Ch 24 Multiple Sclerosis

Question 1

Describe MS

Answer 1

chronic, progressive inflammatory autoimmune disorder of the CNS
immune system response results in attack on myelin sheathing in the brain and spinal cord -> axonal damage and slowing axonal signal transmission

Question 2

in simple words, describe MS

Answer 2

person’s immune system becomes dysregulated and attacks the CNS

Question 3

brain biopsy studies of MS show?

Answer 3

MS lesions are characterized by perivascular inflammation and demyelination
acute neuroimaging findings - lesions show infiltrates of immune system T cells, B cells and macrophages
chronically affected regions - demyelination and assoicated gliosis, axonal damage

Question 4

Is MS primarily a white matter disease

Answer 4

previously thought of a white matter disease only

actually - gray matter also involved

Question 5

age groups primarily affected by MS

Answer 5

20-40 (average 30 year old onset)
can occur in peds or as late as 80s
2-5% onset before age 18
very few before puberty

Question 6

genetic contribution

Answer 6

30% concordance rate in twins

6-8x more likely in those with first degree relatives with MS

Question 7

male to female ratio in MS

Answer 7

2.5 : 1

Question 8

how does MS progression differ for men and women

Answer 8

women more likely to be diagnosed
men more likely to develop progressive disease with more disability and cog impairment
females earlier onset than men

Question 9

racial contributions in MS

Answer 9

White more common than minorities in adult population (not in peds)

Question 10

geographic location and MS

Answer 10

lowest rate near equator

increased rates if moving north or south

Question 11

environmental contributions to MS

Answer 11

ped MS associated with Epstein Barr virus, and other pathogens and bacterial infections

cigarettes higher rates

breastfeeding lower rates

lower Vitamin D higher rates

Question 12

prevention of MS

Answer 12

use Vitamin D in pregnant women to reduce likelihood of MS in children

Question 13

mortality of MS

Answer 13

90-95% average life expectancy

about 5-10 years shorter in comparison to normal

Question 14

determinants of severity of MS

Answer 14

onset of younger age - lower relapse rate, slower rate of progression
racial minorities - less likely, although course is more severe
lower levels of vitamin D - higher relapse rate
pregnant women - fewer relapses, see improvement in neurologic fx, maybe b/c of hormones

Question 15

cognitive reserve theory and MS

Answer 15

explanatory model to account for individual diff in expression of cog impairment

• education
• literacy
• enrichment activities

cognitive processing speed declines - moderated by high cog reserve - can withstand greater neuropathology (e.g. brain atrophy) without more speed deficits

Question 16

Dx of MS is a diagnosis of ?

Answer 16

diagnosis of exclusion because presentation of symptoms is heterogeneous

Question 17

is there definitive lab tests for MS?

Answer 17

no

Question 18

Dx criteria of MS must include

Answer 18

evidence of CNS lesions disseminated across both space AND time

2 or more clinical attacks with positive MRI findings

dissemination of lesions in space demonstrated in at least one T2 lesion in 2 of 4 areas in CNS

• periventricular
• juxtacortical
• infratentorial
• spinal cord

EVENTS NO LONGER NEED TO BE SEPARATED IN TIME BY 3O DAYS

Question 19

MS neuropsych eval results typicall show

Answer 19

declines in processing speed
learning
free recall

Question 20

factors to consider when selecting battery for MS

Answer 20

fatigue
processing speed
speech
upper motor deficiency

Question 21

tests to use for MS

Answer 21

Brief Repeatable Battery of Neuropsychological Tests (BRB-N)

Minimal assessment of cog function in MS (MACFIMS)

Question 22

presentation of MS can vary because

Answer 22

lesions can occur anywhere in the CNS

Question 23

Typical Clinical Symptoms of MS

Answer 23

CCF SOS

Optic neuritis - inflammation of the optic nerve resulting in blurring of vision. Occur unilaterally

Somatosensory - 21% to 55% in early phase, increased to 70% over the course. Paresthesia, numbness and tingling.

Corticospinal tract - 32% of 41% in early stages, 50% over the course. Bladder and bowel dysfunction

Cerebellar brainstem - ataxia, speech problems, diplopia.

Fatigue- 80% of adults, 50% of children. Most disabling symptom. Exacerbated by heat. Biggest reason for unemployment

Sleep.- 26% children. Restless syndrome, sleep disordered breathing. Can be due to pain and spatisticity

Question 24

clinically isolated syndrome

Answer 24

not met criteria for MS
had one episode of event similar to MS
describes first episode that lasts at least 24 hours
first episode - single symptom - numbness on one side cause by single lesion (monofocal) OR multiple symptoms (multifocal) caused by lesions in more than one location in the CNS.

Question 25

radiologically isolated syndrome RIS

Answer 25

Lesions are seen on imaging, no clinical correlation | 1/3 of RIS converts to MS within five years.

Question 26

Four types of MS

Answer 26

Relapsing remitting Secondary progressive Primary progressive Progressive relapsing

Question 27

Relapsing remitting

Answer 27

85% all peds case belong HERE Most common initial diagnosis distinct development of symptoms followed by variable recovery stability and improvement between episodes if not treated, becomes secondary progressive in 10-15 years

Question 28

Secondary progressive

Answer 28

Initially presents as relapsing remitting, but becomes progressive worsening relative to baseline functioning between attacks No periods of remission Greatest deficits in cognitive functioning

Question 29

Primary progressive

Answer 29

10% Continuous gradual worsening of functions from the onset with minor fluctuations No distinct exacerbations or remission *Mobility difficulty such as stiffness or weakness More likely to occur in older individuals

Question 30

Progressive relapsing

Answer 30

5% Progressive deterioration of function from the onset Distinct acute exacerbations or relapses Gradual deterioration between exacerbations

Question 31

Treatment for MS

Answer 31

acute relapses overall disease progression specific symptoms of the disease

Question 32

Treatment for acute relapses

Answer 32

Treated through IV corticosteroids and oral prednisone taper. Steroids are ineffective, plasmapheresis or intravenous immunoglobulin

Question 33

Treatment for disease progression

Answer 33

Disease Modifying therapies (DMT) | -injectable drugs for delay of relapses, and slow diseases progression

Question 34

Rules outs for MS

Answer 34

``` Leukodystrophies Progressive multifocal leukoencephalopathy PML ADEM Transverse myelitis Neuromyelitis optica Anti MOG assoicated encephalomyelitis autoimmune disease - lupus, sarcoidosis, sjogren's syndrome guillain barre syndrome toxic opic neuropathy brain tumor ```

Question 35

timeframe for NP testing for MS

Answer 35

no testing within 30 days from acute relapse or steroid use

Question 36

NP testing in MS

Answer 36

cog impairment 45-60% deficits progress over time (Esp with progressive disease course) most affected: processing speed, sustained attention learning and declarative memory Less affected: language VS perception remote memory

Question 37

what NP factors are most predictive of vocational status

Answer 37

Verbal memory | EF

Question 38

IQ and MS

Answer 38

early stage - IQ unaffected | processing speed affects PRI and other tasks of speed

Question 39

attention and MS

Answer 39

sustained, complex attention bad | simple attention span ok

Question 40

processing speed and MS

Answer 40

most affected (processing speed decrease related to thinning of corpus callosum) reduced processing speed affects learning and EF needs to r/o psychomotor speed

Question 41

Language and MS

Answer 41

language not affected early on aphasia very rare primary speech difficulties (e.g. dysarthria, hypophonia) scanning speech children show greater language impairment than adults

Question 42

Scanning speech

Answer 42

spoken words are broken up with interrupted syllables, pauses, varying intonation caused by cerebellar lesions common sx of MS

Question 43

visuospatial and MS

Answer 43

timed visual scanning | visuospatial learning

Question 44

memory and MS

Answer 44

learning and memory (encoding and retrieval) earliest problems explicit memory affected early reduced memory due to reduced learning curve, not impaired recall (forgetting) semantic and implicit memory are not affected until later

Question 45

executive function and MS

Answer 45

common impairment - attention, concentration, mental flexibility - word list generation - concept formation, abstraction affects jobs and IADLs frontal lobe lesion associated

Question 46

sensorimotor function and MS

Answer 46

commonly affected motor speed bilaterally affected can be lateralized, depending on lesion location

Question 47

mood and MS

Answer 47

depression anxiety lability possibly due to frontal lobe white matter lesions depression may be related to lesions and cytokine effects pseudobulbar episodes may occur

Question 48

pediatric considerations and MS

Answer 48

greater risk for cog impairment than adults because consequences on developing brain during ongoing myelinogenesis

Question 49

prevalence of MS in peds

Answer 49

2-5% under 18 years most commonly in teenage years exclusively relapse remitting

Question 50

racial difference in MS in peds cases

Answer 50

more common in minorities (VS in adults, more whites than minorities)

Question 51

gender in MS in peds

Answer 51

girls more than boys | before age 10 - about the same

Question 52

differential diagnosis in MS in peds

Answer 52

ADEM (CNS demyelinating condition) Neuromyelitis optica antiMOG

Question 53

how to distinguish between MS in peds and ADEM in peds?

Answer 53

presence of encephalopathy (e.g. change in mental status, personality) observed in the early stages of ADEM

Question 54

cognition in Peds MS

Answer 54

1/3 of peds with MS have cog deficits memory, attention, EF, VS, processing speed more difficulty with language based skills (e.g. verbal knowledge, receptive/expressive language, verbal fluency) IQ may be lower

Question 55

schooling in peds in MS

Answer 55

need accommodations

Question 56

psych functions in peds in MS

Answer 56

higher risk for psych problems | 30-50% internalizing disorders eg depression/anxiety

Question 57

treatment adherence in peds with MS

Answer 57

DMT - injection (struggle with adherence b/c of needles)

Question 58

adult treatment with MS

Answer 58

cant drive 2/2 physical impairment, slow RT, VS, mental flexibility work - can work, depends on nature and disease course. fatigue plays a role

Question 59

mood in adults with MS

Answer 59

depression 50% (shared sx like fatigue, poor initiation) anxiety 25% (less than depression) euphoria, apathy - related to demyelination of drontal lobe pseudobulbar - lesion in corticobulbar tracts hypomania or mania - corticosteroids

Question 60

medication in adult MS

Answer 60

fatigue: amantadine, modafinil antidepressants (which can also treat inflammation) fatigue and cog enhancer: stimulant (dextroamphetamine, methylphenidate)

Question 61

cog rehab in MS

Answer 61

need more research

Question 62

geriatric considerations in MS

Answer 62

longer disease duration greater disability if dx at old age, progressive subtype

Question 63

geriatric patients complications with MS

Answer 63

``` recurrent infections (pneumonia, pulmonary embolism, infection ulcers, suicide) other comorbid- heart disease, HTN, DM aspiration pneumonia bladder or kidney infection osteoporosis ```

Question 64

BRB test short version includes what tests

Answer 64

PASAT selective reminding SDMT