Multiple Sclerosis Flashcards
Is MS a common or rare neurological disease?
common
MS is said to be a disease of _____ people
young
Describe the presentation, severity and prognosis of MS
presentation - diverse
severity - variable
prognosis - uncretain
Are all MS cases similar?
no they are all very different
Where does MS effect in the body?
the CNS
Where in the CNS does MS effect?
white matter
Causes demyelination - ”inflammation”
What is the common course of MS?
relapsing remitting course - presentation of disease symptoms that become worse over time (relapsing), followed by periods of less severe symptoms that do not completely cease (remitting)
What do most patietns with MS develop?
most patients develop progressive disability
What is the prevalence of MS?
Approx 5 new cases per week in Scotland
50-60 new cases per year in Aberdeen
Average GP has 2-4 patients
1-3 of whom are significantly disabled
When does MS usualy present?
symtpoms often first seen 20-40 but can appear later
What is the initial presentation of MS?
relapse - demyelination/“inflammation”
Gradual onset over days
Stabilises days to weeks
Gradual resolution
Partial or complete recovery
After a patient has their first relapse what happens after?
they may make a partial or full recovery and may not have any more symptoms for a long time or may have problems affecting them for a long time
What do the symptoms of a relapse of MS depend upon?
where the inflammation has occured
this means that there is many different presentations
What are the different things that may be experienced during a relapse?
- Optic neuritis
- Sensory symptoms
- Limb weakness
- Brainstem
Diplopia
Vertigo/Ataxia
• Spinal cord
bilateral motor and sensory symptoms
Bladder involvement
What is optic/retrobulbar neuritis?
when your optic nerve becomes inflamed
ON can flare up suddenly from an infection or nerve disease
What may been seen in a patient with optic neuritis?
Subacute visual loss
Pain on moving eye
Colour vision disturbed
Initial swelling optic disc
Optic atrophy seen later
Relative afferent pupillary defect
How long does it take for optic neuritis to resolve?
Usually resolves over weeks
How do you test for optic neuritis?
Light in normal eye causes constriction in both eyes which is normal
but if you shine it in the bad eye then it is very slow to react and looks like it isn’t contracting
What can happen during brainstem relapse?
Cranial nerve involvement - may cause double vision
Pons - internuclear ophthalmoplegia - affected eye shows imapirment of adduction
Cerebellum - vertigo, nystagmus, ataxia
Upper motor neurone signs limbs
Sensory involvement
What is myelitis?
inflammation of the spinal cord which can disrupt the normal responses from the brain to the rest of the body, and from the rest of the body to the brain
Inflammation in the spinal cord, can cause the myelin and axon to be damaged resulting in symptoms such as paralysis and sensory loss
What are the 2 forms of myelitis?
Partial or Transverse (complete)
What does myelitis cause?
Sensory level often with band of hyperaesthesia (excessive physical sensitivity, especially of the skin)
Weakness/upper motor neurone changes below level of demyelination
Bladder and bowel involvement
What is demyelination?
An auto immune process
Activated T cells cross blood brain barrier causing demyelination
Acute inflammation of myelin sheath
Loss of function
Nerve impulses slow or even stop, causing neurological problems.
What happens after the inflammation?
Repair
Recovery of function
What symptoms may be experienced after inflammation?
Post inflammatory gliosis -nonspecific reactive change of glial cells in response to damage to the central nervous system (CNS). proliferation or hypertrophy of several different types of glial cells, including astrocytes, microglia, and oligodendrocytes
may have functional deficit
How does demyelination show on a MRI scan?
Lesions or plaques on MRI scan
What is clinically isolated syndrome (CIS)?
no further episodes of relapse
What is MS classed as?
episodes of demyelination disseminated in space and time - so mor than one episode at different locations
When do further relapses of MS occus?
may occur within months of years of the first relapse
What sites may be effected on a further relapse?
Optic nerve
Sensory
Limb weakness
Diplopia
Vertigo
Ataxia
Sphincter disturbance
During a relapse there is often a underlying _______ 25-30%
Underlying infection 25-30%
How do relapses chance change during pregnancy?
Fewer relapses during pregnancy
Increased risk in first 3 months post partum (after birth)
What is important in the disease progression and the development of a persistent disability?
Axonal loss important in disease progression and development of persistent disability
How does axonal loss appear on a MRI?
black holes
later seen as cerebral atrophy
What happens during a progessive phase of MS?
• Accumulation of symptoms and signs:
Fatigue, temperature sensitivity
Sensory
Stiffness or spasms
Balance, slurred speech
Bladder & bowel
Diplopia/oscillopsia (when you look at something it moves)/visual loss
Swallowing
Cognitive-dementia/emotional lability
What would be seen on examination of a patient with MS?
Depends on where demyelination has occurred and stage of disease:
Afferent pupillary defect
Nystagmus or abnormal eye movements
Cerebellar signs - ataxia (affects co-ordination, balance and speech)
Sensory signs
Weakness
Spasticity
Hyperreflexia
Plantars extensor
What are the different types of MS?
Relapsing remitting - 85% at outset (RRMS)
Secondary progressive (SPMS) (may not get picked up until this stage)
Primary progressive - 10-15% (PPMS) - no relapses, just a build up of disability
What type of MS is shown
RELAPSING REMITTING
What type of MS is shown
SECONDARY PROGRESSIVE
What type of MS is shown
PRIMARY PROGRESSIVE
When does primary progressive MS often present?
often presents in the 5th or 6th decade
Does a person have relapses in primary progressive MS?
no relapses
What symptoms may be experienced in primary progressive MS?
Spinal symptoms
Bladder symptoms
What is the prognosis of primary progressive MS like?
poor prognosis
What is the ratio of males to females for primary progressive MS?
M:F = 1:1
This image hsows the different courses tht MS could take
How is the diagnosis of MS made?
Evidence of demyelination separated in time and space (at least two different times that myelination has happened and in at least 2 different areas)
May be clinical or MRI based diagnosis:
Posers criteria (clinical)
Macdonald Criteria (MRI)
How do areas of demyelination show on a MRI?
lesions or plaques
What is gadolinium?
And why when contrast is used that some lesions/plaques are no longer there?
Gadolinium is a contrast medium
Areas that light up are new and active as takes the gadolinium up but the older white blobs are form previous episodes of demyelination and this shows relapses
What is a differential diagnosis?
the process of differentiating between two or more conditions which share similar signs or symptoms
Can a differential diagnosis occur with MS?
Depends on symptoms and signs and on whether a first relapse or progressive disease
What are some differential diagnosis of MS?
- Acute Disseminated Encephalomyelitis (ADEM) - an immune-mediated inflammatory demyelinating condition that predominately affects the white matter of the brain and spinal cord
- Other Auto-immune conditions eg SLE
- Sarcoidosis - disease involving abnormal collections of inflammatory cells that form lumps known as granulomas
- Vasculitis - inflammation of your blood vessels
- Infection eg Lyme disease, HTLV-1
- Adrenoleucodystrophy - congenital - a result of fatty acid buildup caused by the relevant enzymes not functioning properly, which then causes damage to the myelin sheath of the nerves, resulting in seizures and hyperactivity
WHat are differnetial diagnosis for optic neuritis?
- Neuromyelitis optica - central nervous system disorder that primarily affects the eye nerves (optic neuritis) and the spinal cord (myelitis)
- Sarcoidosis
- Ischaemic optic neuropathy
- Toxic/drugs/B12 deficiency
- Wegeners granulomatosis
- Local compression
- Lebers hereditary optic neuropathy
- Infection - TB, HIV
What are the differential diagnosis for myelitis?
• Inflammation
- Neuromyelitis optica
- SLE
- sarcoidosis
• Infection…or post infection
- (HIV, HTLV, HSV, TB, borrelia, mycoplasma etc)
- Tumour
- Paraneoplastic process
- Stroke
What other investigations may be carried out for MS?
All depends on clinical picture if other investigations are needed or not
Lumbar puncture - oligoclonal bands (bands of immunoglobulins) present in CSF but not serum
Visual/somatosensory evoked response:
Looking for a delay in a response as demyelination causes nerves to pass a signal more slowly e.g. stimulating foot and recording the signal in the brain with a EEG
Bloods - exclude other inflammatory conditions
Chest X Ray
What is the progression of MS?
1/4 MS never affects activities of daily living (ADL)
15% severely disabled quickly
1 in 4 will require a wheel chair at some point
Very difficult to predict
What is the pathogenesis of MS?
- Complex genetic inheritance
- Association with autoimmune disease
What is the male to female ratio for MS?
Female:male
2-3:1
Where in the world is MS more common?
Commoner in temperate climate
more common the further a person is from the equator
What is the treatment of MS?
Treatment of relapse
Disease modifying treatment
General health and diet
Symptomatic treatment
Multi-disciplinary approach
How do you treat an acute relapse of MS?
LOOK FOR UNDERLYING INFECTION
Exclude worsening of usual symptoms with intercurrent illness
Oral prednisolone (Intravenous)
Rehabilitation
Symptomatic treatment
What are the 2 main categories of disease modifying agents?
1st line treatments
2nd line treatments
What are disease modifying agents?
Reduce the relapse rate
Don’t slow progression of disease
Have to be having relapses often enough to be given one of these
What are some 1st line disease modifying agents?
• s/c or i/m injections
- Beta-interferons
- glatiramer acetate
• Oral treatments
- Teriflunomide
- Dimethyl Fumarate (anti-inflammatory)
What are some 2nd line disease modifying agents?
- Natalizumab
- Fingolimod
- Cladribine
- Ocrelizumab (?PPMS)
- Alemtuzumub
When would you change a patient form 1st line to 2nd line treatment?
If a patient continues to have relapses on a first line agent then they get a second line agent
Stronger the treatment = more monitoring and more risks
What do disease modifying agents do?
Not a cure
Reduce relapse rate
Do not slow progression
Side effects
How do disease modifying agents affect the immune system?
• Immunosuppression
? Risks
Progressive multifocal leukoencephalopathy (PML) - a demyelinating disease of the central nervous system (CNS) characterized by widespread lesions due to infection of oligodendrocytes by JC virus. Immunosupprsion allows JC virus to reactivate and be worse
What symptomatic treatment can be sued for MS?
Spasiticity - muscle relaxants/antispasmodics/physiotherapy
Dysaesthesia - amitriptyline, gabapentin etc.
Urinary - anticholinergic Rx, bladder stimulator/catheterisation
Constipation - laxatives
Sexual dysfunction - sildenafil
Fatigue - graded exercise, medication
Depression - CBT, medication
Cognitive - memory aids etc
Tremor - aids/medication
Vision/oscillopsia - carbamazepine
Speech/swallowing - SALT
Motor/sensory impairment - multi-disciplinary team
WHat needs ot be considered when using symptomatic treatment?
Ask directed questions about symptoms Eg bladder and mood
Treat the symptom not the “MS Symptom”
Who is involved in a multi-disciplinary team for the care of MS?
- MS nurse
- Physiotherapy
- Occupational therapy
- Speech and language therapy
- Dietician
- Rehabilitation specialists
- Continence advisor
- Psychology/psychiatry
