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