MS

Question 1

Investigations that may be requested for suspected MS?

Answer 1

blood tests-exclude other inflammatory disorders e.g. sarcoidosis-ACE, Ca2+, SLE-haemolytic anaemia, low wcc, raised ESR, ANA, anti-dsDNA, anti-smith Abs, or other causes of paraparesis e.g. HIV, vit B12 deficiency
lumbar puncture: looking for oligocloncal bands-multiple IgG antibodies causing elevated protein count in CSF, but not specific for MS
MRI brain and spinal cord-looking for plaques of demyelination
visually evoked potentials-assess occipital areas response to visual stimulation to look for optic nerve demyelination causing slower electrical conduction.

Question 2

most common presentation of MS?

Answer 2

optic neuritis-acute painful loss of vision

pain on eye movement if retrobulbar neuritis as rectus contraction pulls on the optic nerve sheath

Question 3

possible presenting features of MS?

Answer 3

optic: optic neuritis
internuclear ophthalmoplegia
motor: UMN signs-spastic weakness-most commonly seen in legs
sensory: paraesthesia, numbness, trigeminal neuralgia
neuropathic pain
clumsy/useless hand or limb due to loss of proprioception
fatigue
bladder:incontinence, bladder hypereflexia causes urinary frequency and urgency, can be tx with antimuscarinics e.g. oxybutynin
cerebellum:ataxia, intention tremor
others: intellectual deterioration-as axons degenerate, atrophy of brain occurs over time
sexual dysfunction
temperature sensitivity
epilepsy
tonic spasms-frequent brief spasms of 1 limb

Question 4

presenting features of internuclear ophthalmoplegia?

Answer 4

failed adduction of eye of affected side
nystagmus in abduction of contralateral eye

e.g. R MLF affected: on looking to the L, R eye will not adduct, L eye will abduct but there will by nystagmus.

Question 5

causes of INO?

Answer 5

MS
vascular disease
?tumour-causing compression

Question 6

what does the left medial longitudinal fasciculus connect?

Answer 6

this allows left eye to adduct when the eyes are looking to the right (R eye is abducting)
so connects the right abducens nerve nucleus to the left oculomotor nerve nucleus

Question 7

in someone with MLF syndrome e.g. an MS patient, why is there normal convergence (both eyes able to adduct simultaenously)?

Answer 7

this is achieved via cerebral cortex signalling to the oculomotor nerve nuclei, which does not require the use of the MLF

Question 8

define MS

Answer 8

a chronic T cell mediated autoimmune inflammatory demyelinating condition of the CNS causing multiple plaques of demyelination throughout the brain and SC which are separated in time in space, appearing over years.

Question 9

who is affected by MS?

Answer 9

women more commonly then men
px usually around 20-40yrs, but diagnosis may be delayed-occurring many yrs after 1st onset of symptoms
caucasians
further away from the equator-increased risk, even north south divide in the UK-highest prevalence in Scotland
other AI disorders-occur more commonly in MS pts and their relatives

Question 10

possible environmental factors implicated in pathogenesis of MS?

Answer 10

infections-EBV, herpes virus 6-also causes the skin condition pityriasis rosea-‘herald patch’
?low levels of sunlight and Vit D deficiency
?smoking

EBV is the main environmental factor implicated

Question 11

where do the plaques of demyelination affect in MS?

Answer 11

can occur anywhere in the CNS white mater, but part. affect:
optic nerves
peri-ventricular
corpus callosum
brainstem and its cerebellar connections
cervical cord-dorsal columns and corticospinal tracts

Question 12

pathological basis of progressive disability in progressive forms of MS?

Answer 12

progressive axonal damage, with permanent axonal destruction and inability for remyelination to take place

Question 13

characteristic common presentation of brainstem demyelination in MS?

Answer 13

sudden diplopia and vertigo with nystagmus

but without tinnitus or deafness

Question 14

what problems does an increase in temp in MS patients cause?

Answer 14

e.g. post exercise or a hot bath, can be temporary worsening of pre-existing symptoms
=Uhthoff’s phenomenon

Question 15

what criteria can be used to formalise diagnosis of MS?

Answer 15

McDonald criteria

Question 16

at initial px, what is it part. important to ask about in the hx when suspecting MS?

Answer 16

previous episodes of neurological symptoms, may be many years ago e.g. acute painful loss of vision, severe episode of vertigo lasting wks.

Question 17

definitive investigation for MS?

Answer 17

MRI brain and SC
acute lesions enhanced with contrast
can see multiple lesions disseminated in space,

Question 18

types of MS?

Answer 18

relapsing-remitting (85-90%)
secondary progressive-gradual progression of disability unrelated to relapses after an initial relapsing-remitting course
primary progressive-typically presents later and with less inflammatory changes on MRI
relapsing-progressive (less than 5%)-similar to primary progressive but with supra-added relapses on a background of progressive disability from the outset.

Question 19

why is MRI brain not wholly specific for MS?

Answer 19

other lesions that can appear similar in the older population are small ischaemic lesions
and in younger pts neuroinflammtory disorders such as sarcoidosis, behcet syndrome and vasculitis.

spinal cord lesions quite specific for inflammatory disorders

Question 20

what are the Mcdonald criteria for MS diagnosis?

Answer 20

assessing that episodes are consistent with an inflammatory process
establishing lesions have developed at different times and are in different anatomical locations for a diagnosis of relapsing-remitting MS
excluding alternative diagnoses
establishing progressive neurological deterioration over 1yr or more for a diagnosis of primary progressive MS

Question 21

name given to the presentation of a pt for the 1st time with neuroinflammatory symptoms?

Answer 21

the clinically isolated syndrome

a diagnosis of MS cannot be made by definition

Question 22

non-pharmacological treatment of MS?

Answer 22

patients require MDT input including MS nurse specialist, consultant neurologist, GP, physio, OT, SALT, psychologist, dietician, social care and continence specialist
education, provision of written information
physio and OT especially helpful for persisting impairment between relapses
encourage exercise
advise not to smoke-smoking may increase the progression of disability
immunisations are safe (but NOT live vaccines if on disease-modifying drugs)

Question 23

why must infection be treated promptly in MS patients?

Answer 23

may precipitate relapses of lead to worsening of existing symptoms

Question 24

tx of urinary symptoms in MS?

Answer 24

bladder training exercises
antimuscarinics e.g. oxybutynin, tolterodine
botulinum toxin type A intrathecal injections
intermittent self catheterisation
indwelling catheter

Question 25

tx of spasticity in MS patients?

Answer 25

physio, stretching, splinting
muscle relaxants-baclofen, clonazepam
gabapentin-Ca2+ cannel blocker
botulinum toxin type A for focal spasticity
intrathecal baclofen pump
oromucosal cannabis spray

Question 26

tx of neuropathic pain in MS patients?

Answer 26

gabapentin
carbamazepine
amitriptyline

Question 27

tx of fatigue in MS patients?

Answer 27

fatigue management programme, treat depression

amantadine (glutamate antagonist)

Question 28

problematic LT symptoms in MS patients?

Answer 28

neuropathic pain
spasticity
fatigue
urinary incontinence
swallowing difficulty
depression
tremor
erectile dysfunction
impaired mobility-fampridine (K+ channel blocker) may improve walking significantly

Question 29

disease modifying drugs recommended by NICE for treatment of active relapsing-remitting MS (2 clinically significant relapses in the previous 2 years)?

Answer 29

dimethyl fumarate-PO
alemtuzumab
teriflunomide-PO
fingolimod-PO
natalizumab-for rapidly evolving severe relapsing-remitting MS

Question 30

what is transverse myelitis?

Answer 30

acute inflammatory disorder affecting the SC with cord swelling and loss of function
causes:
para-infectious AI inflammatory response
infection
MS
neuromyelitis optica
systemic inflammtory disorders e.g. SLE, sarcoidosis, sjogrens

Treatment=high dose steroids

Question 31

most common px of transverse myelitis?

Answer 31

spastic diplegia

as most common location of lesion is thoracic SC

Question 32

why does MS only affect the CNS?

Answer 32

MS is an autoimmune condition where the IS attacks myelin and the myelin producing cells, thought that the antigen that is targeted is a protein released from myelin into the blood
although myelin in CNS and PNS, it is chemically different in both, due to production of CNS myelin by oligodendrocytes, and PNS myelin by schwann cells.

Question 33

features o/e of patient with optic neuritis?

Answer 33

reduced visual acuity
reduced colour vision
RAPD due to reduced optic nerve conduction
central scotoma on field testing
swollen disc in papillitis (optic nerve head affected)

loss if vision may progress over a few days, and then slowly improve-wks-mnths, as plaque of demyelination resolves with remyelination of the optic nerve.

Question 34

define highly active MS

Answer 34

2 relapses or more in 1 year

Question 35

what type of MS is a gentleman presenting for the 1st time in his 40s-50s likely to have?

Answer 35

primary progressive

Question 36

acute treatment of MS relapse?

Answer 36

high dose steroids-IV methylprednisolone, 5 day course 500mg/day, given with gastric protection e.g. omeprazole or ranitidine.
steroids shorten the duration of a relapse but do not alter the degree of recovery

additional tment may be required in severe impairment unresponsive to steroids e.g. plasma exchange

Question 37

what is a relapse in MS?

Answer 37

development of new symptoms
or has worsening of existing symptoms?
and these last for more than 24hrs in the absence of infection or any other cause after a stable period of a t least 1 month.

Question 38

what complication do patients taking dimethyl fumarate require counselling on?

Answer 38

progressive multifocal leukoencephalopathy:
risk of this increases with prolonged lymphopenia. it is a demyelinating disease of the CNS characterised by widespread lesions due to infection of oligodendrocytes with JC virus (a human polyomavirus). must monitor pt for neurological symptoms and opportunistic infections.

Question 39

what is the concern if a pt on alemtuzumab develops severe dyspnoea?

Answer 39

severe cytokine release syndrome

drug also causes hepatic impairment, should be aware that drug should be discontinued if signs or symps of hepatic injury develop, or if liver enzymes exceed 3 times upper limit of reference range.