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51 Cards in this Set
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- Back
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Multiple sclerosis defined: |
chronic and progressive inflammatory autoimmune disease of the CNS |
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immune response primarily results in : |
attack on myelin sheathing which results in axonal damage |
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Brain biopsy studies show MS lesions are characterized by: |
perivascular inflammation and demyelination |
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acute lesions in MS: |
infiltrates of immune system T cells, B cells, and macrophages |
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chronic affected regions: |
demyelination and associated gliosis and axonal damage |
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MS primarily affects young adults between what ages? |
20-40 (average age of onset of 30)
can occur in younger ages and as late as 80's
5% have onset before age 18 |
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Genetic contribution to development of MS: |
first degree relative os patients with MS are 6-8 times more likely to develop the disease |
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Men Vs. Women in MS |
women>men (2:5:1)
men develop more destructive lesions and greater cognitive impairment
med less likely to experience a primarily inflammatory response
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MS more common in this ethinicity |
Caucasians of norther European descent |
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MS rates and geographic area |
lowest near the equator and increasingly more common as one moves father north or south |
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environmental factors contributing to susceptibility to MS: |
pediatric MS - has been associated with an increased frequency of Epstein-Barr virus
exposure to cigarette smoke may increase the risk of MS development
breast-fed children are less likely to develop MS
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mortality in MS |
90-95% will experience an average life expectancy |
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Severity of MS |
-young age of onset associated with lower relapse rate and rate of disease progression -racial/ethnic minorities less likely to contract MS, but have a more pernicious course - Vitamin D (serum vitamin D levels) associated with higher relapse rate -women with MS during pregnancy tend to have fewer relapses
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cognitive reserve in MS |
processing speed declines may be moderated by high cognitive reserve |
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Diagnosis of MS |
essentially a diagnosis of exclusion due to heterogenous symptom presentation |
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What is McDonald criteria? |
newest MS diagnostic criteria that allows for more rapid diagnosis
Must be evidence of CNC lesions disseminated across both space and time |
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Specific criteria: |
-patient must have two or more objective clinical attack with positive MRI findings - dissemination of lesion in space can be demonstrated in at least one T2 lesion in two of four areas in CNS (periventricular, juxtacortical, infratentorial, or spinal cord -a new MRI lesion may establish dissemination in time (regardless of time from baseline MRI) other explanations |
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neuropsychological findings for MS |
declines in psychomotor processing speed, learning, and free recall |
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Brief batteries developed for serial assessment of individuals with MS |
Brief Repeatable Battery (BRB) Minimal Assessment of Cognitive Function in Multiple Sclerosis (MACFIMS) |
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most common initial clinical symptoms |
*optic neuritis - inflammation of optic nerve *somtosensory - 21-55% of early symptoms, parathesias *corticospinal tract - 32-41% of early symptoms, bladder and bowel dysfunction cerebellar/brainstem - ataxia, speech problems, or diplopia *fatigue - 75-90% experience. Most cited reason for unemployment in MS *SLeep - sleep disorders common
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Clinically isolated syndrome (CIS) |
patients who have not yet met criteria for a definite diagnosis of MS, but had one episode of a neurologic even similar to MS
term used to describe first episode that lasts at least 24 hours and results in inflammation or demyelination in CNS |
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Four types of MS |
relapsing-remitting secondary-progressive primary-progressive progressive-relapsing |
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Most common initial diagnosis of MS |
relapsing-remitting
85% of patients with MS initially present with a course consistent with this type |
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Relapsing-remitting and converstion to secondary progressive MS occurs from : |
remaining untreated, half of persons with RRMS will convert to secondary-progressive MS within 10-15 years after initial disease onset |
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primary-progressive prevalence
progressive-relapsing prevalence |
10%
5% |
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Three components of treatment for MS |
treatment for acute relapses treatment for the overall disease progression treatments for specific symptoms of the disease |
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Treatment of acute relapses |
treated via intravenous corticosteroids followed by oral prednisone taper
for individuals where steroids are ineffective, plasmapheresis or intravenous immunoglobulin (IVIG) may be used
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Treatment of disease progression |
disease modifying therapies (DMTs) - injectable drugs that delay relapses and slow disease progression
medications include formulations of interferon (IFN) such as IFN beta 1a (Avonex and Rebif), IFN beta 1b (Betaseron), and glatiramer acetate (Copaxone) for injection
those with very active disease that do not respond to first line treatments may also use mitoxantone (Novantrone) that is an immunosuppresant
Another second line agent - Natalizumab (Tysabri) administered by infusion
Fingolimod (Gilenya) first oral agent for use in MS, recently approved by US FDA, but second line treatment due to risks |
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Diagnostic rule outs |
leukodystrophies progressive multifocal leukoencephalopathy acute disseminated encephalomyelitis systemic autoimmune disease Guillain-barre syndrome toxic optic neuropathy neuromyelitis optica |
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incidence of cognitive impairment in MS |
40-65% |
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Most common cognitive sequelea |
processing speed (associated with thinning of CC), concentration, and declarative memory |
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Cognitive deficits most predictive of vocational status |
verbal memory and executive dysfunction |
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Prevalence of MS in pediatric population |
5% under age of 18, most commonly onset in the teen years |
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Most cases of pediatric MS are this type: |
Relapsing-remitting |
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Ethnicity and pediatric MS |
may be more common in more racially and ethnically diverse populations when compared to adults with MS |
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Gender in pediatric MS |
female to male ratio varies with age
girls generally outnumber boys
BUT, boys slightly outnumber girls prior to age 6 |
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Differentiating diagnosis in pediatric MS |
most common is differentiating MS from acute disseminated encephalomyelitis (ADEM)
discriminating clinical feature is the presence of severe encephalopahty in early phases of ADEM.
Children may experience seizures and mental status changes that may not be as common in adults |
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Cognitive deficits in pediatric MS |
deficits found in 30-53% of those sampled
similar to adults, show deficits in memory, attention, executive functioning, visual-motor skills, and processing speed
children show more difficulty with language-based skills and less difficulty with verbal fluency |
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psychological considerations in pediatric MS |
children with chronic illness at higher risk for psychiatric problems
ped MS - associated with internalizing disorders such as anxiety and depression
consider referral for family therapy or therapeutic camp interventions to allow for communication improvement and connection with similar peers |
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Adult considerations in MS |
-need for supervision in later stages -discontinuation of driving due to physical and cognitive changes -work outcomes depending on severity and type of job -education may require accommodations -capacity may be affected when frontal system dysfunction occurs -psychological factors affecting outcomes -interpersonal relationships - financial issues and caregiver burnout
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psychological and emotional issues in adult MS |
-prevalence of depression common (50%) -anxiety less common -pathological symptoms of crying or laughing can occur with lesions to corticobulbar tracts -euphoria and apathy can also occur from demyelination of frontal lobes -adjustment issues due to lifestyle changes -nonpharmacological interventions show promise (CBT and support groups) |
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COgnitive rehabiliation outcomes in MS |
limited evidence regarding cognitive rehabilitation
recent Cochrane Review of cog rehab in MS concluded "low evidence" that cog rehab improved neuropsych functioning in MS (Rosti-Otajarvi & Hamalainen, 2011) |
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Geriatric considerations in MS |
-have longer disease duration -associated with greater physical and cognitive disability -require high level of support |
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End stage disease MS |
-MS is not a fatal disease -patients die from complications during final stage -typically die from recurrent infections (pneumonia, PE, ulcer infections) -occasionally suicide is reported
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Physical complications in advanced MS |
-decubitus ulcers due to lack of mobility -aspiration pneumonia due to swallowing problems -severe bladder or kidney infections and/or bladder control problems due to chronic urinary dysfunction -osteoporosis due to reduced mobility and corticosteroid treatment |
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Brief Repeatable Battery of Neuropsychological Tests (BRB) |
40 minute battery administered to screen for cognitive dysfunction
a short version of the BRB has been validated and includes three tests: selective reminding test, PASAT-3, and symbol digit modalities test
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Expanded Disability Status Scale (EDSS) |
One method of quantifying disability in MS
quantifies disability based on eight functional systems including pyramidal, cerebellar, brainstem, sensory, bowel/bladder, visual, cerebral, and other.
scores range from 0 to 10, with higher scores associated with greater disability |
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Fatigue Severity Scale |
A method of evaluating serenity of fatigue in MS.
scale consists of a brief questionnaire requiring the patient to rate his or her level of fatigue based on a 7-point scale indicating the rater's degree of agreement with the statment
scale is available in multiple languages |
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Minimal Assessment of Cognitive Function in Multiple Sclerosis (MACFIMS) |
90 minute battery including seven subtests targeting areas most commonly impacted in MS:
COWA, JOLO, CVLT-II, BVMT-R, Symbol digit modalities test, PASAT, and DKEFS Sorting Test |
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Multiple Sclerosis Functional Composite (MSFC) |
A screening battery validated in adults with MS and includes: 9 hole peg test, Timed 25-Foot walk, and PASAT. |
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Scanning speech defined: |
(aka explosive speech)
A speech disorder in which spoken words are broken up with interrupted syllables, noticeable pauses, and varying intonation.
syllables can be spoke with variable force and incorrectly placed syllable stress
typically cased by cerebellar lesions and common in MS
characteristic of ataxic dysarthria and one of three symptoms of Charcot's neurologic triad |
