Multiple Sclerosis Flashcards
Describe Multiple Sclerosis (MS).
A chronic and progressive inflammatory autoimmune disease of the CNS.
Immune system response results in attack on myelin sheathing which results in axonal damage.
What are brain biopsy findings of MS?
MS lesions are characterized by perivascular inflammation and demyelination.
Describe the differences between acute and chronic lesions in MS.
Acute lesions: infiltrates of immune system T cells, B cells, and macrophages
Chronic lesions: Demyelination and associated gliosis, with axonal damage.
Gray matter can also be involved even in the earliest stages but less so than WM involvement
In what age range and ethnicity is MS most likely to occur?
20-40, but can occur in younger individuals and up to the 8th decade of life
5% have onset prior to age 18
More common in Caucasians than minorities.
Describe the differences between women and men with regard to MS.
Women are more likely than men to develop the disease (2.5:1).
Men are more likely to develop destructive lesions and greater cog impairment.
Men are less likely to experience a primarily inflammatory response.
Does genetics contribute to the development of MS?
Yes. First degree relatives are 6-8 times more likely to develop the disease
Do rates of MS increases as you get farther from or closer to the equator?
Farther from the equator
What are environmental factors that contribute to the susceptibility of MS>
Onset of MS is thought to be a response to an environmental exposure that occurred many years prior.
Pediatric MS has been associated with Epstein-Barr virus.
Exposure to cigarette smoke may increase the risk.
Babies who are breast fed are less likely to develop MS later in life.
What percentage of patients with MS experience an average life expectancy?
90-95%
What factors are associated with severity in MS?
Younger age of onset= lower relapse rate and slower rate of disease progression
Racial and ethnic minorities less likely to contract MS but if they do it is a worse disease course
Lower levels of Vitamin D= higher relapse rate
Pregnant women with MS tend to experience fewer relapses and may notice improvement (may experience rebound after giving birth)
What is the role of cognitive reserve in MS?
Cognitive processing speed declines may be moderated by high cognitive reserve. Those with MS and high cog reserve may withstand greater neuropathology w/out showing information processing speed deficits.
How do you diagnose MS?
It is a diagnosis of exclusion.
Evidence of CNS lesions that are disseminated across brain areas and time.
- 2 or more objective clinical attacks w/ + MRI findings
- At least 1 T2 lesion in 2 of 4 areas: periventricular, juxtacortical, infratentorial, and spinal cord
- A new MRI lesion may establish disseimination in time regardless of time from baseline MRI
What cognitive changes typically occur very early in the disease and progress in later stages?
Processing speed, learning, and free recall
What considerations should be made when assessing a patient with MS?
Fatigue, slowed mental processing, and speech and upper extremity motor deficiencies
What are the most common initial motor and sensory changes?
- Optic neuritis: inflammation of optic nerve causing blurred vision (unilaterally)
- Somatosensory: 21-55% of early symptoms (parasethsias)
- Corticospinal tract: 32-41% of early sx, bladder and bowel dysfunction
- Cerebellar/brainstem: ataxia, speech problems, diplopia
- Fatigue: often the most disabling symptom and most common reason for unemployment. Exacerbated by heat
- Sleep: insomina, sleep disordered breathing, resltess leg syndrom
What is diagnosed if a person has one episode of a neurologic event similar to MS>?
Clinically Isolated Syndrome
Describes the first episode that lasts at least 24 hours and results from inflammation/demylenation. Can be monofocal or multifocal,
What are the 4 disease courses in MS?
1) Relapsing-Remitting: Distinct development of neurologic sx followed by variable recovery of function (85% of patients with MS)
2) Secondary-Progressive: Initially presents as RRMS but then progressive worsening with no periods of remission
3) Primary-Progressive: Continuous gradual worsening of functions; mobility difficulty is most common sx’ more likely in older patients; 10% of those with MS
4) Progressive-Relapsing: Progressive deterioration but distinct exacerbations/relapses (5% of patients with MS)
Which disease course in MS is associated with the greatest deficits in cognitive functioning?
Secondary-Progressive
50% of patients with RRMS left untreated with convert to secondary progressive MS w/in 10-15 years
How are acute relapses treated?
- IV corticosteroids w/ oral prednisone taper
- IVIG if steroids are ineffective
How is disease progression treated?
With the use of disease modifying therapies.
Injectable drugs that delay relapses and slow progression.
Examples: interferon drugs (Betaseron, Copaxone), mitoxantrone, Tysabari, fingolimod
What are disorders that adversely impact WM and have similar symptom presentations as MS?
Leukodystrophies, progressive multifocal leukoencepalopathy, acute disseminated encephalomyelitis, systemic autoimmune diseases, Gullain-Barre Syndrome, toxic optic neuropathy, brain tumor, and meuromyelitis optica
What percentage of patients with MS have cognitive impairment?
40-65%
Whole brain and thalamic atrophy are specifically implicated
What are typical expectations for neuropsych results in those with MS?
Intelligence/achievement: unaffected
Attention/Concentration: poor sustained and complex attention but spared simple attention
Processing speed: most commonly affected function (d/t thinning of corpus callosum)
Language: not typically affected but children may show some language impairment
Visuospatial: commonly affected (esp visuo learning)
Memory: Encoding and retrieval affected. Explicit mem affected early and semantic/implicit mem affected later (if at all)
Executive functions: Typically affected, esp mental flexibility and fluency (d/t frontal lesions)
Sensorimotor function: commonly affected
Emotion/personality: Dep, anxiety, and lability are common (d/t lesions and cytokine effects)
Are adults or pediatric patients at greater risk for cognitive dysfunction?
Pediatric patients due to the consequences of the disease process on the developing brain during ongoing myelinogenesis.
What is the female to male ratio in pediatric MS?
It varies with age.
Girls outnumber boys in general but prior to age 6 boys slightly outnumber girls.
What disease course of MS do pediatric patients typically present with?
Relapsing Remitting.
What is the most common disease that is differentiated from MS in pediatric patients?
Acute desseminated encephalomyelitis (ADEM).
Discriminating clinical feature: presence of severe encephalopathy observed in early stages of ADEM
What percent of children with MS exhibit cognitive deficits?
30-53% and they typically show a decline over time.
Similar cog profile to that of adults but may show more difficulty with language-based skills and less difficulty with verbal fluency.
What are treatment considerations for pediatric patients with MS?
- Academic accommodations (esp d/t absences associated with fatigue)
- Treatment for internalizing disorders
- Barriers to medication compliance/treatment adherence given that most DMTs are administered through injection
How common is depression in MS
50% prevalence rate
Depression can have a negative impact on disease course
CBT is equally effective as medication in MS
What psychiatric complication can occur when diffuse lesions interrupt the corticobulbar tracts?
Pseudobulbar palsy (pathological crying or laughing(
What are common medications for fatigue, attention problems, and walking speed in MS?
Amantadine (Symmetrel) or modafinil (Provigil) for fatigue.
Stimulant meds for attentiont.
Dalfampridine (Ampyra) for walking speed
Does cognitive rehabilitation improve neuropsychological functioning in MS?
A recent Chochrane Review determined that there is lo evidence.
Are pediatric, adults, or older patients with MS more likely to have longer disease duration?
Older patients- associated with greater physical and cognitive disability.
Is MS a fatal disease?
No. Patients typically die from recurrent infections, pneumonia, pulmonary embolism, infections from decubitus ulcers, and suicide.
What is the Brief Repeatable Battery of Neuropsychological Tests?
40-minute battery to screen for cognitive dysfunction.
Short-version includes three tests: Selective Reminding Test, PASAT, and SDMT
What is the Expanded Disability Status Scale?
A method for quantifying disability in MS.
Based on 8 functional systems: pyramidal, cerebellar, brainstem, sensory, bowel/bladder, visual, cerebral, and other.
Score of 0-10 w/ higher socres= greater disability
What is the Fatigue Severity Scale?
Evaluates fatigue in MS- brief questionnaire that the patient rates their level of fatigue on a 7-point scale.
What is the Minimal Assessment of Cognitive Function in Multiple Sclerosis?
90-minute battery that includes 7 subtests: COWA, JLO, CVLT, BVMT-R, SDMT, PASAT, and DKEFS Sorting test
What is the Multiple Sclerosis Functional Composite?
Screening battery validated in adults: 9-hole peg test, timed 25-foot walk, and PASAT
What is scanning speech?
Speech disorder in which spoken words are broken up with interrupted syllables, noticable pauses, and varying inotation.
Typically caused by cerebellar lesions. It is a characteristic of ataxic dysarthria. It is 1 of 3 symptoms of Charcot’s neurologic triad.
What is Charcot’s neurologic triad?
The combination of nystagmus, intention tremor, and scanning or staccato speech. This triad is associated with multiple sclerosis.
