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Paediatrics > neurology > Flashcards

neurology Flashcards

1
Q

What investigations should you complete a neonate with seizures of unknown cause

A 
glucose/calcium/Mg/U&Es and acid/base status
FBC and film
CSF analysis
blood, urine and CSF cultures
cranial US
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2
Q

What additional investigations may you consider once the standard seizure investigations are done

A 
ammonia
lactate
uric acid
liver enzymes
biotinidase levels
blood and urine amino acids
Torch screen
MRI brain
EEG
genetic testing
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3
Q

What are the most common bacterial causes of meningitis in neonates

A

group B streptococcus
E. Coli
Listeria monocytogenes

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4
Q

What is the most common cause of neonatal encephalitis

A

vertical transmission of herpes simplex virus

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5
Q

How would you diagnose bacterial meningitis on CSF

A

isolation of the bacterium
increased WCC
Raised protein
decreased glucose

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6
Q

What does an infantile seizure (west syndrome) present as

A

sudden, tonic clonic contractions lasting 5-10 seconds with spasms often occurring in quick succession

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7
Q

When does West syndrome usually occur

A

4-8 months

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8
Q

How does West syndrome present

A

infantile spasms, often when child is waking up

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9
Q

Who is at risk of developing West syndrome

A
  • previous brain injury <6 months
  • brain malformation
  • genetic abnormalities
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10
Q

What is the management of West syndrome (infantile spasm)

A
  1. prednisilone or vibigatran
  2. the other of the above
  3. pyroxidine
  4. AEDs
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11
Q

What type of spasms do you see in West syndrome

A

Both extensor and flexor spasms

  • Extensor: extend neck and trunk and abduct limbs
  • flexor - ‘self hugging’
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12
Q

What would you see on EEG in West Syndrome (infantile spasms)

A

Hypsarrythmia - chaotic high to very high voltage polymorphic EEG

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13
Q

What are the common causes of seizures in neonates

A
  • hypoxic ischaemic
  • encephalopathy
  • intracranial haemorrhage
  • intracranial infections
  • congenital cerebral malformations
  • metabolic disorders
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14
Q

What investigations should you do if you feel there is an underlying metabolic disorder causing seizures

A 
glucose
electrolyte
magnesium
calcium
metabolic screen (urine and plasma)
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15
Q

What is the most common cause of seizures in a term infant

A

hypoxic ischaemic encephalopathy

Usually occurs in the first 24-48 hours of life

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16
Q

What is the most common cause of seizures in a pre-term infant

A

intra-cranial haemorrhage - Cranial US

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17
Q

What are the differential diagnoses of seizures in a neonate

A

jitteriness + benign neonatal sleep myoclonus

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18
Q

How can you tell between true epileptiform movements and non-epileptiform

A

stopped by gentle restraint and can be reproduced by sensory stimuli

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19
Q

What is Benign neonatal sleep myoclonus

A
  • bilateral or localised myoclonic jerks only in sleep,
  • consistent cessation with arousal
  • Normal EEG
  • normal neurological examination,
  • good outcome
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20
Q

Feautires of jitteriness

A

movements are stimuli sensitive
movements stop with gentle constraint
predominantly a tremor as opposed to tonic clonic
No CVS changes or eye movements

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21
Q

Feautires of jitteriness

A

movements are stimuli sensitive
movements stop with gentle constraint
predominantly a tremor as opposed to tonic clonic
No CVS changes or eye movements

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22
Q

Most common presentation of tuberous sclerosis

A

epilepsy (infantile spasms)

  • autism
  • cognitive impairment
  • neonatal cardiac rhabdomyomas
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23
Q

What is tuberous scelerosis

A

autosomal-dominant, neurocutaneous, multi-system disorder characterised by cellular hyperplasia, tissue dysplasia, and multiple organ hamartomas

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24
Q

What is a reflex anoxic seizure (reflex asystolic syncope)

A

Occurs from early infancy and onwards

unpleasant, sudden stimulus leads to a profound vagal discharge resulting in a fast drop in HR and transient asystole.

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25
Q

WHat skin lesions may you see in tuberous sclerosis

A 
cafe au lait spots
shagreen patches
hypo-pigmented as leaf patches
adenoma sebacum - acne like rash
subungal/periungal fibromatoma
26
Q

What are the most common CNS lesions in tuberous sclerosis

A

cortical tumours

subependymal nodules

27
Q

What neuroimaging is reocmmended in patients with tuberous sclerorsis

A

MRI every 1-3 years until the age of 25

28
Q

What is Landau Kleffner Syndrome

A

subacute onsent of aphasia and seizure activity (usually focal) with abnormal EEG
Usually between 3-7
treated as an epileptic disorder

29
Q

How do benzodiazepines terminate seizures

A

They enhance the effect of GABA on GABA receptors leading to neural inhibition

30
Q

Why is buccal medicine accepted to terminate a seizure

A

avoids first pass hepatic metabolism

31
Q

How do you caclultate the dose of benzodiazepines as a rescue med

A

by age

Age 5-9 is 7.5mg

32
Q

What advice should be given to parents about seizures

A
  • wait 5 mins before rescue meds
  • 999 if not slowed/stopped in 5 mins
  • 999 if unable to give meds
  • 999 it stops breathing/difficulty breathing
  • ONLY give ONE dose at home
33
Q

What is the AED of Choice for absence seizures

A

ethosuximide or sodium valproate (boy only)

34
Q

What is the AED of Choice for focal seizures

A

carbamazepine or lamotrigine

35
Q

What is the AED of Choice for generalised tonic clonic

A

sodium valproate

Lamotrogine if sodium valproate inappropriate

36
Q

What is the AED of Choice for myoclonic seizures

A
  • sodium valrpoate

- levetiracetam or topiramate

37
Q

What is Dravets syndrome

A
  • severe prolonged seizures triggered by high body temp
  • drug resistant
  • start in first year of life
  • associated with learning difficulties etc and autism
38
Q

What is the management of Dravets syndrome

A

toperimate/sodium valproate

39
Q

Diagnosis criteria of Lennox-Gasteau

A
  • multiple generalized seizure types
  • a slow spike-and-wave pattern (less than 2.5 Hz) on EEG
  • cognitive dysfunction
40
Q

How does juvenile myoclonic epilepsy present

A
  • sudden brief bilateral jerks in morning. conciousness unimpaired
  • few years later generalised tonic clonic seizure
41
Q

what is the management of juvenile myoclonic epilepsy

A

lamotrigine

valproate

42
Q

Benign epilepsy of childhood with centrotemporal spikes

A
  • brief, simple partial and hemifacial motor seizures with somatosensory symptoms
  • often evolve into tonic clonic
43
Q

complex partial seizures

A

absences, lip smacking and repetitive movements

44
Q

Causes of raised ICP

A
  • traumatic brain injury
  • hydrocephalus
  • brain tumours
  • intracranial infections
  • hepatic encephalopathy
  • impaired CNS outflow
45
Q

Causes of cerebral oedema

A
  • Head trauma
  • tumour
  • hydrocephallus
  • hypoxic/ischaemix encephalopathy
  • infectious
  • DKA
  • stroke
  • venous thrombosis
  • AV malformations
  • vasculitis
46
Q

Features of acutely raised ICP

A
  • headache
  • vomiting
  • altered mental state
  • papilloedema
  • HTN with brady/tachycardia
  • seizures
47
Q

Features of chronically elevated ICP

A
  • Headache
  • vomiting
  • Parinaud syndrome - abnormalities with vertical gaze
  • Visual changes
  • papiloedema
  • neurology e.g ataxia
48
Q

What is the initial investigations of choice if ? raised ICP

A

CT without contrast

MRI

49
Q

What features on CT suggest raised ICP

A
  • midline shift
  • effacement of basilar cisterns
  • effacement of the sulci
  • Thumb printing - increase gyral marking on inner table of the skull.
50
Q

Key features of medulloblastoma

A 
headaches
early morning vomiting
unsteadiness
6th nerve palsy
(posterior fossa tumour)
51
Q

Key features of neurofibromatosis 1

A 
C - cafe au lait spot >5
A - axillary freckling
F - fibromas (2 or more)
E - Eye - lisch nodules
S - skeletal anomalies
P - Positive FH
OT - Optic tumour - gliomas
(learning difficulties)
52
Q

What skeletal anomalies may you seen in neurofibromatosis

A

leg bowing

sphenoid dysplasia

53
Q

What is neurofibromatosis 1

A
  • genetic condition causing benign tumours to grow along nerves
  • usually presents by age 1
54
Q

Where is chromosomal anomaly in neurofibromatosis 1

A

NF1 on Cr 17

55
Q

How does neurosarcoidosis present

A

rash
uveitis
arithitis
neuro involvement is rare in children

56
Q

What is the management of Benign epilepsy of childhood with centrotemporal spikes

A
  • No AED
  • If frequent seizures - carbmazepine or lamotrigine
  • until 14-16yrs or 2 years seizure free
57
Q

First line agent in neonatal seizures

A

phenobarbitone

58
Q

Features of WIlson’s disease

A 
Jaundice
tremor
dysphagia
dysarthria
poor co-ordination
lethargy (anaemia 2ry to liver dysfunction)
easy bruising
Kaiser Fleisher rings (copper rings)
psychiatric manifestations
menstrual irregularities
59
Q

Management of Wilsons’

A

chelating agents - penacillamine, zine trientine

60
Q

WHat is neuroancyanthosis

A
  • group of conditions with mishapen spiny red blood cells + neurological anomalies.
61
Q

Features of neuroancyanthosis

A

chorea

can begin of childhood to adulthood and can progress and different speeds

Paediatrics (21 decks)

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