MedEd chronic neuro

Question 1

what is MS

Answer 1

a chroninc inflammatory multifocal demyelinating disease of the CNS

Question 2

what are RF for MS?

Answer 2

nordic origin/ living at high latitude
autoimmune disease
female gender

Question 3

why are nordic people/those who live at high latitudes at risk for ms?

Answer 3

they get less vit D and vit D is protective of MS

Question 4

what are the patterns of MS and which is most common

Answer 4

relapsing remitting is most common

there is also primary and secondary progressive

Question 5

what are signs and symptoms of MS?

Answer 5

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

Question 6

what sign is specific to MS, how do you ellicit it and what do you see?

Answer 6

lhermitte’s sign- when they flex their leg forward they get an electric shock at the back of their neck

Question 7

how do you diagnose MS? what are the 3 catagories

Answer 7

absence of alternative diagnosis
dissemination in time
dissemination in space

Question 8

what ix are done for MS? what will you see

Answer 8

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

Question 9

what contrast is used in MRI for MS? why? what do you see?

Answer 9

gadolinium- it can show lesions over time
new lesions= white
old leasions= dark

Question 10

what colour are old lesions on MRI in MS? explain why

Answer 10

dark- gadolinium leaks out of leaky capillaries so if there is an old lesion it won;t leak out as much

Question 11

what colour are new lesions on MRI in MS? explain why

Answer 11

white- gadolinium leaks out of leaky capillaries so if there is an new lesion it leaks out a lot

Question 12

what imaging is used in MS to see lesions?

Answer 12

MRI with gadolinium contrats

Question 13

what is seen in csf in MS? where is this not seen?

Answer 13

oligoclonal bands

this is not seen in the serum

Question 14

when you see an SBA about diagnosis of MS what should you think?

Answer 14

Which answer shows that there is both dissemination in time and in space?

Question 15

what is MG?

Answer 15

autoantibodies against acetylcholine at neuromuscular jucntion

Question 16

what antibodies are produced in MG?

Answer 16

anti acteylcholine

anti MuSK

Question 17

what gland is often associated with MG?

Answer 17

thymus

Question 18

who is more likely to get MG?

Answer 18

young women

old men

Question 19

what are signs and symptoms for MG?

Answer 19

ptosis
diplopia
dysathria
dysphagia
SOB
normal reflexes

Question 20

what is the key defining feature about how muscles are affected in MG?

Answer 20

the more you use them the weaker they get

Question 21

how do you differentiate MG from lambert eaton myasthenic syndrome?

Answer 21

there is hyperreflexia in LEMS but reflexes are normal in MG

Question 22

what specific test can be done for MG, how is it done and what is seen?

Answer 22

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

Question 23

what ix are done for MG? why are they done and what will you see?

Answer 23

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

Question 24

what chronic neuro condition is the thymus associated with and involved in?

Answer 24

myasthenia gravis

Question 25

what is MND?

Answer 25

build up of ubiquinated proteins in the cytoplasm causing muscle wasting and paralysis due to death os corticospinal tract

Question 26

what is the prognosis of MND? how do people usually die?

Answer 26

3-4 years, due to resp failure

Question 27

what are signs and symptoms of MND?

Answer 27

``` progressive muscle weakness dysphagia SOB wasting of thenar muscles wasting of tongue base ```

Question 28

what is spared in MND?

Answer 28

oculumotor sensory autonomic function- no constipation etc

Question 29

what are signs and symptoms of UMN lesions?

Answer 29

``` spasticity hypereflexia clonus positive babinski's sign paresis ```

Question 30

what are signs and symptoms of LMN lesions?

Answer 30

``` hypotonia hyporeflexia muscle atrophy fasciculations and fibrillation paresis ```

Question 31

what are the 3 types of parkinsonism? explain what they are

Answer 31

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

Question 32

what is the pathophysiology behind parkisnons disease?

Answer 32

loss of dopaminergic neurones in the substantia nigra in the midbrain misfolded alpha synuclein builds up lewy bodies and lewy neurites

Question 33

where in the brain is the substantia nigra?

Answer 33

midbrain

Question 34

what are rf for parkinsons?

Answer 34

age living in the countryside genetic (fhx)

Question 35

what is the classic triad for parkinsons?

Answer 35

resting tremor rigidity bradykinesia

Question 36

what are the 6 ms of parkinsons?

Answer 36

``` monotonous hypotnic speech micrographia- small handwritting march a petit pas- shuffling gait misery- depression memory loss- dementia hypomiMesis- lack of facial movement ```

Question 37

what type of rigidity is seen in parkinsons?

Answer 37

cogwheel

Question 38

how is huntigtons inheried?

Answer 38

autosomal dominant

Question 39

in what gene is there a mutation in huntingtons? what is the mutation?

Answer 39

HTT- expansion of CAG trinucleotide

Question 40

what is there atrophy of in the brain in huntingtons?

Answer 40

straitum

Question 41

what is the role of huntington for proteins?

Answer 41

It stabilises it and prevents apoptosis

Question 42

what are motor and cognitive symptoms of huntingtons?

Answer 42

``` motor= chorea, athetosis, motor, dysphagia, ataxia cognitive= depression, personality change, lack of concentration, dementia ```

Question 43

what is the main motor symptom of huntingtons?

Answer 43

chorea

Question 44

what is the main ix for huntingtons?

Answer 44

CAG repeat testing

Question 45

how many CAG repeats do you need for huntingtons?

Answer 45

40 or more

Question 46

how does increasing cag repeats affect huntingtons?

Answer 46

higher cags= earlier onset and more severe disease

Question 47

what do you see on MRI in huntingtons?

Answer 47

straitum atrophy

Question 48

what is dementia?

Answer 48

severe loss of memory and other cognitive abilities leading to impaired daily function

Question 49

what are rf for dementia?

Answer 49

``` increasing age downs syndrome cardiovascular rf= hyperlipidaemia, hypertension head trauma (sports eg heading the ball in football) ```

Question 50

what is pathophysiology in dementia?

Answer 50

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

Question 51

what is present intra v extracellularly in dementia and what does this cause?

Answer 51

``` extracellular= amyloid plaques intracellular= neurofibrillary triangles ``` both cause a loss in neurones and synapses

Question 52

what is protective of alzheimers?

Answer 52

exercise | education

Question 53

what are the 5 as of alzheimers?

Answer 53

``` 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 ```

Question 54

how is alzheimer's diagnosed?

Answer 54

Clinically based on hx

Question 55

what would you see on csf in alzheimer's?

Answer 55

high tau and low beta amyloid

Question 56

what ix might you do for alzheimers?

Answer 56

CT MRI PET

Question 57

what cognitive tests might be done for someone with alzheimers?

Answer 57

MMSE addenbrooke's cognitive assessment MOCA

Question 58

what are signs and symptoms of frontotemporal dementia?

Answer 58

``` personality change disinhibition overeating prefer sweet foods emotional blunting relative preservation of memore ```

Question 59

in what type of dementia is there relative preservation of memory?

Answer 59

fronto temproal

Question 60

what is the pathophysiology behind vascular dementia?

Answer 60

tiny strokes and as the strokes occur it gets worse affects small and medium vessels this causes step wise progression

Question 61

what is progression of disease like in vascular dementia?

Answer 61

step wise progression

Question 62

what is another name for frontotemporal dementia?

Answer 62

picks disease

Question 63

what is picks disease?

Answer 63

fronto temporal dementia

Question 64

what is another name for MND?

Answer 64

ALS

Question 65

what is wernicke's encephalopathy?

Answer 65

thiamine deficiency

Question 66

what are rf for wernicke's encpehaloapthy?

Answer 66

alcoholism- decreases thiamine absorption and they eat less malabsorption bariatric surgery dialysis- may dialyse the thiamine out

Question 67

what is there build up of in the brain in hepatic encephalopathy?

Answer 67

ammonia

Question 68

what is the triad for wernicke's encephalopathy?

Answer 68

ataxia eye signs confusion

Question 69

what are signs and symptoms of wernickes encephalopathy?

Answer 69

``` ataxia confusion eye signs alcoholism malnourishment ```

Question 70

what are ix for wernickes encephalopathy?

Answer 70

Bloods- albumin, vit b1, LFTs ECG CT neuropsychology- check if they are cinfused

Question 71

how serious is wernicke's encephalopathy?

Answer 71

very it is a medical emergency and must be treated straight away

Question 72

what is korsakoff's encephalopathy?

Answer 72

chronic wernicke's encephalopathy

Question 73

how is korsakoff's encephalopathy different to wernicke's?

Answer 73

korsakoffs is chronic and there is no confusion | wernicke's is acute and there is confusion

Question 74

what are signs and symptoms of korsakoff's encephalopathy?

Answer 74

chronic and alert amnesia and confabulation irreversible