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Year 4 Paediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Define Ataxia

A

Abnormality in gait that is wide based/staggering/unsteady

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2
Q

Give three causes of Ataxia in children

A

Posterior fossa tumours
Brainstem Encephalitis
Friedreich’s Ataxia

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3
Q

Give four other clinical features that might be associated with a presentation of Ataxia

A

Increased separation of speech syllables
Abnormal Proprioception
Positive Romberg
Nystagmus

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4
Q

How could you investigate Ataxia in a child?

A

Cerebral Imaging

Plasma and CSF samples (particularly for Varicella, Strep, and Inborn Errors of Metabolism)

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5
Q

What is Chorea? Give four causes

A

Jerk like movements that may involve face/arms/legs

Drugs (anticonvulsants), SLE, Sydenhams Chorea, Benign Familial

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6
Q

What is Sydenham’s Chorea?

A

Chorea often associated with streptococcal infection, occurring in older children

Child is normally well

20% Rheumatic Fever

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7
Q

How is Syndehams Chorea managed?

A

High dose Pen V then daily prophylaxis

If IEM is excluded, then start Sodium Valproate

Can give Benzodiazepines/Haloperidol as symptom management

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8
Q

What is PANDAS?

A

Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus

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9
Q

How does PANDAS present?

A

OCD
Tics
Irritability
Anxiety

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10
Q

Define Athetosis

A

Sinuous, slow, involuntary writhing movements affecting fingers/hands/toes/feet

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11
Q

Name three causes of Athetosis

A

Asphyxia
Neonatal Jaundice
Trauma

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12
Q

How is Athetosis managed?

A

Diazepam/Haloperidol

Surgery and restraining technique

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13
Q

What is Primary Pure Dystonia?

A

Autosomal Dominant dystonic spasms of legs on walking

Progresses to whole body within ten years

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14
Q

What are Tics?

A

Repetitive stereotyped movements that can be initiated voluntarily and also suppressed voluntarily
Can be simple (twitch in same site, occurring in 25%) or complex (multiple tics, associated with tourettes)

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15
Q

What could the term ‘floppy’ mean?

A

Decrease in muscle tone
Decrease in muscle power
Ligamentous laxity/increased ROM

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16
Q

Define Hypotonia

A

Low resistance to passive stretch around the joint

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17
Q

What is Phasic Tone?

A

Response of muscles to rapid stretch

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18
Q

What is Postural Tone?

A

Response to sustained low intensity stretch

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19
Q

What in their antenatal history would you want to know about a ‘Floppy Infant’?

A

Reduced foetal movements
Polyhydramnios
Breech

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20
Q

What in their family history would you want to know about a ‘Floppy Infant’?

A

Muscle Disease
Stillbirth
Consanguinity

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21
Q

What in their birth history would you want to know about a ‘Floppy Infant’?

A 
Duration of labour 
Method of delivery
Rescucitation
Apgar Score
Cord Gases
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22
Q

What associated features might you see in a ‘Floppy Infant’?

A

Poor swallowing

Weak Cry

Paradoxical Breathing (intercostal muscles paralysed but intact diaphragm)

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23
Q

If the cause of the ‘Floppy Infant’ was central/UMN, how would it present?

A

Poor truncal tone
Normal reflexes
Loud cry

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24
Q

If the cause of the ‘Floppy Infant’ was Peripheral/LMN, how would it present?

A

Frog like posture
Reduced/Absent reflexes
Weak Cry

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25
Q

Describe three central causes of the ‘Floppy Infant’

A

Dysgenesis - Downs Syndrome, Prader Willi

Degeneration - Metabolic Disorders, Enzyme Disorders (eg Tay Sachs)

Encephalopathy (HIE, ICH, Congenital or Acquired infections)

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26
Q

Give 5 classes of non central causes of the ‘Floppy Infant’

A

Spinal Cord (Syringomyelia, Birth Trauma)

Anterior Horn (SMA, Poliomyelitis)

Peripheral Nerve (Guillaine Barre, Hereditary - Charcot Marie Tooth)

Neuromuscular Junction (Neonatal Myasthenia Gravis, Infantile Botulism)

Muscle (Muscular Dystrophy, Metabolic Myopathies, Congenital Myopathies)

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27
Q

How can you investigate central causes of the ‘Floppy Infant’?

A 
Range of bloods
Sepsis screen
Plasma Amino Acids
Cranial Ultrasound and MRI 
Microarray
LP
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28
Q

How can you investigate peripheral causes of the ‘Floppy Infant’?

A

CK
Muscle Biopsy
Microarray
Conduction studies

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29
Q

Name 7 causes of headaches in children

A 
Tension
Migraines
ENT Infection
Vision Problems
Raised ICP
Meningitis
Brain Tumours
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30
Q

How do young children with tension headaches present?

A

Non Specific
Quiet
Stop Playing
Turn Pale

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31
Q

How do older children with tension headaches present?

A

Mild ache across forehead in band like pattern

Gradual onset and resolution with no pulsing/visual changes

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32
Q

Migraines are a complex condition that causes headache attacks and other symptoms. Name five different types.

A 
Migraine without aura
Migraine with aura
Silent migraine 
Hemiplegic
Abdominal
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33
Q

How do Migraines present?

A

Unilateral, throbbing headache

Associated - visual aura, photophobia, nausea and vomiting

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34
Q

If Migraines are impacting on QoL, what drugs can you prevent them with?

A

Propanolol (avoided in Asthma)
Pizotifen (Drowsiness)
Topirimate (Teratogenic - COCP required)

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35
Q

Abdominal Migraines may be the start of traditional migraines developing when they’re older. How do they present?

A

Episodes of central abdominal pain lasting for more than one hour, with associated Nausea and Vomiting/Anorexia/Headache/Pallor

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36
Q

How are Abdominal Migraines managed?

A

Pizotifen, Sumatriptans

Avoid Caffiene/Cheese/Marmite

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37
Q

Give three causes of Focal Seizures

A

Cortical Dysplasia
Trauma
Tumour

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38
Q

Temporal lobe is the most common type of focal epilepsy. How can it present?

A

Odd Smell/Sensation
Deja Vu
Repetitive Automatisms

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39
Q

How do Frontal Lobe Seizures present?

A
  • Often night waking
  • Thrashing and Bicycle Movements
  • Jacksonian March (movements travelling proximally)
  • Tonic arms raised in air
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40
Q

How do Occipital Lobe Seizures present?

A

Often mistaken for migraines

Flickering light, Visual Hallucinations

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41
Q

How do Parietal Lobe tumours present?

A

Tingling in hands/feet

Body image distortion

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42
Q

Generalised seizures occur in both hemispheres. How does an Absence Seizure present?

A

Generally starts between age of 4-7 and resolves in adolescence

<10seconds unresponsive

Occasional Automatisms

Triggered by hyperventilation

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43
Q

Generalised seizures occur in both hemispheres. How does an Myoclonic Seizures present?

A

Jerking Movements with retained conscious

Child may require helmet

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44
Q

What is Progressive Myoclonic Epilepsy?

A

Seizures + Unsteadiness+ Rigidity + Mental Deterioration

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45
Q

Ohtahara Syndrome is a cause of seizures in the first 28d of life. How does it present?

A

Primary tonic seizures occuring in the first two weeks
Caused by metabolic disorders or brain damage

Often die within weeks/months

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46
Q

Benign Familial Seizures is a cause of seizures in the first 28d of life. How does it present?

A

Recurrent seizures in newborns lasting one to two minutes

Usually stops at four months old

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47
Q

Give two causes of INFANTILE seizures

A

West Syndrome (Jerking followed by stiffening, often due to Hypoxic birth injury)

Dravet Syndrome (Sodium Channel Mutation)

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48
Q

Give four Neurological causes of Seizures

A

Rett Syndrome
Angelman Syndrome
Tuberous Sclerosis
Sturge Weber (Port Wine Stain)

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49
Q

Define Epilepsy

A

Umbrella term for condition where there is a tendency to have seizures (transient episodes of abnormal electrical activity)

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50
Q

How does a Generalised Tonic Clonic Seizure present?

A

Loss of consciousness then tonic clonic movements

Associated tongue biting, incontinence, irregular breathing

Post Ictal - Prolonged drowsiness, confusion, irritability

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51
Q

How are Generalised Tonic Clonic seizures managed?

A

1st line - Sodium Valproate

2nd line - Lamotrigine or Carbemazepine

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52
Q

How are Focal Seizures managed?

A

1st line - Lamotrigine/Carbemazepine
2nd line - Sodium Valproate

IE opposite to generalised tonic Clonic

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53
Q

How are Absence Seizures managed?

A

Typically grow out of them

1st line - Sodium Valproate or Ethosuxamide

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54
Q

What are Atonic Seziures?

A

AKA Drop Attacks

Brief lapses in muscle tone, normally not lasting longer than 3 minutes

No post ictal drowsiness

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55
Q

Describe the typical EEG of childhood absence seizures

A

3Hz spike and wave

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56
Q

How are Myoclonic Seizures managed?

A

First line - Sodium Valproate

Second line - Lamotrigine/Topirimate

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57
Q

How is Infantile Spasms/West Syndrome managed?

A

Prednisolone and Vigabatrin

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58
Q

How is Epilepsy investigated?

A

EEG (after SECOND tonic Clonic seizure)
Bloods/LP/Cultures
ECG

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59
Q

When would you do an MRI brain in Epilepsy?

A

If the first seizure is when they’re <2

Focal Seizures

No response to antiepileptic medication

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60
Q

What general advice should be given to epileptic children? Give four points

A

Take showers rather than baths

Be cautious with swimming

Be cautious with heights and traffic

Older teenagers may need to avoid driving

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61
Q

Describe Sodium Valproate’s MOA

A

Increases GABA Activity

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62
Q

Name three side effects of Sodium Valproate

A

Teratogenic
Hepatitis
Tremor

63
Q

Name two side effects of Carbemazepine

A

Agranulocytosis

CYP inducer

64
Q

Describe Carbemazepine’s MOA

A

Sodium Channel Blocker

65
Q

State three side effects of Phenytoin

A

Folate deficiency
Vit D Deficiency
Osteomalacia

66
Q

State two side effects of Ethosuxamide

A

Night terrors

Rashes

67
Q

State two side effects of Lamotrigine

A

Steven Johnson Syndrome

Leucopenia

68
Q

How should you manage an acute seizure in the community?

A

Recovery position with soft item under head

Note start and end of seizure

Call an ambulance if first episode or lasts more than five minutes

69
Q

Define Status Epilepticus

A

Seizures lasting >5 minutes, or >3 in an hour

70
Q

How is Status Epilepticus managed in hospital?

A

A to E approach
IV Lorazepam
Secure airway

If persists - IV Phenytoin

71
Q

How should Status Epilepticus be managed in the community?

A

A to E approach

Buccal Midazolam or Rectal Diazepam

72
Q

What is an Extradural Haemorrhage?

A

Collection of blood in potential space between dura and skull

Normally a venous bleed in children

Typically caused by trauma to temple

73
Q

How does an Extradural Haemorrhage present in a child?

A

Headache
Nausea and Vomiting
Lucid Interval until consciousness deteriorates

May experience seizures

74
Q

How should Extradural haemorrhages be investigated?

75
Q

How should an Extradural Haemorrhage be managed?

A

A to E

Small bleed - conservative
Raised ICP - Mannitol, Burr Holes

> 30cm2 bleed - Surgical Evacuation

76
Q

The prognosis of Extradural haemorrhage in children is excellent. Name two complications.

A

Neurological deficits

Post traumatic seizures

77
Q

Define Migraine

A

Recurrent headache occurring with or without aura, lasting from 30 minutes to 48 hours

78
Q

A Basilar Migraine is a type of migraine. What is it associated with?

A

Dizziness

Syncope

79
Q

Childhood Periodic Syndromes are commonly migraine precursors. Name three.

A 
Cyclical Vomiting (intense vomiting at night)
Abdominal Migraine
BPPV
80
Q

Describe the aura of a migraine

A

Can be visual, sensory, cognitive or motor

Aura may be more distressing than the headache itself

81
Q

When does a Migraine require further investigation?

A

Neurological abnormalities
Seizure history
Head trauma

82
Q

What drugs can be used in an Acute Migraine

A

Simple Analgesia

Sumatriptans (only if over 6y and simple is ineffective)

Domperidone/Prochlorperazine

83
Q

Define Muscular Dystrophy

A

Umbrella term for genetic conditions causing gradual wasting and weakness of muscles

84
Q

What is Gower’s Sign?

A

Children with proximal muscle weaknessGet on their hands and knees, push hips back and up, then walk hands up legs in order to stand

Due to weakness of pelvic muscles

85
Q

The most well known Muscular Dystrophy is Duchennes. Describe the pathophysiology

A

X Linked defective gene for Dystrophin, which normally connects muscular cytoskeleton to ECM

Results in myofibre necrosis

86
Q

Name four presenting features of Duchennes MD

A

Waddling Gait
Language Delay
Gowers Sign
Pseudo Hypertrophy of Calves (muscle replaces with fat and subcut tissue)

87
Q

What is the prognosis of Muscular Dystrophy?

A

Usually wheelchair bound by teenage years

Life expectancy 25-35

88
Q

How is Duchennes MD managed?

A

Oral steroids to slow progression
Creatine supplements
Physiotherapy

May require overnight CPAP

Ataluren

89
Q

How does Ataluren work for MD?

A

Skips non sense mutations allowing production of small amounts of dystrophin

90
Q

What is Becker’s Muscular Dystrophy?

A

Similar mutation to Duchennes, but some dystrophin is produced

91
Q

How does Becker’s MD vary from Duchennes?

A

Clinical course is less predictable
Symptoms start at 8-12y
Patients may require wheelchair in late 20s to 30s

92
Q

What is Myotonic Dystrophy?

A

Genetic disorder that normally presents in adulthood
Progressive muscular weakness and prolonged muscle contractions (unable to let go after shaking hand)
Associated cataracts and cardiac arrhythmias

93
Q

What is Fascioscapulohumeral MD?

A

Presents in childhood with weakness around the face progressing to shoulders and arms

Initial signs - sleeping with eyes open, weakness in pursing lips, unable to blow out cheeks

94
Q

What is Oculopharyngeal Muscular Dystrophy?

A

Usually presents into late adulthood

Weakness of ocular muscles and pharynx

Bilateral ptosis, restricted eye movements, swallowing problems

95
Q

What is Limb Girdle Muscular Dystrophy?

A

Presents in teenage years with progressive weakness around limb girdle

96
Q

What is Emery Dreifuss MD?

A

Contracture occurring at elbows and ankles

97
Q

A raised ICP is a pressure above 20mmHg. Describe the normal ranges in infants, young children and older children respectively

A

Infants - 1.5 to 6
Young - 3 to 7
Older - 10 to 15

98
Q

What is the Monroe Doctrine?

A

An increase in any constituents of the brain will cause a compensatory decrease in others in order to maintain ICP

Constituents being blood, CSF and brain tissue

99
Q

Give five causes of raised ICP?

A 
Masses
Haemorrhage
Impaired CSF flow
Meningitis
Reyes Syndrome
100
Q

In raised ICP, herniation can occur. State the three different types.

A

Subfalcine (cingulate gyrus displaced under falx, may compress ACA)

Tentorial (medial temporal lobe under tentorium cerebelli)

Tonsillar (cerebellar tonsils through foramen magnum, can compress respiratory centres)

101
Q

Describe five classical features of raised ICP

A 
Headache worse on waking
Vomiting
Visual Disturbance
Change in mood
Abnormal Pupils

Note: May be subtle if mild

102
Q

How does Severely Raised ICP present?

A

Papilloedema
Sun setting Eyes
Cushing’s Response (bradycardia and hypertension)

103
Q

Name three red flags with raised ICP

A

GCS<8
Abnormal respiratory pattern
Abnormal Posture (decorticate, decerebrate)

104
Q

How is raised ICP managed?

A

A to E approach and high flow O2
Tilt 20-30 degrees up

Consider Mannitol, Antibacterials, Antivirals

Urgent CT when stable

105
Q

Define Spinal Muscular Atrophy

A

Rare autosomal recessive condition that causes progressive loss of motor neurones, leading to muscular weakness

106
Q

As Spinal Muscular Atrophy affects lower motor neurones, how does it present?

A 
Fasciculations
Reduced Muscle Bulk
Reduced Tone
Reduced Power 
Reduced reflexes
107
Q

What are the five types of Spinal Muscular Atrophy?

A

Type 0 - Prenatal onset, death within 6 months

Type 1 - onset in first few months, progressing to death within 2 years

Type 2 - onset in first 18 months, most never walk but survive into adulthood

Type 3 - onset after first year, walk without support then lose that ability

Type 4 - onset in 20s, able to walk short distances but easily fatigued

Note: in Type 3 and 4 life expectancy is not affected

108
Q

How is SMA managed?

A

Supportive only
Physiotherapy
PEG?

May have scoliosis and respiratory failure that needs managing

109
Q

Give four causes of Spinal Cord Injury in children

A

Road Traffic Accidents
Breech Delivery
Shoulder Dystocia
Horse Riding

110
Q

Why are children at risk of Spinal Cord Injury?

A

Vertebral column is relatively elastic and dura doesn’t provide a lot of mechanical protection

Head is relatively large compared to weak neck

111
Q

How could Spinal Cord Injury present?

A

Cervical - Tetraplegia with Sensory Loss

Thoracic - Weak Intercostals, breathing assistance with ventilation required

Lumber and Sacral - Paraplegia and loss of control of bladder/bowel/sexual organs

112
Q

How should a suspected SCI be investigated

A

XRay and CT first, if high suspicion then MRI

Beware of SCIWORA due to flexibility of paediatric spine

113
Q

Describe the Frankel Grading of Spinal Cord Function

A 
A - Complete Paralysis
B - Sensory function below injury
C - Incomplete motor function below injury
D - Good Motor function
E - Normal Function
114
Q

How are Spinal Cord Injuries in children managed?

A

A to E
Rehab
Corticosteroids to reduce swelling

115
Q

Name four causes of Subdural Haemorrhage in children

A

Trauma (inc Birth and NAI)
Cerebral Infections
Coagulation disorders
Hypernatraemia

116
Q

How does a Subdural Haemorrhage present?

A 
Encephalopathy 
Vomiting
Pallor
Tense Fontanelle
Seizures
117
Q

If considering NAI in a Subdural Haemorrhage presentation, what associated features should you look out for?

A

Retinal Haemorrhages

Skull Fractures that are depressed/branching

118
Q

How should a Subdural Haemorrhage be investigated?

A

CT and MRI

119
Q

Name five potential causes of SAH in children

A 
AV Malformation
Cerebral Aneurysm 
Neoplasms
Leukaemia
Haemophilia
120
Q

How does SAH present in <6 month old?

A

Seizures in 65%

Apnoea, Irritability, Bulging Fontanelle

121
Q

How does SAH present in >6 month old?

A

Severe headaches
Seizures
Focal neurology

122
Q

How should suspected SAH be investigated?

A

USS in neonates
CT
Clotting
LP - Xanthechromia

123
Q

How is SAH managed?

A

Intubation and ventilation as required
Analgesia
Nifedipine (prevents Vasospasm)
Clipping/coiling where appropriate

124
Q

What is required to diagnose Tension Headaches?

A

Atleast 10 headaches for 30 minutes of 7 days with atleast two of:

Bilateral Pain
Tightening quality
Not worsened by physical activity

125
Q

What is a Squint/Strabismus?

A

Misalignment of the eyes

Images on retina will not match and patient will experience double vision

126
Q

Describe the physiology of a squint in childhood

A

Brain copes by reducing signal from less dominant eye, creating dominant eye and lazy eye

If lazy eye is not treated it becomes worse (Amblyopia)

127
Q

What are concomitant squints?

A

Differences in control of Extra- Ocular muscles

128
Q

State the different types of eye movement in Strabismus

A

Esotropia - Inward moving
Exotropia - Outward moving
Hypertropia - Upward moving
Hypotropia - Downward moving

129
Q

The cause of Strabismus is normally idiopathic. Describe two tests that can be done OE

A

Hirschbergs - shine pen torch, reflection should be equal (if not then its a squint)

Cover Test - cover one eye, ask patient to focus on an object then switch cover. If eye moves back in then it was an Exotropia etc

130
Q

Management of Strabismus needs to start before 8 years while visual field still develops. How is this done?

A

Occlusive patch over good eye

Atropine drops over good eye

131
Q

What is a Scotoma?

A

Defect surrounded by a normal visual field

Can be relative (ie high luminance objects seen) or absolute

132
Q

What are the five different types of Hemianopia?

A 
Bitemporal
Homonymous
Altitudinal 
Quadrantopia
Sectoral
133
Q

What is Glaucoma?

A

Loss of Optic Nerve axons associated with raises Intraocular Pressure, manifesting in optic disc cupping and visual loss

In children less than 3y the sclera is stretchy so raised IOP causes globular enlargement and corneal blurring

134
Q

Give three causes of Glaucoma in Children

A

Congenital
Implicated in Sturge Weber (Port Wine Stain)
Steroid Therapy

135
Q

How can Glaucoma be managed medically?

A

Topical Beta Blockers
Topical Prostaglandin Analogues
Topical Miotics
Acetazolamide

136
Q

Give 5 causes of Sensorineural Hearing Loss in Children

A

Lesion at Cochlea/Auditory nerve
Genetic
Antenatal (infection, HIE)
Postnatal (Meningitis,Encephalitis, Aminoglycosides)

137
Q

How is Sensorineural Hearing Loss managed in Children?

A

Amplification or Cochlear Implant

138
Q

Give three causes of Conductive Hearing Loss

A

Glue Ear
Wax
Eustacian Tube Dysfunction (Downs, Cleft Palate)

139
Q

How is Conductive Hearing Loss managed?

A

Conservative
Amplification
Grommets

140
Q

What is Tay Sach’s Disease?

A

Accumulation of lipids in brain tissue

Associated with: Muscle weakness, Myoclonic jerks, exaggerated startled response, gradual visual deterioration, loss of previously learned skills

141
Q

Name 5 childhood paroxysmal events that could be mistaken for seizures

A 
Breath Holding Attacks 
Reflex Asystolic Syncope
Migraine
BPPV
Pseudo seizures
142
Q

What are Reflex Asystolic Syncope episodes?

A

Vagally induced cardiac asystole, secondary to cold food/fright/head bump

Child stops breathing, goes limp and blue

Hypoxia may induce Tonic Clonic

143
Q

How might BPPV present in toddlers?

A

Pale and frightened
May vomit

Exclude temporal lobe epilepsy

144
Q

How might Pseudoseizures present?

A

Typically adolescent girls
Dramatic
Rolling around
Flinging limbs

Gradual onset

145
Q

West Syndrome is an example of a childhood Seizure Syndrome. How does it present?

A

Typically onset between 3 and 12 months

Multiple busts of flexor spasms causing distress

Salaam attacks (rapid flexion extension)

Gradually lose skills and develop learning disability

146
Q

How do Infantile Spasms/West Syndrome present on EEG?

A

Hypsarrhythmia

Burst supression

147
Q

Lennox Gastaut Syndrome is an example of a childhood Seizure Syndrome. How does it present?

A

Can be : Atonic, Absence (with blinking or head nodding) or Tonic (stiffening of arms and legs at night)

Poor neurodevelopment progression

148
Q

How does Lennox Gastaut Syndrome present on EEG?

A

Slow generalised spike and wave discharge (1-3 Hz)

149
Q

Benign Rolandic Epilepsy is an example of a childhood Seizure Syndrome. How does it present?

A

Starts during sleep or when about to wake

Pins and needles on one side of mouth

Can get Associated twitching or can involve throat (affecting speech)

Occasionally the whole body is affected

Most grow out at Puberty

150
Q

How does Benign Rolandic Epilepsy present on EEG

A

Posterior sharp waves

Occipital discharges

151
Q

Juvenile Myoclonic Epilepsy Syndrome is an example of a childhood Seizure Syndrome. How does it present?

A

Normally onset between 10-20y

Can be Myoclonic or tonic Clonic

Unlikely to grow out of but control is good

152
Q

How does Juvenile Myoclonic Epilepsy present on EEG?

A

3-6 Hz Polyspike and Wave discharge

153
Q

Describe some hearing test milestones for children

A

Newborn - Otoacoustic Emission (looking for a soft echo meaning normal tympanic membrane)

If Otoacoustic Emission abnormal - Auditory Brainstem

Distraction test at 6-9 months

Pure Tone Audiometry upon school entry

Year 4 Paediatrics (15 decks)

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