Week 3 Flashcards
1
Q
what is the most common subtype of motor neurone disease?
A
Amyotrophic lateral sclerosis (ALS)
1
Q
what is the median survival of MND after symptom onset
A
3 years
2
Q
what is the average age of MND diagnosis
A
65
3
Q
ALS is more common Caucasian populations, true or false
A
true
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
5
Q
Primary Lateral Sclerosis is common, true or false
A
false, it is very rare.
6
Q
Primary Lateral Sclerosis prognosis
A
years and years to go!
7
Q
is muscle wasting an upper or lower motor neurone sign?
A
lower
8
Q
is increased tone (hypertonia) an upper or lower motor neurone sign?
A
upper
9
Q
is hyperreflexia an upper or lower motor neurone sign?
A
upper
10
Q
is extensor plantar responses (babinksi reflex) an upper or lower motor neurone sign?
A
upper
11
Q
are fasciculations an upper or lower motor neurone sign?
A
lower
12
Q
absent or reduced deep tendon reflexes - upper or lower motor neurone sign?
A
lower
13
Q
spastic gait - upper or lower motor neurone sign?
A
upper
14
Q
exaggerated jerk - upper or lower motor neurone sign?
A
upper
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
16
Q
what is the most common site of onset of MND
A
a limb
17
Q
is primary bulbar onset of MND more common in men or women
A
women
18
Q
bulbar dysfunction
A
- wasted tongue
- rippling tongue
- weak droopy face
- difficulty closing mouth properly (so can get oral thrush)
- dry mouth
19
Q
C9orf72*
A
gene to do with dementia in MND
frequent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)
20
Q
why is nutritional support particularly important in bulbar MND?
A
make sure airway and swallowing are safe - prevent aspiration
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)
22
Q
is motor neurone disease usually upper, lower or mixed motor neurone signs?
A
mixed
23
Q
true or false: weight loss is expected in MND
A
true
- hypermetabolism
24
does gastrostomy prolong survival in MND?
no
25
treatment for muscle cramps in MND
- baclofen
- quinine
26
treatment for muscle spasms in MND
- baclofen
- tizanidine
- dantrolene
- gabapentin
27
treatment for shortness of breath in MND
lorazepam
28
treatment for coughing in MND
- breath stacking
- cough assist
29
what percentage of MND patients have cognitive impairment?
50%
30
making muscle contract simple pathway
motor neurone --> neuromuscular junction --> muscle
31
neuromuscular junctions have invaginations, true or false
true. they are flattened in disease
32
what is the motor end plate
the synapses formed between motor neurons and muscle
33
which are more common in disorders of the neuromuscular junction: pre-synaptic issues or postsynaptic issues
postsynaptic
34
who is most at risk of botulism
IV drug users
35
is myasthenia gravis a pre or postsynaptic issue
post
36
what is the most common neuromuscular junction disorder
myasthenia gravis
37
what are the antibodies in the majority of myasthenia gravis cases
antibodies against the muscle acetylcholine receptor (AChR)
38
what percentage of acetylcholine receptors are lost before symptoms occur in myasthenia gravis
30%
39
myasthenia gravis symptoms
- extraocular weakness
- facial and bulbar weakness
- weakness typically fluctuating
- muscle fatiguability (get them to blink 10 times for example)
- limb weakness typically proximal
40
what percentage of myasthenia gravis patients have hyperplasia or thymoma
75%
41
acute treatment for myasthenia gravis
- acetylcholinesterase inhibitor - pyridostigmine (symptoms relief only)
- IV immunoglobulin - acute rescue for severe case
42
what is Pyridostigmine used for
symptomatic treatment of myasthenia gravis
43
name a drug that should be avoided in myasthenia gravis patients
gentamicin
44
what are fasciculations?
visible, fast, fine, spontaneous twitch
45
what is myotonia
failure of muscle relaxation after use
46
name 2 immune-mediated muscle diseases
- dermatomyositis
- polymyositis
47
polymyositis CK level
raised
48
what is the commonest muscular dystrophy
myotonic dystrophy
49
myotonic dystrophy mode of inheritance
autosomal dominant
50
what are the most common muscular dystrophies?
- Duchenne's
- Becker's
51
what would grade 5 in muscle power grading suggest
normal strength
52
peripheral nerve damage - what two ways can they be damaged
- axonal loss
- peripheral nerve demyelination
53
how do we determine when peripheral nerves are damaged by axonal loss or demyelination
electrical study
54
true or false, pain is very common in Guillain barre
true
55
what days into onset do the symptoms of Guillain barre peak?
days 10-14
56
Guillain barre syndrome treatments
IV infusion of immunoglobulin
and/or plasma exchange
57
hereditary neuropathy - what signs can be seen in the feet?
pes cavus
58
treatment of axonal (vasculitic) peripheral neuropathy
pulsed IV methylprednisolone and cyclophosphamide
59
Is multiple sclerosis a CNS or PNS problem
CNS
60
clinical courses of MS
- relapsing remitting
- secondary progressive
- primary progressive
61
what is the condition: "plaques are disseminated in time and place"
Multiple sclerosis
62
is MS more common in males or females
females
63
is optic neuritis painful or painless vision loss
painful
64
what causes internuclear ophthalmoplegia
- MS (very common)
- stroke
65
what is internuclear ophthalmoplegia
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
differentials of MS
- vasculitis
- granulomatous disorder
- vascular disease
- structural lesion
- infection
- metabolic disorder
67
on investigation of CSF, is the presence of oligoclonal bands normal or abnormal?
abnormal
68
MS acute relapse treatment
mild - symptomatic treatment
moderate - oral steroids
severe - admit/IV steroids
69
MS sensory symptoms management
- anticonvulsant e.g. gabapentin
- antidepressant e.g. amitriptyline
- tens machine
- acupuncture
70
MS lower urinary tract dysfunction treatment
- bladder drill
- anticholinergics e.g. oxybutynin
- desmopressin
- catheterisation
71
what symptom can amantadine help in MS?
fatigue
72
disease modifying therapy first line therapy for MS
- Tecfidera, Aubagio
- interferon Beta
- Glatiramer Acetate
73
Tecfidera administration route
oral (tablet)
74
disease modifying therapy second line therapy for MS
- fingolimod
- cladrabine
- monoclonal antibody
75
which MS treatment does the patient need to be JC virus negative for?
the monoclonal antibodies
76
parkinson's pathology
loss of dopaminergic neurons from the pars compacta region of the substantia nigra
77
what % of neurons are lost in parkinson's before symptoms?
60% loss
78
where are the basal ganglia
near the centre of your brain
79
what movement disorder group does parkinsons fit into
hypokinetic (stiff,slow)
80
is parkinsons more common in males or females?
males
81
what percentage of parkinson's cases are idiopathic
95%
82
what is the main symptom of parkinson's disease required for diagnosis?
bradykinesia (slow movement)
83
Parkinson's motor symptoms
- tremor
- bradykinesia
- postural instability - bending forward, shuffling gait, loss of arm swing
- rigidity (test tone at wrist - lead pipe or cog wheel)
84
parkinson's non-motor symptoms
- sleep disorders
- hallucinations
- GI dysfunction
- depression
- cognitive impairment/dementia
- anosmia
85
what is often an early sign of parkinson's
constipation
86
diagnosis of parkinsons
bradykinesia and one or more of:
- resting tremor
- rigidity (cog wheel or lead pipe)
- postural instability
87
what is DaTSCAN for
can distinguish between essential and non-essential tremor.
so tells us if a tremor is because of parkinsons??
88
parkinsons environmental risk factors
- pesticide exposure
- prior head injury
- rural living
- beta blocker use
- agricultural occupation
- well water drinking
89
true or false, smoking, coffee, NSAID use, CCB use, and alcohol increase risk of parkinson's
false, they decrease the risk!
90
which is the best parkinsons drug
levodopa
91
when would you consider a different drug to levodopa in parkinsons
- patient under 50
- motor symptoms are mild
- patient preference
92
which shows excellent bony detail - CT or MRI
CT
93
if someone presents to a&E with stroke, which imaging are they likely to get
CT - cause faster than MRI
94
two types of parasomnias
rem and non-rem
95
which type of parasomnia is usually in the latter third of the night, dreaming
REM
96
which type of parasomnia: confusional arousals, sleep walking, bruxism
non-REM
97
what is bruxism
teeth grinding
98
typical age of narcolepsy onset
teenage years
99
which type of parasomnia is more common in kids
non-REM
100
what condition does cataplexy occur as part of
narcolepsy
101
what is cataplexy
a sudden loss of muscle tone and power in response to strong emotion - occurs as part of narcolepsy
102
pathophysiology of narcolepsy
caused by abnormalities of the brain neurotransmitter hypocretin (orexin), which is a regulator of sleep
103
are narcolepsy patients allowed to drive
once satisfactory control of symptoms has been achieved
104
what are the two main bacteria causing meningitis?
- strep pneumoniae (aka pneumococcus)
- neisseria meningitidis (aka meningococcus)
105
treatment of most bacterial meningitis
ceftriaxone high dose IV
steroids
106
what additional medication is needed if the meningitis is caused by listeria
amoxicillin
107
do you need to inform health protection in bacterial meningitis cases?
yes
108
are there normally red blood cells in the CSF
no
109
are there normally white cells in the csf
not many at all, maybe like 5
110
what does a positive Kernig's sign show?
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
treatment for viral meningitis
should be fine in a couple of weeks!
Acyclovir can be used to treat suspected or confirmed HSV or VZV infection
112
most common cause of viral meningitis
enteroviruses
113
what is encephalitis?
brain becomes inflamed
114
viral encephalitis treatment
IV aciclovir
115
hyperkinetic meaning
too much movement e.g. tremor
116
hypokinetic meaning
too little movement, e.g. in parkinsons
117
can you see an essential tremor at rest?
no, unless the essential tremor is severe
118
are upper or lower limbs usually affected in essential tremor
upper
119
when does tourettes start/onset of condition
childhood
120
can you suppress tics
usually yes, although inner tension mounts then they can all come out in a flurry
121
what is it called when we suddenly jerk in bed
physiological myoclonus
122
dystonia onset
usually young, average age 12. but can get adult onset
123
which part of the brain is degenerating in Alzheimer's disease?
hippocampus
(and later the parietal lobes)
124
prophylaxis drugs for migraines
- propanolol
- amitriptyline
125
migraine acute medication
NSAIDs or triptans
126
types of Trigeminal Autonomic Cephalgias
- cluster headache (CH)
- paroxysmal hemicrania (PH)
- hemicrania continua (HC)
127
cluster headache
- very painful
- worse if they smoke
- evolves over minutes
128
how long does a cluster headache last
average 90 mins, can have 1-8 times a day
129
cluster headache acute treatment
- high flow O2 100% for 20 mins
- sumatriptan (injection subcutaneous 6mg)
130
is hemicrania continua more commons in males or females
females
131
what medication will hemicrania continua always respond to?
indomethacin
