Multiple Sclerosis Flashcards
Definition of multiple sclerosis
a chronic progressive inflammatory disease involving damage to the myelin in the brain and spinal cord
- autoimmune disease (genetics, environment, 1st degree relatives)
- range of medical issues (physical function, depression, participation, personal social)
MS characteristics
the most common demyelinating nervous system disease to affect young adults
- mean onset 30 yrs old
- 70% onset 20-40 yrs old
- 10-20% after 60 yrs old
- more in females
- genetic predisposition
- triggered by environmental factors (sunshine, vit D, smoking)
MS is a dysfunction of?
oligendrocytes
- pro inflammatory agents released by microglial cells cause dysfunction
Which cells are reactive in MS?
T cells
- they are auto reactive in the periphery of the CNS
- T & B cells cross the weakened blood brain barrier
- T cells interact with B cells & make aberrant antibodies that target oligodendrocytes
Gray matter dysfunction in MS
gray matter pathology is closely associated with clinical disability than white matter (white matter injury is said to come independently)
Epidemiology of MS
- women > men
- men have a worse prognosis/aggressive disease
- extreme north/south latitudes
- least common in warm climates
- people who migrate from high risk to low risk regions before puberty reduce risk
What are some geography/environmental triggers?
- Epstein Barr virus
- low vit D levels
- reduced sunlight exposure
- more common above the 45° latitude
Inflammatory demyelination
damage to the transmission of nerve impulses
1. inflammation to oligodendrocytes
2. oligodendrocytes demyelinate
3. inflammation subsides and symptoms reduce
4. oligodentrocytes remyelinate
5. more/repeated inflammation causes reduced repairing leading to axonal damage
What does axonal damage equate to?
irreversible disability
Plaques
focal loss of myeline leads to fibrous astrocytes and undergoes gliosis leading to plaques
–> axonal loss is present
50% spinal cord
25% optic nerve
25% brainstem/cerebellum
** plaques are the hallmark of MS
MS classification: Relapsing-Remitting
(RRMS) unpredictable attacks which may or may not leave permanent deficits followed by periods of remission
~ 85% of cases
MS classification: Secondary Progressive
(SPMS) initial relapsing remitting that suddenly begins to have decline without periods of remission
MS classification: Primary Progressive
stead increase in disability without attack, gradual worsening of symptoms that does not respond to treatment prescriptions
–> progressive myelopathy
MS classification: Benign
one occurrence with no reoccurrence
MS classification: Progressive Relapsing
steady decline since onset with superimposed attacks, progressive course with clear relapses
~ 5% of cases
MS Expanded Disability Status Scale 0-4
0 = not affected
1 = no disability
2 = minimal disability
3 = moderate disability
4 = relatively severe disability
MS Expanded Disability Status Scale 5-9
5 = disability affects daily routine
6 = assistance required to work
7 = restricted to wheelchair
8 = restricted to bed or wheelchair
9 = confined to bed
Diagnosing MS
there is no single test, a collection of medical tests to confirm diagnosis (MRI, lumbar puncture)
- 2 lesions in at least 2 separate areas of the brain, spinal cord, or optic nerve
AND
- evidence of damage at 2 different points in time
AND
- rule out other diagnoses
Differential diagnosis for MS
- chronic low back pain (common 1st issue)
- fibromyalgia
- cervical spondyosis
- herniated disc
- mitochondrial disease
Suspected MS symptom picture
significant lapse between the initial s/s (flares) and an additional episode of MS (can sometimes be years)
- some people may have a clinical isolated symptom (one event) and no other signs after that
Lumbar puncture: MS
the basic protein found in the CSF will be elevated during acute exacerbations of MS
Visual evoked potential (VEP)
tests that measure the electrical activity of the brain (optic nerve) in response to stimulation of visual nerve pathways
What are the initial symptoms of MS?
- females in their 20s
- usually transient
- fatigue
- visual disturbances (double vision)
- paresthesias, numbness, weakness, pins/needles (usually accompanied by LBP)
- pain in B LEs (sudden and spontaneous electrical shock)
What are some red flags in terms of MS symptoms?
double vision
pain in B LEs with a sudden electrical shock
S/S of MS flare/relapse/exacerbation
- numbness/tingling
- fatigue
- visual changes
- weakness
- changes in gait
- brain fog
- tremors
- incontinence
** last longer than 24 hours (days, weeks, months)
Disease pathway of MS
- initial diagnosis
- exacerbation
- period of remission
- disease of management
Exacerbation and damage
the longer the length of an exacerbation the more damage may occur
Pseudo exacerbation
brought on by stress, over exertion, or heat
- causes fatigue, brain fog, pain
- will resolve in less than 24 hours on its own
- DOES NOT MEAN THAT THE NERVOUS SYSTEM IS UNDERGOING DAMAGE
Fatigue
a subjective response perceived by an individual
Primary fatigue
autoimmune, lassitude (lethargy)
Indirect fatigue
medications that cause sleep
Neurologic fatigue
spasms, weakness, heat exposure, energy failure
Mitochondrial genetic variants and MS fatigue
deficient mitochondrial metabolism may generate more reactive oxygen specific (ROS) that wreak havoc in cells
–> mitochondrial injury and subsequent energy failure are key factors in the induction of demyelination and neurodegeneration
Fatigue in MS
greater exposure to stress (deadlines at work, family conflict) and low mood were associated with higher fatigue levels while positive moods are associated with lower levels of fatigue
* increased fatigue was associated with recent physical activity in people with MS
Uhtoff phenomenon
increase in core body temp (70% of people with MS experience)
- physical exertions, hot water, warm temps are all correlated with fatigue
- temporary symptoms usually don’t increase MS symptoms
- rapidly reversed with symptoms are removed
Motor system in MS
weakness (brain and spinal cord)
- mono, hemi, quad paresis
- deconditioning
- dysarthria
ataxia (80% of people have it)
- cerebellar
- dysmetria or dysdiadochokinesia
- tremor
Spasms
sudden contractions/jumping of extremities (occurs in spinal cord pathology)
- can be flexor or extensor
- LE > UE
Hypertonicity/spasticity in MS
velocity sensitive resistance to muscle stretch, highly correlated with reduced function and QOL
- spasticity seen with position changes, noxious stimuli, or physiologic stress
- may be seen with increased pain, cold weather, tight clothing, or increased body temps
What can spasticity lead to?
contractures, skin breakdown, pain/sleep disturbances
Acute neuropathic pain
brought on by demyelination of sensory neurons (anteriolateral tract)
Trigeminal neuralgia
stabbing pain in the face or jaw, pain is not constant
Lhermitte’s sign
brief stabbing electrical shock through the spinal cord
Chronic neuropathic pain
- brought on by stress (disease stress), fatigue, illness, or overheating
- patient may feel dysesthesias or pruritis
Dysesthesia
painful sensations of the upper and lower limbs that are burning, lancing, prickling, stabbing, or icy
Pruritis
a form of dysesthesia (pins and needles), itching, burning, stabbing
Musculoskeletal pain
- muscular tightness/loss of ROM
- spasticity, weakness, abnormal motion
- compensatory patterns during movement
- muscular pain from abnormal use or loading
- muscular fatigue
Sensory impairments in MS
VISUAL
- optic neuritis (20% will have in the first MS event)
- double vision, blurry, painful eye movements
- visual field loss, color desaturation
- pupillary deficits
SOMATOSENSORY/PROPRIOCEPTIVE
- dysethesias (tingling, vibrations)
- anesthesias
- parasthesias
- vestibular (dizziness/vertigo)
NEUROPATHIC PAIN
Gait dysfunction in MS
decreased mobility due to other deficits
- spasticity, weakness, sensory loss
- coordination/ataxia
- loss of sensation
Postural control and falls in MS
complex interaction between sensory inputs, motor output, processing, and context
- leads to fractures from falls
What are the three common problems in MS in terms of postural control/falls?
- delayed response to postural perturbations
- increased body sway to quiet standing
- inability to move outside of BOS
Bowel problems in MS
35-58% experience this
- fecal retention
- incontinence
- constipation
Bladder problems in MS
52-97% experience this
- urinary urgency
- incontinence
- overactive detrusor
Psychosocial problems in MS
depression is 2-3x more likely compared to other neuro conditions
- associated with lower QOL
Cognitive problems in MS
- decreased verbal fluency and verbal memory
- decreased processing speed
- decreased executive functioning
Cycle of symptoms
- increase in fatigue and depression
- exercise decreases
- spasticity and constipation increase
- bladder problems
- decrease in sleep
- decreased cognitive function
Disease modifying drugs: relapsing remitting MS
prevention of new inflammatory lesion and prevention of the development of secondary progressive MS
Disease modifying drugs: secondary and primary MS
prevention of loss of nerve fibers (neuroprotection) and allows for remyelination
Disease modifying drugs: Ocrevus
used in people with relapsing and progressive forms of MS
- 2x/yr
- 10 min IV drug
- suppresses relapse acitivity and MRI lesions in 97% of people in 48 weeks
Outcomes of MS
- life expecacntny 6 years earlier than general population (72 women, 68 men)
- higher mortality risk in patients with primary progressive MS
Progression of MS in the field
- it is highly variable
- disease modifying therapy has reduced level of burden and progression
- research on progressive forms
- there has been earlier diagnosis and prompt medical intervention
Clinical picture: clinical isolated syndrome
- aucte/subacute onset of a monophasic episode suggestive of MS (longer than 24 hrs and affects optic nerve, brainstem, or spinal cord)
- 30-70% of patients will develop MS
- patients with optic neuritis have higher risk of MS development
- age of onset 20-45 yrs, more in women
Clinical picture: relapsing remitting
- relapses over days to weeks with complete or partial remissions over months to years (~85% of cases)
- age of onset 20-30 yrs, more in women
Clinical picture: progressive
progressive accumulation of disability after initial relapsing
Clinical picture: secondary progressive
~75% of relapsing remitting case within 15 years of initial diagnosis will develop this
Clinical picture: primary progressive
- steady functional worsening from the onset of disease
- ~15% of cases
- later onset than relapsing remitting
- more in female
SUMMARY: MS
- progressive autoimmune CNS disease
- myelin, spinal cord, cerebellum, brain
- variable course and presentation
- no cure, reduce amount of attacks
SUMMARY: functional impairments
- all facets of function and mobility
- progressive loss of function
- motor, cognitive, integumentary systems affected
- risk for secondary complications from lack of movement and mobility