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Paeds FOP/TAS > Neuro > Flashcards

Neuro Flashcards

1
Q

1st line medications to treat absence seizures

A

1) Ethosuximide
2) Sodium valporate

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2
Q

EEG findings in absence / petit mal seizures

A

Regular, symmetrical, generalised 3Hz spike and wave complexes of 3 cycles per second

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3
Q

Define encephalitis

A

Inflammation of the brain parenchyma

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4
Q

Most common cause of encephalitis

A

Viral (HSV, influenza, adenovirus, enterovirus, arbovirus)
- HSV most common (90% cases HSV-1, 10% HSV-2)
- CMV should be considered in the immunocompromised

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5
Q

Area of the brain most commonly affected in HSV encephalitis?

A

Medial temporal and inferior frontal lobes (in adults and children >3 months)

Generalised brain involvement in neonates

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6
Q

Causes of Encephalitis

A

Viral:
- HSV, influenza, adenovirus, enterovirus, arbovirus)
- HSV most common (90% cases HSV-1, 10% HSV-2)
- CMV should be considered in the immunocompromised

Bacterial:
- Lyme disease
- TB
- Listeria
- Legionella
- Syphilis

Parasites:
- Toxoplasmosis

Funagl:
- Candida
- Histoplasmosis

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7
Q

What infections can cause a secondary encephalitis?

A

Chickenpox
Measles
Mumps
Rubella
Flu A or B
EBV
Hep A or B
HIV
Enterovirus
Coronavirus

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8
Q

How does a secondary encephalitis present?

A

1-3 weeks post illness

Usually presents as an acute disseminated encephalomyelitis

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9
Q

x3 routes by which HSV infiltrates the CNS in HSE

A

1) Trigeminal or Olfactory tracts

2) CNS activation after recurrent infection

3) Latent HSV: CNS infection without a primary or recurrent infection

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10
Q

Pathophysiology of Herpes Simplex Encephalitis

A

HSV invades and replicates in neurones and glia, causing necrotising encephalitis with widespread haemorrhagic necrosis.

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11
Q

Duration of treatment for confirmed HSE?

A

14-21 days of IV Aciclovir (then needs repeat LP to confirm CSF is negative)

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12
Q

Drug of choice for CMV encephalitis?

A

Ganciclovir

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13
Q

What is the only indication to use corticosteroids in the treatment of encephalitis?

A

Encephalitis due to an immunologic reaction (e.g. Autoimmune encephalitis)
- Corticosteroids
- Plasma exchange or IVIG

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14
Q

Mortality rate of HSE?

A

70% in untreated HSE (often fatal within 7-14 days)

19% in treated HSE

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15
Q

Define meningoencephalitis

A

Inflammation of meninges and brain

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16
Q

Define myeloencephalitis

A

Inflammation of spinal cord and brain

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17
Q

Why should you use cefotaxime instead of Ceftriaxone to treat suspected meningitis in children <3 months

A

Ceftriaxone displaces bilirubin from albumin and can worsen jaundice

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18
Q

First line Abx choice for suspected bacterial meningitis?

A

> 3 months:
- Ceftriaxone

<3 months:
- Amoxicillin (listeria cover) and Cefotaxime

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19
Q

Contraindications for using corticosteroids (Dexamethasone) to treat severe bacterial meningitis?

A

<3 months
TB meningitis
>12 hours lapsed since first dose of Abx

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20
Q

When to follow up bacterial meningitis and what Ix should be done?

A

4-6 weeks post discharge

They all need a hearing test - high risk of sensorineural hearing loss and may need a cochlear implant

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21
Q

Define meningitis

A

Inflammation of the meninges caused by penetration of the blood-brain barrier and proliferation in the CSF by a particular pathogen

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22
Q

Most common form of meningitis

A

Viral meningitis (accounts for >50% cases)

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23
Q

Who is at the greatest risk of a bacterial meningitis?

A

Infants

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24
Q

What is the annual incidence of bacterial meningitis?

A

1-2 per 100,000

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25
Q

Risk factors for meningitis?

A

Immunosuppressed
CSF shunts/surgery
Renal or adrenal insufficiency
Thalassaemia
Cystic Fibrosis
Croweded living conditions
UNVACCINATED children

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26
Q

Common viral causes of meningitis

A

Enterovirus (most common)
Coxsackie virus
Echo virus
Measles
Mumps
HSV
VZV

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27
Q

Bacterial causes of meningitis

A

Neonates / <1 month old:
- Group B Strep
- E Coli
- Listeria
- Staph aureus

First 2 months of age:
- Group B Strep
- E Coli
- H influenzae
- Strep pneumoniae

Older infants / children:
- Meningococcus
- H influenzae
- Strep pneumoniae

TB is a risk at any age

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28
Q

LP findings in viral meningitis

A

AWPG

Appearance - clear
WCC - raised (lymphocytes)
Protein - normal or slightly raised
Glucose - normal or slightly reduced

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29
Q

LP findings in bacterial meningitis

A

AWPG

Appearance - Turbid
WCC - raised, usually polymorphs (typically >90%)
Protein - raised
Glucose - low (<40% serum glucose)

Same for TB although appearance can be turbid, viscous or clear

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30
Q

What is a normal CSF glucose level?

A

> 50% blood glucose ratio

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31
Q

Which infection can cause a subacute sclerosing panencephalitis?

A

Measles

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32
Q

What time period after measles infection would a subacute sclerosing panencephalitis develop?

A

2-10 years (median interval is 8 years)

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33
Q

Why are neonates/immature brains more excitable and likely to develop seizures?

A

Higher expression of NKCC1 and lower expression of KCC2 which causes a high neuronal chloride concentration. This causes GABA-ergic signalling to be excitatory rather than inhibitory and increases the brain’s excitability.

Ischaemia also increases NKCC1 expression
HIE reduces KCC2 expression

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34
Q

Types of neonatal seizure

A

Tonic -> stiffening of trunk/extremitiess

Multifocal clonic -> rhythmic clonic movements of different parts of the body

Focal clonic -> repetitive clonic movements of the same part of the body

Subtle -> stereotyped bicycling, tongue thrusting and swallowing movements

Myoclonic -> isolated repetitive brief (shock like) jerks of the body

35
Q

Drug choice for neonatal seizures

A

1) Phenobarbitone
2) Phenytoin
3) Keppra or midazolam

36
Q

What are the three conditions required for a seizure to occur?

A

Population of pathologically excitable neurones

Increase in excitatory glutaminergic activity through recurrent connections to spread the discharge

Reduction in the normal activity of GABA-ergic inhibitory projections

37
Q

Types of generalised epileptic seizure

A

Absence / petit mal -> transient LOC with abrupt onset and termination where they stare off into space

Myoclonic -> brief, shock like jerks of limbs, neck or trunk

Tonic -> sudden stiffness in muscles

Tonic - clonic -> stiffness in muscles followed by general herking movements. LOC. may bite tongue, become cyanosed or incontinent of urine

Atonic -> part or all of the body becomes limp. Drop attacks.

38
Q

Types of focal epileptic seizure

A

Focal seizure = activity arising from part of or in one hemisphere only

Frontal - motor phenomena

Temporal - auditory, taste or smell phenomena

Parietal - contralateral altered sensation

Occipital - positive or negative visual phenomena

39
Q

Contraindications to a ketogenic diet

A

Carnitine deficiency
Beta oxidation defects
Porphyrias
Pyruvate carboxylase deficiency

40
Q

1st line medication for most partial seizures

A

Oxcarbazepine
Keppra

41
Q

1st line medication for generalised tonic-clonic seizures

A

Sodium Valporate

Alternative - Lamotrigine

42
Q

1st line medication for myoclonic seizures

A

Sodium Valporate

2nd line - Topiramate or Keppra

43
Q

1st line medication for atonic / tonic seizures

A

Sodium valporate

2nd line - lamotrigine

44
Q

1st line medication for benign rolandic epilepsy / BECTS

A

Carbamazepine
Lamotrigine
Keppra
Sodium valporate

45
Q

Teratogenic effects of sodium valporate / features of fetal valporate syndrome

A

Limb defects
Spina bifida
Cleft lip / palate
Heart defects (?VSD)
Reduced fetal growth

Dysmorphic - epicanthic folds, long thin upper lip, flat nasal bridge

46
Q

Teratogenic effects of phenytoin / fetal hydantoin syndrome

A

Cleft palate / lip
Reduced growth
Mental deficiency
Congenital heart defects (VSD/ASD/PDA)
Hypoplastic nails and digits

Dysmorphic - indistinct philtrum, micrognathia, short palpebral fissures, epicanthic folds

47
Q

Mutation most commonly seen in Rett syndrome

A

Sporadic mutation in MECP2 gene

Can also be X-linked dominant inheritance

48
Q

Movement stereotypies seen in Rett syndrome

A

Hand wringing
Clapping
Hand to mouth movements

49
Q

What cardiac condition is seen in Rett syndrome?

A

Long QT syndrome

  • Note important to avoid medications which prolong the QTc
50
Q

Which is the most common form of neurofibromatosis?

A

NF type 1 (makes up 96% of all NF cases)

51
Q

Clinical / diagnostic features of NF type 1?

A

Cafe au lait macules (>6)
Lisch nodules (iris hamartomas) (>2)
Skin folding
Neurofibromas
Axillary freckling
Bone lesions (sphenoid dysplasia, scoliosis, long bone abnormalities)
Optic glioma

52
Q

Inheritance pattern of NF type 1? (and which chromosome is affected?)

A

Autosomal dominant

53
Q

Inheritance pattern of NF type 1? (and which chromosome is affected?)

A

Autosomal dominant

Mutation in NF1 gene on chromosome 17 (gene locus 17q11.2)

54
Q

What is the biggest risk associated with NF type 1?

A

Overall risk of malignancy is 2.5-4 times that of the general population

This is because the mutation causes decreased production of neurofibromin which is a tumour suppressor protein

55
Q

Which specific tumour is highly suggestive of NF2?

A

Posterior subcapsular tumours

56
Q

Is there a cure / treatment for NF?

A

No

57
Q

Which medication can reduce the size of plexiform neurofibromas?

A

Imatinib

58
Q

Consequences / sequelae of NF type 1?

A

HTN (phaeochromacytomas or renal artery stenosis)
Epilepsy
Pathological bone fractures
Malignancy (brain tumours, leukaemia)
Learning difficulties +/- ADHD

59
Q

Acute management of raised ICP

A

A-E approach;
- Protect airway if GCS <8
- 3ml/kg of 3% hypertonic saline (reduces cerebral oedema and improves cerebral perfusion)
- Nurse in midline and at 30 degrees
- IV Dex if RICP due to SOL

60
Q

Acute management of status epilepticus

A

First line: IV Lorazepam or PR Diazepam - x2 doses

Second line: Phenytoin or Keppra

Last option: Rapid sequence induction / general anaesthesia

61
Q

Inheritance pattern of tuberous sclerosis? (and which chromosome is affected?)

A

Autosomal dominant

Mutations in TSC1 and TSC2 genes found on chromosomes 9 and 16

62
Q

Clinical features / diagnostic criteria of tuberous sclerosis

A

> 3 hypopigmented macules (ash leaf spots) - can use wood lamp to help visualise
3 angiofibromas
2 ungal fibromas
Shagreen patch
Retinal harmatomas
2 subependymal nodules (brain)
Subependymal giant cell astrocytoma (brain)
Cardiac rhabdomyoma
Lymphagiomyomatosis
2 angiomyolipomas (kidneys/liver)
Confetti skin lesions
3 dental enamel pits
Adenoma sebaceum (acne like rash)

63
Q

Which neurocutaneous disorder requires regular suveillance?

A

TS

Need blood pressure, ECG + echo, U&E, EEG, MRI head, MRI abdomen

64
Q

In which organs would you see growth of benign tumours / harmatomas in tuberous sclerosis

A

Brain
Heart
Kidneys
Lungs
Skin

65
Q

Which medication is 1st line for infantile spasms caused by tuberous sclerosis

A

Vigabatrin

66
Q

What do the TSC1 and TSC2 genes code for?

A mutation in which gene will cause more severe clinical manifestations?

A

TSC1 codes for hamartin

TSC2 codes for tuberin (MORE SEVERE CLINICAL MANIFESTATIONS)

Both are tumour suppressor genes

67
Q

Organs affected in Friedrich’s ataxia

A

CNS
- Spinocerebellar tracts
- Dorsal columns
- Pyramidal tracts
- Cerebellum
- Medulla

Heart
- Hypertrophic cardiomyopathy

Pancreas
- DM

68
Q

Inheritance / genetic abnormality causing Friedrich’s ataxia

A

Autosomal recessive

GAA repeat expansion in frataxin gene (FXN gene) - codes for the frataxin protein in mitochondria

69
Q

Novel pharmacological therapies for Friedrich’s ataxia

A

trials have focussed on antioxidant therapy (reduce oxidative injury caused by iron deposits in mitochondria)
- Vitamin E or Co-enzyme Q10

70
Q

Life expectancy in Friedrich’s ataxia

A

40-50

71
Q

Life expectancy in NF type 1

A

Live long healthy lives (average life expectancy only reduced by 10-15 years)

72
Q

Life expectancy in TS

A

Depends on clinical mainfestations of the disease - most have a normal life span

73
Q

Typical clinical presentation of TS

A

Seizures and cutaneous signs

74
Q

Timescales of neurological sequelae seen following measles infection

A

Encephalitis
- Usually 5 days following the rash

Acute disseminated encephalomyelitis (ADEM)
- 2 weeks following the rash

Subacute sclerosing panencephalitis
- 7-10 years following the infection

75
Q

What has been shown to reduce the incidence of sensorineural hearing loss in Hib meningitis

A

Early administration of IV Dex

76
Q

Cause of Angelman syndrome

A

Neurodevelopmental disorder caused by loss of expression of the maternally inherited UBE3A gene

77
Q

Common presentation in juvenile myoclonic epilepsy

A

Myoclonic seizures (jerk like movements) soon after waking up / being clumsy first thing in the morning

Up to 80% will also develop generalised TC and absence seizures

78
Q

Common presentation of frontal lobe epilepsy

A

Arm posturing / hypermotor activity during sleep

79
Q

Common presentation of Panayiotopoulos syndrome?

A

Autonomic features (vomiting) are followed by tonic eye deviation, impairment of consciousness and sometimes evolution to secondary generalised tonic clonic seizure

Note low seizure frequency!

80
Q

1st line drug for Panayiotopoulos syndrome

A

Nil - trick question

Most children go into spontaneous remission after a few seizures and don’t require treatment

81
Q

Seizures seen in Lennox-Gastaut syndrome

A

Multiple seizure types including atonic, tonic, tonic-clonic and atypical absence seizures

Children also have cognitive impairment

82
Q

Clinical presentation of language regression with EEG abnormalities seen in sleep

A

Landau-Kleffner syndrome

83
Q

In which epilepsy syndrome do seizures most commonly occur at night?

A

Benign childhood epilepsy with centrotemporal spikes / benign rolandic epilepsy

Paeds FOP/TAS (12 decks)

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