grows)

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18
Q

Mx CP saliva

A

SLT
Anticholinergics - hyoscine hydrobromide
Botox into salivary glands

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19
Q

Difference between Duchenne’s and Becker’s

A

Some functional dystrophin is produced in Becker’s, unlike DMD.
Later onset and slower progression of disease in Becker’s

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20
Q

Ix West syndrome

A

EEG - hypsarrythmia

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21
Q

Commonest type focal seizure

A

Temporal

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22
Q

Two examples inflammatory myopathies

A

Dermatomyositis

Myotonic dystrophy

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23
Q

Two broad categories of epilepsy

A

Generalised

Focal

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24
Q

Commonest cause of motor impairment in children

A

CP

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25
Q

Which type of CP does PVL typically predipose to?

A

Spastic diplegia (legs>arms)

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26
Q

Ix in child with seizures and developmental delay

A

Metabolic screen

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27
Q

Life expectancy Becker’s MD

A

Late 40s

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28
Q

Red flags for other neuro conditions (not CP)

A

o Absence of risk factors
o Family history of progressive neurological disorder
oLoss of already attained cognitive or developmental abilities
o Development of unexpected focal neurological signs
o MRI findings suggestive of progressive neurological disorder o MRI findings not in keeping with CP

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29
Q

Age presentation febrile convulsions

A

6mos-3yrs

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30
Q

Side effects gabapentin

A

Insomnia

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31
Q

Genetic abnormality in DMD

A

Deletion of Xp21 = mutation in dystrophin gene

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32
Q

Mx CP sleep

A

Sleep hygiene,

Melatonin

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33
Q

Mx CP eating and drinking

A

SLT assessment
Thickened fluids
Postural management
Optimise nutrition

Video fluoroscopy if necessary

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34
Q

Two broad categories of motor disorders

A

Central

Peripheral

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35
Q

Average age of diagnosis DMD

A

5.5 yrs

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36
Q

3 types of CP

A

Spastic
Dyskinetic
Ataxic

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37
Q

Features of dermatomyositis

A

Fever, misery
Symmetrical, proximal muscle weakness

Helioptrope rash
Gottren’s nodules

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38
Q

Mx headaches

A

Assess the impact on daily life
EXCLUDE RED FLAGS

Rescue treatment

  • analgesia
  • anti-emetics
  • triptans (nasal)

Prophylactic treatment

  • topiramate (Na channel blocker)
  • beta blockers
  • tricyclics
  • acupuncture

Psychosocial suport

  • identifying stressors
  • relaxation techniques
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39
Q

Rescue therapy epilepsy

A

Buccal midazolam for seizures lasting more than 5 minutes

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40
Q

Advice for parents for febrile convulsion

A 
Do not restrain child 
Protect from injury 
Do not put anything in their mouth 
Check airway 
Recovery position once stops 
Medical advice if >5 mins
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41
Q

Tuberous sclerosis management

A

Laser therapy for ash leaf patches and shargreen patches

AEDs for epilepsy

Anti-arrhythmics for cardiac involvement

anti-HTNs for PCKD

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42
Q

Features of spasms in West syndrome

A

Violent flexor spasms of head, trunk, limbs followed by extensor spasms of the arms

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43
Q

Features reflex anoxic episode

A

Trigger eg. fear, cold, head trauma
Child goes pale and falls to floor
Hypoxia may induce a GTC
Fast recovery

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44
Q

Commonest muscular dystrophy

A

DMD

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45
Q

Side effects lamotrigine

A

Rash

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46
Q

Features of motor cortex disorder

A

Loss of fine finger movements
Brisk hyperreflexia
Extended plantars

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47
Q

Ix epilepsy

A

History (?triggers)

EEG - asymmetry, hyperexcitation

MRI (if focal neuro signs) - structural and functional

Metabolic screen if developmental delay

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48
Q

Status epilepticus

A

Seizure lasting more than 5 minutes

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49
Q

Function of dystrophin (absent/mutated in MD)

A

Connects cytoskeleton to ECM

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50
Q

West syndrome prognosis

A

Poor - loss of skills, learning disabilities and continuing epilepsy

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51
Q

Prevalence of epilepsy in children

A

0.5%

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52
Q

Ix dermatomyositis

A

Bloods: Raised ESR, CRP

Muscle biopsy: inflammatory infiltrations/atrophy

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53
Q

Tuberous sclerosis inheritance patterm

A

AD

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54
Q

Which type of migraine has a genetic predisposition?

A

With aura

55
Q

What % of children suffer from febrile convulsions?

A

3%

56
Q

Cr USS finding predisposes to CP

A

Periventricular leukomalacia (HIE)

57
Q

Delayed motor milestones in CP

A

Not sitting unsupported by 8 mos
Not walking before 18 mos
Hand preference before 1 yr

58
Q

Mx status epilepticus (5 steps)

A

1) ABCDE. Secure airway, check hypoglycaemia, O2.
2) IV lorazepam.
3) If no response after 10 mins, given second dose IV lorazepam
4) Phenytoin infusion 20mg/kg for 20 mins (phenobarbitone if already on phenytoin)
5) RSI

59
Q

SOL presentation

A 
Morning/wake up headaches 
Change in mood, personality, school performance, 
Visual field changes 
Cranial nerve abnormalities 
Abnormal gait 
Growth failure
60
Q

Which surgeries may be appropriate in muscular dystrophy

A

Tendoachilles lengthening and scoliosis surgery

61
Q

AED for focal seizures

A

Carbamazepine

Lamotrigine

62
Q

Inheritance pattern neurofibromatosis 1

A

AD - highly penetrant

63
Q

When can children come off AED

A

2 years seizure free

64
Q

Features of temporal focal seizure

A

Warning/aura, lip-smacking/plucking/automations. Impaired consciousness

65
Q

Mx if febrile convulsion lasts longer than 5 min

A

Call ambulance
Measure blood glucose
Bucal midazolam/ PR diazepam

66
Q

Age presentation West Sydrome

A

4-6 months

67
Q

What is neurofibromatosis associated with?

A

MEN, phaeo, renal artery stenosis, HTN, gliomatous change

68
Q

Initial mx epilepsy

A

Refer all patients to neurologist (first fit clinic)
Specialist epilepsy nurse
Record future seizures
Start a diary

INFORM SCHOOL

69
Q

Pathophysiology of muscular dystrophy

A

Reduced dystrophin
Reduced intracellular firing = reduced calmodulin = increased free radicals = necrosis = muscle destruction and release of CK

70
Q

Causes of CP

A

80% antenatal
10% HIE
10% postnatal

71
Q

Migraine ddx

A

Abdominal migraine
Cyclical vomiting
BPPV

72
Q

What % of childhood migraines are with aura?

A

10%

73
Q

Example NMJ dysfx disorder

A

Myasthenia gravis

74
Q

Features of CP

A

Abnormal limb/trunk posture
Delayed motor milestones
Slowed head growth

Feeding difficulties due to locomotor coordination

Abnormal gait (scissoring, tip toes)

Asymmetric hand function

75
Q

Example central motor disorder

A

Cerebral palsy

76
Q

Epilepsy

A

Recurrent, unprovoked seizures - transient signs and symptoms of abnormal/asynchrous/excessive firing of neurons

77
Q

4 types focal seizure

A

Frontal - motor cortex with clonic/atonic features

Temporal - warning/aura, lip-smacking/plucking/automations. Impaired consciousness

Parietal - impaired sensation

Occipital - visual distortion

78
Q

Two examples peripheral neuropathies

A

GBS

Facial nerve palsies e.g. Bell’s

79
Q

4 types neurocutaneous syndromes

A

NF1
NF2
Tuberous sclerosis
Sturge-Weber

80
Q

Cerebral palsy

A

Abnormality of movement and posture disturbance

81
Q

Life expectancy of DMD

A

Late 20s

Eventual resp failure

82
Q

Presenting features DMD

A

Waddling gait
Gower sign
Language delay
Pseudohypertrophy of calves (muscle replaced with fat/fibrous tissue)

83
Q

Mx CP speech, language, comms

A

SLT
Posture control, breath control,
Alternative communication - comms boards, pictures, etc.

84
Q

Age presentation dermatomyositis

A

5-10 years

85
Q

Mx CP pain

A

Reducig regimen of paracetamol

86
Q

Pathophysiology reflex anoxic episode

A

Cardiac asystole from vagal inhibition - trigger

87
Q

Features of hydrocephalus

A

Increases HC centiles
Bulging fontanelle
Sun-setting gaze (fixed-downward)
Distended scalp veins

88
Q

4 types of peripheral neuro disorders and their features

A

Anterior horn cell - weakness, hyporeflexia, fasciculations

Neuropathy - weakness, reduced sensation

NMJ dysfunction - progressive weakness

Myopathy - proximal weakness, wasting, abnormal gait,

89
Q

Causes of postnatal CP

A

Kernicterus, meningitis/encephalitis, hypoglycaemia

90
Q

Advice for parents epilepsy

A

Parents/carers - how to recognise a seizure
Record future seizures
Start a diary
Avoid dangerous activities (e.g. swimming until seizures under control)

91
Q

Average age wheelchair dependence DMD

A

10-14 yrs

92
Q

Ix for DMD

A

CK
Muscle biopsy
DNA
USS/MRI

93
Q

Mx hydrocephalus

A

Ventriculo-peritoneal shunt

Ventriculostomy

94
Q

What % of childhood migraines are without aura?

A

90%

95
Q

Causes of secondary epilepsy

A

Infection, HIE, tumour, trauma,

Neurodegenerative disorder

96
Q

Average age onset Becker’s MD

A

11 years

97
Q

Mx myelomeningocele

A

Surgical repair

Physiotherapy

98
Q

Mx neurofibromatosis

A

Medical - regular r/v ophtalmology, BP, skeletal complications)
Surgery - laser removal of nodules

99
Q

Inheritance pattern DMD

A

XR

100
Q

Features of myelomeningocoele

A

Meninges protrude with cord. Paralysis of legs, sensory loss, bladder/bowel dennervation, hydrocephalus

101
Q

Causes of hydrocephalus

A

Increased CSF production
CSF flow obstruction
Inhibited CSF reabsorption

= ventricular dilatation

102
Q

Mx CP bones

A

Ca, vit D, phosphate

Non-weight bearing

103
Q

Example of anterior horn cell disease

A

Spinal muscular atrophy

104
Q

Side effects sodium valproate

A

Weight gain, hair loss, liver failure

105
Q

Features of nocturnal hypoxia

A

Daytime headache
Irritability
Reduced feed

106
Q

Features myotonic dystrophy

A

Hypotonia

Feeding/resp difficulty

107
Q

Features of Sturge-Weber syndrome

A

Port-wine stain in trigeminal region (V2)
Similar intracranial lesion

Haemangiomatous

108
Q

Examples peripheral motor disorder

A 
Neuromuscular 
Anterior horn cell
Neuropathy 
NMJ
Myopathy
109
Q

Mx dermatomyositis

A

Physiotherapy to reduce risk of contractures
Steroids
Methotrexate

110
Q

Features of GBS

A

2-3 weeks after GI infection (e.g. campylobacter)

Ascending, symmetrical muscle weakness

Increased protein in CSF

111
Q

Three types of primary headache

A

Tension
Migraine
Cluster

112
Q

Members of MDT CP

A 
Paediatrician
Nurse 
Physio
OT
SLT
Dietetics 
Psych
113
Q

Mx West syndrome

A

Prednisolone

Vigabatrin

114
Q

Limitation of using EEG as ix for epilepsy

A

Need to catch the seizure

115
Q

AED for generalised seizures

A

Sodium valproate

Keppra

116
Q

Two categories of muscule disorders

A

Muscular dystrophies

Inflammatory myopathies

117
Q

When does the neural plate fuse?

A

Within 28 days of conception

118
Q

Features of basal ganglia disorder

A

Dystonia
Dyskinesia
Difficulty initiating movements
Chorea

119
Q

Mx of DMD

A

Physiotherapy to help prevent contractures
Exercise
Psych support
CPAP (weakened ICM lead to nocturnal hypoxia)

Surgery - tendoachilles lengthening and scoliosis surgery

Prednisolone (delay wheelchair dependence)

Cardioprotective drugs if LVEF drops

120
Q

Mx myotonic dystrophy

A 
Physiotherapy
OT (utensils)
Ortho (foot drop)
SLT - E,D, speech 
Pain - gabapentin/NSAIDs 
Mixeletine - for myotonia
121
Q

Inheritance pattern myotonic dystrophy

A

AD - nucleotide triplet expansion, with anticipation thru generations

122
Q

Three categories of headache

A

Primary (tension, migraine, cluster, other)
Secondary (Raised ICP, trauma, infection)
Neuralgias

123
Q

Two types of migraine

A

w/ aura

w/o aura

124
Q

When do febrile convulsions result in an increased risk of later epilepsy

A

If they are prolonged and/or recurrent

125
Q

Mx absence seiures

A
  • aimed at maximsing education potential and supporting social development.
126
Q

Features of a migraine without aura

A

Lasts 1-72 hours
Pulsatile over the temporal/parietal region
Unpleasant GI disturbance - Nausea, vomiting, pain
Photophobia
Worsened by physical activity

127
Q

What is the decision to treat epilepsy based on?

A

Risk of recurrence
Impact on life
How dangerous/impairing seizures are

128
Q

What are common triggers for a migraine with aura?

A

Reduced stress

Certain foods

129
Q

What % of epilepsy is idiopathic?

A

70-80%

130
Q

What proportion of West syndrome have underlying neuro cause?

A

2/3

131
Q

When appropriate to r/f febrile convulsion to paediatrician

A 
If <18 mos 
Diagnostic uncertainty over cause 
Seizure lasted >15 mins 
Focal features during seizure 
Incomplete recovery after 1 hour
132
Q

Mx CP muscle stiffness

A

Baclofen/diazepam

133
Q

Mx myasthaenia gravis

A

Neostygmine
Prednisolone
Azathioprine

Sarah Paeds (20 decks)

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