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
Steven Johnson syndrome associated with ______ especially with given with carbamazepine
Lamatrogine
26
Why do you get non-communicating hydrocephalus?
Because of failure to communicate within the brain?
27
"Sun setting sign" - eye gaze downwards?
Hydrocephalus
28
Adenoma sebaceum is related to ______
Tuberous sclerosis
29
What is a port wine stain related to?
Sturge Weber disease
30
NF 1 is more common/uncommon? | Autosomal dominant/recessive
More common - dominant
31
Bilateral acoustic neuroma is related to _____
Neurofibromatosis 2
32
Small angiokeratoms seen on face - often in malar distribution are called adenoma sebaceum. This is related to _____
Tuberous sclerosis
33
A child with hypopigmentation (ash leaf spots) and isolated raised plaque over lower black (Shrageen patch)
Tuberous Sclerosis
34
Complications of Tuberous Sclerosis?
Epilepsy (generally presented within 1 st of life) | - generally present with Infantile spasms
35
How do you manage infantile spasms?
ACTH, Vigabatrin
36
Port wine nevus syndrome in V1 distribution with associated ________-
Angiomatous Malformation _ sturge weber syndrome
37
Cherry red spot, hypotonia, developmental regression in infancy, death 2-5 years. Jewish!
Tay Sach's disease
38
Hypersplenism (Splenomegaly), bone marrow suppression, neurological degeneration, seizures
Gaucher disease
39
Delayed response to previous measles infection...
Subacute sclerosing panencephalitis (SSPE)
40
Ddx for seiures?
Breath holding attacks | Reflex anoxic seizures (head trauma, cold food, frightened)
41
Bright eye appearance - and severe hypotonia (frog like posture)
SMA Type I
42
Waddling gait, pseudohypertrophy of the calf, run flower than their peers, one step at a time
Duchene dystrophy
43
Gower's signs - what is is and what is it related to?
It's a weird way that kids with peripheral weakness get up and it is related to Duchene Dystrophy
44
Temporal lobe seizures are partial seizures with ______ aura
Sensory Aura
45
Infantile spasms can look like _____
colic pain.
46
Infantile spasms with hypsarrythmia and developmental regression point to
West Syndrome
47
In infantile spasms CT demonstrates diffuse or localised brain disease in 70% which can be due to _______
Tuberous Sclerosis
48
Most common cause of Cerebral palsy?
Antenatal causes: Cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
49
Which one of the following does not cross suture lines? Cephalohaematoma or Caput?
Cephalohaematoma and it develops after birth
50
A common complication of Cephalohaematoma?
Jaundice may develop
51
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?
Seborrhoeic dermatitis
52
Which diet is helpful in epilepsy?
Ketogenic diet
53
Which seizure syndrome? | 1-5 year old affected, atypical absences, falls and jerks ; EED shows slow spikes...
Lenox-Gastruat Syndrome
54
Teenage girl, with infrequent generalised seizure in the morning, with daytime absences and sudden shock like myoclonic seizure...
Janz Syndrome - Juvenile Myoclonic Epilepsy
55
Which anti epileptic has a good response to Juvenile Myoclonic Epilepsy?
Sodium Valproate
56
Which anti epileptic has the following side effects? | Alopecia, Ataxia, tremor, hepatitis, pancreatitis, thrombocytopaenia,
Sodium Valproate
57
Which anti-epileptic has the following side effects? Dizziness and ataxia, leukopenia with agranulocytosis, syndrome of inappropriate ADH secretions, and visual disturbances (Diplopia)
Lamotrigine
58
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
Phenytoin
59
Which anti-epileptic has the following side effects? | Sore throat, fatigue, ulcers in the mouth - reduced ability to eat or drink. "Toxic epidermal necrolysis"
Stevens Johnson Syndrome
60
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?
Strawberry Naevus - not present at birth.
61
If a Strawberry naevus is causing visual field obstruction, how would you treat it?
Propanolol
62
3-5 year old with multiple seizure types, diffuse cognitive dysfunction and slow generalised spike and slow wave EEG
Lennox gastruat syndrome
63
How do you manage benign focal epilepsy of childhood with Rolandic centerotemporal spikes?
Carbamazapine
64
Seizure leads to disruption of talking --> Infantile acquired aphasia
Laundau Kleffner syyndrome
65
Atresia of foramine of Magendie and Luschka, resulting in complete or incomplete agenesis of the cerebellar vermis with widely separated, hypoplastic cerebellar hemispheres.
Dandy walker malformation
66
In Dandy Walker malformation you get two widely separated cerebral hemispheres and can also get _______ formation
Posterior cyst formation
67
________ is responsible for 2-4% of paediatric hydrocephalus
Dandy Walker malformation
68
Average age of type I Chairi malformation presentation is ____
15 yo
69
In Chiari Malformation type I the ________ _____ lie below the level of the foramen magnum
Cerebellar tonsils
70
3 common presentations of Chiari malformations Type I?
Central chord syndrome Syringiomyelia Foramen maganum compression syndrome
71
How does central chord syndrome present?
It is characterized by loss of motion and sensation in arms and hands - Upper limb affected more than lower limb
72
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.
Brown Sequard syndrome
73
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 ______
Myelomeningocoele
74
What are common clinical features in Type II Chiari Malformations?
Findings due to brainstem and lower cranial nerve dysfunction
75
Most common type/angle of craniosyntosis?
Sagittal!
76
Sagittal Craniosyntosis results in what shaped head?
Long narrow shaped head
77
What are common clinical features of Craniosyntosis?
Skull deformity, Raised ICP, hydrocephuls | May also get opthalmologic problems due raised ICP to bony abnormalities of the orbit.
78
When do you really need surgery for Craniosyntosis?
More than 2 sutures involved
79
What are key differentiating features between deformative plagiocephaly and craniosyntosis?
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
How do you manage Deformity plagiocephaly?
Managed by GP or paediatrician. Counter positioning and/or helmet therapy
81
How do you manage Craniosyntosis?
Craniofacial surgeon - expansion surgery.
82
60% of all paediatric brain tumours are _______
Infratentorial