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Neurology > Week 3 > Flashcards

Week 3 Flashcards

1
Q

what is the most common subtype of motor neurone disease?

A

Amyotrophic lateral sclerosis (ALS)

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1
Q

what is the median survival of MND after symptom onset

A

3 years

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2
Q

what is the average age of MND diagnosis

A

65

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3
Q

ALS is more common Caucasian populations, true or false

A

true

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4
Q

what comes under the umbrella term of ‘motor neurone disease’?

A
  • Amyotropic Lateral Sclerosis (ALS)
  • Progressive Muscular Atrophy
  • Primary Lateral Sclerosis
  • Progressive Bulbar Palsy
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5
Q

Primary Lateral Sclerosis is common, true or false

A

false, it is very rare.

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6
Q

Primary Lateral Sclerosis prognosis

A

years and years to go!

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7
Q

is muscle wasting an upper or lower motor neurone sign?

A

lower

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8
Q

is increased tone (hypertonia) an upper or lower motor neurone sign?

A

upper

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9
Q

is hyperreflexia an upper or lower motor neurone sign?

A

upper

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10
Q

is extensor plantar responses (babinksi reflex) an upper or lower motor neurone sign?

A

upper

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11
Q

are fasciculations an upper or lower motor neurone sign?

A

lower

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12
Q

absent or reduced deep tendon reflexes - upper or lower motor neurone sign?

A

lower

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13
Q

spastic gait - upper or lower motor neurone sign?

A

upper

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14
Q

exaggerated jerk - upper or lower motor neurone sign?

A

upper

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15
Q

in general, what is the difference between upper motor neurone and lower motor neurone signs

A

upper - stiffness (‘spasticity’)

lower - muscle weakness and twitching

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16
Q

what is the most common site of onset of MND

A

a limb

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17
Q

is primary bulbar onset of MND more common in men or women

A

women

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18
Q

bulbar dysfunction

A
  • wasted tongue
  • rippling tongue
  • weak droopy face
  • difficulty closing mouth properly (so can get oral thrush)
  • dry mouth
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19
Q

C9orf72*

A

gene to do with dementia in MND

frequent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

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20
Q

why is nutritional support particularly important in bulbar MND?

A

make sure airway and swallowing are safe - prevent aspiration

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21
Q

would these ALS variant good or bad prognosis:
- flail arm syndrome
- flail leg syndrome
- primary lateral sclerosis

A

good (well better anyway. more benign prognosis)

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22
Q

is motor neurone disease usually upper, lower or mixed motor neurone signs?

A

mixed

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23
Q

true or false: weight loss is expected in MND

A

true
- hypermetabolism

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24
Q

does gastrostomy prolong survival in MND?

A

no

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25
Q

treatment for muscle cramps in MND

A
  • baclofen
  • quinine
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26
Q

treatment for muscle spasms in MND

A
  • baclofen
  • tizanidine
  • dantrolene
  • gabapentin
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27
Q

treatment for shortness of breath in MND

A

lorazepam

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28
Q

treatment for coughing in MND

A
  • breath stacking
  • cough assist
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29
Q

what percentage of MND patients have cognitive impairment?

A

50%

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30
Q

making muscle contract simple pathway

A

motor neurone –> neuromuscular junction –> muscle

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31
Q

neuromuscular junctions have invaginations, true or false

A

true. they are flattened in disease

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32
Q

what is the motor end plate

A

the synapses formed between motor neurons and muscle

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33
Q

which are more common in disorders of the neuromuscular junction: pre-synaptic issues or postsynaptic issues

A

postsynaptic

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34
Q

who is most at risk of botulism

A

IV drug users

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35
Q

is myasthenia gravis a pre or postsynaptic issue

A

post

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36
Q

what is the most common neuromuscular junction disorder

A

myasthenia gravis

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37
Q

what are the antibodies in the majority of myasthenia gravis cases

A

antibodies against the muscle acetylcholine receptor (AChR)

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38
Q

what percentage of acetylcholine receptors are lost before symptoms occur in myasthenia gravis

A

30%

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39
Q

myasthenia gravis symptoms

A
  • extraocular weakness
  • facial and bulbar weakness
  • weakness typically fluctuating
  • muscle fatiguability (get them to blink 10 times for example)
  • limb weakness typically proximal
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40
Q

what percentage of myasthenia gravis patients have hyperplasia or thymoma

A

75%

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41
Q

acute treatment for myasthenia gravis

A
  • acetylcholinesterase inhibitor - pyridostigmine (symptoms relief only)
  • IV immunoglobulin - acute rescue for severe case
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42
Q

what is Pyridostigmine used for

A

symptomatic treatment of myasthenia gravis

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43
Q

name a drug that should be avoided in myasthenia gravis patients

A

gentamicin

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44
Q

what are fasciculations?

A

visible, fast, fine, spontaneous twitch

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45
Q

what is myotonia

A

failure of muscle relaxation after use

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46
Q

name 2 immune-mediated muscle diseases

A
  • dermatomyositis
  • polymyositis
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47
Q

polymyositis CK level

A

raised

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48
Q

what is the commonest muscular dystrophy

A

myotonic dystrophy

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49
Q

myotonic dystrophy mode of inheritance

A

autosomal dominant

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50
Q

what are the most common muscular dystrophies?

A
  • Duchenne’s
  • Becker’s
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51
Q

what would grade 5 in muscle power grading suggest

A

normal strength

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52
Q

peripheral nerve damage - what two ways can they be damaged

A
  • axonal loss
  • peripheral nerve demyelination
53
Q

how do we determine when peripheral nerves are damaged by axonal loss or demyelination

A

electrical study

54
Q

true or false, pain is very common in Guillain barre

A

true

55
Q

what days into onset do the symptoms of Guillain barre peak?

A

days 10-14

56
Q

Guillain barre syndrome treatments

A

IV infusion of immunoglobulin

and/or plasma exchange

57
Q

hereditary neuropathy - what signs can be seen in the feet?

A

pes cavus

58
Q

treatment of axonal (vasculitic) peripheral neuropathy

A

pulsed IV methylprednisolone and cyclophosphamide

59
Q

Is multiple sclerosis a CNS or PNS problem

A

CNS

60
Q

clinical courses of MS

A
  • relapsing remitting
  • secondary progressive
  • primary progressive
61
Q

what is the condition: “plaques are disseminated in time and place”

A

Multiple sclerosis

62
Q

is MS more common in males or females

A

females

63
Q

is optic neuritis painful or painless vision loss

A

painful

64
Q

what causes internuclear ophthalmoplegia

A
  • MS (very common)
  • stroke
65
Q

what is internuclear ophthalmoplegia

A

when the two eyes don’t move together.

Message gets to one before the other, e.g. lateral rectus fine, but medial rectus (of other eye?) slow

66
Q

differentials of MS

A
  • vasculitis
  • granulomatous disorder
  • vascular disease
  • structural lesion
  • infection
  • metabolic disorder
67
Q

on investigation of CSF, is the presence of oligoclonal bands normal or abnormal?

A

abnormal

68
Q

MS acute relapse treatment

A

mild - symptomatic treatment

moderate - oral steroids

severe - admit/IV steroids

69
Q

MS sensory symptoms management

A
  • anticonvulsant e.g. gabapentin
  • antidepressant e.g. amitriptyline
  • tens machine
  • acupuncture
70
Q

MS lower urinary tract dysfunction treatment

A
  • bladder drill
  • anticholinergics e.g. oxybutynin
  • desmopressin
  • catheterisation
71
Q

what symptom can amantadine help in MS?

A

fatigue

72
Q

disease modifying therapy first line therapy for MS

A
  • Tecfidera, Aubagio
  • interferon Beta
  • Glatiramer Acetate
73
Q

Tecfidera administration route

A

oral (tablet)

74
Q

disease modifying therapy second line therapy for MS

A
  • fingolimod
  • cladrabine
  • monoclonal antibody
75
Q

which MS treatment does the patient need to be JC virus negative for?

A

the monoclonal antibodies

76
Q

parkinson’s pathology

A

loss of dopaminergic neurons from the pars compacta region of the substantia nigra

77
Q

what % of neurons are lost in parkinson’s before symptoms?

A

60% loss

78
Q

where are the basal ganglia

A

near the centre of your brain

79
Q

what movement disorder group does parkinsons fit into

A

hypokinetic (stiff,slow)

80
Q

is parkinsons more common in males or females?

A

males

81
Q

what percentage of parkinson’s cases are idiopathic

A

95%

82
Q

what is the main symptom of parkinson’s disease required for diagnosis?

A

bradykinesia (slow movement)

83
Q

Parkinson’s motor symptoms

A
  • tremor
  • bradykinesia
  • postural instability - bending forward, shuffling gait, loss of arm swing
  • rigidity (test tone at wrist - lead pipe or cog wheel)
84
Q

parkinson’s non-motor symptoms

A
  • sleep disorders
  • hallucinations
  • GI dysfunction
  • depression
  • cognitive impairment/dementia
  • anosmia
85
Q

what is often an early sign of parkinson’s

A

constipation

86
Q

diagnosis of parkinsons

A

bradykinesia and one or more of:
- resting tremor
- rigidity (cog wheel or lead pipe)
- postural instability

87
Q

what is DaTSCAN for

A

can distinguish between essential and non-essential tremor.

so tells us if a tremor is because of parkinsons??

88
Q

parkinsons environmental risk factors

A
  • pesticide exposure
  • prior head injury
  • rural living
  • beta blocker use
  • agricultural occupation
  • well water drinking
89
Q

true or false, smoking, coffee, NSAID use, CCB use, and alcohol increase risk of parkinson’s

A

false, they decrease the risk!

90
Q

which is the best parkinsons drug

A

levodopa

91
Q

when would you consider a different drug to levodopa in parkinsons

A
  • patient under 50
  • motor symptoms are mild
  • patient preference
92
Q

which shows excellent bony detail - CT or MRI

A

CT

93
Q

if someone presents to a&E with stroke, which imaging are they likely to get

A

CT - cause faster than MRI

94
Q

two types of parasomnias

A

rem and non-rem

95
Q

which type of parasomnia is usually in the latter third of the night, dreaming

A

REM

96
Q

which type of parasomnia: confusional arousals, sleep walking, bruxism

A

non-REM

97
Q

what is bruxism

A

teeth grinding

98
Q

typical age of narcolepsy onset

A

teenage years

99
Q

which type of parasomnia is more common in kids

A

non-REM

100
Q

what condition does cataplexy occur as part of

A

narcolepsy

101
Q

what is cataplexy

A

a sudden loss of muscle tone and power in response to strong emotion - occurs as part of narcolepsy

102
Q

pathophysiology of narcolepsy

A

caused by abnormalities of the brain neurotransmitter hypocretin (orexin), which is a regulator of sleep

103
Q

are narcolepsy patients allowed to drive

A

once satisfactory control of symptoms has been achieved

104
Q

what are the two main bacteria causing meningitis?

A
  • strep pneumoniae (aka pneumococcus)
  • neisseria meningitidis (aka meningococcus)
105
Q

treatment of most bacterial meningitis

A

ceftriaxone high dose IV
steroids

106
Q

what additional medication is needed if the meningitis is caused by listeria

A

amoxicillin

107
Q

do you need to inform health protection in bacterial meningitis cases?

A

yes

108
Q

are there normally red blood cells in the CSF

A

no

109
Q

are there normally white cells in the csf

A

not many at all, maybe like 5

110
Q

what does a positive Kernig’s sign show?

A

pain when they bend over and putting neck forwards, basically showing that the meninges are inflamed (because they go all the way down the back) - meningitis

111
Q

treatment for viral meningitis

A

should be fine in a couple of weeks!
Acyclovir can be used to treat suspected or confirmed HSV or VZV infection

112
Q

most common cause of viral meningitis

A

enteroviruses

113
Q

what is encephalitis?

A

brain becomes inflamed

114
Q

viral encephalitis treatment

A

IV aciclovir

115
Q

hyperkinetic meaning

A

too much movement e.g. tremor

116
Q

hypokinetic meaning

A

too little movement, e.g. in parkinsons

117
Q

can you see an essential tremor at rest?

A

no, unless the essential tremor is severe

118
Q

are upper or lower limbs usually affected in essential tremor

A

upper

119
Q

when does tourettes start/onset of condition

A

childhood

120
Q

can you suppress tics

A

usually yes, although inner tension mounts then they can all come out in a flurry

121
Q

what is it called when we suddenly jerk in bed

A

physiological myoclonus

122
Q

dystonia onset

A

usually young, average age 12. but can get adult onset

123
Q

which part of the brain is degenerating in Alzheimer’s disease?

A

hippocampus
(and later the parietal lobes)

124
Q

prophylaxis drugs for migraines

A
  • propanolol
  • amitriptyline
125
Q

migraine acute medication

A

NSAIDs or triptans

126
Q

types of Trigeminal Autonomic Cephalgias

A
  • cluster headache (CH)
  • paroxysmal hemicrania (PH)
  • hemicrania continua (HC)
127
Q

cluster headache

A
  • very painful
  • worse if they smoke
  • evolves over minutes
128
Q

how long does a cluster headache last

A

average 90 mins, can have 1-8 times a day

129
Q

cluster headache acute treatment

A
  • high flow O2 100% for 20 mins
  • sumatriptan (injection subcutaneous 6mg)
130
Q

is hemicrania continua more commons in males or females

A

females

131
Q

what medication will hemicrania continua always respond to?

A

indomethacin

Neurology (12 decks)

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