CNS Demyelinating Disorders Flashcards
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MS is a ___ ____ ___ disease of the ___
Chronic, autoimmune, inflammatory disease of the CNS
MS is often initially episodes of what, followed by what
Episodes of reversible neurological deficits followed by progressive neurological deterioration over time
MS - rates are higher in what geographic area
Farther from the equator
MS - cause
True cause is unknown
Involves combination of genetic and non genetic triggers
Pathophysiology MS
Genetic susceptibility plus viral trigger and then leads to a higher immune response
Pathophysiology MS - genetic susceptibility plus viral trigger and then heightened immune response leads to what
T cell antibodies attack oligodendrocytes
The T cells are attacking the myelin in the CNS “friendly fire”
Can oligodendrocytes survive the insult from T cell attack?
Initially they can remyelinate but over time they can’t repair themselves and will end up with demyelination
What happens to the areas of the axon that are demyelinated
Gliosis! accumulation of the astrocytes and microglia
This gliosis forms glial scars (plaques) and the axon will degenerate
What can gliosis do to synaptic transmission
Slow it down at first and then block it completely
What is the primary cause of permanent clinical disability
Axonal damage!!!
What areas are most vulnerable to demyelination
Optic nerves Periventricular white matter CST tracts Posterior (dorsal) columns Cerebellar peduncles
Pro Inflammatory and Anti Inflammatory Cytokines
Thought to be that people with MS have heightened response of these T cells but it has been shown that exercise might help tilt the balance more towards the anti-inflammatory T2 cytokines
Why is there a higher incidence of MS in regions with less sunlight
Vit D is important for down regulating immune response - so being in a place without sunlight already puts them at risk for having a heightened immune response
What is one of the independent risk factors for MS
Vitamin D insufficiency
Forms of MS
Relapsing Remitting MS (RRMS)
Secondary Progressive MS (SPMS)
Primary Progressive MS (PPMS)
Progressive Relapsing MS (PRMS)
Relapsing Remitting MS (RRMS)
Periods of attacks (flare-ups) separated by periods of recovery (partial or full)
With each relapse there are new s/s and old ones worsen
Relapsing Remitting MS - how common
Most common
85%
Secondary Progressive MS
Begins as relapsing remitting but then at some point turns progressive and steady decline over time, with or without acute attacks
Primary Progressive MS
Worsens continuously from onset - usually without distinct attacks
Flare up 100% of the time and just keeps adding s/s
Primary Progressive MS - how common is it
15%
Progressive Relapsing MS
Primary progressive (so continues to get worse) plus periods of more severe acute attacks and no remission
How common is progressive relapsing MS
less than 5% - rare
Diagnosis of MS is based on the presence of
CNS lesions that are disseminated in time and space with no better explanation for the disease process
So they occur in at least 2 different parts of the CNS at least 1 month apart
Confirmatory studies
1 CNS Imaging - MRI is particularly useful as the demyelinated area apepars white
2 Evoke Potentials - visual, auditory, brainstem, somatosensory
3 CSF analysis - when myelin breaks down there is a protein that is released and it can be found in CSF
Blood tests - to eliminate other diseases
What usually gets someone a diagnosis of MS
One of the confirmatory studies showing it and the definition of 2 CNS areas impacted 1 month apart
Drug therapy - what do they start with
Immunomodulatory therapy as soon as possible to prevent disability
Drug therapy - examples of disease modifying drugs
Interferon
Mitoxantrone (chemotherapy)
Glatiramer Acetate
What do they use for flare up?
High dose of corticosteroids - usually intravenously so will be in hospital a few days
Why do we need to be careful with repeated corticosteroid use
HTN
DM
Osteopenia
Other drugs also used to treat secondary complications such as
Spasticity Fatigue Depression Erectile dysfunction Bowel function UTIs Bladder spasms
Common s/s of MS
Early vision changes, paresthesias
CST involvement - UMN lesion symptoms - weakness first followed by spasticity
CBM involvement - ataxia, tremors
FATIGUE with MS
Central/Primary Fatigue - overwhelming tiredness, exhaustion
Fatigue with MS - how common
75 - 95% experience it
50% say it is their most troubling sx
Fatigue with MS - worse with and better with what
Not related to disease severity
Worsen throughout the day, worse with heat/humidity
Better with cooling
What does heat do?
Examples
Worsens the symptoms for the patient Core temp inc by 1/4 to 1/2 a degree!!! Hot baths Hot, humid weather Fever Exercise!
Does heat permanently or temporarily worsen their symptoms
Temporarily - reversed with cooling
Pain experienced by what percent
80%
Pain with MS is described how
Paroxysmal pain - intense, sharp, shooting
Can be trigeminal neuralgia can happen from demyelination of sensory division of trigeminal
Chronic neuropathic pain too from demyelination of spinothalamic tracts
Medications for pain
Cimbalta, antidepressants, wearing compression garments
MM weakness
Decreased strength
Neurologic effects that decrease recruitment and firing rates
MM effects like atrophy
Spasticity is experienced by what percent
75%
Spasticity - where more common
LEs more than UEs
Adductors and foot
Increased DTRs, clonus
Can fluctuate on a daily basis
Spasticity is exacerbated by what
Fatigue, stress, overheating
Does spasticity abate with during remission
NO! Not typically because they have axons that are degenerated - there is permanent axonal damage
Meds for spasticity
Baclofen
Stretching, HEP, Orthotics
Balance/Falls - Inc risk with MS why
Neuronal effects can impact vision, proprioception, vestibular function
Dec mm strength leads to dec ability to recover from perturbations
Spasticity may also contribute to gait impairments
Risk of fracture from a fall is ____ than controls
Higher!
Respiratory effects
Decreased strength of respiratory mm
Dec effectiveness of coughs which leads to inc risk of pulm disease
Most common associated cause of death with MS
Pneumonias
Bone health
Inc risk of OP - dec physical activity, steroid use long term
Bone health - what can they do to monitor
DEXA scans - at least annually if not more
Autonomic effects
Bladder/Bowel
Sexual Dysfunction
Decreased sweating
Bladder function
80%
Small spastic bladder, failure to store
Flaccid, big bladder, failure to empty, UTI, kidney damage
Bowel function
Bowel dysfunction in 60%
Constipation is most common
Rehab - Aerobic exercise benefits
Dec constipation
Improve fatigue
Inc VO2 max and functional capacity
Maybe neuroprotection and maybe immunomodulation
Rehab - Aerobic exercise - need to monitor
Blunted HR response to exercise Blunted BP response to exercise RPE should stay between 11 and 14 Monitor heart stress!!! Aquatics can be beneficial! Just be careful of water temp!
Rehab - Resistance exercise
Inc mm strength/power
Inc mm mass
Bone benefits
ACSM resistance training guidelines are appropriate - but err on side of caution with progression
