MS Flashcards
Define MS
progressive long term neurological disorder of CNS affecting brain SC and optic nerves
acquired chronic autoimmune mediated inflammatory disease
multiple areas of scarring sclerotic tissue found disseminated throughout the CNS white and grey matter
BS SC optic nerve
prevalance
MS is the most prevalent chronic inflammatory disease of the CNS and the most common cause of non-traumatic neurological disability in young adults:
8,000 people living with MS in Ireland
100,000 people in the UK
400,000 people in the US
2.5 million people worldwide
Globally, the median estimated prevalence of MS is 30 per 100,000
(range, 2–80)
epidemiology
Peak age onset 25-35 years 70% between 21 and 40
Rarely in childhood and after 60 years
Females > Males (3:1)
White races mostly
Risk 1:750-800 for Northern Europeans
causes
not known if it has single or multiple causes known risk factors genetic environmental viral immunological
genetic factors
Incidence in first-degree relatives is 20 x higher than in general population (2-4% compared to 0.1%)
Risk higher for females
Concordance rate in monozygotic twins is 30%
Women transmit to offspring more frequently than males
Genome-wide association studies from 1000s of patients have
identified >200 gene variants that raise the risk of disease
Most risk alleles are associated with immune-pathway genes
environmental factors
Incidence higher with increasing geographic latitude
Europe and North America – 120 per 100,000 population (4-6x higher than south)
Equatorial countries – rare (1 per 100,000)
Differences in the same latitude – lower prevalence in Korea compared to Japan
Reasons unclear
viral factors
“Epidemics” after WWII Faroe Islands
Orkney and Shetland Islands
Iceland
Viral agent with long incubation period?
Increased incidence after history of mononucleosis or Epstein- Barr virus?
No proof
immunology
Genetically susceptible individuals may have an abnormality in immune response that results in an attack on their own neural tissue
Analysis of active lesions suggests that a single immune- effector mechanism dominates in each person
No single specific antigen
pathophysiology
MS attacks myelin in the CNS
Complex interplay between the immune system, glial cells
(myelin-making oligodendrocytes) and neurons
Destruction of oligodendrocytes → Myelin degenerates → Less
effective conduction by neuron
pathology
Sclerotic plaques in the cortex (grey and white matter), spinal cord and optic nerve
Acute lesions are inflammatory, mediated by T cells (CD4+ and
CD8+)
Relative sparing of axon itself initially
Possible incomplete re-myelination following acute demyelinating episode
Later stages of disease: damage to axons
investigations
no single definitive test
lumbar puncture
visual evoked response
MRI
clinical presentation
initial symptoms
Vague: lack of energy, headache, depression, aches in limbs
Precise neurological deficit Sensory disturbance 40% Retrobulbar or optic neuritis 17% Limb weakness 12%
Diplopia 11%
Vertigo, Ataxia, Sphincter disturbance - 20%
FCDE: First clinical demyelinating event
clinical symptoms
motor symptoms and signs
Muscle weakness: Often asymmetric paraparesis Monoparesis Hemiparesis Tetraparesis Spasticity (80%) Clonus Spasms Hyperreflexia, positive Babinski’s sign Abnormal posture and movement
visual symptoms
diplopia
blurred vision
loss of visual activity
sudden onset of optic neuritis without any other CNS signs or symptoms
pupillary abnormalities
irregularities in outline of pupil
partial constriction and loss of light reflex
cerebellar signs
hypotonia ataxia intention tremor poor coordination nystagmus dysarthria
sensory systems
somatosensory symptoms position sense / proprioception vibratory sense pain, temp, touch Lhermitte's symptoms - flexing head - sensory symptoms spine of LL paraesthesias and numbness
cranial nerves / brainstem
visual disturbance
vertigo
UMN signs
autonomic
bladder dysfunction
bowel
sexual dysfunction
sweating and vascular abnormalities
cognitive psycho psychiatric
Cognitive dysfunction 43-70%
Affects memory and higher executive functions
Euphoria
Emotional lability
Psychiatric symptoms – mood swings, anxiety, hypomanic states
Depression common (15% to 50%)
Sleeping problems
fatigue
Common in initial presentation as well as established disease
Relapses often associated with fatigue
33-55% of people with MS report it as a troubling symptom
Worse as day progresses
Link to temperature (Uhthoff’s phenomenon)
Exacerbated by poor sleep, pain, anxiety
clinical course
relapsing
remising MS
Most common type of MS – up to 85% of cases
Characterized by attacks (relapses) and remissions (recovery).
During remission , fewer or no symptoms. Relapses: unpredictable and unclear etiology
During a relapse new symptoms may occur or previous symptoms may
return
A relapse is usually defined as the appearance of new symptoms lasting more than 24 hours. They can last any length of time.
40% will develop secondary progressive MS
primary progressive MS
Some people with MS never have distinct relapses and
remissions.
From the onset of disease, individuals experience steadily worsening symptoms and progressive disability.
This may level off at any time, or may continue to get worse.
Around 10-15% of people with MS have PPMS
secondary progressive SPMS
Starts as RRMS but after repeated attacks the remissions stop
and the disease moves into a progressive phase.
40% of people with initial RRMS develop secondary progressive MS.
The time it takes to move into the secondary progressive phase varies (median 15-20 years from first onset)
Less progression with:
Younger age Early treatment
Fewer spinal cord lesions
benign MS
Usually starts with mild attacks followed by a recovery
It does not worsen over time and there is rarely permanent
disability. The first symptoms are usually sensory.
It is only possible to classify people as having benign MS when they have little or no sign of disability 10 to 15 years after the onset of the disease. However, occasionally disability may develop even after many years of the disease remaining inactive.
Around 10% per cent of people with MS have the benign form.
course of disease
Impact on life expectancy depends on course of disease and access to disease-modifying treatments
On average people with MS die 6 years earlier
15 y after diagnosis: 50% use walking aids, 29% need a
wheelchair
In first 10 years 50-80% become unable to work
treatment
exercise modifying pharmacology
exercise and lifestyle
symptomatic pharmacology and treatment
pharm managment
Immunomodulators (disease modifiers) Copaxone® (Glatiramer acetate) Novantrone® (Mitoxantrone)
Tysabri® (Natalizumab)
Gilenya® (Fingolimod) Lemtrada® (Alemtuzumab) Leustatin® (Cladribine) Dimethyl fumarate Ocrevus® (Ocrelizumab) Aubagio® (Teriflunomide)
Steroids (treatment of relapses)
0.5g daily orally
1g IV 3-5 days
-Interferons (prevention of relapses) Avonex Rebif Betaferon Most effective in FCDE and early stages
symptomatic pharm management
Spasticity: Zanaflex, Baclofen, Dantrolene, Baclofen pump
Urinary Symptoms:
Intermittent self catheterisation (Residual vol < 100mls)
Oxybutin, Tolterodine Impramine
Fatigue: Amantadine
Oscillopsia: Gabapentin
Neuropathic pain: Lyrica
symptomatic treatment
Sexual dysfunction 60-70% of males impotent 90% improved with Viagra Cognitive problems neuropsychology cognitive retraining strategies Depression Amitryptilline
assessment
subjective
objective
outcome measures
subjective
Presenting Complaint History of Presenting Complaint Investigations Past Medical History Social History Family History Take time Develop a rapport Open ended questions Avoid interrupting (if possible!) Summarise or paraphrase as you go on Don’t have to take entire history sitting down face to face
new diagnosis
1. Database
Has the diagnosis been confirmed by a neurologist? Is the patient aware?
How has the neurologist classified the disease?
Currently in a relapse? Medications (steroids, disease modifiers, other)
- Subjective questions
What problems did you notice that
brought you to hospital?
Have you ever had anything like this before?
How is it affecting your daily life (mobility, ADLs, participation)? How are you coping?
Did the steroids make a difference?
subjective assessment in MS:
known diagnosis
Since you were last seen or assessed, has any activity you used to undertake been limited, stopped or affected?”
“Are you still able to undertake, as far as you wish:
Your work / employment
Leisure activities
Family roles
Washing, dressing and using the toilet
Getting about and getting in and out of the house
Controlling your environment – opening doors, switching things on and
off, using appliances, using your phone
Shopping, going to church, community activities
Household and domestic activities?”
objective assessment
observation neuro exam ROM functional assessment activities
outcome measures
Impairment
Oxford scale, 10RM, 5TSTS
Modified Ashworth Scale
Nine Hole Peg Test
Activity limitation
Functional Assessment Measure (FAM)
*Multiple Sclerosis Functional Composite (MSFC) scale
Gait tests (Timed 10MWT, 6MWT)
Balance (TUG, Berg Balance Scale, Mini-Best Test)
*MS Fatigue Impact Scale (MFIS)
Participation restriction
* Kurtzke’s Extended Disability Status Scale (EDSS)
aims of physio RX
Maintain soft tissue length and manage tonal abnormalities
Maximise muscle activity and strength
Education about the importance of exercise
Posture re-education
Gait training
Balance re-education / Vestibular rehabilitation / Falls prevention
Fatigue management
Management of incontinence
Pain management
education
A wealth of evidence has established that exercise in MS is safe and effective
Aerobic training (low to moderate intensity) can improve fatigue and aerobic fitness
Balance exercises reduce fall rates and improve balance
Resistance training may improve fatigue and ambulation
Flexibility exercises may diminish spasticity and potentially prevent future contractures
Lifestyle and exercise
encourage to exercise
may have beneficial effects on MS
exercise testing in MS
6 minute walk test
with HR monitor, RPE
Leg or arm cycle ergometry with HR monitor, RPE (Physiological Cost Index)
30s STS 10 rep max 5T STS 1 min STS goniometry sit and reach test
exercise prescription
FITT principle
Considerations: ICF, patient goals Feasibility, safety Patient choice Facilitators – enjoyment, peers Barriers – fear, fatigue, facilities, conflicting advice