MedEd chronic neuro Flashcards
what is MS
a chroninc inflammatory multifocal demyelinating disease of the CNS
what are RF for MS?
nordic origin/ living at high latitude
autoimmune disease
female gender
why are nordic people/those who live at high latitudes at risk for ms?
they get less vit D and vit D is protective of MS
what are the patterns of MS and which is most common
relapsing remitting is most common
there is also primary and secondary progressive
what are signs and symptoms of MS?
optic neuritis motor weakness sensory disturbance faitgue hemiparesis lhermitte's sign- when they flex their leg forward they get an electric shock at the back of their neck
what sign is specific to MS, how do you ellicit it and what do you see?
lhermitte’s sign- when they flex their leg forward they get an electric shock at the back of their neck
how do you diagnose MS? what are the 3 catagories
absence of alternative diagnosis
dissemination in time
dissemination in space
what ix are done for MS? what will you see
hx
MRI with gadolinium contrast- old lesions are dark, new lesions are white
CSF- oligoclonal lesions in CSF but not in plasma
visually evoked potentials
what contrast is used in MRI for MS? why? what do you see?
gadolinium- it can show lesions over time
new lesions= white
old leasions= dark
what colour are old lesions on MRI in MS? explain why
dark- gadolinium leaks out of leaky capillaries so if there is an old lesion it won;t leak out as much
what colour are new lesions on MRI in MS? explain why
white- gadolinium leaks out of leaky capillaries so if there is an new lesion it leaks out a lot
what imaging is used in MS to see lesions?
MRI with gadolinium contrats
what is seen in csf in MS? where is this not seen?
oligoclonal bands
this is not seen in the serum
when you see an SBA about diagnosis of MS what should you think?
Which answer shows that there is both dissemination in time and in space?
what is MG?
autoantibodies against acetylcholine at neuromuscular jucntion
what antibodies are produced in MG?
anti acteylcholine
anti MuSK
what gland is often associated with MG?
thymus
who is more likely to get MG?
young women
old men
what are signs and symptoms for MG?
ptosis diplopia dysathria dysphagia SOB normal reflexes
what is the key defining feature about how muscles are affected in MG?
the more you use them the weaker they get
how do you differentiate MG from lambert eaton myasthenic syndrome?
there is hyperreflexia in LEMS but reflexes are normal in MG
what specific test can be done for MG, how is it done and what is seen?
simpson plus recovery test- you ask them to look up for 10 seconds then close thier eyes then open the eyes.
when they open the eyelids there will be ptosis in MG
what ix are done for MG? why are they done and what will you see?
bloods- anti acetyl and anti MuSK antibodies (these can be negative!!)
EMG- as you keep stimulating a nerve it will fire less and less as the condition is fatigueable
CT/MRI- to identify thymus involvement
what chronic neuro condition is the thymus associated with and involved in?
myasthenia gravis
what is MND?
build up of ubiquinated proteins in the cytoplasm causing muscle wasting and paralysis due to death os corticospinal tract
what is the prognosis of MND? how do people usually die?
3-4 years, due to resp failure
what are signs and symptoms of MND?
progressive muscle weakness dysphagia SOB wasting of thenar muscles wasting of tongue base
what is spared in MND?
oculumotor
sensory
autonomic function- no constipation etc
what are signs and symptoms of UMN lesions?
spasticity hypereflexia clonus positive babinski's sign paresis
what are signs and symptoms of LMN lesions?
hypotonia hyporeflexia muscle atrophy fasciculations and fibrillation paresis
what are the 3 types of parkinsonism? explain what they are
parkinsons disease
drug induced- symptoms of parkinsons disease but due to drugs not parkinsons disease pathophysiology
atypical parkinsonism- disorders that present like parkinsons disease but have different pathophysiolgy
what is the pathophysiology behind parkisnons disease?
loss of dopaminergic neurones in the substantia nigra in the midbrain
misfolded alpha synuclein builds up
lewy bodies and lewy neurites
where in the brain is the substantia nigra?
midbrain
what are rf for parkinsons?
age
living in the countryside
genetic (fhx)
what is the classic triad for parkinsons?
resting tremor
rigidity
bradykinesia
what are the 6 ms of parkinsons?
monotonous hypotnic speech micrographia- small handwritting march a petit pas- shuffling gait misery- depression memory loss- dementia hypomiMesis- lack of facial movement
what type of rigidity is seen in parkinsons?
cogwheel
how is huntigtons inheried?
autosomal dominant
in what gene is there a mutation in huntingtons? what is the mutation?
HTT- expansion of CAG trinucleotide
what is there atrophy of in the brain in huntingtons?
straitum
what is the role of huntington for proteins?
It stabilises it and prevents apoptosis
what are motor and cognitive symptoms of huntingtons?
motor= chorea, athetosis, motor, dysphagia, ataxia cognitive= depression, personality change, lack of concentration, dementia
what is the main motor symptom of huntingtons?
chorea
what is the main ix for huntingtons?
CAG repeat testing
how many CAG repeats do you need for huntingtons?
40 or more
how does increasing cag repeats affect huntingtons?
higher cags= earlier onset and more severe disease
what do you see on MRI in huntingtons?
straitum atrophy
what is dementia?
severe loss of memory and other cognitive abilities leading to impaired daily function
what are rf for dementia?
increasing age downs syndrome cardiovascular rf= hyperlipidaemia, hypertension head trauma (sports eg heading the ball in football)
what is pathophysiology in dementia?
build up of amyloid precursor protein
abnormal Abeta and gamma secretase is formed
extracellular amyloid plaques build up, intracellular neurofibrillary triangles made causing loss of neurones and synapses
what is present intra v extracellularly in dementia and what does this cause?
extracellular= amyloid plaques intracellular= neurofibrillary triangles
both cause a loss in neurones and synapses
what is protective of alzheimers?
exercise
education
what are the 5 as of alzheimers?
amnesia anomia- cant name things (they know what a book is but they cant think of the word book when asked to say what it is) apraxia- cant carry out normal tasks agnosia- cant recognise things aphasia- cant speak
how is alzheimer’s diagnosed?
Clinically based on hx
what would you see on csf in alzheimer’s?
high tau and low beta amyloid
what ix might you do for alzheimers?
CT
MRI
PET
what cognitive tests might be done for someone with alzheimers?
MMSE
addenbrooke’s cognitive assessment
MOCA
what are signs and symptoms of frontotemporal dementia?
personality change disinhibition overeating prefer sweet foods emotional blunting relative preservation of memore
in what type of dementia is there relative preservation of memory?
fronto temproal
what is the pathophysiology behind vascular dementia?
tiny strokes and as the strokes occur it gets worse
affects small and medium vessels
this causes step wise progression
what is progression of disease like in vascular dementia?
step wise progression
what is another name for frontotemporal dementia?
picks disease
what is picks disease?
fronto temporal dementia
what is another name for MND?
ALS
what is wernicke’s encephalopathy?
thiamine deficiency
what are rf for wernicke’s encpehaloapthy?
alcoholism- decreases thiamine absorption and they eat less
malabsorption
bariatric surgery
dialysis- may dialyse the thiamine out
what is there build up of in the brain in hepatic encephalopathy?
ammonia
what is the triad for wernicke’s encephalopathy?
ataxia
eye signs
confusion
what are signs and symptoms of wernickes encephalopathy?
ataxia confusion eye signs alcoholism malnourishment
what are ix for wernickes encephalopathy?
Bloods- albumin, vit b1, LFTs
ECG
CT
neuropsychology- check if they are cinfused
how serious is wernicke’s encephalopathy?
very it is a medical emergency and must be treated straight away
what is korsakoff’s encephalopathy?
chronic wernicke’s encephalopathy
how is korsakoff’s encephalopathy different to wernicke’s?
korsakoffs is chronic and there is no confusion
wernicke’s is acute and there is confusion
what are signs and symptoms of korsakoff’s encephalopathy?
chronic and alert
amnesia and confabulation
irreversible
