Neurology Flashcards

1
Q

When might you see febrile convulsions in a child?

A

6 months to 5 years

FHx

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2
Q

How are febrile convulsions treated?

A

Paracetamol/ibuprofen

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3
Q

What are the differentials for febrile convulsions?

A

Rigors

Intracranial infections

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4
Q

What do infantile spasms look like on EEG?

A

Hyposarrythmia

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5
Q

What can infantile spasm cause?

A

Developmental delay

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6
Q

What is the most common cause of infantile spasm?

A

Tuberous sclerosis

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7
Q

What others things should you look for in infantile spasm?

A

Dysmorphism

Skin changes

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8
Q

How is infantile spasm treated?

A

Vigabatrin

Prednisolon

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9
Q

What do myoclonic jerks mean in children?

A

Often benign

No treatment

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10
Q

What is a reflex anoxic seizure?

A
Triggered by pain/strong emotion
Results in decreased perfusion to brain
ECG - ?arrhythmia
EEG - normal
Most will grow out of it
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11
Q

What is juvenile myoclonic epilepsy?

A

Myoclonic jerks
Absence seizures
Worse when tired
Treatment - sodium valproate NOT carbamazepine

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12
Q

How is status epilepticus treated?

A
ABCDE
5 mins - benzodiazepine
10 mins later - further benzodiazepine eg IV lorazepam
Prepare PR paraldehyde
Prep phenytoin
Call for senior support 
10 mins later - IV phenytoin
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13
Q

How do you take a neurological history and what questions should you ask?

A

From parent/carer (but don’t forget child)
If for funny turns need proper witness description
Thorough history helps plan examination
Family history (neurogenetic/neurometabolic)
Birth history - prenatal, perinatal and postnatal (25% referrals for developmental impairment)
Developmental milestones
- Need to know if there is developmental delay/developmental regression)
- Normal for one age may be abnormal for another age
- Reflection of maturation of child’s nervous system
- Delay and abnormal pattern are indicators of underlying neurological diseases

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14
Q

What are the key principles in neurodevelopment?

A

Motor development proceeds head to toe fashion
Primitive reflexes are normally present in the term infant and diminish over the next 4 to 6 months of life
Postural reflexes emerge at 3 to 8 months of life
Persistence of primitive reflexes and lack of development of postural reflexes are hallmark of UMN abnormality of infant

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15
Q

Why do we do head circumference in babies?

A
Accurate reflection of brain size and development
Serial important so can plot them on graph
Small head (microcephaly) or a large head (macrocephaly or hydrocephalus) can be key findings in explaining the neurological abnormalities of a child
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16
Q

What are the different types of abnormal head shape (sysnostosis)?

A

Trigonocephaly - fusion of metopic suture - pointed shaped forehead
Brachycephaly - fusion of coronal suture - flat back of head
Solichocephaly/scaphocephaly - fusion of sagittal suture - AP breadth of skull much bigger than lateral breadth - seen in extreme preterm
Plagiocephaly - unilateral premature closure of lambdoid and coronal sutures - flattening of sutures
Oxycephaly - fusion of coronal and lambdoidal sutures - tower skull

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17
Q

What is Gower’s sign?

A

Hip girdle weakness, when asked to rise from prone position patient uses hands to walk up legs to compensate for proximal lower leg extremity

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18
Q

What should you do in a general physical examination in neurology?

A

Somatic growth - measure height and weight, compare percentiles with head circumference
Skin search - stigmata of neurocutaneous syndromes eg cafe au lait spot, ash leaf macule, iris lisch nodule, port wine stain, adenoma sebaceum, neurofibroma
Dysmorphic features - look especially at the midface, face reflects the brain, anomalies of the midface often associated with underlying brain malformations
- Facial measurements can be done to determine these
- Williams syndrome, Angelman syndrome, Rett syndrome, Smith-Magenis syndrome
Eyes - can be difficult due to poor concentration, retina is window to brain, fundoscopy

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19
Q

What should you do in a neurological examination to make it easier for you and the child?

A
Stop look listen
- Don't cooperate for standard neuro examination
- Tailor to child's age
- Learn more by hands off observation
- Mental status, cranial nerve examination, co-ordination by watching spontaneous activity
Make it a game
- Engage curiosity and imagination
- Less threatening and child more cooperative when toys used
- Not much gained testing power and reflexes
Save the worst for last
- Undressing child
- Looking at fundus
- Using auroscope
- Testing gag reflex (if essential)
- Measuring head circumference
Cranial nerves including eye movements
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20
Q

What eye movement problems can you see in children?

A
Paroxysmal tonic upgaze
Opsoclonus myoclonus syndrome
Tick disorders
Nystagmus
Horner's syndrome
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21
Q

What is paroxysmal tonic upgaze?

A

Initially described as benign phenomenon with negative investigations and eventual complete resolution of symptoms
Similar clinical picture may arise from structural brain lesions, channelopathies, neurotransmitter disorders, and epileptic seizures
CACNA1A related disorders - spectrum of episodic ataxia 2, hemiplegic migraine, benign paroxysmal torticollis of infancy, and paroxysmal vertigo

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22
Q

What is opsoclonus myoclonus syndrome?

A

Associated with neuroblastoma
Eyes flick in all different directions
Also get myoclonus

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23
Q

What should you do with someone with nystagmus and why?

A

CT scan to make sure no cerebellar lesion

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24
Q

What is palatal myoclonus?

A

Rhythmic involuntary jerking movement of soft palate and pharyngopalatine arch
Surprisingly little effect on swallowing

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25
Q

How is palatal myoclonus treated?

A

Drugs used - clonazepam, carbamazepine, baclofen, anticholinergics, tetrabenazine, valproic acid, phenytoin, lamotrigine
Botulinum toxin but caution as spread of toxin may cause dysphagia and other problems

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26
Q

What are 4 common neurological problems?

A

Seizures
Syncope
Movement disorders
Cerebral palsy

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27
Q

Name 3 common types of seizure in children

A

Atonic seizures
Absence seizures
GTCS
Frontal lobe seizures - can progress to tonic-clonic

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28
Q

Name 3 common types of movement disorders in children

A
Hyperekplexia 
Chorea
Shuddering
Sleep myoclonus
Tremors
Motor tics
Motor stereotypy
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29
Q

What is hyperekplexia?

A

Tonic phase after shocking baby, baby will stop breathing, flex neck will abort attack, genetic condition

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30
Q

What is motor stereotypy associated with?

A

ASD

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31
Q

What is cerebral palsy?

A

Umbrella terms covering group of motor impairment syndromes secondary to non progressive lesions or anomalies in the brain arising in the early stages of development
In most cases anomaly occurs pre or perinatally

32
Q

What nutrition concerns are there in children with disabilities?

A

10-50% severely impaired children may be undernourished
Growth retardation due to inadequate intake as a result of self-feeding inadequacy and/or oromotor coordination difficulties
On the whole, the more severe disability the more severe the feeding difficulties and growth retardation

33
Q

Name 2 consequences of undernutrition?

A

Poor growth with reduced muscle strength
Poor circulation due to reduced activity
Increased susceptibility to infection

34
Q

What can cause feeding difficulties?

A

Oral motor dysfunction
Disrupted sensitive period
Dystonia
- Extensor patterns, limiting oral movements becoming stereotyped and abnormal
- Tactile hypersensitivity may be greatest around mouth Postural deformity - control of head/trunk/compression abdomen
Drug treatment - baclofen/diazepam/tone/sedation/anticonvulsants/sedation, effect of appetite
Impaired hand function
Immobility
Vision impairment
Deafness
GORD - pain/irritability, Sandifer syndrome

35
Q

When do stereotypies present?

A

< 2 years

36
Q

What do stereotypies look like?

A
Fixed, identical, foreseeable
Arm-hands, wavelike, fluttering, jiggling
Rhythmic
Intermittent, repeated, prolonged
No pre-movement sensorimotor phenomena
Triggered by excitement, stress
Suppress by distraction, seldom conscious effort
May be positive family history
Rarely responsive to medication
37
Q

When might tics develop?

A

6-7 years

38
Q

What do tics look like?

A
Variable pattern
Blinking, grimacing, warping, jerking
Quick, sudden, aimless, but not rhythmic
Intermittent, short, abrupt
Pre-movement sensorimotor phenomena
Triggered by excitement/stress
Self-directed, short suppressibility
Often positive family history
Primary neuroleptic treatment
39
Q

How would you manage a child presenting with DKA in shock?

A
ABCDE
Consider intubation
Blood gases
20ml/kg bolus of 0.9% NaCl over 10-15mins
Manage in resus
40
Q

What fluids would you give to a child presenting with DKA who is NOT in shock?

A

10ml/kg over 60mins

41
Q

What fluids would you give someone with DKA over the next 48 hours?

A

0.9% NaCl
Dehydration correction over 48 hours minus bolus
Maintenance over 24 hours
Give insulin after fluids for an hour

42
Q

How can you treat cerebral oedema?

A

3% NaCl

43
Q

Name 3 differentials for a seizure

A
Febrile convulsion
Epilepsy
Meningoencephalitis
Tumour
Metabolic - hypoglycaemia
Head injury
NEAD
44
Q

What can cause non-epileptic seizures?

A

Organic - infection, hypoglycaemia, tumour, trauma

Non-organic - NEAD

45
Q

What is a febrile convulsion?

A

Seizure in a febrile child with the absence of CNS infection, metabolic imbalance, or other neurological cause

46
Q

How are febrile convulsions classified?

A

Simple < 15 mins
Complex > 15 mins
Status > 30 mins

47
Q

What are the potential risk factors of febrile convulsions?

A

FHx

Viral infection

48
Q

What does a febrile convulsion look like?

A

Generalised tonic-clonic seizure lasting < 5 mins, rapid and full neurological recovery

49
Q

What are the rules of 6 for febrile convulsions?

A

Usually occur in 6 months - 6 year olds
Occur in 6% of pre-school children
1/6 chance of recurrence after 1 episode of febrile convulsion

50
Q

What education should you give to parents about seizures?

A

How to manage seizure
Call ambulance if seizure lasts > 5 mins
When to admit
Risk of epilepsy

51
Q

When should you admit a child with seizures/

A
First seizure
Seizure recurring within the same febrile illness
Incomplete recovery after 1 hr
Suspected serious infection
< 18 months
Anxious parent
52
Q

What is the risk of epilepsy in children with febrile seizures?

A

2% risk
(1% in child w/o febrile seizures)
Complex seizures have higher risk

53
Q

What is West syndrome/

A

Salaam attacks
Hypsarrhythmia on EEG
Developmental delay

54
Q

What is childhood absence epilepsy?

A

5-20 second episode of arrest of movement and awareness - blankly starring into space
Can have automatism
No developmental delay but impaired learning due to multiple episodes during the day
Spontaneous remission in adolescent

55
Q

What differentials can you have for seizures in childhood?

A

Myoclonic jerks during sleep in babies (normal)
Jittering - can be normal but check BM
Breath holding attacks - when child gets angry or frustrated they can hold their breath and go cyanotic and lose consciousness
Reflex anoxic seizures - pain, minor head trauma can trigger a vaso-vagal where the child gets a short episode of asystole or bradycardia and goes pale and faint and has seizure activity with spontaneous resolution

56
Q

Name 3 viruses that can cause meningitis in children

A
Enteroviruses
EBV
CMV
VZV
Adenovirus
Herpes simplex - from birth canal
57
Q

What bacterias can cause meningitis in children?

A

< 3 months - listeria, E coli, GBS

> 3 months - N meningitidis, strep pneumo, HiB

58
Q

What can cause meningitis in immunocompromised people?

A

Fungal - cryptococcus

59
Q

How do you manage meningitis?

A

3rd gen cephalosporin IV
Resuscitate
Add amox in < 3 months to cover listeria
Report to PHE

60
Q

What are the complications of meningitis?

A
Sepsis
DIC
SIADH
Hydrocephalus
Hearing loss - all children get hearing tests after 6 weeks
LD
61
Q

What is cerebral palsy?

A

Disorder of movement and posture
Due to non-progressive lesion of the motor pathways in the developing brain
First 2 years of life - after 2 years it’s termed ABI

62
Q

What can cause cerebral palsy antenatally?

A
Congenital infections (TORCH)
Radiation
IVH
Metabolic
Ischaemic
Periventricular leukomalacia
63
Q

What can cause cerebral palsy intrapartum?

A

Birth asphyxia

64
Q

What can cause cerebral palsy post-natally?

A

Neonate - IVH, meningitis, head trauma, meconium aspiration, hypogylcaemia, jaundice (kernicterus)
Infant - hydrocephalus, hypoglycaemia, head injury - NAI, CNS infection

65
Q

What are the TORCH organisms?

A
Toxoplasmosis
Other - VZV, parvovirus B19, syphilis, listeria
Rubella
CMV
HSV
66
Q

How is cerebral palsy classified?

A

Spastic/pyramidal - cortex UMN - tremors, hypertonicity, brisk reflexes, scissor gait
Dyskinetic/extrapyramidal - basal ganglia - involuntary movements - chorea, dystonia, athetosis
Ataxic - cerebellum - broad based gait
Mixed

67
Q

What is the most common type of cerebral palsy?

A

Spastic/pyramidal

68
Q

What do you get in spastic hemiplegic CP?

A

Tiptoe gait
Circumduction gait
Arm bent - hand spastic or floppy, often of little use
Other side almost completely normal

69
Q

What do you get with diplegic/paraplegic spastic CP?

A

Both legs with slight involvement elsewhere
Upper body normal or with minor signs
Child may develop contractures of ankles and feet

70
Q

What do you get with quadriplegia?

A

Child may develop contractures of ankles and feet
When walks arms, head, and mouth may twist strangely
Children 4 limbs affected often have such severe brain damage that they are never able to walk
Knees press together
Legs and feet turned inward

71
Q

What happens in a dyskinetic baby?

A

Initially baby hypotonic with poor head control

Increased tone and dyskinetic movements develop

72
Q

What is dystonia?

A

Involuntary, sustained contraction of opposing muscles leading to abnormal posture, twisting repetitive movements

73
Q

What is athetosis?

A

Slow involuntary writhing movements

74
Q

What is chorea?

A

Irregular, non-repetitive dance like movements

75
Q

What non-motor features can you get in CP?

A
Epilepsy
Swallowing problems - bulbar palsy
GOR
Hearing and visual - strabismus
Communication problems
Bladder/bowel problems
Behaviour difficulties
MSK - scoliosis, contractures, hip dislocation
76
Q

How is CP diagnosed?

A

MRI
Chromosome testing
Metabolic workup

77
Q

How is CP managed?

A

MDT!!
- Nutrition - faltering growth so may need NG/PEG
SALT - assess swallow
OT and PT - promote mobility and physical activity to prevent contractures
Adaptive equipment -walkers, splints, wheelchairs
Special needs education
Antimuscarinics to prevent drooling
Anti-reflux medication
Anti-spasmodics
Bisphosphonates - prevent osteoporosis
Surgery - achilles tendon release, fix scoliosis