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Paediatric > Neurology > Flashcards

Neurology Flashcards

1
Q

What age group is affected by febrile convulsions?

A

6mo-6yr

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2
Q

What is the criteria for a seizure to be Simple Febrile seizure

A
  1. 95%
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3
Q

Brief repeated symmetric contractions of neck, trunk, extremities (flexion and extension) lasting 10-30s

A

Infantile Spasm

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4
Q

What is West Syndrome?

A

Infantile spasm, Developmental regression, hypsarhythmia on EEG

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5
Q

What age group is affected by infantile spasm?

A

4-8mo

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6
Q

80% of infantile spasms are due to ________ abnormalities, _____ or are associated with_____

A

80% due to metabolic or developmental abnormalities, encephalopathies or are associated with neurocutaneous syndromes (

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7
Q

What is seen on a Typical EEG: hypsarrhytmia

A

High voltage slow waves, spikes and polyspikes, background disorganization

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8
Q

Management of infantile spasms:

A

ACTH, benzodiazepines, Corticosteroids (for an acute period of time), vigabatrin (if diagnosed dura sclerosis)

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9
Q

Centrotemporal sharp waves on EEG?

A

Benign Focal epilepsy (rolandic spikes)

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10
Q

Focal motor seizures involving tongue, mouth, face, upper extremity usually occurring in sleep-wake transition states

A

Benign Rolandic Epilepsy

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11
Q

What age group affected by Benign Rolandic Epilepsy?

A

5-10 years of age - remit spontaneously in adolescence

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12
Q

Manage Benign Rolandic epilepsy with _______

A

Carbamazepine,

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13
Q

What age group affected by Childhood absence seizures?

A

6-7 years and usually grow out of it by teenage years

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14
Q

What medication is used for Childhood absence seizures?

and if there’s also generalised seizures?

A

Ethosuximide for just absent

Valproic acid (if also generalized)

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15
Q

Typical EEG: 3Hz spike and wave

A

Childhood absence seizures

Normal EEG in between seizures

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16
Q

Multiple daily absence seizures lasting

A

Childhood absence seizures

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17
Q

Myoclonus particularly in morning; frequently presents as generalized tonic-clonic seizures

A

Juvenile Myoclonic Epilepsy (Janz Syndrome)

  • Can also have absence seizures
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18
Q

What age group is affected by Janz Syndrome

A

12-16year olds, autosomal dominance with variable penentrance

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19
Q

What would exacerbate Janz Syndrome?

A

Sleep deprivation, Illness, photic stimulation

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20
Q

Triad for Lennox-Gastraut Syndrome?

A
  1. Multiple seizure types
  2. Diffuse congnitive dysfunction
  3. Slow generalised spike and slow EEG wave
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21
Q

Causes for Lennox Gastraut syndrome?

A

Seen with underlying encephalopathy and brain malformations

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22
Q

Management of Janz Syndrome?

A

Life long treatment - Valproic acid most commonly.

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23
Q

Mx of Lennox-Gastraut syndrome?

A

Valproic acid, benzodiazepines and ketogenic diet; however, response often poor

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24
Q

infantile acquired aphasia post seizure?

A

Landau Kleffner Syndrome:

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25
Q

Steven Johnson syndrome associated with ______ especially with given with carbamazepine

A

Lamatrogine

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26
Q

Why do you get non-communicating hydrocephalus?

A

Because of failure to communicate within the brain?

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27
Q

“Sun setting sign” - eye gaze downwards?

A

Hydrocephalus

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28
Q

Adenoma sebaceum is related to ______

A

Tuberous sclerosis

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29
Q

What is a port wine stain related to?

A

Sturge Weber disease

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30
Q

NF 1 is more common/uncommon?

Autosomal dominant/recessive

A

More common - dominant

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31
Q

Bilateral acoustic neuroma is related to _____

A

Neurofibromatosis 2

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32
Q

Small angiokeratoms seen on face - often in malar distribution are called adenoma sebaceum. This is related to _____

A

Tuberous sclerosis

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33
Q

A child with hypopigmentation (ash leaf spots) and isolated raised plaque over lower black (Shrageen patch)

A

Tuberous Sclerosis

34
Q

Complications of Tuberous Sclerosis?

A

Epilepsy (generally presented within 1 st of life)

- generally present with Infantile spasms

35
Q

How do you manage infantile spasms?

A

ACTH, Vigabatrin

36
Q

Port wine nevus syndrome in V1 distribution with associated ________-

A

Angiomatous Malformation _ sturge weber syndrome

37
Q

Cherry red spot, hypotonia, developmental regression in infancy, death 2-5 years. Jewish!

A

Tay Sach’s disease

38
Q

Hypersplenism (Splenomegaly), bone marrow suppression, neurological degeneration, seizures

A

Gaucher disease

39
Q

Delayed response to previous measles infection…

A

Subacute sclerosing panencephalitis (SSPE)

40
Q

Ddx for seiures?

A

Breath holding attacks

Reflex anoxic seizures (head trauma, cold food, frightened)

41
Q

Bright eye appearance - and severe hypotonia (frog like posture)

A

SMA Type I

42
Q

Waddling gait, pseudohypertrophy of the calf, run flower than their peers, one step at a time

A

Duchene dystrophy

43
Q

Gower’s signs - what is is and what is it related to?

A

It’s a weird way that kids with peripheral weakness get up and it is related to Duchene Dystrophy

44
Q

Temporal lobe seizures are partial seizures with ______ aura

A

Sensory Aura

45
Q

Infantile spasms can look like _____

A

colic pain.

46
Q

Infantile spasms with hypsarrythmia and developmental regression point to

A

West Syndrome

47
Q

In infantile spasms CT demonstrates diffuse or localised brain disease in 70% which can be due to _______

A

Tuberous Sclerosis

48
Q

Most common cause of Cerebral palsy?

A

Antenatal causes: Cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)

49
Q

Which one of the following does not cross suture lines?

Cephalohaematoma or Caput?

A

Cephalohaematoma and it develops after birth

50
Q

A common complication of Cephalohaematoma?

A

Jaundice may develop

51
Q

A 4-week-old child is brought to clinic with a red rash on her scalp associated with yellow flakes. What is the most likely diagnosis?

A

Seborrhoeic dermatitis

52
Q

Which diet is helpful in epilepsy?

A

Ketogenic diet

53
Q

Which seizure syndrome?

1-5 year old affected, atypical absences, falls and jerks ; EED shows slow spikes…

A

Lenox-Gastruat Syndrome

54
Q

Teenage girl, with infrequent generalised seizure in the morning, with daytime absences and sudden shock like myoclonic seizure…

A

Janz Syndrome - Juvenile Myoclonic Epilepsy

55
Q

Which anti epileptic has a good response to Juvenile Myoclonic Epilepsy?

A

Sodium Valproate

56
Q

Which anti epileptic has the following side effects?

Alopecia, Ataxia, tremor, hepatitis, pancreatitis, thrombocytopaenia,

A

Sodium Valproate

57
Q

Which anti-epileptic has the following side effects?
Dizziness and ataxia, leukopenia with agranulocytosis, syndrome of inappropriate ADH secretions, and visual disturbances (Diplopia)

A

Lamotrigine

58
Q

Which anti-epileptic has the following side effects?
Gingival hyperplasia, Hirsutism, coarsening of facial features, megaloblastic anaemia, peripheral neuropathy, enhanced Vitamin D metabolism causing osteomalacia, Lymphadenopathy

A

Phenytoin

59
Q

Which anti-epileptic has the following side effects?

Sore throat, fatigue, ulcers in the mouth - reduced ability to eat or drink. “Toxic epidermal necrolysis”

A

Stevens Johnson Syndrome

60
Q

A mother brings her 2-week-old baby girl into the surgery for review. She has noted a bright red, well-circumscribed and lobulated lesion developing on her right temple. This wasn’t noted at birth but is now 5 mm in diameter… What is it?

A

Strawberry Naevus - not present at birth.

61
Q

If a Strawberry naevus is causing visual field obstruction, how would you treat it?

A

Propanolol

62
Q

3-5 year old with multiple seizure types, diffuse cognitive dysfunction and slow generalised spike and slow wave EEG

A

Lennox gastruat syndrome

63
Q

How do you manage benign focal epilepsy of childhood with Rolandic centerotemporal spikes?

A

Carbamazapine

64
Q

Seizure leads to disruption of talking –> Infantile acquired aphasia

A

Laundau Kleffner syyndrome

65
Q

Atresia of foramine of Magendie and Luschka, resulting in complete or incomplete agenesis of the cerebellar vermis with widely separated, hypoplastic cerebellar hemispheres.

A

Dandy walker malformation

66
Q

In Dandy Walker malformation you get two widely separated cerebral hemispheres and can also get _______ formation

A

Posterior cyst formation

67
Q

________ is responsible for 2-4% of paediatric hydrocephalus

A

Dandy Walker malformation

68
Q

Average age of type I Chairi malformation presentation is ____

A

15 yo

69
Q

In Chiari Malformation type I the ________ _____ lie below the level of the foramen magnum

A

Cerebellar tonsils

70
Q

3 common presentations of Chiari malformations Type I?

A

Central chord syndrome
Syringiomyelia
Foramen maganum compression syndrome

71
Q

How does central chord syndrome present?

A

It is characterized by loss of motion and sensation in arms and hands - Upper limb affected more than lower limb

72
Q

Which syndrome causes is damage to one half of the spinal cord, resulting in paralysis and loss of proprioception on the same (or ipsilateral) side as the injury or lesion, and loss of pain and temperature sensation on the opposite (or contralateral) side as the lesion.

A

Brown Sequard syndrome

73
Q

Type II Chiari malformations are when parts of the cerebellar vermis and medulla and 4th ventricle protrude below the foramen magnum. It is almost always associated with ______

A

Myelomeningocoele

74
Q

What are common clinical features in Type II Chiari Malformations?

A

Findings due to brainstem and lower cranial nerve dysfunction

75
Q

Most common type/angle of craniosyntosis?

A

Sagittal!

76
Q

Sagittal Craniosyntosis results in what shaped head?

A

Long narrow shaped head

77
Q

What are common clinical features of Craniosyntosis?

A

Skull deformity, Raised ICP, hydrocephuls

May also get opthalmologic problems due raised ICP to bony abnormalities of the orbit.

78
Q

When do you really need surgery for Craniosyntosis?

A

More than 2 sutures involved

79
Q

What are key differentiating features between deformative plagiocephaly and craniosyntosis?

A

Deformative plagiocephaly results in normal growth rate of head, parallelogram shaped head, Nose generally straight, ears anterior and there will be external pressures

Craniosyntosis - rhomboid shaped head, nose pointing towards fused suture, ears posterior, slow growing head!

80
Q

How do you manage Deformity plagiocephaly?

A

Managed by GP or paediatrician. Counter
positioning
and/or helmet
therapy

81
Q

How do you manage Craniosyntosis?

A

Craniofacial surgeon - expansion surgery.

82
Q

60% of all paediatric brain tumours are _______

A

Infratentorial

Paediatric (13 decks)

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