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Nervous System > CM- MS & White Matter Disease > Flashcards

CM- MS & White Matter Disease Flashcards

1
Q

During the natural progression of MS, what percent of patients will require ambulatory assistance [cane or wheelchair] after 10 years?

What percent of people with untreated relapsing remitting MS will develop secondarily progressive MS after 10 years?

A

In 10 years:
50% will need a cane
15% will need a wheelchair

50% of untreated relapsing remitting will develop secondary progressive MS in 10 years and 85-90% in 25 years.

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2
Q

During the natural progression of MS, what percent of patients will require ambulatory assistance [cane or wheelchair] after 10 years?

What percent of people with untreated relapsing remitting MS will develop secondarily progressive MS after 10 years?

A

In 10 years:
50% will need a cane
15% will need a wheelchair

50% of untreated relapsing remitting will develop secondary progressive MS in 10 years and 85-90% in 25 years.

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3
Q

What parts of the nervous system are affected by MS?

A

ONLY the CNS

  • brain
  • optic nerves
  • spinal cord
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4
Q

Describe the epidemiology of MS.

What age, race, geography, etc are most affected?

A

MS occurs generally in:

  • women 20-40
  • western European descent
  • northern climate before age 15
  • genetics
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5
Q

What happens to the relapse rate of MS during pregnancy and during postpartum period?
What does this suggest about the nature of the disease?

A

Relapse rates decrease in pregnancy and increase during postpartum period

This suggests that there is a complex hormonal component to MS

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6
Q

What are the 4 factors that predict a more severe course of MS?

A
  1. motor or cerebellar symptoms
  2. disability after the first attack
  3. shorter time intervals between attacks/numerous relapses in the first year
  4. heavy lesion burden on MRI
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7
Q

What are 4 factors predict a less severe course of MS?

A
  1. sensory symptoms only
  2. infrequent attacks
  3. full neuro recovery after initial attack
  4. low level of disability after 5-7 years
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8
Q

What are the 4 disease classifications for MS? What do 85% of people present with?

A
  1. Relapsing remitting [RRMS] - 85% of people
    - relapses and exacerbations of optic neuritis [blurring], diplopia, vertigo, numbness, paresthesia or weakness
  2. Relapsing progressive [RPMS]
    - presents like RRMS but there will be residual symptoms detected on exam after the attacks
    - cumulative disability in the long term
  3. Secondary Progressive [SPMS]
    - 50% of RRMS in 10yrs and 85-90% in 25yrs
    - more pronounced, steady, progressive decline in function w/ or w/o relapses
    - ambulation difficulty, bladder/bowel dysfunction, sexual dysfunction
    - cognitive, visual, cerebellar, sensory decline
  4. Primary Progressive [PPMS]
    - gradual decline from ONSET with myelopathic features [ambulation, bladder/bowel/sex dysfunc.]
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9
Q

How does the MRI lesion burden differ amongst RRMS and PPMS?
How does treatment differ?
How does presentation differ?

A

PPMS will have LESS MRI lesions on the brain and spinal cord than RRMS.
It cannot be treated with immunomodulatory drugs [because it is degenerative, not inflammatory]

PPMS affects older individuals and affect men and women equally

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10
Q

How is the diagnosis of MS made?

How is it confirmed? [2 ways]

A

You need evidence of MULTIPLE lesions occurring at different times and locations

MRI will show:

  1. periventricular, perpendicular, ovoid lesions in hemispheres or infratentorially [Dawson’s fingers]
    - seen best with FLAIR
  2. deep white matter and juxtacortical lesions [not specific and can be seen in migraines]
    - seen best with T2 imaging
  3. cigar-shaped spinal cord lesions [most often cervical]
    - seen best with T2 imaging

Confirms with:
CSF analysis showing:
1. oligoclonal bands
2. elevated IgG index

Evoked potentials showing slow conduction [indicative of demyelination] in auditory, visual and sensory neural pathways

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11
Q

How can active inflammation and chronic lesions of MS be differentiated via imaging?

A

Gadolinium enhancement will persist for 2-4wks

Active = enhanced
Chronic = no enhancement
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12
Q

A patient has had one attack of optic neuritis with blurred vision and diplopia. The attack lasts at least 24 hours and occurs in the absence of infection. She shows no evidence clinically or by history of a second relapse.
What is the diagnosis?
What is the most significant predictor of disease?

A

We cannot call this clinically definite MS [CDMS] because MS needs to have multiple lesions over time and space.

This is clinically isolated syndrome [CIS]
The most significant predictor of disease is the MRI

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13
Q

For clinically isolated syndrome, what will be the presenting symptom in 50% of people? 25% of people? 15%?

A

50% will have a spinal cord syndrome [myelopathy]
25% will have optic neuritis
15% will have brainstem symptoms

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14
Q

What occurs in the early stages of RRMS that differentiates it from SPMS?

A

Early stages of RRMS:

  • recurrent inflammation with damage to myelin, oligodendrocytes, and axons –> gliosis
  • INFLAMMATION

SPMS:

  • infrequent new lesions, but increase of spinal and brain atrophy
  • DEGENERATION
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15
Q

What occurs in the early stages of RRMS that differentiates it from SPMS?

A

Early sta

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16
Q

What parts of the nervous system are affected by MS?

A

ONLY the CNS

  • brain
  • optic nerves
  • spinal cord
How well did you know this?
1
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2
3
4
5
Perfectly
17
Q

Describe the epidemiology of MS.

What age, race, geography, etc are most affected?

A

MS occurs generally in:

  • women 20-40
  • western European descent
  • northern climate before age 15
  • genetics
18
Q

What happens to the relapse rate of MS during pregnancy and during postpartum period?
What does this suggest about the nature of the disease?

A

Relapse rates decrease in pregnancy and increase during postpartum period

This suggests that there is a complex hormonal component to MS

19
Q

What are the 4 factors that predict a more severe course of MS?

A
  1. motor or cerebellar symptoms
  2. disability after the first attack
  3. shorter time intervals between attacks/numerous relapses in the first year
  4. heavy lesion burden on MRI
20
Q

What are 4 factors predict a less severe course of MS?

A
  1. sensory symptoms only
  2. infrequent attacks
  3. full neuro recovery after initial attack
  4. low level of disability after 5-7 years
21
Q

What are the 4 disease classifications for MS? What do 85% of people present with?

A
  1. Relapsing remitting [RRMS] - 85% of people
    - relapses and exacerbations of optic neuritis [blurring], diplopia, vertigo, numbness, paresthesia or weakness
  2. Relapsing progressive [RPMS]
    - presents like RRMS but there will be residual symptoms detected on exam after the attacks
    - cumulative disability in the long term
  3. Secondary Progressive [SPMS]
    - 50% of RRMS in 10yrs and 85-90% in 25yrs
    - more pronounced, steady, progressive decline in function w/ or w/o relapses
    - ambulation difficulty, bladder/bowel dysfunction, sexual dysfunction
    - cognitive, visual, cerebellar, sensory decline
  4. Primary Progressive [PPMS]
    - gradual decline from ONSET with myelopathic features [ambulation, bladder/bowel/sex dysfunc.]
22
Q

How does the MRI lesion burden differ amongst RRMS and PPMS?
How does treatment differ?
How does presentation differ?

A

PPMS will have LESS MRI lesions on the brain and spinal cord than RRMS.
It cannot be treated with immunomodulatory drugs [because it is degenerative, not inflammatory]

PPMS affects older individuals and affect men and women equally

23
Q

How is the diagnosis of MS made?

How is it confirmed? [2 ways]

A

You need evidence of MULTIPLE lesions occurring at different times and locations

MRI will show:

  1. periventricular, perpendicular, ovoid lesions in hemispheres or infratentorially [Dawson’s fingers]
    - seen best with FLAIR
  2. deep white matter and juxtacortical lesions [not specific and can be seen in migraines]
    - seen best with T2 imaging
  3. cigar-shaped spinal cord lesions [most often cervical]
    - seen best with T2 imaging

Confirms with:
CSF analysis showing:
1. oligoclonal bands
2. elevated IgG index

Evoked potentials showing slow conduction [indicative of demyelination] in auditory, visual and sensory neural pathways

24
Q

How can active inflammation and chronic lesions of MS be differentiated via imaging?

A

Gadolinium enhancement will persist for 2-4wks

Active = enhanced
Chronic = no enhancement
25
Q

A patient has had one attack of optic neuritis with blurred vision and diplopia. The attack lasts at least 24 hours and occurs in the absence of infection. She shows no evidence clinically or by history of a second relapse.
What is the diagnosis?
What is the most significant predictor of disease?

A

We cannot call this clinically definite MS [CDMS] because MS needs to have multiple lesions over time and space.

This is clinically isolated syndrome [CIS]
The most significant predictor of disease is the MRI

26
Q

For clinically isolated syndrome, what will be the presenting symptom in 50% of people? 25% of people? 15%?

A

50% will have a spinal cord syndrome [myelopathy]
25% will have optic neuritis
15% will have brainstem symptoms

27
Q

How can you differentiate an exacerbation of an old MS lesion from a true relapse?

A

Exacerbations will:

  1. have symptoms that fluctuate over the course of the day
  2. lack new gadolinium uptake on MRI
  3. have inciting factors present [heat, UTI, fatigue]
  4. after removal of inciting factor, symptoms resolve in 1 to 2 days
28
Q

What are the 3 most commonly seen manifestations of MS?

A
  1. optic neuritis
  2. transverse myelitis
  3. intranuclear ophthalmoplegia [bilateral]
29
Q

A 25 year old woman presents with blurred vision and concomitant pain with ocular movement. Neurological exam defines a central defect with quandrantanopia, and enlargement of the blind spot.
She has a dimming of color perception, but her acuity is fine. She has a Marcus Gunn pupil.

What is the likely problem?

A

This is the presentation of optic neuritis

30
Q

How is optic neuritis differentiated from ischemic optic neuropathy?

A

optic neuritis has pain with ocular movement while ischemic optic neuropathy does not

31
Q

A 35 year old woman presents with numbness and paresthesia, weakness, spasticity, pain, and bladder/bowel dysfunction that has developed over the course of hours.
She has a positive Babinski.
When she flexes her neck, an electrical sensation travels down her arms and legs.

What is the problem?

A

Transverse myelitis

32
Q

In MS where is the predilection for transverse myelitis?

A

posterior columns

33
Q

What is Lhermitte’s sign?

A

Electrical sensation down the arms and legs when the neck is flexed.
It is associated with transverse myelitis

34
Q

Where are MS lesions of the brainstem most likely to occur?

What does this anatomical preference lead to?

A

Periventricular pontine tegementum

This leads to increased incidence of intranuclear ophthalmoplegia, vertigo, incoordination.

INO–> lesions of the MLF slow adduction in the affected eye with no change in abduction

35
Q

What is intranuclear ophthalmoplegia?

A

It is a defect in MLF that disrupts coordinated eye movement.
When looking laterally, CN 6 usually controls the lateral rectus and the MLF crosses to the contralateral CN3 to move the medial rectus of the other eye.

INO results in slowed adduction of the contralateral eye.

36
Q

A patient presents with bilateral optic neuropathy and myelitis simultaneously.
You recognize a serum antibody against aquaporin 4 water channel in the plasma membrane of astrocytes of the BBB [NMO-IgG].
MRI of the brain shows few hyperintensities and multi-segmental myelitis. What is the likely diagnosis?

A

nueromyelitis optica

37
Q

Following vaccination or viral infection, a patient presents with multiple enhancing lesions in the brain. They have multifocal symptoms.
What is the diagnosis?

A

Acute disseminated encephalomyelitis

38
Q

A malnourished, chronic alcoholic is hyponatremic. Rapid correction is done. Now he seems confused and has gaze paralysis and quadroplegia.

MRI shows abnormal signal in the pons.
What is the likely diagnosis?

A

Central pontine myelinolysis

  • non inflammatory demyelinating condition of the pons that occurs in overcorrection of hyponatremia
  • midbrain, thalamus, cerebellum may also be affected
39
Q

If a child presents with gradual decline of motor function and cognition, what is the first thing you want to consider?

A

Leukodystrophy because they are inherited conditions associated with inborn errors of metabolism.

The gradual decline is another feature that differentiates it from MS because it is not relapse/remitting

40
Q

What is the cause of X-linked adrenoleukodystrophy?

A

mutation in peroxismal function causing accumulation of VLCFA which breakdown the myelin sheath

41
Q

What is the inheritance pattern and gene mutated in metachromatic leukodystophy?

A

AR mutation in arylsulfatase [ASA] which is an enzyme that degrades sulfatides.
Without this enzyme, the myelin sheath is disrupted in CNS and PNS

42
Q

A late teen presents to your office. He had normal development and growth.
Now he is experiencing vision loss due to retinitis pigmentosa, deafness, and peripheral neuropathy.

What is the disease and what is the defect?

A

Refsum’s disease - defect in ability to breakdown phytanic acid [in dairy and animal fats]

Nervous System (32 decks)

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