Multiple Sclerosis Flashcards

1
Q

Describe Multiple Sclerosis (MS).

A

A chronic and progressive inflammatory autoimmune disease of the CNS.

Immune system response results in attack on myelin sheathing which results in axonal damage.

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2
Q

What are brain biopsy findings of MS?

A

MS lesions are characterized by perivascular inflammation and demyelination.

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3
Q

Describe the differences between acute and chronic lesions in MS.

A

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

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4
Q

In what age range and ethnicity is MS most likely to occur?

A

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.

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5
Q

Describe the differences between women and men with regard to MS.

A

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.

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6
Q

Does genetics contribute to the development of MS?

A

Yes. First degree relatives are 6-8 times more likely to develop the disease

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7
Q

Do rates of MS increases as you get farther from or closer to the equator?

A

Farther from the equator

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8
Q

What are environmental factors that contribute to the susceptibility of MS>

A

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.

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9
Q

What percentage of patients with MS experience an average life expectancy?

A

90-95%

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10
Q

What factors are associated with severity in MS?

A

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)

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11
Q

What is the role of cognitive reserve in MS?

A

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.

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12
Q

How do you diagnose MS?

A

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
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13
Q

What cognitive changes typically occur very early in the disease and progress in later stages?

A

Processing speed, learning, and free recall

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14
Q

What considerations should be made when assessing a patient with MS?

A

Fatigue, slowed mental processing, and speech and upper extremity motor deficiencies

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15
Q

What are the most common initial motor and sensory changes?

A
  • 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
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16
Q

What is diagnosed if a person has one episode of a neurologic event similar to MS>?

A

Clinically Isolated Syndrome

Describes the first episode that lasts at least 24 hours and results from inflammation/demylenation. Can be monofocal or multifocal,

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17
Q

What are the 4 disease courses in MS?

A

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)

18
Q

Which disease course in MS is associated with the greatest deficits in cognitive functioning?

A

Secondary-Progressive

50% of patients with RRMS left untreated with convert to secondary progressive MS w/in 10-15 years

19
Q

How are acute relapses treated?

A
  • IV corticosteroids w/ oral prednisone taper

- IVIG if steroids are ineffective

20
Q

How is disease progression treated?

A

With the use of disease modifying therapies.

Injectable drugs that delay relapses and slow progression.

Examples: interferon drugs (Betaseron, Copaxone), mitoxantrone, Tysabari, fingolimod

21
Q

What are disorders that adversely impact WM and have similar symptom presentations as MS?

A

Leukodystrophies, progressive multifocal leukoencepalopathy, acute disseminated encephalomyelitis, systemic autoimmune diseases, Gullain-Barre Syndrome, toxic optic neuropathy, brain tumor, and meuromyelitis optica

22
Q

What percentage of patients with MS have cognitive impairment?

A

40-65%

Whole brain and thalamic atrophy are specifically implicated

23
Q

What are typical expectations for neuropsych results in those with MS?

A

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)

24
Q

Are adults or pediatric patients at greater risk for cognitive dysfunction?

A

Pediatric patients due to the consequences of the disease process on the developing brain during ongoing myelinogenesis.

25
Q

What is the female to male ratio in pediatric MS?

A

It varies with age.

Girls outnumber boys in general but prior to age 6 boys slightly outnumber girls.

26
Q

What disease course of MS do pediatric patients typically present with?

A

Relapsing Remitting.

27
Q

What is the most common disease that is differentiated from MS in pediatric patients?

A

Acute desseminated encephalomyelitis (ADEM).

Discriminating clinical feature: presence of severe encephalopathy observed in early stages of ADEM

28
Q

What percent of children with MS exhibit cognitive deficits?

A

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.

29
Q

What are treatment considerations for pediatric patients with MS?

A
  • 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
30
Q

How common is depression in MS

A

50% prevalence rate

Depression can have a negative impact on disease course

CBT is equally effective as medication in MS

31
Q

What psychiatric complication can occur when diffuse lesions interrupt the corticobulbar tracts?

A

Pseudobulbar palsy (pathological crying or laughing(

32
Q

What are common medications for fatigue, attention problems, and walking speed in MS?

A

Amantadine (Symmetrel) or modafinil (Provigil) for fatigue.

Stimulant meds for attentiont.

Dalfampridine (Ampyra) for walking speed

33
Q

Does cognitive rehabilitation improve neuropsychological functioning in MS?

A

A recent Chochrane Review determined that there is lo evidence.

34
Q

Are pediatric, adults, or older patients with MS more likely to have longer disease duration?

A

Older patients- associated with greater physical and cognitive disability.

35
Q

Is MS a fatal disease?

A

No. Patients typically die from recurrent infections, pneumonia, pulmonary embolism, infections from decubitus ulcers, and suicide.

36
Q

What is the Brief Repeatable Battery of Neuropsychological Tests?

A

40-minute battery to screen for cognitive dysfunction.

Short-version includes three tests: Selective Reminding Test, PASAT, and SDMT

37
Q

What is the Expanded Disability Status Scale?

A

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

38
Q

What is the Fatigue Severity Scale?

A

Evaluates fatigue in MS- brief questionnaire that the patient rates their level of fatigue on a 7-point scale.

39
Q

What is the Minimal Assessment of Cognitive Function in Multiple Sclerosis?

A

90-minute battery that includes 7 subtests: COWA, JLO, CVLT, BVMT-R, SDMT, PASAT, and DKEFS Sorting test

40
Q

What is the Multiple Sclerosis Functional Composite?

A

Screening battery validated in adults: 9-hole peg test, timed 25-foot walk, and PASAT

41
Q

What is scanning speech?

A

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.

42
Q

What is Charcot’s neurologic triad?

A

The combination of nystagmus, intention tremor, and scanning or staccato speech. This triad is associated with multiple sclerosis.