Paediatric neurology Flashcards

1
Q

What is a febrile seizure ?

A

A seizure that occurs in children with a high fever.

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2
Q

What age group suffer from febrile seizures?

A

6 mnths -> 5 years

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3
Q

What are simple febrile convulsions ?

A
  • Generalised, tonic clonic seizures
  • Last <15 mins
  • Occur once during a single febrile illness
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4
Q

What are complex febrile convulsions ?

A

Any of the following :

  • Partial or focal seizures
  • Last >15 mins
  • Multiple occur during the same febrile illness

-Febrile status epilepticus = >30 mins

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5
Q

What needs to be excluded before febrile convulsions are diagnosed ?

A
  • Epilepsy
  • Meningitis, encephalitis or other neurological infection
  • Intracranial space occupying lesions
  • Syncopal episode
  • Electrolyte abnormality
  • Traume
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6
Q

How are febrile convulsions managed ?

A
  • Identify and treat underlying infection
  • Fever : paracetamol and ibuprofen
  • Period of observation
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7
Q

altered consciousness
altered cognition
unusual behaviour
acute onset of neurological symptoms
acute onset of focal seizures
fever

A

encephalitis

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8
Q

how is encephalitis investigated

A

LP for CSF with viral PCR testing

CT if LP is CI

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9
Q

how is encephalitis managed

A

Aciclovir for HSV and VZV

Ganciclovir for CMV

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10
Q

When would a child be admitted with a febrile seizure

A

-> First seizure or features of complex seizure

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11
Q

Benign Rolandic seizures (benign focal epilepsy) : presentation, EEG

A
  • 3 to 10 yrs
  • Boys and during sleep
  • Tonic seizure : wake up on floor or with messy sheets
  • EEG : centro-temporal spikes
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12
Q

Infatile spasms (west syndrome) : presentation, diagnosis

A
  • Boys : 4-8 mnths
  • Presentation : Salam attacks (flexion of head, trunk, arms, follwed by extension of arms).
  • Lasts 1-2 seconds, repeated up to 50 times.
  • EEG : hypsarrhymthmia
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13
Q

Inheritance of juvenile myoclonic epilepsy

A

AD

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14
Q

Management of a generalised Tonic-Clonic seizure

A
  • First line: sodium valproate
  • Second line: lamotrigine or carbamazepine
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15
Q

Management of focal seizures

A
  • First line: carbamazepine or lamotrigine
  • Second line: sodium valproate or levetiracetam
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16
Q

Management of absence seizures

A

First line: sodium valproate or ethosuximide

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17
Q

Management of atonic seizures (drop attacks

A
  • First line: sodium valproate
  • Second line: lamotrigine
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18
Q

Management of myoclonic seizures

A
  • First line: sodium valproate
  • Other options: lamotrigine, levetiracetam or topiramate
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19
Q

Management of west syndrome

A
  • Prednisolone
  • Vigabatrin
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20
Q

How does SV work and what are 4 notable SE

A
  • Increasing activity of GABA
  • Teratogenic (avoided in girls)
  • Liver damage and hepatitis
  • Hair loss
  • Tremor
21
Q

3 notable SE of carbamazepine

A
  • Agranulocytosis
  • Aplastic anaemia
  • Induces the P450 system so many drug interactionms
22
Q

3 notable SE of phenytoin

A
  • Folate and vitamin D deficiency
  • Megaloblastic anaemia (folate deficiency)
  • Osteomalacia (vitamin D deficiency)
23
Q

2 notable SE of ethosuximide

A

Night tremors
Rashes

24
Q

2 notable SE of lamotrigine

A
  • SJS or DRESS
  • Leukopenia
25
Q

How is status epilepticus defined

A
  • > Seizure >5 minutes, 2 or more seizures without regaining consciousness
26
Q

How is status epilepticus managed

A
  • Secure the airway
  • Give high-concentration oxygen
  • Check blood glucose levels
  • Gain IV access
  • IV lorazepam, repeated after 10 minutes if the seizure continues
  • If seizure persists : IV phenytoin or phenobarbital
27
Q

Medical options for management of status epilepticus in community

A

Buccal midazolam
Rectal diazepam

28
Q

What can cause CP

A
  • Antenatal : maternal infection, trauma in pregnancy
  • Perinatal : birth asphyxia, prematurity
  • Postnatal : meningitis, severe neonatal jaundice, head injury.
29
Q

What are the 4 types of CP ?

A
  • Spastic: hypertonia and reduced function resulting from damage to upper motor neurones.
  • Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
  • Ataxic: problems with coordinated movement resulting from damage to the cerebellum
  • Mixed: a mix of spastic, dyskinetic and/or ataxic features
30
Q

What are the 4 patterns of spastic CP

A
  • Monoplegia: one limb affected
  • Hemiplegia: one side of the body affected
  • Diplegia: four limbs are affects, but mostly the legs
  • Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
31
Q

What can the gait tell you about a child with CP

A
  • Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
  • Broad based gait / ataxic gait: indicates a cerebellar lesion
  • High stepping gait: indicates foot drop or a lower motor neurone lesion
  • Waddling gait: indicates pelvic muscle weakness due to myopathy
  • Antalgic gait (limp): indicates localised pain
32
Q

If not idiopathic, what are 4 underlying causes of strabismus ?

A
  • Hydrocephalus
  • Cerebral palsy
  • Space occupying lesions, for example retinoblastoma
  • Trauma
33
Q

What can be done to manage strabismus ?

A

-> Start before 8 yrs of age.
-> Cover the good eye with an occlusive patch, forcing the weaker eye to develop.

34
Q

What 6 definitions are used when described a strabismus ?

A
  • Strabismus: the eyes are misaligned
  • Amblyopia: the affected eye becomes passive and has reduced function compared to the other dominant eye
  • Esotropia: inward positioned squint
  • Exotropia: outward positioned squint
  • Hypertropia: upward moving affected eye
  • Hypotropia: downward moving affected eye
35
Q

How can a cover test be used to define a strabismus ?

A
  • Cover one eye and ask the pt to focus on an object in front of you
  • Move the cover to the opposite eye
  • Watch movement of previously covered eye
  • If the eye moves in, it drifted outwards when covered = EXOTROPIA
  • If the eye moves outward, it drifted inwards when covered = ESOTROPIA
36
Q

What is the most common cause of hydrocephalus in children ?

A
  • Aqueductal stenosis -> the cerebral aqueduct connecting third and fourth ventricle is stenosed blocking outflow of CSF
37
Q

How will hydrocephalus present in children

A
  • Rapidly increasing head circumference (sutures have not fused)
  • Bulging anterior fontanelle
  • Poor feeding and vomiting
  • Poor tone
  • Sleepiness
38
Q

Wat the mainstay of treatment for hydrocephalus ?

A
  • Ventriculoperitoneal (VP) shunt
  • CSF is drained from the ventricles into the peritoneal cavity
39
Q

Give 5 complications of a VP shunt

A
  • Infection
  • Blockage
  • Excessive drainage
  • Intraventricular haemorrhage during shunt related surgery
  • Outgrowing them (they typically need replacing around every 2 years as the child grows)
40
Q

What is craniosynostosis and its complication if left untreated

A
  • > Premature closure of skull sutures
  • > Complications : raised ICP = developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neurological symptoms and seizures
41
Q

what is the inheritance of Duchennes muscualr dystrophy ?

A
  • X-linked recessive
  • Due to defective gene for dystrophin on X chromosome required for normal muscular function
42
Q

How does duchennes muscular dystrophy present, what is a positive finding and how is progression slowed ?

A
  • Boys, aged 3-5 with weakness around the pelvis
  • Gower’s sign = Using hands on legs to help them stand up due to proximal muscle weakness
  • Oral steroids can help
43
Q

what is the difference in presentation between Duchenne’s and Beckers muscular dystrophy

A
  • Beckers presents later (around 8-12 yrs)
  • Less severe
44
Q

Give 4 features of myotonic dystrophy

A
  • Progressive muscle weakness
  • Prolonged muscle contractions = pt unable to let go after shaking hand etc
  • Cataracts
  • Cardiac arrhythmias
45
Q

What is spinal muscular atrophy ?

A
  • > Autosomal recessive condition causing progressive loss of motor neurones = progressive muscular weakness
  • > Affects LMN !
46
Q

Most common complication of meningitis

A

Sensorineural hearing loss

47
Q

how will herpes simplex encephalitis present on CT and what will be present in the history?

A
  • CT : bilateral hyperdensities of the middle temporal gyri
  • History : confusion, fever, general malaise. + possible focal temporal seizures - rising feeling in stomach before generalised seizure
48
Q
A