Multiple Sclerosis Flashcards
What is MS
Demyelination of the white matter of brain and spinal cord
Causes abnormal conduction and loss of axons
UMN Signs only
Rarely affects peripheral nervous system
What causes MS
Autoimmune - HLA
Complex genetics
T cells cross BBB causing inflammation and demyelination of glial cells (oligodendrocyte) that form myelin sheath
Post inflammatory gliosis
2 axon damage lead to irreversible loss of function
What are the RF for MS
Genetics F>M Autoimmune Temperate climate > tropical Increased distance from equator Viral - EBV / HPV6 / chlamydia Vitamin D deficiency Smoking
What is the diagnostic criteria for MS
Mcdonald criteria
2 episodes of demyelination in different regions of CNS +- 2+ lesions on scan
Dissmeninated in time (2 attacks or different enhancement) and space (2 diff lesions MRI)
What are lesions seen as on MRI
White blobs of demyelination of myelin sheath
Axonal loss may account for persistent disability - seen as black holes
What is the most common type of MS
Relapsing remitting
What happens in relapsing remitting MS
Episode of demyelination which stabilises to complete or partial recovery
Will develop into secondary progressive where symptoms present all the time
What is secondary progressive MS
10-35 years after relapse
Disability increases
Gait and bladder disturbance dominate
What is primary progressive MS
Progressive deterioration from onset No relapse 5/6th decade Poor prognosis Spinal and bladder symptoms common
What is sensory MS
What is acute fulminate MS
Only sensory inflammation
Very rare, rapidly detiorating from that leads to death over days
What suggests a positive prognosis
Female Young age of onset Present with optic neuritis Relapsing remitting Long interval between relapse
What is signs of -ve prognosis
Male Age Multifocal S+S Motor S+S at onset - pyramidal, brain stem or cerebellar Early relapse
What are the features of MS
Fatigue / lethargy / poor concentration + memory - Due to neurone conducting more slowly Optic neuritis = common Demyelinating myelitis Trigeminal neuralgia Cerebellar Eye movement abnormalities Internuclear ophthalmoplegia Oscillipsia - visual fields oscillate Unpleasant sensation - Neurone discharge spontaneously Pain, paraesthesia, heat disturbance Conduction can fail with increased temperature so Sx worse in hot / after exercise Autonomic - incontinente, retention and problems emptying Cognition - depression + mild cognitive
How does optic neuritis present (15% present with this)
Central scrotoma - blind spot Unilateral visual loss Pain when moving eye and behind eye Light flashes precipitated by eye movement Optic disc swelling but can be normal Afferent pupillary defect Often get gradual return in vision Can't see bright red
What are cerebellar signs
Vertigo
Ataxia
Tremor
DANISH P
What is internuclear ophthalmoplegia
- Symptoms
- What type of nystagmus and where
Lesion in MLF of pons - connects CN 3,4,6
Can be MS of vascular
Causes lateral conjugate gaze so if one eye moves other doesn’t
Failure of adduction on side of lesion
- e.g. R INO = failure adduction of R eye when looking to L
Other
Horizontal nystagmus on abduction on contralateral side e.g. L eye
Diplopia on lateral gaze
What is myelitis
Inflammation of spinal cord
What are signs of myelitis and what is Ddx
Presents like cord compression without obstruction on imaging
UMN signs
Can be partial or transverse
Hyperaesthesia / pins and needles
Weakness - LL > UL
Spastic paralysis
Hyperreflexia
Hypertonia
Babinski +Ve
Impairment in sensation - pain, temp, prorpiception
Bladder, bowel and sexual issues due to autonomic dysfunction
DDX
- Spinal cord compression
- Cauda equina
Will require urgent MRI to see if benefit from surgical decompression
What do you do for relapse
Look for infection + treat Oral prednisone or IV for any acute PPI protection Explain risk vs benefit Not >3 weeks and not >3x a year Symptomatic Rx
When do you start disease modifying drugs
If 2 relapses in 2 years
What is 1st line disease modifying drugs
Beta-interferon - Adminstered weekly IM - Improve integrity of BBB Dimethyl fumarate Immunosuppression
What are SE
Flu-like symptoms
Abnormal blood count + LFT
CI if severe depression / psychosis
What is 2nd line if regularly relapses
Biologic infusion - Almetizumab
SE of 2nd line
Pain so admit for 5 days Reactivation TB Infusion reaction Immunosuppression High risk of autoimmune thyroid or kidney ITP PML Malignancy - skin / lymphoma
What do you need annual
MRI to look for PML
What are symptomatic treatment
Analgesia Muscle relaxant for spasticity - Gabapentin / baclofen = 1st line for spasticit - Diazepam if fails - Physio Anti-convulsant for dysasthesia Anti-cholinergic / catheter for urinary incontience Oscillipsia - Gabapentin Laxatives Graded exercise for fatigue or amantadine CBT OT / PT Aids for walking Botilum toxin for tremor
What is PML and what causes causes
Progressive multifocal leukoencephalopathy
Fatal disease of white matter
JC virus reactivation
What are RF
HIV
Biologics
How do you Dx or screen
Annual MRI
JC Ab + urine 6 monthly
What is Devic’s
Bilateral optic neuritis + spinal cord myelitis
- UMN signs
- Limb weakness
- Sensory loss
- Urinary incontinence
- Bilateral visual loss / swollen optic disc
Relapsing remitting
What causes Devic’s
Autoimmune IgG Ab against aquaporin-4
Immune system attacks astrocytes
Myelin sheath destroyed
How do you Dx Devic’s
Ab in blood - aquaporin 4
Perivascuar lesion on MRI
How do you treat Devics
Immunosuppression
High dose steroids during attack
Plasma exchange
How do you Dx MS
Primary clinical Can do urgent CT head MRI = main diagnostic - plaque CSF - oligoclonal bands not present in serum if unsure of Dx Delayed VEP if spinal lesion - Litle help if optic neuritis
What type of MRI
MRI with contrast
What are differentials
Motor neurone Bells palsy Brain abscess Devics GBS - peripheral HIV Trauma Sarcoid Lumbar disc herniation Spinal cord infection Stroke - look at patient presenting but CT if concern Subdural Syphillis SLE TIA Trigeminal neuralgia
If patient presents with signs that could be attributed to MS what is needed
Urgent discussion with neurologist and MRI
What other investigations
CT brain for DDx if think acute MRI to exclude compression of cord LP for CSF analysis Serum glucose U+E
If patient with known MS presents
FBC to look for infection
Urinanalysis to exclude UTI
Bladder scan
UTI in MS
Much more common due to incomplete voiding and retention
Rx pyrexia quick as can worsen Sx
Catheter to drain urine
What needs to be aggressively managed
Fever and all infections
If LL weakness + incontinence what do you think
Some form of cord compression
Obstructing cause must be excluded first
What are common area’s affected
Optic nerve - neuritis (20%)
Cerebellum - co-ordination / cerebellar syndrome
Brain stem - eye movement + INO
Spinal cord - paraparesis + transversemyelitis