Ch 24 Multiple Sclerosis Flashcards

1
Q

Describe MS

A

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

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2
Q

in simple words, describe MS

A

person’s immune system becomes dysregulated and attacks the CNS

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3
Q

brain biopsy studies of MS show?

A

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

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4
Q

Is MS primarily a white matter disease

A

previously thought of a white matter disease only

actually - gray matter also involved

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5
Q

age groups primarily affected by MS

A

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

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6
Q

genetic contribution

A

30% concordance rate in twins

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

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7
Q

male to female ratio in MS

A

2.5 : 1

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8
Q

how does MS progression differ for men and women

A

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

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9
Q

racial contributions in MS

A

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

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10
Q

geographic location and MS

A

lowest rate near equator

increased rates if moving north or south

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11
Q

environmental contributions to MS

A

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

cigarettes higher rates

breastfeeding lower rates

lower Vitamin D higher rates

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12
Q

prevention of MS

A

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

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13
Q

mortality of MS

A

90-95% average life expectancy

about 5-10 years shorter in comparison to normal

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14
Q

determinants of severity of MS

A

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

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15
Q

cognitive reserve theory and MS

A

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

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16
Q

Dx of MS is a diagnosis of ?

A

diagnosis of exclusion because presentation of symptoms is heterogeneous

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17
Q

is there definitive lab tests for MS?

A

no

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18
Q

Dx criteria of MS must include

A

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

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19
Q

MS neuropsych eval results typicall show

A

declines in processing speed
learning
free recall

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20
Q

factors to consider when selecting battery for MS

A

fatigue
processing speed
speech
upper motor deficiency

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21
Q

tests to use for MS

A

Brief Repeatable Battery of Neuropsychological Tests (BRB-N)

Minimal assessment of cog function in MS (MACFIMS)

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22
Q

presentation of MS can vary because

A

lesions can occur anywhere in the CNS

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23
Q

Typical Clinical Symptoms of MS

A

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

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24
Q

clinically isolated syndrome

A

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.

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25
Q

radiologically isolated syndrome RIS

A

Lesions are seen on imaging, no clinical correlation

1/3 of RIS converts to MS within five years.

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26
Q

Four types of MS

A

Relapsing remitting
Secondary progressive
Primary progressive
Progressive relapsing

27
Q

Relapsing remitting

A

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

28
Q

Secondary progressive

A

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

29
Q

Primary progressive

A

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

30
Q

Progressive relapsing

A

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

31
Q

Treatment for MS

A

acute relapses
overall disease progression
specific symptoms of the disease

32
Q

Treatment for acute relapses

A

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

33
Q

Treatment for disease progression

A

Disease Modifying therapies (DMT)

-injectable drugs for delay of relapses, and slow diseases progression

34
Q

Rules outs for MS

A
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
35
Q

timeframe for NP testing for MS

A

no testing within 30 days from acute relapse or steroid use

36
Q

NP testing in MS

A

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

37
Q

what NP factors are most predictive of vocational status

A

Verbal memory

EF

38
Q

IQ and MS

A

early stage - IQ unaffected

processing speed affects PRI and other tasks of speed

39
Q

attention and MS

A

sustained, complex attention bad

simple attention span ok

40
Q

processing speed and MS

A

most affected
(processing speed decrease related to thinning of corpus callosum)

reduced processing speed affects learning and EF
needs to r/o psychomotor speed

41
Q

Language and MS

A

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

42
Q

Scanning speech

A

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

43
Q

visuospatial and MS

A

timed visual scanning

visuospatial learning

44
Q

memory and MS

A

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

45
Q

executive function and MS

A

common impairment

  • attention, concentration, mental flexibility
  • word list generation
  • concept formation, abstraction

affects jobs and IADLs
frontal lobe lesion associated

46
Q

sensorimotor function and MS

A

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

47
Q

mood and MS

A

depression
anxiety
lability

possibly due to frontal lobe white matter lesions
depression may be related to lesions and cytokine effects

pseudobulbar episodes may occur

48
Q

pediatric considerations and MS

A

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

49
Q

prevalence of MS in peds

A

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

50
Q

racial difference in MS in peds cases

A

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

51
Q

gender in MS in peds

A

girls more than boys

before age 10 - about the same

52
Q

differential diagnosis in MS in peds

A

ADEM (CNS demyelinating condition)
Neuromyelitis optica
antiMOG

53
Q

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

A

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

54
Q

cognition in Peds MS

A

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

55
Q

schooling in peds in MS

A

need accommodations

56
Q

psych functions in peds in MS

A

higher risk for psych problems

30-50% internalizing disorders eg depression/anxiety

57
Q

treatment adherence in peds with MS

A

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

58
Q

adult treatment with MS

A

cant drive 2/2 physical impairment, slow RT, VS, mental flexibility

work - can work, depends on nature and disease course. fatigue plays a role

59
Q

mood in adults with MS

A

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

60
Q

medication in adult MS

A

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

61
Q

cog rehab in MS

A

need more research

62
Q

geriatric considerations in MS

A

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

63
Q

geriatric patients complications with MS

A
recurrent infections (pneumonia, pulmonary embolism, infection ulcers, suicide)
other comorbid- heart disease, HTN, DM
aspiration pneumonia
bladder or kidney infection
osteoporosis
64
Q

BRB test short version includes what tests

A

PASAT
selective reminding
SDMT