Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Adams 10th ed > MS And Other Inflammatory Demyelinating Diseases > Flashcards

MS And Other Inflammatory Demyelinating Diseases Flashcards

1
Q

Pathologic criteria of a demyelinating disease

A
  1. Destruction of myelin sheaths of nerve fibers with relative sparing of other elements of nervous tissue
  2. Infiltration of inflammatory cells
  3. Lesions primarily in the white matter
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Topography of lesions in MS

A

Periventricular (where subependymal veins line the ventricles)
Optic nerves and chiasm
Spinal cord (where pial veins lie next to or w/in white matter)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Gross Pathologic findings in MS

A

Numerous scattered patches sl depressed from cut surface

Pink-gray color

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pathology of relatively recent MS lesions

A

Partial or complete destruction and loss of myelin throughout zone of confluence of perivenous foci
Slight degeneration of oligodendroglia
Astrocytic reaction
Perivascular and para-adventitial infiltration w mononuclear cells and lymphocytes

Axons relatively spared or less affected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Histopathology of long-standing MS lesions

A

Thickly matted
relatively acellular glial tissue
Only occasional perivascular lymphocytes and macrophages
Few intact axons or descending and ascending Wallerian degeneration
“Shadow patches” - from partial remyelination or incompletely demyelinated axons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Critical age of immigration after which risk of MS becomes similar to that of person’s birthplace

A

15years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Histocompatibility locus antigen with strongest association to MS is located at

A

DR locus chromosome 6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

HLA-DR2, to a lesser extent, -DR3, -B7, and -A3 are thought to be markers for an MS “susceptibility gene”. The presence of one of these increases risk for developing MS by what factor?

A

Any one would inc risk by a factor of 3-5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Heritable risk factors for MS

A 
HLA-DR2
HLA-DR3
HLA-B7
HLA-A3
IL-2Ralpha
IL7Ralpha
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

In optic neuritis, what color reduces in intensity?

A

Red

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Uhthoff phenomenon

A

Temporary induction, by heat or exercise, of sx such as unilateral blurring of vision.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Syndromes typical of MS and may be initial manifestations

A

Optic neuritis
Transverse myelitis
Cerebellar ataxia
Brainstem syndromes (vertigo, facial pain or numbness, dysarthria, diplopia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Lhermitte sign

A

Flexion of the neck induces a tingling electric-like feeling down the shoulders and back and, less commonly, down the anterior thighs
- also occurs in other conditions such ad cervical spondylosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pulfrich effect

A

An object swinging perpendicular to patient’s line of sight, appears to be moving in a 3D circular motion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Charcot triad of MS (often seen in advanced stages of the disease)

A

Nystagmus
Scanning speech
Intention tremor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Finding in a young adult that is virtually diagnostic of MS

A

Bilateral Internuclear Ophthalmoplegia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Occurrence of this in a young adult should always suggest the diagnosis of MS

A

Transient facial hypesthesia or anesthesia or of trigeminal neuralgia
(Intramedullary fibers of CNV)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Characteristics of cognitive impairment seen in about 1/2 of patients with long-standing MS

A

Reduced attention
Diminished processing speed and executive skills
Memory decline

(Language and other intellectual functions preserved)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

la belle indifférence

A

Euphoria, a pathologic cheerfulness or elation that seems inappropriate in the face of obvious neurologic deficit
Also stupid indifference or morbid optimism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What drug/s is/are used in symptomatic management of these manifestations of MS

  1. Paroxysmal attacks of neuro deficit
  2. Painful tonic spasms
  3. Fatigue
  4. Depression
A
  1. Paroxysmal attacks of neuro deficit - CBZ
  2. Painful tonic spasms - acetazolamide
  3. Fatigue - modafinil 200-400mg/d, amantadine 100mg am&nn, pemoline 20-75mg OD AM
  4. Depression - antidepressants
21
Q

Marburg variant

A

Rapidly progressive highly malignant form of MS rendering pt stuporous comatose or decerebrate with prominent CN and corticospinal abnormalities.
Death may end illness within few weeks or months

22
Q

Lesions have a series of concentric rings that represent alternating areas of myelin loss and preservation. High incidence in the Philippines

A

Concentric sclerosis of Balo

23
Q

Oligoclonal bands seen in more than 90% of MS cases and used to confirm dx is also appear in CSF of patients with what other conditions?

A

Syphilis, Lyme, SSPE

24
Q

A positive test of IgG index

A

Greater than 12% of the total protein

25
Q

Why is MBP not useful as a diagnostic test for MS

A

Other lesions that destroy myelin can also increase level of MBP in the CSF.
It only reflects destruction of central myelin

26
Q

Usual BAER findings in MS

A

Prolonged interwave latency

Decreased amplitude of wave5

27
Q

Feature most predictive of long-term disability

A

Degree of disability at 5y from the first symptom

28
Q

MS associated with little change over time in MRI findings, negligible response to therapy, and poor outcome

A

Primary Progressive MS

29
Q

In whom of the following is a higher rate of exacerbation and of MS expected
A. 28yo woman with MS in her first trimester of pregnancy
B. 30yo man diagnosed with MS who had loc after a vehicular crash while driving his motorcycle
C. 30yo woman with MS who gave birth 2 weeks ago
D. A 20yo woman who underwent a lumbar puncture for the 1st episode of optic neuritis

A

C. There appears to be an increased risk of exacerbations, up to 2-fold in the first few months postpartum

30
Q

Dosing regimen of methylprednisolone for acute or subacute exacerbation of MS or optic neuritis

A

IV Methylprednisolone 500-1000mg daily x 3-5days
Followed by
Prednisone 60-80mg daily tapering over 12-20days

If IV cannot be given
Methylprednisolone 48mg OD x 1wk then 24mg OD x 1wk then 12mg OD x 1wk or equivalent doses of prednisone

31
Q

This monoclonal antibody has been shown to be two times more efficacious in the treatment of relapsing-remitting MS but the complication of Progressive Multifocal Leukoencephalopathy hs precluded its use

A

Natalizumab
Directed against alpha-integrin in order to block lymphocyte and monocyte adhesion to endothelial cells and their migration through the vessel wall.

32
Q

Distinctive clinical and pathologic features of NMO from MS

A
  1. Failure to develop Cerebral demyelinating lesions even years after
  2. Absence of oligoclonal bands in CSF
  3. Tendency to CSF pleocytosis
  4. Necrotizing and cavitary nature of spinal cord lesion (affects white and gray matter alike w prominent thickening of vessels and minimal inflammatory infiltrates
  5. Tendency to involve several contiguous longitudinal segments of the spinal cord
33
Q

Typical rash of acute graft vs host disease

A

Tender erythematous macular rash

34
Q

Pathologic findings of Acute Necrotizing Hemorrhagic Leukoencephalitis of Weston Hurst

A

Gross: white matter destroyed to almost liquefaction, pink or yellow-gray flecked with multiple petechial hemorrhages
Histo: widespread necrosis of small blood vessels and brain tissue around vessels, intense cellular infiltration, multiple small hemorrhages and inflammatory reaction in meninges
(Resembles adem save for more widespread necrosis and large foci)

35
Q

A variant of post-infectious encephalomyelitis that involves solely or predominantly the cerebellum is particularly associated with this virus among others.

A

Varicella

36
Q

Treatment for Hurst Syndrome

A

High dose intravenous corticosteroids

37
Q

T/F

Recovery from ADEM is good among adult patients

A

T

38
Q

Postexanthem encephalomyelitis symptoms generally begin ___ days after the appearance of the rash

A

2-4days

39
Q

This drug may be used as symptomatic treatment for postural tremor in MS, albeit mechanism for its beneficial effects is unknown

A

Isoniazid
300mg initial daily inc by weekly increments of 300mg to a dose of 1200mg daily
To be given with Pyridoxine 100mg daily

40
Q

T/F vaccinations are prohibited in patients with MS

A

F

41
Q

Distribution of the NMO antibody

A

Located in astrocytic end feet adjacent to capillaries, pia, and Virchow-Robin spaces all in the periventricular region and surrounding central canal of spinal cord

42
Q

Sn and Sp of the presence of NMO antibody

A

76% sensitive

94% specific

43
Q

Treatment for PML complicating Natalizumab treatment

A

Stop Natalizumab

Plasma exchange

44
Q

This oral therapy for MS that interferes with egress of lymphocytes from lymph nodes has a short term effect on MRI lesion burden and relapse rate

A

Fingolimod

45
Q

Progressive Multifocal Leukoencephalopathy is associated with which virus

A

JC virus

46
Q

T/F

All protein components of the respiratory chain are encoded in mitochondrial DNA.

A

F

About 83% of respiratory chain proteins are encoded in nuclear DNA.

47
Q

Which complex of the respiratory chain is most often disordered leading to lactic acidosis

A

Complex IV (cytochrome-c oxidase)

48
Q

First definite indication of disordered nervous system function in neonates with metabolic disease

A

Seizures

Adams 10th ed (11 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions