CNS Demyelinating Disorders

Question 1

Article

Answer 1

READ it! There will be a question from it!

Question 2

MS is a ___ ____ ___ disease of the ___

Answer 2

Chronic, autoimmune, inflammatory disease of the CNS

Question 3

MS is often initially episodes of what, followed by what

Answer 3

Episodes of reversible neurological deficits followed by progressive neurological deterioration over time

Question 4

MS - rates are higher in what geographic area

Answer 4

Farther from the equator

Question 5

MS - cause

Answer 5

True cause is unknown

Involves combination of genetic and non genetic triggers

Question 6

Pathophysiology MS

Answer 6

Genetic susceptibility plus viral trigger and then leads to a higher immune response

Question 7

Pathophysiology MS - genetic susceptibility plus viral trigger and then heightened immune response leads to what

Answer 7

T cell antibodies attack oligodendrocytes

The T cells are attacking the myelin in the CNS “friendly fire”

Question 8

Can oligodendrocytes survive the insult from T cell attack?

Answer 8

Initially they can remyelinate but over time they can’t repair themselves and will end up with demyelination

Question 9

What happens to the areas of the axon that are demyelinated

Answer 9

Gliosis! accumulation of the astrocytes and microglia

This gliosis forms glial scars (plaques) and the axon will degenerate

Question 10

What can gliosis do to synaptic transmission

Answer 10

Slow it down at first and then block it completely

Question 11

What is the primary cause of permanent clinical disability

Answer 11

Axonal damage!!!

Question 12

What areas are most vulnerable to demyelination

Answer 12

Optic nerves 
Periventricular white matter
CST tracts
Posterior (dorsal) columns
Cerebellar peduncles

Question 13

Pro Inflammatory and Anti Inflammatory Cytokines

Answer 13

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

Question 14

Why is there a higher incidence of MS in regions with less sunlight

Answer 14

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

Question 15

What is one of the independent risk factors for MS

Answer 15

Vitamin D insufficiency

Question 16

Forms of MS

Answer 16

Relapsing Remitting MS (RRMS)
Secondary Progressive MS (SPMS)
Primary Progressive MS (PPMS)
Progressive Relapsing MS (PRMS)

Question 17

Relapsing Remitting MS (RRMS)

Answer 17

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

Question 18

Relapsing Remitting MS - how common

Answer 18

Most common

85%

Question 19

Secondary Progressive MS

Answer 19

Begins as relapsing remitting but then at some point turns progressive and steady decline over time, with or without acute attacks

Question 20

Primary Progressive MS

Answer 20

Worsens continuously from onset - usually without distinct attacks
Flare up 100% of the time and just keeps adding s/s

Question 21

Primary Progressive MS - how common is it

Answer 21

15%

Question 22

Progressive Relapsing MS

Answer 22

Primary progressive (so continues to get worse) plus periods of more severe acute attacks and no remission

Question 23

How common is progressive relapsing MS

Answer 23

less than 5% - rare

Question 24

Diagnosis of MS is based on the presence of

Answer 24

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

Question 25

Confirmatory studies

Answer 25

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

Question 26

What usually gets someone a diagnosis of MS

Answer 26

One of the confirmatory studies showing it and the definition of 2 CNS areas impacted 1 month apart

Question 27

Drug therapy - what do they start with

Answer 27

Immunomodulatory therapy as soon as possible to prevent disability

Question 28

Drug therapy - examples of disease modifying drugs

Answer 28

Interferon Mitoxantrone (chemotherapy) Glatiramer Acetate

Question 29

What do they use for flare up?

Answer 29

High dose of corticosteroids - usually intravenously so will be in hospital a few days

Question 30

Why do we need to be careful with repeated corticosteroid use

Answer 30

HTN DM Osteopenia

Question 31

Other drugs also used to treat secondary complications such as

Answer 31

``` Spasticity Fatigue Depression Erectile dysfunction Bowel function UTIs Bladder spasms ```

Question 32

Common s/s of MS

Answer 32

Early vision changes, paresthesias CST involvement - UMN lesion symptoms - weakness first followed by spasticity CBM involvement - ataxia, tremors

Question 33

FATIGUE with MS

Answer 33

Central/Primary Fatigue - overwhelming tiredness, exhaustion

Question 34

Fatigue with MS - how common

Answer 34

75 - 95% experience it | 50% say it is their most troubling sx

Question 35

Fatigue with MS - worse with and better with what

Answer 35

Not related to disease severity Worsen throughout the day, worse with heat/humidity Better with cooling

Question 36

What does heat do? | Examples

Answer 36

``` Worsens the symptoms for the patient Core temp inc by 1/4 to 1/2 a degree!!! Hot baths Hot, humid weather Fever Exercise! ```

Question 37

Does heat permanently or temporarily worsen their symptoms

Answer 37

Temporarily - reversed with cooling

Question 38

Pain experienced by what percent

Answer 38

80%

Question 39

Pain with MS is described how

Answer 39

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

Question 40

Medications for pain

Answer 40

Cimbalta, antidepressants, wearing compression garments

Question 41

MM weakness

Answer 41

Decreased strength Neurologic effects that decrease recruitment and firing rates MM effects like atrophy

Question 42

Spasticity is experienced by what percent

Answer 42

75%

Question 43

Spasticity - where more common

Answer 43

LEs more than UEs Adductors and foot Increased DTRs, clonus Can fluctuate on a daily basis

Question 44

Spasticity is exacerbated by what

Answer 44

Fatigue, stress, overheating

Question 45

Does spasticity abate with during remission

Answer 45

NO! Not typically because they have axons that are degenerated - there is permanent axonal damage

Question 46

Meds for spasticity

Answer 46

Baclofen | Stretching, HEP, Orthotics

Question 47

Balance/Falls - Inc risk with MS why

Answer 47

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

Question 48

Risk of fracture from a fall is ____ than controls

Answer 48

Higher!

Question 49

Respiratory effects

Answer 49

Decreased strength of respiratory mm | Dec effectiveness of coughs which leads to inc risk of pulm disease

Question 50

Most common associated cause of death with MS

Answer 50

Pneumonias

Question 51

Bone health

Answer 51

Inc risk of OP - dec physical activity, steroid use long term

Question 52

Bone health - what can they do to monitor

Answer 52

DEXA scans - at least annually if not more

Question 53

Autonomic effects

Answer 53

Bladder/Bowel Sexual Dysfunction Decreased sweating

Question 54

Bladder function

Answer 54

80% Small spastic bladder, failure to store Flaccid, big bladder, failure to empty, UTI, kidney damage

Question 55

Bowel function

Answer 55

Bowel dysfunction in 60% | Constipation is most common

Question 56

Rehab - Aerobic exercise benefits

Answer 56

Dec constipation Improve fatigue Inc VO2 max and functional capacity Maybe neuroprotection and maybe immunomodulation

Question 57

Rehab - Aerobic exercise - need to monitor

Answer 57

``` 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! ```

Question 58

Rehab - Resistance exercise

Answer 58

Inc mm strength/power Inc mm mass Bone benefits ACSM resistance training guidelines are appropriate - but err on side of caution with progression