Neurology Flashcards

1
Q

What is it important to ask about in history of seizures?

A

Do the seizures occur when the child is awake or asleep

Could be benign sleep myoclonus - not an epileptic seizure and will disappear with age; no change in colour, saturations, otherwise well

Was the child in distress before the episode?
= breath holding spells, reflex anoxic seizures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is epilepsy?

A

Umbrella term - tendency to have seizures - which are transient episodes of abnormal electrical activity in the brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the types of seizures?

A
Tonic-clonic
Focal seizures 
Absence seizures
Atonic seizures
Myoclonic seizures
Infantile spasms

Febrile convulsions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is seen in a tonic clonic seizure?

A

loss of consciousness, tonic muscle tensing, clonic muscle jerking
Associated tongue biting, incontinence, irregular breathing
Drowsy post-octal period

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is seen in a focal seizure?

A
start in temporal lobe
Affect hearing, speech, memory, emotions
Hallucinations
Memory flashbacks
Deja vu
Doing strange things on autopilot
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the management of tonic clonic seizures?

A

First line - sodium valproate

Second line - lamotrigine or carbamazepine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the management of focal seizures?

A

First line: carbamazepine or lamotrigine

Second line: sodium valproate, levetiracetam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are absence seizures?

A

Patient becomes blank
Stares into space then abruptly returns to normal
During episode unaware of surroundings
Lasts 10-20 seconds

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the management of absence seizures?

A

First line - sodium valproate or ethosuximide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are atonic seizures?

A

Drop attacks
Brief lapses in muscle tone
Usually don’t last more than 3 minutes
May be indicate of Lennox-Gastaut syndrome?

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is lennox-gastaut syndrome?

A

rare type of severe childhood-onset epilepsy
associated with gene mutations, perinatal injuries, brain tumours, malformations
treatment does not tend to end seizure recurrence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the treatment of atonic seizures?

A

First line sodium valproate

Second line lamotrigine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are myoclonic seizures?

A

Sudden brief muscle contractions like a sudden jump
Patient usually awake during
Typically happen as part of juvenile myoclonic epilepsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the management of myoclonic seizures?

A

First line - sodium valproate

Other options - lamotrigine, levetiracetam, topiramate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is West syndrome?

A

Infantile spasms
Clusters of full body spasms
Difficult to treat

First line prednisolone or vigabatrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are febrile convulsions?

A

Seizures in children whilst they have a fever
Not caused by epilepsy or underlying neurological pathology

Occur only in children between the ages of 6 months - 5 years

Slightly increases risk of developing epilepsy in future

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are the investigations for epilepsy?

A

Good history, type of seizure
EEG
MRI brain - first seizure in under 2, focal seizures, no response to first line drugs

ECG to exclude heart problems
Blood electrolytes
Blood glucose - hypoglycaemia and diabetes
Blood cultures, urine cultures, LP - infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What general advice is key in those with epilepsy?

A

Take showers rather than baths
Be cautious with swimming unless seizures well controlled and closely supervised
Cautious with heights
Cautious with traffic
Cautious with heavy, hot or electrical equipment
Likely will avoid driving

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the effect of sodium valproate?

A

Increasing GABA

Teratogenic
Liver damage, hepatitis
Hair loss
Tremor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are some of the side effects of carbamazepine?

A

Agranulocytosis
Aplastic anaemia
Induces P450 system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are some of the side effects of phenytoin?

A

Folate and Vit D deficiency
Megaloblastic anaemia
Osteomalacia - Vit D def

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are some of the side effects of lamotrigine?

A

Stevens Johnson syndrome

Leukopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the management of a seizure?

A

Safe position
Recovery position if possible
Something soft under head
Remove obstacles that could lead to injury
Record start and end time
Call ambulance if lasts more than 5 mins, or first seizure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the definition of status epilepticus?

A

Seizures lasting more than 5 minutes

More than 3 seizures in one hour

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is the management of status epilepticus?

A
Secure airway
High conc oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain IV access
IV lorazepam repeated after 10 mins if continues

If still persists - infusion of IV phenobarbital or phenytoin
Then intubation, ventilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What are the medical options in the community for status epilepticus?

A

Buccal midazolam

Rectal diazepam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What questions need to be asked about episodes - fits, faints or funny turns?

A

Freq - how many in day, week, month
Timing - certain time of day or behavioural states
Relationship to sleep - on falling asleep, during sleep, waking
Triggers - tired, travel, excitement, exercise
Warnings beforehand
Duration - if clustered how long between episodes
Colour change - pallor, early cyanosis
Alterations in conscious level
Motor phenomena - act it out
Recovery and symptoms after
Family history

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What defines epilepsy?

A

At least two unprovoked seizures occurring more than 24 hours apart

One unprovoked seizure and a probability of further seizures similar to the general recurrence risk after two unprovoked seizures occurring over next 10 years

Diagnosis of an epilepsy syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What are acute asymptomatic seizures?

A

Result of acute insults

e.g. hypoglycaemia, hypoxia, electrolyte derangements, infection, trauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are the common paediatric epilepsy syndromes?

A
West Syndrome - infantile spasms
Age 4-7
Sudden jerks of neck, trunk
Shortly after wakening, or when falling asleep, clustered
Cries at end
Loss of visual alertness

Benign epilepsy with Centro-temporal spikes
3-12 years, spontaneous remission by adolesence
From sleep, focal onset
Sensory or motor
Tingling, drooling, aphasia

Childhood absence epilepsy
4-8 years, more common in girls
Brief arrest of speech, activity
Perioral, periocular flickering
Unrousable, rapid recovery
Can be induced by hyperventilation

Juvenile myoclonic epilepsy
12-18 years
Present with first generalised tonic-clonic seizure
Awareness retained during jerks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What are examples of non-epileptic paroxysmal episodes which can occur during sleep?

A

Benign neonatal sleep myoclonus
Myoclonic movements not involving the face
Occur in infants few wks old

Parasomnias
Sleep paralysis
Confusional arousal
Sleep walking
Night terrors
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are examples of non-epileptic paroxysmal episodes which can occur during feeding?

A

GORD/Sandifer syndrome

GORD can cause apnoea and dystonic posturing of the head, neck and back
Associated with feeding is Sandifer syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What are examples of non-epileptic paroxysmal episodes which can occur during fevers?

A

Febrile seizures
Brief, generalised convulsions
6 months to 6 years

Vaso-vagal syncope
In older children
Triggers e.g. intercurrent illness, heat, low food and water intake, prolonged standing

Prodromal pallor, nausea, visual greying, dizziness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What are examples of non-epileptic paroxysmal episodes which can occur due to pain, shock or startles?

A

Reflex asystolic syncope/reflex anoxic seizure:
Sudden unexpected pain or discomfort e.g. headbanging
Child becomes pale, hypotensive, loses consciousness
May have limb stiffening, clonic jerking, vagally mediated

Cyanotic breath holding
End expiratory apnoea following crying

Hyperekplexia:
Whole body stiffening in response to sudden noises, being touched or handled
Severe neonatal form can result in life threatening apnoea - forcible flexion of the neck can terminate it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What are examples of non-epileptic paroxysmal episodes which can occur due to boredom, tiredness, stress?

A
Self gratification behaviour:
Self comforting
Can occur in older children with a neurological disability
Adducted, crossed legs
Unresponsive to interruption

Tics:
Compulsive movements
Gestures
Occur in isolation, or may have vocal tics or Tourette’s

Daydreaming

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What are examples of non-epileptic paroxysmal episodes which can occur due to excitement?

A

Shuddering spells
Excitement from toy or food - provokes shivering as if chilld
Benign

Cataplexy
Strong emotion e.g. laughter provokes sudden loss of tone
Temporary
Brief sagging of the legs
Associated with sleep-wake fragment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What are the types of primary headaches?

A

Migraine
Tension type headache
Cluster headache and other trigeminal autonomic cephalagias
other primary headaches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What are causes of secondary headaches?

A
Headaches due to an underlying pathology
Head or neck trauma
Intracranial haemorrhage
Vascular malformation
Substance or withdrawal
Infection
Disorder of homeostasis
Psychiatric disorder
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What are tension headaches?

A

Mild ache across forehead
Pain or pressure in band like pattern
Come on and resolve gradually

Children become quiet, stop playing, turn pale, tired
Resolve quicker

Triggers include
stress, fear, discomfort 
Skipping meals
dehydration
infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is the management of tension headaches in children?

A

Reassurance
Analgesia
Regular meals
Avoid dehydration, stress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What are the types of migraine?

A
Migraine with aura
Migraine without aura
Silent migraine - migraine with aura but no headache
Hemiplegic migraine
Abdominal migraine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What is an abdominal migraine?

A

May occur in younger children before they develop traditional migraines
Episodes of central abdominal pain lasting more than one hour
Examination normal
May have associated N&V, anorexia, headache, pallor

In child with possible migraines, ask about history of recurrent central abdominal pain as a child

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What may be the presentation of migraine?

A

Unilateral
More severe
Throbbing
Take longer to resolve

Associated with visual aura
Photophobia, phonophobia
Nausea and vomiting
Abdominal pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is the management of migraines in children?

A
Rest, fluids, low stimulus environment
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics e.g. domperidone unless contraindicated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What are options for migraine prophylaxis?

A

Propranolol - but avoid in asthma
Pizotifen - causes drowsiness
Topiramate - girls with child bearing potential need good contraceptive as teratogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What are options for migraine prophylaxis?

A

Propranolol - but avoid in asthma
Pizotifen - causes drowsiness
Topiramate - girls with child bearing potential need good contraceptive as teratogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

How can infections cause a headache in a child?

A

Sinusitis causes headaches with inflammation of ethmoidal, maxillary, frontal or sphenoidal sinuses

Viral URTI, otitis media, sinusitis, tonsillitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

How might a headache suggest a space occupying lesion?

A

Worse when lying down
Morning vomiting
Night time wakening
Change in mood, personality

Visual field defects
Cranial nerve abnormalities
Abducens often affected - squint with diplopia
Abnormal gait
Torticollis - tilting of head
Growth failure
Papilloedema - late feature
Cranial bruits
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What are other causes of funny turns to consider in a child?

A
Cardiac arrhythmia - prolonged QT interval
Non epileptic attack disorder
Pseudoseizures - child feigns seizures
Fabricated
Paroxysmal movement disorders
50
Q

What are the types of muscular dystrophies?

A

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

Duchennes
Beckers
Myotonic dystrophy
Fascioscapulohumeral
Limb-girdle
Emery-Dreifuss
51
Q

What is Gower’s sign?

A

Standing up from a lying position - using hands on their legs to help them stand up
Shift weight backwards
Muscles around the pelvis not strong enough to keep upper body erect without arms

52
Q

What is the management of muscular dystrophy?

A

No curative treatment
Aid quality of life
Occupational therapy, physio, medical appliances e.g. wheelchairs, braces
Management of complications e.g. spinal scoliosis, heart failure

53
Q

What is the inheritance of Duchennes?

A

Defective gene for dystrophin on the X chromosome
X-linked recessive

If mother is carrier with one faulty gene - 50% chance being carrier as female, 50% chance of having condition if male

54
Q

What is the presentation of Duchennes?

A

Waddling gait
and/or language delay
Mount stairs slowly
Psuedohypertrophy of the calves, due to replacement of muscle fibres with fat and fibrous tissue

Progressive muscle atrophy and weakness
No longer ambulant by 10-14
Respiratory failure
Associated cardiomyopathy
Scoliosis
55
Q

What is the management of Duchennes?

A

Exercise to maintain muscle power and mobility
Passive stretching and night splints prevent contractures
Orthoses for walking
Truncal brace for scoliosis
CPAP or NIPPV for nocturnal hypoxia, due to weakness of intercostal muscles
Oral steroids shown to slow progression of weakness
Creatine supplementation

56
Q

What is Beckers muscular dystrophy?

A

Similar to Duchennes
Dystrophin gene less severely affected
Clinical course less predictable, starts later

57
Q

What is myotonic dystrophy?

A
Type of muscular dystrophy
Progressive muscle weakness
Prolonged muscle contractions e.g. unable to let go after shaking hand, cannot release grip
Cataracts
Cardiac arrhythmias

Usually presents in adulthood

58
Q

What is facioscapulohumeral muscular dystrophy?

A
Presents in childhood
Weakness around face
Progresses to shoulders and arms
Sleeping with eyes open
Weakness pursing lips
Cannot blow cheeks out without air leaking
59
Q

What is oculopharyngeal muscular dystrophy?

A
Presents in late adulthood
Weakness of ocular muscles
Weakness around pharynx
Bilateral ptosis
Swallowing problems
60
Q

What are the central movement control centres?

A

Motor cortex in the pre-central gyrus which passes down corticospinal tracts to basal ganglia

Basal ganglia - stores patterns of movement

Cerebellum

61
Q

What are examples of corticospinal tract disorders?

A
Cerebral dysgenesis
Global hypoxia ischaemia
Cerebral tumour
Hemiplegic migraine
Arterial ischaemic stroke
62
Q

What are examples of basal ganglia disorders?

A

Acquired brain injury e.g. CO poisoning, ischaemia, post cardiopulmonary bypass chorea
Wilson disease
Huntington’s

63
Q

What are examples of cerebellar disorders?

A

Medication, drugs
Post viral
Medulloblastoma
Genetic and degenerative disorders e.g. ataxia-telangiectasia

64
Q

How might a child present with a neuromuscular disorder?

A
Weakness - progressive or static
Floppiness
Delayed motor milestones
Muscle weakness
Unsteady-abnormal gait 
Fatiguability
Muscle cramps - metabolic myopathy
65
Q

How may a myopathy present?

A

Waddling gait or positive Gowers

Gowers positive is normal up until the age of 3

66
Q

What are examples of neuromuscular disorders?

A

Disorders of anterior horn:
Poliomyelitis
Spinal muscular atrophy

Disorders of peripheral nerve:
Hereditary motor sensory neuropathies
Acute post-infectious polyneuropathy - Guillan-Barre
Bell’s

Disorder of neuromuscular transmission: myasthenia gravis

Muscle disorders:
Muscle dystrophies
Inflammatory myopathies e.g. benign acute myositis, polymyositis
Myotonic disorders
Metabolic myopathies
Congenital myopathies
67
Q

What are the investigations for a myopathy?

A

Serum creatine phosphokinase elevated in Duchenne and Becker’s, and in inflammatory myopathies

Muscle biopsy
DNA testing
USS or MRI of muscles

68
Q

What are the investigations for a neuropathy?

A

Nerve conduction studies
DNA testing
Nerve biopsy (rare)
EMG - electromyography e.g. shows fatiguability in repetitive nerve stimulation in myasthenia

69
Q

What is spinal muscular atrophy?

A

Rare autosomal recessive condition
Progressive loss of motor neurones and progressive muscular weakness

Affects lower motor neurones in spinal cord - LMN signs:
fasciculations, reduced muscle bulk, reduced tone and power
Reduced or absent reflexes

70
Q

What are the types of SMA?

A

1 - onset in first few months of life, death within 2 years
2 - onset within first 18 months, most never walk, survive to adulthood
3 - onset after first year of life, most walk without support
4 - onset in 20s, retain ability to walk short distances, tasks are tiring, rest muscles and life expectancy not affected

71
Q

What is the management of spinal muscular atrophy?

A

Supportive with MDT
Physio to maximise muscles
Retain resp function
Use of splints, braces, wheelchairs

Resp support with NIPPV to prevent hypoventilation and resp failure, part in sleep

SMA 1 may need tracheostomy and mechanical ventilation

Percutaneous endoscopic gastrostomy PEG tube if weak swallow

72
Q

What are signs of SMA Type 1?

A

Lack of antigravity power in hip flexors
Absent deep tendon reflexes
Intercostal recession
Fasciculation of the tongue

73
Q

What is Guillain Barre syndrome?

A

Acute post infectious polyneuropathy
2-3 weeks after URTI or campylobacter gastroenteritis
Fleeting abnormal sensory symptoms in legs
Ascending symmetrical weakness, loss of reflexes
Involvement of bulbar muscles can lead to difficulty chewing
Difficulty swallowing

Recovery can take up to 2 years
Management supportive
Resp support

74
Q

What is Bell’s palsy?

A

Isolated lower motor neurone paresis of facial nerve
Probably post infectious
Corticosteroids can reduce oedema in first week

Main complication conjunctival infection due to inability to close eye

75
Q

What is a key differential of Bell’s palsy?

A

If symptoms of vestibulocochlear nerve paresis present; due to compressive lesion

Herpes virus can invade geniculate ganglion, cause facial palsy treated with aciclovir

HTN excluded as Bell’s palsy can be associated with coarctation of the aorta

Bilateral facial weakness can be due to sarcoidosis or Lyme disease

76
Q

What is juvenile myasthenia?

A

Similar to adult autoimmune myasthenia
Due to binding of antibodies to acetylcholine receptors on post synaptic membrane

Reduces no. of functional receptors

Presentation usually after 10
Ophthalmoplegia, ptosis
Loss of facial expression
Difficulty chewing
Facial weakness

Diagnosis confirmed with acetylcholine receptor antibody tests or anti muscle specific kinase antibodies.

77
Q

What is the treatment for myasthenia gravis/juvenile myasthenia?

A

Anti-muscarinic drugs neostigmine or pyridostigmine

Immunosuppressive therapy with prednisolone, azathioprine

78
Q

What are metabolic myopathies and causes?

A

Metabolic conditions can affect muscles
Lead to floppy infant
Or child with muscle weakness or cramps on exercise

Glycogen storage disorders
Disorders of lipid metabolism
Mitochondrial cytopathies

79
Q

What is found on examination of a floppy infant?

A

Hang like a rag doll
Marked head lag

Central hypotonia associated with poor truncal tone, but preserved limb tone
Dysmorphic features suggest genetic cause

Lower motor neurone lesions - frog like posture, poor antigravity movements and absent reflexes

80
Q

What are causes of a central hypotonic infant (floppy)?

A

Cortical:
Hypoxic ischaemic encephalopathy
Cortical malformations

Genetics:
Down’s
Prader-Willi

Metabolic:
Hypothyroidism
Hypocalcaemia

81
Q

What are the causes of a peripheral hypotonic infant?

A

Spinal muscular atrophy
Myopathy
Myotonia
Congenital myasthenia

82
Q

What is Friedreich ataxia?

A
Autosomal recessive condition
Presents with worsening ataxia
Distal wasting in legs
Absent lower limb reflexes
Extensor plantar responses due to pyramidal involvement
Pes cavus - high arch
Dysarthria

Kyphoscoliosis and cardiomyopathy can worsen with age, cause death at 40-50

83
Q

What is ataxia telangiectasia?

A

Autosomal recessive
Mild delay in motor development
Oculomotor problems
Incoordination
Difficulty with balance and coordination
Dystonia, cerebellar signs
Telangiectasia in conjunctiva, neck and shoulders

Increased susceptibility to infection
Develop malignant disorders
Raised AFP
Increased white cell sensitivity to irradiation

84
Q

What are causes of cerebellar ataxia?

A

Medication, drugs
Varicella infection
Tumours
Degenerative disorders - ataxic cerebral palsy, Freidreich ataxia, ataxia telangiectasia

85
Q

What is an extradural haemorrhage?

A

Follows direct head trauma

Skull fracture and tearing of middle meningeal artery

86
Q

What is the presentation of an extradural haemorrhage?

A

Lucid interval
Then consciousness drops
Seizures secondary to increasing size of haematoma

Focal neurological signs may be present - dilatation of ipsilateral pupil, paresis of contralateral limbs.
In young child, may be anaemic and in shock

87
Q

What is the diagnosis and management of an extradural haemorrhage?

A

CT scan
Correct hypovolaemia
Urgent evacuation of haematoma, arrest bleeding

88
Q

What is a subdural haematoma?

A

Tearing of veins as they cross subdural space
Retinal haemorrhages present

Characteristic non-accidental injury e.g. shaking or direct trauma

89
Q

What is a subarachnoid haemorrhage and its presentation?

A
Less common in kids
Acute onset of head pain
Neck stiffness
Occasional fever 
Retinal haemorrhage
Seizures and coma may occur
90
Q

What are the investigations and treatment for a subarachnoid haemorrhage?

A

CT scan identifies blood in CSF
LP in acute situation avoided - haemorrhage may then extend following releasing of ICP
Cause often aneurysm or arteriovenous malformation identifiable with MR angiography, CT or conventional angiography

Treatment with neurosurgery or interventional radiography

91
Q

What are causes of rising intracranial pressure?

A
Meningoencephalitis
Head injury
Subdural/extradural bleeds - NAI?
Hypoxia e.g. near drowning 
Ketoacidosis
Tumours
Thrombosis
Reye's
92
Q

What are the sections of children’s coma scale?

A

Best motor response
Best verbal response
Eye opening

93
Q

What are the signs of raised intracranial pressure?

A
Irritable
Drowsy
Headache
Diplopia
Vomiting
Tense fontanelle
Level of responsiveness

If unconscious look for: pupil changes (ipsilateral dilatation) abnormal posturing, Cushing’s triad warns of imminent coning

94
Q

What are the types of abnormal posturing?

A

Decorticate - arms flexed over chest

Decerebrate - arms extended at sides, and head and back arched backward

95
Q

What is Cushing’s triad?

A

Slow pulse, widened pulse pressure
Raised BP
Breathing pattern abnormalities

96
Q

What is the management of rising ICP?

A
Prevent ischaemia - keep head in midline elevated at 25 degrees
Give O2
Treat any hypoglycaemia
Control seizures
Don't do LP - risk of coning
Intubate, dexamethasone 
Fluid restriction and diuresis
97
Q

What is the main rule of visual field defects?

A

Pre-chiasmal lesions cause ipsilateral monocular defect

Post-chiasmal leads to homonymous visual field defects on contralateral side

98
Q

What do lesions at the optic chiasm cause?

A

Bitemporal hemianopia

99
Q

Why is there sparing of the macula in the occipital lobe?

A

As there is dual blood supply in the anterior portion of the visual centre - PCA and MCA
Homonymous hemianopia

100
Q

What are typical lesions of the optic nerve?

A

Optic neuritis
Amaurosis fugax
Trauma
Retrobulbar optic neuropathy

Leads to ipsilateral monocular blindness

101
Q

What are typical lesions of the central portion of the optic chasm?

A

Pituitary adenoma
Suprasellar aneurysm

Leads to bitemporal hemianopia

102
Q

What are typical lesions of the optic tract?

A

MCA stroke
Tumours

Contralateral homonymous hemianopia

103
Q

What are typical lesions of the optic radiations?

A

MCA stroke
Tumour
Trauma

Contralateral homonymous quadrantopis

104
Q

What are causes of acquired spinal cord injury?

A

Trauma
Inflammatory - post infectious, abscesses, tuberculous
Vascular - acute anterior spinal artery infarction - sparing of dorsal cord leads to preservation of dorsal column - vibration, joint position, sensation
Compression - SOL, paraspinal neuroblastoma, syringomyelia

105
Q

What is the acute management of a spinal cord injury?

A
Neurosurgical evaluation
High dose steroids
Ventilatory support for high lesions
Urinary catheterisation, management of constipation
Aggressive contracture prevention
106
Q

What is autonomic dysreflexia?

A

Important problem of complete lesions above T6
Stimuli below this level, e.g. even full bladder and not aware can lead to increased reflex sympathetic activity, HTN, vasoconstriction
This is sensed by CNS above lesion, causing increased vagal tone and bradycardia so vasodilation above lesion leads to pounding headache and sweating

Needs emergency treatment, relief of cause, sit upright

107
Q

What are long term complications of a spinal cord injury?

A
Motor
Weakness, spasticity, contractures
Spinal deformity
Chest infection
Postural abnormalities
Pathological fractures

Sensory
Skin breakdown due to lack of pain sensation from pressure
Scalds, hot weather etc

Sphincters
Incontinence, bladder dysfunction
Unsafe bladder not well managed - renal failure

Emotional
Lack of independence, depression, sexual dysfunction

108
Q

What is syringomyelia?

A

Reversible cause
Expansion of central cavity of cord due to abnormal CSF flow and pressure
Loss of pain, pinprick and temperature

109
Q

What are some causes of vision loss pre-chiasmatic/chiasmatic?

A

Cornea - scarring, vit A deficiency, keratoconus (Trisomy 21)

Lens - cataract
Macula - damage, dystrophy
Retina - trauma, NAI, detached retina, intraocular tumour
Optic nerve - inflammation, optic neuritis, optic atrophy
Compression; raised ICP, trauma, bone disease, infection, hypertension
Ischaemia, optic glioma

Chiasm - tumour

110
Q

What are examples of neural tube defects?

A

Anencephaly - failure of most of cranium and brain
Encephalocele - extrusion of brain and meninges through midline skull defect

Spina bifida:
Spina bifida occulta - vertebral arch does not fuse properly, underlying tethering of cord causes neurological defects
Meningocele - sac of fluid, meninges herniate but nervous system remains undamaged
Myelomeningocele - spinal cord protrudes out as well as meninges containing it

111
Q

What problems is myelomeningoceles associated with?

A
Variable paralysis
Muscle imbalance
Dislocation of hips, talipes
Sensory loss
Bladder dernervation
Bowel denervation 
Scoliosis
Hydrocephalus
112
Q

What is seen in spina bifida occulta?

A

Associated overlying skin lesion
Tuft of hair, lipoma, birth mark
Small dermal sinus

113
Q

What is the criteria for immediate request of CT head in children?

A

LOC more than 5 mins
Amnesia more than 5 mins
Abnormal drowsiness
Three or more discrete episodes of vomiting
Clinical suspicion of NAI
Post traumatic seizure but no hx of epilepsy
GCS <14 or if less than 1 <16
Suspicion of open or depressed skull injury, tense fontanelle
Any sign of basal skull fracture
Focal neurological deficit
Presence of bruise, swelling, laceration >5cm on head if less than 1 year
Dangerous mechanism of injury

114
Q

What are some of the causes of microcephaly?

A
Normal variation, familial
Congenital infection
Perinatal brain injury e.g. hypoxic ischaemic encephalopathy 
Fetal alcohol syndrome
Patau's
Craniosynostosis
115
Q

What is hydrocephalus?

A

CSF building up abnormally within the brain and spinal cord

Either due to overproduction or problem draining and absorbing

116
Q

What is normal CSF physiology?

A

Four ventricles - two lateral, third and fourth
Ventricles contain CSF
Created in four choroid plexuses passes through to arachnoid granulations where it is absorbed

117
Q

What are congenital causes of hydrocephalus?

A

Aqueductal stenosis - stenosed connection in cerebral aqueduct between third and fourth ventricle so there is a build up in third and lateral ventricles

Arachnoid cysts can block outflow
Arnold-Chiari malformation, seen in spina bifida - cerebellum herniates downwards towards foramen magnum causing blockage
Chromosomal abnormalities and congenital malformations

118
Q

What is the presentation of hydrocephalus?

A

Outward pressure on cranial bones if sutures not fused
They fuse at 2 years
Enlarged and rapidly increasing head circumference

Bulging anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleepiness

119
Q

What is a VP shunt?

A

Drains CSF from ventricles into another body cavity

Usually peritoneal cavity as plenty of space, easily reabsorbed

120
Q

What are the complications of a VP shunt?

A

Infection
Blockage
Excessive drainage
Intraventricular haemorrhage during shunt related surgery
Outgrowing them - typically need replacing every 2 years