Neurology Flashcards

1
Q

What is essential tremor?

A

autosomal dominant tremor
affects both upper limbs

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2
Q

features of essential tremor

A

postural tremor : worse if arms outstretched. nothing on relaxed

improved by alcohol and rest

mc cause of titubation (head tremor)

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3
Q

management of essential tremor

A

1st line: propranolol

primidone sometimes

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4
Q

most common causes of dementia

A

1st: alzheimers
2nd: vascular and lewy body

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5
Q

assesment tools of dementia

A

delayed/difficult diagnosis

recommended assesment tool: 10-cs or 6CIT

non recommended but can do: AMTS, GPCOG, MMSE (24 or less/30 is dementia)

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6
Q

initial investigations in dementia

A

primary care: blood screen to exclude reversible causes eg hypothyroidism

fbc, u+e, lft, calcium, glucose , esr/crp, tft, vit b12, folate.

secondary care: neuroimaging - exclude subdural haematoma, normal pressure hydrocephalus.

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7
Q

Define Delirium

A

acute confusional state.
affects 30% of elderly admitted to hospital.

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8
Q

presdisposing factors to delirium

A

age>65
dementia background
significant injury eg hip fracture
polypharmacy
frailty/multimorbidity

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9
Q

precipitating factors for delirium

A

infection: uti
environmental change
severe pain
alcohol withdrawal
constipation
any significant cv, resp, neuro, endo condition
metabolic: hypercalcaemia, hypoglycaemia, hyperglycaemia, dehydration

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10
Q

features of delirium

A

memory disturbance (loss of short term>long term)
mood change
visual hallucination
disturbed sleep cycle
possibly agitated/withdrawn
poor attention
disoriented

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11
Q

how would you manage delirium?

A

tx underlying cause
change environment

haloperidol 0.5mg : 1st line sedative
possibly olanzapine

if parkinson patient, antipycotic worsen parkinsonism symptom - careful reduction of parkinson medication helpful.

if sx require urgent tx give atypical antipsychotic like quetiapine/clozapine

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12
Q

difference between delirium and dementia (favouring delirium)

A

acute onset
impaired conciousness
fluctuating symptoms - worse @ night, periods of normality

abnormal perception (illusions and hallucinations)

agitation, fear

delusions

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13
Q

depression vs dementia (favouring depression)

A

short history, rapid onset

patient worried about poor memory

reluctant taking tests, disappointed w/ results

MMSE: variable

global memory loss - dementia is recent memory loss

biological sx: wt loss, sleep disturbance

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14
Q

what is alzheimers?

A

progressive degenerative disease of the brain.

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15
Q

rf of alzheimers

A

increasing age
fhx of alzheimers
caucasian
downs syndrome
apoprotein E allele E4 - encodes a cholestrol transport protein

5% cases - inherited autosomal dominant trait. mutation in amyloid precursor protein (chr 21) , presenilin 1 (chr 14) and presenilin 2 (chr 1) genes - thought to cause inherited form

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16
Q

Pathological changes for alzheimers

A

macroscopic: widespread cerebral atrophy, particular involves cortex and hippocampus

microscopic: cortical plaques due to deposition of type A - beta - amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of tau protein
hyperphosphorylation of tau protein linked to AD

biochemical: deficit of acetylcholine from damage to an ascending forebrain projection

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17
Q

what are neurofibrillary tangles?

A

paired helical filaments made from tau protein.

in AD tau proteins excessively phosphorylated= impaired function

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18
Q

what is tau protein?

A

Alzhiemers

protein interacting with tubulin.
stabilises microtubules
promotes tubulin assembly into microtubules

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19
Q

how would you manage alzheimers? (non-pharmacological)

A

activities to promote wellbeing tailored to preference

group cognitive stimulation therapy - mild/moderate dementia

group reminiscence therapy , cognitive rehab

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20
Q

how would you manage alzheimers (pharmacological mx)

A

acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine) - mild/moderate

memantine - nmda receptor antagonist. - 2nd line or monotherapy in severe alzheimers

moderate to severe: acetylcholineesterase inhibitor + memantine

moderate alzheimers intolerant of/contraindication of acetylcholinesterase inhibitors: memantine

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21
Q

how would you manage non cognitive symptoms in alzheimers?

A

NO to antidepressants for mild/moderate depression in patients with dementia

antipsycotics only for patient at risk of harming themselves or others/agitation/hallucination/delusions causing severe distress (increased risk of mortality)

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22
Q

which drug is contraindicated in alzheimers treatement in patients with bradycardia?

A

donepezil (Acetylcholine esterase inhibitor)

adverse effect: insomnia

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23
Q

causes of dementia

A

common : alzheimers,
lewy body (10-20%), cerebrovascular disease : multi-infarct dementia (10-20%)

rarer (5%) - huntingtons, CJD, picks disease (atrophy of frontal/temporal lobes), HIV (50% of AIDS pts)

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24
Q

important differentials for dementia

A

hypothyroid, addisons
b12/folate/thiamine def
syphilis
brain tumour
normal pressure hydrocephalus
subdural haematoma
depression
chronic drug use: alcohol, barbiturates

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25
Q

what is lewy body dementia?

A

20% of dementia cases.

lewy bodies (alpha-synuclein cytoplasmic inclusions) in substantia nigra, paralimbic and neocortical areas.

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26
Q

where would you find lewy bodies

A

substantia nigra
paralimbic
neocortical areas

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27
Q

what percentage of alzherimers patients have lewy bodies?

A

40%

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28
Q

features of lewy body dementia

A

parkinsonism
visual hallucination - delusion and non-visual hallucination too)

progressive cognitive impairement - BEFORE PARKINSONISM. - parkinsons is motor symptoms a yr before cognitive.

FLUCTUATING COGNITION COMPARED WITH DEMENTIA

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29
Q

HOW DOES LEWY BODY DEMENTIA COMPARE WITH ALZHEIMERS

A

IN LEWY BODY EARLY IMPAIREMENT IN ATTENTION AND EXECUTIVE FUNCTION RATHER THAN JUST MEMORY LOSS.

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30
Q

diagnosis of lewy body dementia

A

clinical

single-photon emission computed tomography (SPECT) / DaTscan.
isotope used: 123-I-FP-CIT - radioisotope.

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31
Q

what is the sensitivity and specifity of SPECT in diagnosing lewy body dementia

A

sensitivity: 90%
specificity: 100%

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32
Q

how would you manage lewy body dementia?

A

acetylcholinesterase inhibitors - donepezil, rivastigmine)

AVOID NEUROLEPTICS - pts could get irreversible parkinsonism.

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33
Q

what drug should lewy body dementia patients avoid?

A

NEUROLEPTICS - can develop irreversible parkinsonism.

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34
Q

causes of parkinsonisms

A

parkinson’s
drug-induced eg antipsychotics, metoclopramide

progressive supranuclear palsy - neurological condition causes issue with balance, movement,vision,speech, swallowing.

wilsons disease
post-encephalitis
toxins: carbon monoxide, MPTP

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35
Q

what is the first line anti-emetic ( used for parkinsons?

A

domperidone - doesnt cross blood brain barrier.

no extra-pyramidal side effects (movement disorder, parkinonism, tremor)

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36
Q

what is vascular dementia?

A

group of syndromes of cognitive impairement caused by different mechanisms causing ischaemia or haemorrhage secondary to cerebrovascular disease.

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37
Q

epidemiology of vascular dementia

A

17% of uk dementia.
prevalence following first stroke depends on location/size of infarct, interval after stroke, age. overall stroke doubles risk.

incidence increase with age

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38
Q

subtypes of vascular dementia

A

stroke related - multi-infarct or single-infarct dementia

subcortical - caused by small vessel disease

mixed - presence of both VD and Alzheimers

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39
Q

risk factors of Vascular dementia

A

hx of tia
AF
HTN
DM
SMOKING
OBESITY
CORONARY HEART DISEASE
HYPERLIPIDEMIA
FHX OF STROKE/CV

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40
Q

can vascular dementia be inherited?

A

rarely

if so CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)

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41
Q

presentation of vascular dementia typically

A

several months/years of hx of a sudden or stepwise deterioration of cognitive function.

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42
Q

symptoms of vascular dementia

A

speed of progression and sx may vary :

seizures
gait disturbance
emotional disturbance
memory disturbance
attention and concentration difficulty
focal neurological abnormality: visual disturbance, sensory/motor sx.

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43
Q

how to make diagnosis of vascular dementia?

A

hx and physical exam
formal screen for cognitive impairement
medical review - exclude medication cause of cognitive decline

MRI - show infarcts and extensive white matter changes

44
Q

criteria for diagnosing vascular dementia

A

NINDS-AIREN

presence of cognitive decline interfering with ADLs, not due to secondary effects of cv event. (clinical exam and neuropsychological testing)

cv disease - neurological signs/brain imaging

relationship between the above 2 disorders:
- fluctuating,stepwise deterioration of cognitive deficit
- abrupt deterioration of cognitive function
- onset of dementia within 3 months following recognised stroke

45
Q

how would you manage vascular dementia?

A

symptomatic

non pharma:
cognitive stimulation programme
multisensory stimulation
music/art therapy, animal assisted therapy

address pain, avoid overcrowding, clear communication

pharma:
only with AChE inhibitor/memantine if have VD and Alzheimers,Parkinsons dementia or dementia w/ Lewy Bodies.

NO EVIDENCE OF ASPIRIN TREATING PTS WITH VD

no trails showed statins good to tx of vd

46
Q

what is normal pressure hydrocephalus?

A

reversible cause of dementia. elderly

secondary to reduced CSF absorption at the arachnoid villi.

could be due to head injury, subarachnoid haemorrhage or meningitis.

47
Q

classic triad of features seen in normal pressure hydrocephalus

A

urinary incontinence
dementia and bradyphrenia(slowness of thought)
gait abnormality - similar to parkinsons

sx typically develop over a few months

48
Q

imaging for normal pressure hydrocephalus

A

MRI - ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement

49
Q

how would you manage normal pressure hydrocephalus?

A

ventriculoperitoneal shunting

50
Q

potential complications of ventriculoperitoneal shunting treatment?

A

10% of patients

seizures
infection
intracerebral haemorrhages

51
Q

what is parkinsons diseases

A

progressive
neurodegenerative

caused by degeneration of dopaminergic neurones in substantia nigra. reduction in dopaminergic output results in triad of features.

52
Q

parkinson classic triad

A

bradykinesia - hypokinesia,short shuffling steps , reduced arm swing, difficulty initiating movement

tremor - at rest, 3-5 hz, worse when stressed/tired, voluntary movement improves. - PIN-ROLLING

rigidity - lead pipe. cogwheel: superimposed tremor

53
Q

are parkinsons symtoms symmetrical or asymmetrical?

A

asymmetrical!!

54
Q

epidemiology of parkinsons

A

twice as common in men

mean age of diagnosis: 65

55
Q

other features of parkinsons

A

mask-like facies
flexed posture
micrographia - small writing
drooling of saliva
rem sleep behaviour disorder
fatigue
autonomic disfunction: postural hypotension
impaired olfaction

psychiatric: depression - 40% , dementia, psychosis, sleep disturbed

56
Q

drug-induced parkinsonism features:

A

motor symptoms bilateral and rapid onset.

rigidity and rest tremor uncommon

57
Q

how would you diagnosis parkinsons?

A

clinical.
if hard to differentiate between essential tremor and parkinsons consider : SPECT

single photon emission computed tomography
123 i - fp - cit

58
Q

what colour stain is for lewy body?

A

brown - alpha synuclein

59
Q

how would you manage parkinsons?

A

1st line: levodopa - if motor sx affect QoL

non-ergot derived dopamine agonist (ropinirole,rotigotine,apomorphine), levodopa, monoamine oxidase B inhibitor - if motor sx dont affect QoL

60
Q

give an example of a monoamine oxidase b inhibitor and tell us how it works?

A

selegelline

inhibits breakdown of dopamine secreted by dopaminergic neurones

61
Q

common adverse affects of levodopa

A

dry mouth
anorexia
palpitations
postural hypotension
psychosis

62
Q

give an example of a catechol-o-methyl transferase inhibitor and how is works?

A

entacapone, tolcapone

comt is enzyme involved in dopamine breakdown, could use as adjunct in levodopa therapy.

esp with pts with established pd give with levodopa

63
Q

give examples of antimuscarinics and tell us how they work

A

procyclidine, benzotropine, trihexyphenidyl (benzhexol)

block cholinergic receptors
treat drug-induced parkinsonism rather than idiopathic parkinsons disease
help tremor and rigidity

64
Q

what class of drug is amantadine and what is its function?

A

antidyskinetic

most likely increases dopamine release and inhibits uptake at dopaminergic synapses

se: ataxia, slurred speech, confusion, dizziness, livedo reticularis (spasm of blood vessel near skin surface)

65
Q

give examples of dopamine receptor agonists and potential side effects

A

bromocriptine, ropinirole, cabergoline, apomorphine

ergot derived : pulmonary,retroperitoneal and cardiac fibrosis. DO ECHO, ESR, CREATININE AND CXR BEFORE AND DURING TX.

INFORM PT POTENTIAL IMPULSE CONTROL DISORDER AND EXCESSIVE DAYTIME SOMNOLENCE(sleepy)

hallucination (more likely than levodopa in elder patients)
nasal congestion , postural hypotension.

66
Q

what drug is nearly always combined with levodopa in PD tx and why?

A

decarboxylase inhibitor (carbidopa or benserazide)

prevents peripheral metabolism of levodopa to dopamine outside of brain so reduce side effects

67
Q

what do you give if patient has sx despite optimal levodopa or developed dyskinesia?

A

add dopamine agonist, MAO-B inhibitor or COMT inhibitor as adjunct.

68
Q

in parkinsons, what can cause acute akinesia or neuroleptic malignant syndrome?

A

medication not taken/absorbed - gastroenteritis

no drug holidays

69
Q

causes of impulse control disorder

A

dopamine agonist therapy

hx of previous impulse behaviours

hx of alcohol consumption and/or smoking

70
Q

patient develops excessive daytime sleepiness, he has pmh of parkinsons. how to treat?

A

do not drive.

adjust med to control symptoms.

give modafinil.

71
Q

patient with parkinsons develops orthostatic hypotension. what to do?

A

medication review

midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance)

72
Q

patient with parkinsons develops drooling of sailva. how to treat?

A

glycopyrronium bromide

73
Q

things to know about prescribing levodopa

A

adverse affects due to difficulty achieving steady dose:

end of dose wearing off - sx worsen at end of dosage interval. decline in motor activity.

on-off phenomenon - large variation in motor performance. normal function in on period. weakness/restricted mobility in off.

dyskinesia at peak dose: dystonia (muscle contraction) , chorea(involuntary muscle movements) and athetosis(involuntary writhing movements)

74
Q

rules for administering and stopping levodopa

A

dont acutely stop levodopa

if pt with PD cant take levodopa orally, give dopamine agonist patch as rescue medication to prevent ACUTE DYSTONIA (muscle contraction)

75
Q

what is MND and types??

A

neuro condition of unknown cause
can present with both UMN,LMN signs.

amyotrophic lateral sclerosis
progressive muscular atrophy
bulbar palsy
primary lateral sclerosis

rarely presents before 40.

76
Q

clues pointing towards MND diagnosis

A

asymmetric limb weakness - MC ALS
no sensory deficits
mixed umn and lmn signs

wasting of small hand muscles/ tibialis anterior

fasciculations

you might get vague sensory symptoms in early disease (limb pain) but never sensory signs

77
Q

what muscles are spared in MND?

A

external ocular muscles - eyes are spared.

no cerebellar signs

abdominal reflexes preserved and sphincter dysfunction (late feature)

78
Q

how would you diagnose MND?

A

clinical

nerve conduction study - normal motor conduction - help exclude neuropathy.

elelectromyography shows - reduced number of action potentials with increased amplitude.

MRI - excludes differentials of cervical cord compression and myelopathy

79
Q

mnd type - presentation - ALS - AMYOTROPHIC LATERAL SCLEROSIS

A

50% pts

typically LMN signs in arms
UMN signs in legs

in familial cases, gene responsible lies on chr 21 and codes for superoxide dismutase

80
Q

presentation of primary lateral sclerosis - MND

A

UMN signs only

81
Q

presentation of MND - Progressive Muscular Atrophy

A

LMN signs only
affects distal muscles before proximal
carries best prognosis

82
Q

presentation of progressive bulbar palsy - MND

A

palsy (paralysis) of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

carries worst prognosis

83
Q

how to manage MND?

A

riluzole - prevents stimulation of glutamate receptors . used in ALS mainly - prolongs life by about 3 months

respiratory care - non invasive ventilation (BIPAP) @ night. 7 months survival benefit

PEG tube - nutrition

84
Q

prognosis of mnd

A

50% of patients die within 3 years

85
Q

what is multiple sclerosis?

A

chronic cell-mediated autoimmune disorder characterised by demyelination in the CNS.

86
Q

epidemiology of MS - multiple sclerosis

A

3 times more common in women
20-40
much more common at higher latitudes (5 times more common than in tropics.

87
Q

genetics with multiple sclerosis

A

monozygotic twin concordance = 30%
dizygotic twin concordance = 2%

88
Q

subtypes of multiple sclerosis

A

relapsing remitting - 85% mc - acute attacks (1-2mths) followed by remission periods

secondary progressive - relapsing remitting pts with deteriorating w/ neurological signs and symptoms between relapses. (65% pts develop within 15 yrs) - gait and bladder disorders seen too

primary progressive disease - 10% pts - progressive deterioration from onset. mc older ppl.

89
Q

how would you investigate for multiple sclerosis?

A

YOU NEED TO SEE LESIONS DISSEMINATED IN TIME AND SPACE

MRI
CSF
VISUAL EVOKED POTENTIALS

90
Q

when you do an MRI in someone with multiple sclerosis what will you see?

A

high signal t2 lesions

periventricular plaques

dawson fingers : seen in FLAIR images - hyperintense lesions perpendicular to corpus callosum

91
Q

when you do a lumbar puncture in a multiple sclerosis pt , what would you see?

A

csf:
oligoclonal bands - not in serum

increased intrathecal synthesis of IgG

92
Q

when you do visual evoked potentials in a multiple sclerosis pt, what would you see?

A

delayed, but well preserved waveform

93
Q

how could you diagnose multiple sclerosis?

A

2 or more relapses

and either

objective clinical evidence of 2 or more lesions

or

objective clinical evidence of 1 lesion + reasonable historical evidence of previous relapse

94
Q

visual features of multiple sclerosis

A

optic neuritis : MC
optic atrophy

UHTHOFF’S PHENOMENON - worsening of vision following body temp rise

internuclear opthalmoplegia

95
Q

sensory features of multiple sclerosis

A

pins/needles
numbness
trigeminal neuralgia
lhermitte’s syndrome : paresthesia in limbs on neck flexion

96
Q

motor features of multiple sclerosis

A

spastic weakness: mc legs

97
Q

cerebellar features of multiple sclerosis

A

ataxia : more often in acute relapse than presenting symptom

tremor

98
Q

general features (non specific) of Multiple Sclerosis

A

LETHARGY (85%)

urinary incontinence
sexual dysfunction
intellectual deterioration

99
Q

is there a treatment for multiple sclerosis?

A

tx focused on reducing frequency and duration of relapse.

NO CURE

100
Q

what drug would you give in an acute relapse of multiple sclerosis and for how long?

A

high dose steroids - oral/iv methylprednisolone - 5 DAYS

shortens length of acute relapse. but not degree of recovery

101
Q

why would you give a disease modifying drug in multiple sclerosis , indications and examples?

A

reduce risk of relapse

indications:
relapse remitting disease + 2 relapses in past 2 yrs+ able to walk 100m unaided

secondary progressive + 2 relapses in lasat 2 yrs + able to walk 10m (aided/unaided)

natalizumab - iv - 1st line
ocrelizumab - iv
fingolimod - oral formulation
beta-interferon - subcut/intramuscular (not as effective as others)
qlatiramer acetate - subcut

102
Q

MoA of natalizumab - MS 1st line DMD

A

recombinant monoclonal antibody - antagonises alpha 4 beta 1 integrin on surface of leucocytes.

inhibits migration of leucocytes across endothelium across blood brain barrier

strongest base for preventing relapse - 1st line

give IV

103
Q

class of drug - ocrelizumab - MS tx

A

humanized anti-cd20 monoclonal antibody

high-efficacy drug - often 1st line like natalizumab

GIVE IV

104
Q

what type of drug is fingolimod , MoA ? MS TX

A

sphingosine 1 phosphate receptor modulator

prevents lymphocytes from leaving lymph nodes

ORAL FORMULATION

105
Q

what type of drug is glatiramer acetate? MS TX

A

immunomodulating drug - acts as a immune decoy

GIVE SUBCUT

older drug - less effective like beta interferon - monoclonal antibodies and s1p receptor modulators more effective.

106
Q

if a MS pt experiences fatigue, how could you treat?

A

trial AMANTADINE - if anaemia, thyroid, depression