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Brain Power: Adam and Victor's Neurology > Demyelinating Disease > Flashcards

Demyelinating Disease Flashcards

1
Q

What part of the periventricular area usually harbors demyelinating plaques?

A

Where subependymal veins line the ventricles mainly adjacent to the bodies and atria of the lateral ventricles

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2
Q

What are the different histopathologic types of MS?

A

Pattern
1: T cells + AND Macrophage
2: (+) Ig AND (+) Complement
3: (-) Ig (-) Complement
(+) Apoptosis of oligodendrocytes with remyelination
4: Oligodendrocyte dystrophy (-) Remyelination

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3
Q

What is the peak age for MS?

What is the incidence in children?

A

30 years old

0.3-0.4% of all cases appearing in the first decade

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4
Q

T or F there are no Ig in progressive MS only in relapsing remitting

A

T

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5
Q

What are the 4 common modes of onset of MS?

A
  1. Optic neuritis
  2. Transverse myelitis
  3. Cerebellar ataxia
  4. Brainstem syndromes
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6
Q

What is the Uhthoff phenomenon?

A

Temporary induction of MS symptoms by heat or exercise. An increase in -.5 degrees celsius can causes block in electrical transmission in a thinly myelinated nerve

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7
Q

What percentage of MS presents with Optic Neuritis?

A

25

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8
Q

What distinguishes papillits from papilledema?

A

The severe and acute visual loss– usually found in MS but depends on the location of the demyelinating lesion

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9
Q

What two diagnostics can diagnose optic neuritis?

A

Visual evoked response and optical coherence tomography

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10
Q

What are the 2 persistent optical defects in optic neuritis despite treatment?

A

Dyschromatopsia, perceived desaturation of colors and Pulfritch effect where an object like the pendulum appears to be moving in 3d

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11
Q

T or F the risk for developing MS is higher if ON develops as a child

A

F

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12
Q

T or F Less than 1/2 of patients with ON will develop MS

A

F MORE than

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13
Q

What is the Charcot Triad?

A
  1. Nystagmus
  2. Scanning speech
  3. Intention tremor

Seen in advanced cases of MS

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14
Q

A lesion of the tegmentum of the midbrain that damages the dentatorubrothalamic tracts will result in?

A

Severe ataxia

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15
Q

T or F the presence of bilateral internuclear ophthalmoplegia in an young adult is virtually diagnostic of MS.

A

T

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16
Q

T or F. Cognitive impairment is a presentation of in 1/2 of MS patients

A

F. NOT presentation but in long standing MS

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17
Q

What drug may be used to control the spontaneous and recurrent, transitory symptoms of MS?

A

Carbamazepine

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18
Q

What are the most common triggers for MS attacks?

A

Pregnancy, Infection, Trauma

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19
Q

What is the Marburg variant of MS?

A

Acute and tumor like MS (Tumefactive)
That develops rapidly over a few weeks– can be fatal

On histopath: more lesions that are of the SAME age and there is more obvious confluence of the perivenous zones of demyelination

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20
Q

What is the typical imaging feature of tumefactive MS?

A

Open enhancing ring on cranial MRI with contrast

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21
Q

Balo or Schilder?

  1. Found in the philippines
  2. DDx are gliomatosis, PML, adrenoleukodystrophy
  3. Diffuse sclerosis
  4. Concentric sclerosis
A

B Found in the philippines
S DDx are gliomatosis, PML, adrenoleukodystrophy
S Diffuse sclerosis
B Concentric sclerosis

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22
Q

What percentage of patients with MS will have oligoclobands?

A

90 but less in asians

Remember the presence of bands in the first attack of MS is predictive of a chronic relpasing course

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23
Q

What type of MS lesion has edema?

A

Acute

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24
Q

T or F. T1 hypointensity is inversely proportional to the degree of remyelination

A

T.

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25
Q

Which is the most sensitive?
VER
SSEP
BAER

A

VER 70% in definite MS 60% in probable
SSEP 60/ 40
BAER 40/20

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26
Q

What are the common locations of MS lesions (4) for diagnostic purposes?

A 
J-PIS
Juxtacortical
Periventricular
Infratentorial
Spinal cord
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27
Q

What percentage of MS patients have will have a next attack after the initial attack in the first year?

28
Q

T or F. In MS, pregnancy is associated with clinical stability or even improvement

29
Q

T or F. Atrophy of the first dorsal interosseous muscle is frequent finding in MS and cervical spondylosis.

A

T.

Use oligoclonal bands and IgG to distinguish

30
Q

What are the doses for giving IV and oral antibiotics to MS relapses?

A

IV: 1g per day for 3-5 days then 60-80mg per day tapering over 12-20 days
Oral: 48mg for 7 days, 24mg for 7 days, 12mg for 7 days

31
Q

What are the side effects of beta interferon?

A

Flu like symptoms or malaise.

May give NSAIDs or prednisone

32
Q

What is the main advantage of Glatiramer over Interferon-beta?

A

Antibodies do not develop against it

33
Q

What is the main adverse of effect of Natalizumab that limits its use despite better outcomes compared to Glatiramer and beta-interferon?

A

progressive multifocal leukoencephalopathy

34
Q

What are the MOA?

  1. Natalizumab
  2. Alemtuzumab
  3. Rituximab
  4. Fingolimod
A
  1. Natalizumab: against alpha integrin to block lymphocyte and monocyte adhesion
  2. Alemtuzumab: Target CD 52 on T and B lymphocytes
  3. Rituximab: Target CD 20 lymphocytes
  4. Fingolimod: Interferes with egress of lymphocytes from lymph nodes
35
Q

A postural tremor that develops with MS patients can be treated with what drug?

A

Isoniazid!

36
Q

What are the features of NMO that distinguish it from MS?

DEVIC PROCESS IS A HUMORAL DISEASE IN CONTRAST TO THE CELLULAR MECHANISM THAT IS PROPOSED FOR MS

A
  1. Anti aquaporin-4 IgG
  2. Absence of oligoclonal bands
  3. Several contiguous spinal cord segments are involved
37
Q

What is the se and sp of aquaporin antibodies?

A

Se 76 Sp 94

38
Q

What criteria has 99 se and 90 sp?

A

2/3
Antibodies
Longitudinally extensive myelopathy
Initial MRI not characteristic of MS

39
Q

Which HLA are overrepresented in MS and arte though to be markers for an MS susceptibility gene?

A

HLA DR2 mostly

also DR3, B7, A3

40
Q

What is the pathophysiology of Lhermitte sign?

A

There is increased sensitivity of demyelinated axons to stretch or pressure on the spinal cord induced by neck flexion. ALSO OCCURS WITH CERVICAL SPONDYLOSIS

41
Q

What is the 20-30-20-10 distribution of the temporal mode of onset of MS?

A

20% within minutes to hours
30% over days
20% over weeks
10% over months to years

42
Q

How to dfx papillitis from papilledema?

A

Papillitis has severe and acute visual loss

43
Q

1/10 of optic neuritis patients will have papillitis more often optic nerve head looks normal representing what pathology?

A

retrobulbar neuritis

44
Q

Despite the good prognosis of ON patients (95% 20/40 or better at 5 years) what two visual phenomena usually persist?

A

Dyschromatopsia: Perceived desaturation of colors

Pulfrich effect: 3D moving objects like the pendulum swinging in a circular motion rather than 2D

45
Q

What perecentage of patients with ON develop MS?

A

more than half
74% in women
34% in men
MUCH LOWER IF ON HAPPENS IN CHILDHOOD

46
Q

What other diseases can cause isolated recurrent myelitis or myelopathy?

A

SLE
Sjogren
APAS

47
Q

What is the most common manifestation of progressive MS?

A

An asymmetrical spastic paraparesis with some degree of impaired joint position and vibration sense in the legs

48
Q

What are the CSF characteristics of MS?

A
  1. Increased protein more than 100mg per dl
  2. Increased WBC lymphocytes 6-20 but may even be 1000 in hyperacute
  3. IgG index of more than 12%
49
Q

What are the imaging characteristics that differentiate an old from a new lesion of MS?

A
  1. Enhancement for new lesions. Though this enhancement can last for many weeks
  2. New lesions have evidence of tissue expansion due to edema while old lesions are contracted– recall that the less remyelination the more hypointense on T1 for chronic lesions

Chronic cases also have cerebral atrophy

50
Q

What are the 4 typical locations for MS?

A 
T2 hyperintensity in:
Periventricular
Juxtacortical
Infratentorial
Spinal
51
Q

What percentage of MS patients will be positive for ANA and anti-DSDNA?

52
Q

What are the 2 pathologies seen in SLE lesions?

A
  1. Vascular from small vessel occlusion

2. Demyelinating in a few

53
Q

What can differentiate bechet disease from MS?

A

Iridocyclitis
meningitis
Mucous membrane ulcers

54
Q

How does steroid therapy BENEFIT MS PATIENTS?

500mg to 1g per day for 3-5 days followed by high oral doses of prednisone beginning iwith 60-80mg daily tapering to a lower dosage over 12 to 20 days

A

hastens recovery from an acute attack BUT HAS NO EVIDENCE IN AFFECTING THE ULTIMATE COURSE OF THE DISEASE

55
Q

What type of MS is treatable by glatiramer and interferon beta 1b?

A

Relapsing remitting

56
Q

How is interferon given?
How about glatiramer?
How about natalizumab?

A

IFbeta1b: every other day!
IFbeta1a: weekly
G: Daily
Natalizumab: Once a month IV treatment

57
Q

What is the only drug recommended for relapsing AND primary progressive MS?

A

Ocrelizumab

58
Q

What is the MOA of fingolimod and how is it administered?

A

Once a day– PREVENTS THE EGRESS of lymphocytes from lymph nodes

59
Q

What are the 3 oral treatments for MS?

A

Fingolimod
Dimethylfumarate
Teriflunomide

60
Q

What are useful to reduce paroxysmal symptoms of MS?

A

CBZ and Gabapentin

61
Q

Tremor due to MS can be improved with what medications?

A

INH with pyridoxine

62
Q

What is the location of aquaporin-4?

A

At the astrocytic end feet adjacent to capillaries, pia and Virchow Robin spaces all in the periventricular region SURROUNDING THE CENTRAL CANAL OF THE SPINAL CORD

63
Q

What are the three treatment options for NMO to prevent subsequent attacks?

A

Rituximab, Azathioprine, Mycophenolate mofetil

64
Q

Diagnostic criteria for NMO?

A

If Aq4 positive one core clinical criteria
If negative 2 are required plus MRI criteria

Optic neuritis

Acute myelitis

Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting

Acute brainstem syndrome

Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions

Symptomatic cerebral syndrome with NMOSD-typical brain lesions

65
Q

What is also known as acute necrotizing hemorrhagic encephalomyetlitis?

A

Acute hemorrhagic leukoencephalitis of Weston Hurst

66
Q

What usually precedes acute necrotizing hemorrhagic encephalomyelitis?

A

Respiratory infection of variable duration of 1-14 days sometimes caused by M pneumoniae

Brain Power: Adam and Victor's Neurology (19 decks)

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