Ch 24 Multiple Sclerosis Flashcards
Describe MS
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
in simple words, describe MS
person’s immune system becomes dysregulated and attacks the CNS
brain biopsy studies of MS show?
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
Is MS primarily a white matter disease
previously thought of a white matter disease only
actually - gray matter also involved
age groups primarily affected by MS
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
genetic contribution
30% concordance rate in twins
6-8x more likely in those with first degree relatives with MS
male to female ratio in MS
2.5 : 1
how does MS progression differ for men and women
women more likely to be diagnosed
men more likely to develop progressive disease with more disability and cog impairment
females earlier onset than men
racial contributions in MS
White more common than minorities in adult population (not in peds)
geographic location and MS
lowest rate near equator
increased rates if moving north or south
environmental contributions to MS
ped MS associated with Epstein Barr virus, and other pathogens and bacterial infections
cigarettes higher rates
breastfeeding lower rates
lower Vitamin D higher rates
prevention of MS
use Vitamin D in pregnant women to reduce likelihood of MS in children
mortality of MS
90-95% average life expectancy
about 5-10 years shorter in comparison to normal
determinants of severity of MS
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
cognitive reserve theory and MS
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
Dx of MS is a diagnosis of ?
diagnosis of exclusion because presentation of symptoms is heterogeneous
is there definitive lab tests for MS?
no
Dx criteria of MS must include
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
MS neuropsych eval results typicall show
declines in processing speed
learning
free recall
factors to consider when selecting battery for MS
fatigue
processing speed
speech
upper motor deficiency
tests to use for MS
Brief Repeatable Battery of Neuropsychological Tests (BRB-N)
Minimal assessment of cog function in MS (MACFIMS)
presentation of MS can vary because
lesions can occur anywhere in the CNS
Typical Clinical Symptoms of MS
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
clinically isolated syndrome
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.
radiologically isolated syndrome RIS
Lesions are seen on imaging, no clinical correlation
1/3 of RIS converts to MS within five years.
Four types of MS
Relapsing remitting
Secondary progressive
Primary progressive
Progressive relapsing
Relapsing remitting
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
Secondary progressive
Initially presents as relapsing remitting, but becomes progressive worsening relative to baseline functioning between attacks
No periods of remission
Greatest deficits in cognitive functioning
Primary progressive
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
Progressive relapsing
5%
Progressive deterioration of function from the onset
Distinct acute exacerbations or relapses
Gradual deterioration between exacerbations
Treatment for MS
acute relapses
overall disease progression
specific symptoms of the disease
Treatment for acute relapses
Treated through IV corticosteroids and oral prednisone taper.
Steroids are ineffective, plasmapheresis or intravenous immunoglobulin
Treatment for disease progression
Disease Modifying therapies (DMT)
-injectable drugs for delay of relapses, and slow diseases progression
Rules outs for MS
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
timeframe for NP testing for MS
no testing within 30 days from acute relapse or steroid use
NP testing in MS
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
what NP factors are most predictive of vocational status
Verbal memory
EF
IQ and MS
early stage - IQ unaffected
processing speed affects PRI and other tasks of speed
attention and MS
sustained, complex attention bad
simple attention span ok
processing speed and MS
most affected
(processing speed decrease related to thinning of corpus callosum)
reduced processing speed affects learning and EF
needs to r/o psychomotor speed
Language and MS
language not affected early on
aphasia very rare
primary speech difficulties (e.g. dysarthria, hypophonia)
scanning speech
children show greater language impairment than adults
Scanning speech
spoken words are broken up with interrupted syllables, pauses, varying intonation
caused by cerebellar lesions
common sx of MS
visuospatial and MS
timed visual scanning
visuospatial learning
memory and MS
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
executive function and MS
common impairment
- attention, concentration, mental flexibility
- word list generation
- concept formation, abstraction
affects jobs and IADLs
frontal lobe lesion associated
sensorimotor function and MS
commonly affected
motor speed bilaterally affected
can be lateralized, depending on lesion location
mood and MS
depression
anxiety
lability
possibly due to frontal lobe white matter lesions
depression may be related to lesions and cytokine effects
pseudobulbar episodes may occur
pediatric considerations and MS
greater risk for cog impairment than adults because consequences on developing brain during ongoing myelinogenesis
prevalence of MS in peds
2-5% under 18 years
most commonly in teenage years
exclusively relapse remitting
racial difference in MS in peds cases
more common in minorities (VS in adults, more whites than minorities)
gender in MS in peds
girls more than boys
before age 10 - about the same
differential diagnosis in MS in peds
ADEM (CNS demyelinating condition)
Neuromyelitis optica
antiMOG
how to distinguish between MS in peds and ADEM in peds?
presence of encephalopathy (e.g. change in mental status, personality) observed in the early stages of ADEM
cognition in Peds MS
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
schooling in peds in MS
need accommodations
psych functions in peds in MS
higher risk for psych problems
30-50% internalizing disorders eg depression/anxiety
treatment adherence in peds with MS
DMT - injection (struggle with adherence b/c of needles)
adult treatment with MS
cant drive 2/2 physical impairment, slow RT, VS, mental flexibility
work - can work, depends on nature and disease course. fatigue plays a role
mood in adults with MS
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
medication in adult MS
fatigue: amantadine, modafinil
antidepressants (which can also treat inflammation)
fatigue and cog enhancer: stimulant (dextroamphetamine, methylphenidate)
cog rehab in MS
need more research
geriatric considerations in MS
longer disease duration
greater disability
if dx at old age, progressive subtype
geriatric patients complications with MS
recurrent infections (pneumonia, pulmonary embolism, infection ulcers, suicide) other comorbid- heart disease, HTN, DM aspiration pneumonia bladder or kidney infection osteoporosis
BRB test short version includes what tests
PASAT
selective reminding
SDMT
