neurology Flashcards

(61 cards)

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
WHat skin lesions may you see in tuberous sclerosis
``` cafe au lait spots shagreen patches hypo-pigmented as leaf patches adenoma sebacum - acne like rash subungal/periungal fibromatoma ```
26
What are the most common CNS lesions in tuberous sclerosis
cortical tumours | subependymal nodules
27
What neuroimaging is reocmmended in patients with tuberous sclerorsis
MRI every 1-3 years until the age of 25
28
What is Landau Kleffner Syndrome
subacute onsent of aphasia and seizure activity (usually focal) with abnormal EEG Usually between 3-7 treated as an epileptic disorder
29
How do benzodiazepines terminate seizures
They enhance the effect of GABA on GABA receptors leading to neural inhibition
30
Why is buccal medicine accepted to terminate a seizure
avoids first pass hepatic metabolism
31
How do you caclultate the dose of benzodiazepines as a rescue med
by age | Age 5-9 is 7.5mg
32
What advice should be given to parents about seizures
- 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
What is the AED of Choice for absence seizures
ethosuximide or sodium valproate (boy only)
34
What is the AED of Choice for focal seizures
carbamazepine or lamotrigine
35
What is the AED of Choice for generalised tonic clonic
sodium valproate | Lamotrogine if sodium valproate inappropriate
36
What is the AED of Choice for myoclonic seizures
- sodium valrpoate | - levetiracetam or topiramate
37
What is Dravets syndrome
- severe prolonged seizures triggered by high body temp - drug resistant - start in first year of life - associated with learning difficulties etc and autism
38
What is the management of Dravets syndrome
toperimate/sodium valproate
39
Diagnosis criteria of Lennox-Gasteau
- multiple generalized seizure types - a slow spike-and-wave pattern (less than 2.5 Hz) on EEG - cognitive dysfunction
40
How does juvenile myoclonic epilepsy present
- sudden brief bilateral jerks in morning. conciousness unimpaired - few years later generalised tonic clonic seizure
41
what is the management of juvenile myoclonic epilepsy
lamotrigine | valproate
42
Benign epilepsy of childhood with centrotemporal spikes
- brief, simple partial and hemifacial motor seizures with somatosensory symptoms - often evolve into tonic clonic
43
complex partial seizures
absences, lip smacking and repetitive movements
44
Causes of raised ICP
- traumatic brain injury - hydrocephalus - brain tumours - intracranial infections - hepatic encephalopathy - impaired CNS outflow
45
Causes of cerebral oedema
- Head trauma - tumour - hydrocephallus - hypoxic/ischaemix encephalopathy - infectious - DKA - stroke - venous thrombosis - AV malformations - vasculitis
46
Features of acutely raised ICP
- headache - vomiting - altered mental state - papilloedema - HTN with brady/tachycardia - seizures
47
Features of chronically elevated ICP
- Headache - vomiting - Parinaud syndrome - abnormalities with vertical gaze - Visual changes - papiloedema - neurology e.g ataxia
48
What is the initial investigations of choice if ? raised ICP
CT without contrast | MRI
49
What features on CT suggest raised ICP
- midline shift - effacement of basilar cisterns - effacement of the sulci - Thumb printing - increase gyral marking on inner table of the skull.
50
Key features of medulloblastoma
``` headaches early morning vomiting unsteadiness 6th nerve palsy (posterior fossa tumour) ```
51
Key features of neurofibromatosis 1
``` 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
What skeletal anomalies may you seen in neurofibromatosis
leg bowing | sphenoid dysplasia
53
What is neurofibromatosis 1
- genetic condition causing benign tumours to grow along nerves - usually presents by age 1
54
Where is chromosomal anomaly in neurofibromatosis 1
NF1 on Cr 17
55
How does neurosarcoidosis present
rash uveitis arithitis neuro involvement is rare in children
56
What is the management of Benign epilepsy of childhood with centrotemporal spikes
- No AED - If frequent seizures - carbmazepine or lamotrigine - until 14-16yrs or 2 years seizure free
57
First line agent in neonatal seizures
phenobarbitone
58
Features of WIlson's disease
``` 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
Management of Wilsons'
chelating agents - penacillamine, zine trientine
60
WHat is neuroancyanthosis
- group of conditions with mishapen spiny red blood cells + neurological anomalies.
61
Features of neuroancyanthosis
chorea | can begin of childhood to adulthood and can progress and different speeds