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Paeds FOP/TAS > Neuro > Flashcards

Neuro Flashcards

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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
Risk factors for meningitis?
Immunosuppressed CSF shunts/surgery Renal or adrenal insufficiency Thalassaemia Cystic Fibrosis Croweded living conditions UNVACCINATED children
26
Common viral causes of meningitis
Enterovirus (most common) Coxsackie virus Echo virus Measles Mumps HSV VZV
27
Bacterial causes of meningitis
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
28
LP findings in viral meningitis
AWPG Appearance - clear WCC - raised (lymphocytes) Protein - normal or slightly raised Glucose - normal or slightly reduced
29
LP findings in bacterial meningitis
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
30
What is a normal CSF glucose level?
>50% blood glucose ratio
31
Which infection can cause a subacute sclerosing panencephalitis?
Measles
32
What time period after measles infection would a subacute sclerosing panencephalitis develop?
2-10 years (median interval is 8 years)
33
Why are neonates/immature brains more excitable and likely to develop seizures?
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
34
Types of neonatal seizure
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
Drug choice for neonatal seizures
1) Phenobarbitone 2) Phenytoin 3) Keppra or midazolam
36
What are the three conditions required for a seizure to occur?
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
Types of generalised epileptic seizure
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
Types of focal epileptic seizure
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
Contraindications to a ketogenic diet
Carnitine deficiency Beta oxidation defects Porphyrias Pyruvate carboxylase deficiency
40
1st line medication for most partial seizures
Oxcarbazepine Keppra
41
1st line medication for generalised tonic-clonic seizures
Sodium Valporate Alternative - Lamotrigine
42
1st line medication for myoclonic seizures
Sodium Valporate 2nd line - Topiramate or Keppra
43
1st line medication for atonic / tonic seizures
Sodium valporate 2nd line - lamotrigine
44
1st line medication for benign rolandic epilepsy / BECTS
Carbamazepine Lamotrigine Keppra Sodium valporate
45
Teratogenic effects of sodium valporate / features of fetal valporate syndrome
Limb defects Spina bifida Cleft lip / palate Heart defects (?VSD) Reduced fetal growth Dysmorphic - epicanthic folds, long thin upper lip, flat nasal bridge
46
Teratogenic effects of phenytoin / fetal hydantoin syndrome
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
Mutation most commonly seen in Rett syndrome
Sporadic mutation in MECP2 gene Can also be X-linked dominant inheritance
48
Movement stereotypies seen in Rett syndrome
Hand wringing Clapping Hand to mouth movements
49
What cardiac condition is seen in Rett syndrome?
Long QT syndrome - Note important to avoid medications which prolong the QTc
50
Which is the most common form of neurofibromatosis?
NF type 1 (makes up 96% of all NF cases)
51
Clinical / diagnostic features of NF type 1?
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
Inheritance pattern of NF type 1? (and which chromosome is affected?)
Autosomal dominant
53
Inheritance pattern of NF type 1? (and which chromosome is affected?)
Autosomal dominant Mutation in NF1 gene on chromosome 17 (gene locus 17q11.2)
54
What is the biggest risk associated with NF type 1?
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
Which specific tumour is highly suggestive of NF2?
Posterior subcapsular tumours
56
Is there a cure / treatment for NF?
No
57
Which medication can reduce the size of plexiform neurofibromas?
Imatinib
58
Consequences / sequelae of NF type 1?
HTN (phaeochromacytomas or renal artery stenosis) Epilepsy Pathological bone fractures Malignancy (brain tumours, leukaemia) Learning difficulties +/- ADHD
59
Acute management of raised ICP
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
Acute management of status epilepticus
First line: IV Lorazepam or PR Diazepam - x2 doses Second line: Phenytoin or Keppra Last option: Rapid sequence induction / general anaesthesia
61
Inheritance pattern of tuberous sclerosis? (and which chromosome is affected?)
Autosomal dominant Mutations in TSC1 and TSC2 genes found on chromosomes 9 and 16
62
Clinical features / diagnostic criteria of tuberous sclerosis
>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
Which neurocutaneous disorder requires regular suveillance?
TS Need blood pressure, ECG + echo, U&E, EEG, MRI head, MRI abdomen
64
In which organs would you see growth of benign tumours / harmatomas in tuberous sclerosis
Brain Heart Kidneys Lungs Skin
65
Which medication is 1st line for infantile spasms caused by tuberous sclerosis
Vigabatrin
66
What do the TSC1 and TSC2 genes code for? A mutation in which gene will cause more severe clinical manifestations?
TSC1 codes for hamartin TSC2 codes for tuberin (**MORE SEVERE CLINICAL MANIFESTATIONS**) Both are tumour suppressor genes
67
Organs affected in Friedrich's ataxia
CNS - Spinocerebellar tracts - Dorsal columns - Pyramidal tracts - Cerebellum - Medulla Heart - Hypertrophic cardiomyopathy Pancreas - DM
68
Inheritance / genetic abnormality causing Friedrich's ataxia
Autosomal recessive GAA repeat expansion in frataxin gene (FXN gene) - codes for the frataxin protein in mitochondria
69
Novel pharmacological therapies for Friedrich's ataxia
trials have focussed on antioxidant therapy (reduce oxidative injury caused by iron deposits in mitochondria) - Vitamin E or Co-enzyme Q10
70
Life expectancy in Friedrich's ataxia
40-50
71
Life expectancy in NF type 1
Live long healthy lives (average life expectancy only reduced by 10-15 years)
72
Life expectancy in TS
Depends on clinical mainfestations of the disease - most have a normal life span
73
Typical clinical presentation of TS
Seizures and cutaneous signs
74
Timescales of neurological sequelae seen following measles infection
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
What has been shown to reduce the incidence of sensorineural hearing loss in Hib meningitis
Early administration of IV Dex
76
Cause of Angelman syndrome
Neurodevelopmental disorder caused by loss of expression of the maternally inherited UBE3A gene
77
Common presentation in juvenile myoclonic epilepsy
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
Common presentation of frontal lobe epilepsy
Arm posturing / hypermotor activity during sleep
79
Common presentation of Panayiotopoulos syndrome?
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
1st line drug for Panayiotopoulos syndrome
Nil - trick question Most children go into spontaneous remission after a few seizures and don't require treatment
81
Seizures seen in Lennox-Gastaut syndrome
Multiple seizure types including atonic, tonic, tonic-clonic and atypical absence seizures Children also have cognitive impairment
82
Clinical presentation of language regression with EEG abnormalities seen in sleep
Landau-Kleffner syndrome
83
In which epilepsy syndrome do seizures most commonly occur at night?
Benign childhood epilepsy with centrotemporal spikes / benign rolandic epilepsy

Paeds FOP/TAS (12 decks)

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