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FRACP > Neuro > Flashcards

Neuro Flashcards

1
Q

What are the two major groupings of seizures and what are the differences In the two?

A

Focal- maintained awareness

Generalised- reduced awareness

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2
Q

What is the general population risk of epilepsy

How does it increase with 1 seizure? With 2

A

2%
30%
60%

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3
Q

Benign familial neonatal epilepsy
Gene association
What does it encode for
Do they normally resolve?

A

KCNQ2
Potassium channel
Yes!

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4
Q

Febrile convulsions
What age group do they appear in?
What is the rule of 3s
What defines a complex febrile convulsion

A

6 months to 6 yrs
30% recur, 30% have family history and 3% have epilepsy
>15 mins, focal, rescuers within 24 hours

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5
Q

What are 2 more complicated forms of febrile seizures?

A

Generalised epilepsy with febrile seizures plus

Dravet syndrome

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6
Q

Dravet syndrome

Genetic mutation type of seizures

A

SNC1A mutation

Febrile- more prolonged and frequent that progress to myoclonic seizures with developmental delay

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7
Q

Benign generalised epilepsy

What do you see in juvenile myoclonic epilepsy? What makes it worse
What is seen on eeg?
How many persist? Is lifelong treatment needed?

Is treatment usually needed for benign myoclonic epilepsy of childhood?

A

Morning clumsiness with myoclonic seizures. Alcohol or sleep deprivation.
Photo sensitivity with generalised poly spike and waves
Na valproate- lifelong usually!

No- usually resolves by 2

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8
Q 
Benign focal epilepsy 
What is the most likely cause in children 
What types of seizures are seen?
What is seen on eeg 
Do they usually grow out of it?

What might you see with AD nocturnal frontal lobe epilepsy
What is the defect?
What is the inheritance?

A

Rolandic epilepsy
Focal seizures at night. Might be missed!
Centro-temporal spikes increased with sleep deprivation
Yes by adolescence

Screaming and fencing posturing
ACH receptor defect
AD

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9
Q

Absence seizures
When do they usually present?
Are girls more likely to have them or boys?
What is seen on eeg?
What can they be associated with in the under 4 yrs? How is this found? What is the treatment
What is different about juvenile absence

A

Before school
Girls
3hs spikes, increased with hyperventilation
Channelopathies- glucose 1 transporter- low glucose in the CSF but normal in serum. Ketogenic diet
Less likely to resolve

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10
Q

When can AEDs be stopped?

What is the recurrence rate

A

2 yrs after last seizure

30-40%

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11
Q

What is temporal lobe epilepsy likely to show

A

A focus e.g. tumour, prev meningitis or prev febrile convulsions

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12
Q

What is Todd’s pariesis

A

Focal motor seizure with a prolonged post ictal phase- can last days!

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13
Q

Severe focal epilepsy
What is panyiotopoulos syndrome
What is seen on eeg?

A

Focal seizures with vomiting. Can have migraines or auras

Occipital spikes

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14
Q

Severe generalised epilepsy
What can infant myoclonic seizures progress to? What might be causing it?
What is seen on eeg?

What other syndrome is similar but associated with brain anomalies

A

Early myoclonic encephalopathy
Metabolic problem
Burst suppression

Otahara syndrome

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15
Q

Severe generalised epilepsy
West syndrome- what is the triad
What gene might be involved if ambiguous genitalia is seen?

A

Hypasrrhythmia, developmental delay and reduced iq- either pre or post
ARX gene

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16
Q

Severe generalised epilepsy
What is the triad of Lennox gastaut syndrome
What is seen on eeg
What may be in the history to make it more likely

What is a similar syndrome which is less severe (2 names

A

Difficult to control seizures of multiple types
Typical eeg
Severe dev delay

Slow background with spikes in the sleep

Falls

Dose syndrome- myotonic astatic epilepsy

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17
Q

Aicardi syndrome
What are the features on brain and mri
What types of seizures are likely what is the inheritance. Are girls or boys more likely to get it?

A

Retinal pits or lucencies
Agenesis of the corpus callosum
Infantile spasms
XLD. usually fatal in males

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18
Q

What is seen in a progressive myoclonic epilepsy?

What type is seen in children?

A

Dementia and seizures

Lofara disease

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19
Q

Landau kleffner syndrome
How does it present?
What gene is associated. What is the other presumed cause?
What is seen on eeg

A

Regressed language skills and verbal agnosia in a prev well child
Grin2A or autoimmune
Normal or high spikes in rem sleep or continuous spikes

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20
Q

Pyrodoxine deficient seizures

How do they present
What might be seen in utero
What is tested
How are they treated

A

Progressive seizures from infancy
Increased fetal movements
Urine or serum alpha aminoadipic semialdehyde (Aasa)
Give B6!

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21
Q 
Na valproate 
Side effects
Is it a p450 inducer
What happens with lamotrigine
When is it contraindicated
A

Weight gain, pancreatitis and low plt
No!
Increased risk of SJS
Under 2s

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22
Q

Vigabatrin

Side effects

A

Weight gain and retinopathy

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23
Q 
Carbamazepine 
Side effects 
HLA association
What happens with lamotrogine
What 2 seizure types is it contraindicated in
A

Rash and leukopenia
1502- increased risk for SJS
Increased effect of carbamazepine
Absence and JME

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24
Q

Levetiractam
Side effects
What neurotransmitter does it bind to

A

Fatigue and behaviour change

Synaptic vesicle protein

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25
Q

Topirimate

Side effects

A

Weight loss

Renal stones

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26
Q

Phenytoin
Side effects
Is it a p450 inducer?

A

Rash gum hypertrophy and megaloblastic anaemia

Yes!

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27
Q

SMA
Outline the basic physiology
What causes it?
What chromosome is coded for

A

Progressive degeneration of muscles due to loss to survivor muscle protein. Atrophy of anterior horn cells
Loss of Survivor motor neurone protein
Ch 5

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28
Q

SMA
Type and pattern of weakness?
Does it involve the heart?
What is the severe form called. What are the features
What is the less severe form and how do they differ

A

Progressive
Proximal>distal, lower limbs more than upper, symmetrical. Spares face!
No!
Type 1 werdning Hoffman. Never sit. Poor cry and suck. Frog legs. Areflexic
Type 2- present later, slowed milestones from 18m

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29
Q

SMA
What is the CK level?
What is seen on muscle biopsy?
What will the EMG and NCS show

What is the novel treatment. How is it given

A

Mildly elevated
Giant type 1 fibres and atrophy of other fibres
EMG- fibrillation
NCS- reduces motor amplitude

SMA antisense oligonucleotides- nusinersen- intrathecal

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30
Q

Myaesthenia Gravis
What is the most common cause
What happens

A

Autoimmune

Antibodies to acetylcholine receptors on the post synaptic membrane

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31
Q

Myasthenia gravis
Characteristic weakness
What can be the first sign on presentation- can it be unilateral?

A

Fatiguable. Proximal more than distal. Involves bulbar and diaphragm when severe
Eye signs- ptosis and ophthalmoloplegia. Yes!

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32
Q

Myaesthenia
How does a cholinergic crisis present
What usually causes it
How should it be treated

A

Sweating vomiting nausea and increased weakness
Too many anticholinesterases
Atropine

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33
Q

Myaesthenia
What is a myaesthenic crisis
How should it be treated

A

Sudden increased weakness

Anticholinesterases and resp support

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34
Q

Myaesthenia gravis
What antibodies are found
How many positive in children

A

Antiacetylcholine receptor-IgG

50% only

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35
Q

Myaesthenia

What is the difference between transient neonatal and congenital

A

Transient= mum had myaesthenia. Resolved in a few days. May rarely have arthrogryposis

Congenital= permanent lesions due to AR or AD disorder of NMJ
Don’t have antibodies

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36
Q

What poisoning may mimic myaesthenia

What other autoimmune disease might MG associate with

A

Organophosphates

Hypothyroidism

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37
Q 
Botulism 
Why does it cause weakness 
Characteristic pattern 
Associated signs 
What might precipitate it in a neonate
A

Produces a neurotoxin that binds to the post synaptic membrane
Descending paralysis with bulbar palsy
Dilated pupils
Gent or curling them up for a LP

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38
Q

Guillian barre
What causes the demyelination
What viruses are involved? Timing?
When should it be treated? How

A 
Monocytes damage the myelin scheath 
Zika
Campylobacter 
10d prior
If severe eg bulbar involvement or not walking- steroids and IVIG
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39
Q

Guillian barre
Pattern of weakness
Characteristic sign

A 
Distal to proximal- progressive over a few days
Symmetrical 
Can have sensory and bulbar involvement 
Lower motor neurone pattern
Reduced reflexes
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40
Q 
Miller Fischer syndrome 
What is it 
Pattern of weakness 
What type of encephalitis does it overlap with
What antibodies do the 2 associate with
A

Variant of guillian barre
Cranial nerve 6 ophthalmoplegia, ataxia and reduced reflexes
Bickerstaff
GQ1b

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41
Q

Guillian barre
What is seen on LP
What is seen on NCS
If it doesn’t recover what might it be called

A

High protein level
Reduced conduction velocity
Chronic inflammatory demyelinating polyradiculoneuropathy

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42
Q

ADEM
How quickly after a virus does it come on?
What are the symptoms and signs
What is the association with MOG abs

A

Headaches and back pains with meningitis and confusion- encephalopathy
UMN signs and incontinence
More likely to be recurrent- can therefore actually be MS

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43
Q

Neuromyelitis optica
What 2 ways can it present? Give specifics of the 2
What antibody is most likely involved
Management

A

Transverse myelitis- sudden onset para or quadraplegia. Usually longitudinally extensive
Optic neuritis- sudden eye pain, blindness, reduced visual fields or reduced colour vision
Aquaporin4

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44
Q

Anti MOG disease
How does it present?
Prognosis?

A

Relapsing ADEM or NMo less rarely

Good prognosis- much better than aquaporin 4

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45
Q

What is the difference between a dystrophy and a myopathy?

What is the usual pattern of weakness in muscle disease? What is the exception.

A

Dystrophy= degeneration and damage to a previously normal muscle

Myopathy=functional muscle issues- prev abnormal architecture

Proximal>distal weakness unless myotonic MD

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46
Q

Muscular dystrophies
What is the genetic cause?
What happens in duchennes and Becker’s
What is the inheritance of duchennes

A

Defect in x21.2
Duchennes=non sense mutations normally deletions, lack dystrophin protein
Becker’s=misense, reduces amounts
XLD

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47
Q

Duchennes
What is the classical disease pattern
What are the signs in terms of pattern of weakness
Do they have fasiculations

A

Normal at birth, delayed motor milestones, don’t walk, positive grower and calf pseudohypertrophy, waddling gait. Face gets weaker with age. Wheelchair by 10. Death by 21-resp causes
Symmetrical, proximal, LMN signs
No!

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48
Q

Duchennes
What other body system is likely to be involved
When is treatment needed?
Is the IQ normal?

A

Heart- cardiomyopathy
When EF<55%
No!

49
Q

Duchennes

What medication is proven to be useful?

A

Prednisolone!

50
Q

Becker
Does it involve girls
How does it present
What is the IQ

A

No- XLD too
Normal until adolescence then weak with pseudohypertrophy and cardiomyopathy death in adulthood
Usually more normal

51
Q

What happens to CK levels over life in myotonic dystrophies
Will it be increased in carriers
What hat body system do carriers need screened

A

High +++ then reduces
Yes
Cardiac screening

52
Q

Facioscapulohumeral MD
what is the inheritance
What is the pattern of weakness
What about the weakness is telling

A

AD. Shows anticipation
face first- can’t close eyes when sleeping, puckered Lips. Hearing loss. Winged scapulae. Hips increasingly weak- trendelenberg and positive gower sign
Asymmetrical

53
Q

Limb girdle
How does it present
What is the inheritance

A

Back pain= lumbar lordosis then progressive weakness becoming more distal
AR

54
Q

Emery Dreifuss
What is the pattern of weakness
What other system is severely involved
What do they not have

A

Severe resp weakness and wasting
Severe Dilated cardiomyopathy
No ID or pseudohypertrophy

55
Q

Congenital muscular dystrophies
What are they usually linked to?
How do they present?
What are 2 examples

A

Structural brain anomalies- polymicrogyra, lisencephaly
From birth- low tone, reduced fetal movements, arthrogryposis and contractures
Fukuyama. Walker Warburg syndrome

56
Q

Myotonic muscular dystrophy
What is the inheritance
What is the gene defect -How many causes disease

A

AD showing anticipation

CTG repeats on ch 20->50=pathological

57
Q 
Myotonic MD 
What is the classical presentation-
- weakness 
- 3 other systems 
Is the IQ normal?
Will the CK be raised
A

From childhood- distal weakness with wasting, facial involvement with triangle mouth, high arched palate and tongue wasting
Myotonic- contract and won’t relax
Cataracts
Cardiomyopathy
Endocrine- balding, hypothyroid, adrenal insufficiency
No- mild to mod defect
May be normal or slightly elevated

58
Q

Myotonic MD
How does the severe congenital form present
How many CTG repeats

A

Severe weakness from birth requiring resp support.
Involves the face
>1000

59
Q

Myotonic MD
what is seen on EMG
What might help the weakness?

A

Dive bomber response- continual discharges

Phenytoin

60
Q

Nemaline rod myopathy
Type of inheritance
Pathophysiology

A

AR

Formation of rods within muscles- excessive z band formation and reduced contractility

61
Q

Nemaline rod myopathy
Typical pattern of disease
Typical facial features
Back features

A

Severely weak from birth- distal and proximal. Not progressive but complications
Long face with mouth always open- weak masseters. Facial wasting
Scoliosis and thin muscles to the back

62
Q

Central core disease
pathophysiology
Inheritance

A

Muscle fibres replaced by large chunks of cytoplasm

AD

63
Q

Central core disease
Anaesthetic risk
Likely presenting factor

A

Malignant hypothermia

Congenital hip dysplasia

64
Q

Anti NMDR encephalitis
Classic presentation
Prognosis

A

Psychosis with looping movements- face eyes and limbs

Poor-usually listing defects and long admission required

65
Q

Myotubular myopathy
Pathophysiology
Inheritance
Prognosis

A

Muscles stop developing from around 16 weeks- myotubular phase
XLD
Poor- most die in weeks

66
Q

Pompes disease
Deficiency?
Does it have facial features

A

Maltase

No!

67
Q

Which myopathy causes “muscle builder children”

A

Myotonia congenita

68
Q

What are four CNS causes of neonatal hypotonia

A 
Hypothyroidism 
HIE 
malformations 
T21
Zelwegger syndrome 
Prader willi 
Beckwith widemann- hypoglycaemia
69
Q

Erbs palsy
Pattern of weakness
Inner action involved

A

Arm abducted fingers flexed

C5/6/7

70
Q

Klumpe palsy
Pattern of weakness
Innervation involved

A

Claw hand

C8/T1

71
Q 
Pattern of weakness-
Axillary 
Radial 
Musculocutaneous 
Median
Ulnar
Long thoracic
Sciatic 
Common peroneal
Tibial
A 
Badge sensory loss
Wrist drop
Sensory loss to thumb and forearm, reduced bicep
Lost pincer, thenar wasting
Claw hand
Scapular winging
Can’t flex knee, foot drop
Foot drop
Sole of foot
72
Q

Charcot Marie tooth disease
Gene
Inheritance

A

CMT1

AD

73
Q

Charcot Marie tooth disease
Clinical features
Is the lifespan normal

A 
Unsteadiness then distal weakness. Leg>arms 
Foot drop and reduced handwriting 
Claw hand and pes cavus 
Toe walking 
Pain and paraesthesia

Yes!

74
Q

Charcot Marie tooth disease
What investigation is diagnostic
What is found

A

Sural nerve biopsy- increased collagen and onion bulbs

75
Q

NF1
What chromosome
What is the inheritance?

A

17

AD

76
Q

NF1
What are the 7 diagnostic features
How many do you need to make a diagnosis

A

1) family history
2) >6 cafe au laits >5mm pre puberty, >15 post
3) axillary/ inguinal freckling
4) lisch nodules
5) plexiform neurofibromas
6) optic nerve gliomas
7) fibrous dysplasia- sphenoid wing or pseudoarthrosis

77
Q

NF1

3 Neuro complications

A

Seizures
Macrocephaly
Low IQ

78
Q

NF1
Renal complication
Cardiac complications
Back complications

A

Renal artery stenosis and hypertension
Pulmonary stenosis and coarctation of aorta
Scoliosis

79
Q

What is another syndrome that causes cafe au lait spots

What else do you see

A

McCune Albright syndrome

Precocious puberty, fibrous dysplasia and hyperthyroid

80
Q

NF 2
What is the most common tumour type
What are 2 other tumour types

A

Bilateral acoustic neuromas

Meningiomas, ependymomas

81
Q

TS
Inheritance
How many lesions are sporadic

A

AD

50%

82
Q 
TS 
Skin lesions 
Heart lesions 
Where in the brain are tubers 
Findings in infants
A

Shagreen patch, ash leaf macules, angiomyolipomas to the face
Rhabdomyomas
Subependymal
Infantile spasms with macules in woods lamp

83
Q

What is different in how craniopharyngiomas and pituitary adenomas present

A

Craniopharyngioma- growth slows with visual field defect and headache. DI
Pituitary adenoma- OVERGROWTH with high prolactin

84
Q

What reduces lamotrigine concentration

What increases it

A

Carbamazepine

Na valproate

85
Q 
Sturge Weber 
3 main features 
What is the pathophysiology 
Genetics 
What is seen on ct
A

Port wine stain to face, glaucoma and seizures with hemipariesis
Vascular malformations
Sporadic
Railroad calcification

86
Q

Von hippel Lindau

Types of tumours (4)

A

Cerebellar hamartomas
Retinal angioplasty
Renal cysts
Phaeochromocytomas

87
Q 
Incontientia pigmenti
4 stages of the rash
Characteristic facial feature
Gene 
Do they have seizures and low iq 
The rash in inverse- what is it called. What is the usual genetic pattern
A

Vesicular, verrucous, hyperpigmented then hypopigmented
Conical teeth
NEMO
Yes!
Hypomelanosis of Ito. Sporadic or mosaicism

88
Q

PHACE syndrome
What are the features
Genetics

A 
P- posterior fossa malformations 
H- large facial haemangioma 
A- arterial anomalies- aplastic coronary, aberrant L subclavian 
C- coarctation 
E-eyes- cataract, glaucoma
Unknown
89
Q

Benign intracranial hypertension
How high does the opening pressure need to be
Give 4 associations
What cool sign might been seen on the head

A

> 20mmhg
Pregnancy, female, doxycycline, sinusitis
Cracked pot sign

90
Q

Hydrocephalus
What is the normal pattern of csf flow?
What are the 2 types of hydrocephalus?
Which is the most common subtype

A

Made in the choroid plexus in lateral ventricle, to the 3rd ventricle via foramen of Munro, to the 4th via acqueduct of silvius

Obstructive=most common
Communicative

91
Q

Chiari malformations
What happens in type 1. What is it associated with.
When and how does it present
“ type 2

A

Herniation of cerebellar tonsils.
Syringiomyelia
Teenagers with headaches, neck pains and spasticity

Cerebellum, pons, medulla and 4th ventricle into the cervical canal- myelomeninocoele
Infants with incoordination and spasticity

92
Q

Structurally what is a dandy walker malformation

What is the associated sign

A

Agenesis of the corpus callosum and cerebellar vermis, cystic expansion of the 4th ventricle
Translumination

93
Q

What might a cranial bruit with high output cardiac failure indicate

A

Valen of glen malformation

94
Q

Spina Bifida
On amniocentesis what is seen
Where are the majority of lesions found
What is the asymptomatic form called

A

Raised AFP
Lumbosacral
Spina bifida occulta

95
Q

Spina bifida cystica
What is the most common subtype?
What is another common subtype? What is it commonly associated with?

A

Myelomeningocoele

Meningocoele- cord tethering, syrinx and distematomyelia

96
Q

What is the most common organism found in discitis

A

Staph aureus

97
Q

What is found on CT
Subdural haematoma
Extradural haematoma

A

Crescent shaped

Convex shaped

98
Q 
Ataxia teliengiectasia 
When does it present 
How does it present 
2 other complications 
What should be avoided 
What is the pathognomic blood test
A 
Late childhood 
Teliengiectasia of eyes and skin, progressive ataxia needing a wheelchair by 10 yrs old 
Immunodeficiency and tumours 
Radiation 
AFP
99
Q 
Fredreich ataxia 
What is the gene defect 
What is the inheritance 
When does it present 
Features- heart,skeleton,neuro
How is it diagnosed
A 
Triplet repeat-GAA- abnormal fraxin
AR 
Later than ataxia teliengiectasia 
Cardiomyopathy, absent reflexes and scoliosis
Ataxia with peripheral neuropathy
Chromosomal breakage studies
100
Q 
Abetalipoproteinaemia
What is it? 
Gi presentation 
Neuro presentation 
Eye signs 
What is seen on blood film
A 
Disordered lipid metabolism 
Steatorrhoea and FTT
Ataxia with glove and stocking sensory loss
Retinitis pigmentosa 
Acanthyocytes
101
Q

Childhood stroke
Most common cause
What syndrome might be present with carotid artery narrowing
What metabolic condition can cause recurrent stroke like episodes

A

Arteriopathy
Moya moya
MELAS

102
Q

Which disorder of neuronal migration is miller former syndrome associated with?

A

Lissencephaly

103
Q

Which disorder of lipid metabolism can cause ataxia

What is seen on blood film

A

Abetalipoproteinaemia

Acanthocytes

104
Q

What does a brown sequard lesion show
Why?
What is the only spinal tract not
supplied by the anterior spinal artery

A

Ipsilateral loss of motor function, vibration and proprioception
Contra lateral loss of pain and temp
Spina thalamic tract decussates 1/2 levels not in the medulla

Dorsal column- prop and vibration sense

105
Q

What do the following features on nerve conduction studies tell you ?
Amplitude
Velocity
Repetitive testing

A

Amplitude- number of fibres
Velocity- myelination
Repetitive testing- NMJ

106
Q 
What is neuronal ceroid lipofuscinosis otherwise called?
What is missing 
How do they present 
What is the pathognimic investigation 
What is the treatment
A

Battens disease
TPP1 enzyme
Language regression and seizures- school age
Photoparoxysmal response at low flash frequencies
Enzyme replacement- cerolipase alfa

107
Q

What gene is usually associated with central hypoventilation syndromes
What is different about ROHHAD

A

Phox2B malformations

No “, rapid obesity and polydypsia

108
Q

What is the general aim of the ketogenic diet (2 things)
What type of meds should they not have
When fasting what should be avoided
Do ketone levels correlate to seizure frequency

A

High fat, low carb 4:2.
Aim ketones 2.5-5

No liquid meds
No dextrose containing fluids

No

109
Q

What causes a RAPD
What is seen?
What is it seen in?

A

Pre chiasmatic lesion of CN 2
Normal constriction when normal pupil illuminated, dilatation of both eyes when abnormal eye illuminated
Optic neuritis

110
Q

What will show on a left INO

A

Left eye can’t adduct and right eye will have nystagmus when it abducts

111
Q

What are the afferent and efferent portions of the dolls eye reflex

A

Afferent-8

Efferent- 3,4&6

112
Q 
Embryology 
What is the neural tube derived from 
When does the brain from
What are the three primitive vesicles 
What forms the hemispheres, hypothalamus, midbrain, cerebellum and medulla
A

Ectoderm
Cranial part of the neural tube
Week 5
Prosencephalon, mesencephalon, rhombencephalon

Hemispheres- telencephalon
Hypothalamus- diencephalon
Midbrain- mesencephalon 
Cerebellum- metaencephalon 
Medulla- myelencephalon
113
Q

What is the first visual change to be seen on exam with benign intracranial hypertension. What also occurs.
How high does the opening pressure need to be: sedated. Not obese

A

Visual field defect
Visual acuity
Opening pressure >25-28

114
Q

Which cranial nerves are purely motor

How is this remembered?

A

3,4,6 and 12

All divide by 12

115
Q

Which cranial nerves are in the
Midbrain
Pons
Medulla

A

3-4
5-8
9-12

116
Q

Where does the facial nerve arose from

A

2nd pharyngeal arch

117
Q

What visual field defect can you get after epilepsy surgery

A

Contralateral upper quadrantanopia

Temporal lobe involvement

118
Q

What visual field defect is associated with occipital lobe damage

A

Homonymous hemianopia

FRACP (23 decks)

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