Demyelinating Dz/ Ms - Mcdonald

Front 1

MS affects 1/______ people in US and Europe

Back 1

1/1000

Front 2

MS affects M ___ F

Back 2

F>M
2:1

Front 3

MS = immune response directed against _____

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Myelin sheath

Front 4

MS is associated with the ____ complex

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MHC complex

Front 5

MS is initiated by _________ that react against self antigens and secrete cytokines

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CD4+ Th1 T cells

Front 6

MS is initiated by CD4+ T cells against self antigens --> secrete ____ which activates ____

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IFN gamma which activated macrophages

Front 7

Plaques of MS consist of:

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T cells and macrophages

Front 8

MS may be on the surface of the ______ and along _______

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Surface of brainstem and along spinal cord

Front 9

Irregularily shaped softened depressed lesions

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Plaques of MS

Front 10

MS is frequently in (5)

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Optic nerves
Optic chiasm
Brainstem
Cerebellum
Spinal Cord

Front 11

Ongoing myelin breakdown
Abundant macs with lipid rich debris + lymphocytes + monocytes

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Active plaques

Front 12

Early on in MS, have preservation of axons and depletion of __________

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Oligodendrocytes

Front 13

MS lesions
- Decrease of inflm cell infiltrate and macs

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Quiescent lesion

Front 14

MS lesion
- Little myelin
- Decrease in oligodendrocyte nuclei
- Gliosis astrocyte proliferation

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Inactive plaque

Front 15

MS lesion
- Poorly demarcated
- Thinned out myelin sheaths

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Shadow plaques

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Age for MS

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20-30

Front 17

MS more common geographically

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Above the equator

Front 18

2 viruses that MS is associated with

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- Herpes 6
- EBV

Front 19

MS is associated with this

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Chlamydia pneumoniae

Front 20

HLA associated with MS

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HLA-DR2-DR15

* 1st degree relative = 5%

Front 21

MS is unusual in (2)

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African Americans
Asians

Front 22

22.5% of MS cases exhibited ____

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+ANA = antinuclear antibodies

Front 23

Can dx MS with CSF ______ ______

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Oligoclonal gammopathy

Front 24

Presence of ____________ --> can confuse MS with APLS (antiphospholipid antibody syndrome)

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Antiphospholipid antibodies

Front 25

_____ cells = humeral mediated immunity --> produce antibodies that destroy myelin sheath

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Plasma cells

Front 26

MS is a _____ inflm rxn

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Th1

Front 27

Demyelination in MS follows ______

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Inflammation

Front 28

In MS _________ cells act as antigen presenting cells and also release IL-1 and other cytokines and phagocytiz myelin debris

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Microglial cells

Front 29

Phagocytize myelin debris in MS

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Microglial cells

Front 30

Undergo apoptosis in MS

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Oligodendrocytes

Front 31

End point of inflm in MS is ___________ degeneration and associated _____

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Axonal degeneration and associated astrogliosis

Front 32

MS has antibodies to ___________

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Myelin basic protein

Front 33

___ follows with oligodendrocyte recovery and remyelinization in MS

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Th2

Front 34

Stage 1 of MS = aka

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Initiation

Front 35

Stage 1 of MS (initiation) = age __-

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13-15

Front 36

Stage 1 of MS = initiation = ____________

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Activation of autoreactive lymphocytes

Front 37

Stage 2 of MS = aka

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Latency

Front 38

Stage 2 of MS (latency) = age

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15-30

Front 39

Stage 3 of MS = aka

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Onset

Front 40

Stage 2 of MS = ___________

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Intrathecal lymphocytes appear

Front 41

Stage 3 of MS = onset = Age _

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30

Front 42

Stage 3 of MS (onset) ____________

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Inflm demyleinzation triggered by systemic infection (ex: URI)

Front 43

Stage 4 of MS = aka

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Inflammation-transition

Front 44

Stage 4 of MS = age ____

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30-55

Front 45

Stage 4 of MS = __________

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Recurrent demyelination with later axon injury

Front 46

Stage 5 of MS = aka

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Neurodegeneration

Front 47

Stage 5 of MS (neurodegeneration) = age ______

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45-75

Front 48

Stage 5 of MS = _________________

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Ongoing cortical dz with axon degeneration

Front 49

In MS, serum antibodies to _____ and ______ are not associated with inc risk of progression to clinically definite MS

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- Myelin Oligodendrocyte glycoprotein
- Myelin Basic Protein

Front 50

- Low in areas of active demyelinization
- Normal to increased with remyelinzation
(In MS)

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Oligodendrocytes

Front 51

- Characteristic of MS
- unilateral, painful loss of acuity with decreased color perception

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Optic neuritis

Front 52

- Characteristic of MS
- Sensory and motor signs, Lhermitte's sign, bladder/bowel dsfx, ED, acute dystonias

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Myelitis

Front 53

- Assoc with MS
- Electrical sesation that runs down back (posterior column) from bending neck fwd or bckwd

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Lhermitte's sign

Front 54

- Assoc with MS
- Nystagmus, intention tremor, gait, ataxia, dysarthria, dysmetria dysdiadochokinesia

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Cerebellar signs

Front 55

- Assoc with MS
- Deficits in: sensory and motor deficits with spasticity

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Cerebrum

Front 56

- Assoc with MS
- Ocular muslce palsy, (3,4,6) trigeminal neuralgia, CN7 paralysis, Internuclear Opthalmoplegia

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Mid-brain-brainstem deficiencies

Front 57

MS sign
- Visual blurring during exercise
- And intensification of signs and sx's in hot tubs

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Uhthoff sign

Front 58

Those with optic neuritis and MS ---> with MRI
____ % normal
______ % with 10+ lesions

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35% normal

29% with 10+lesions

Front 59

- If have clinically isolated sx's of MS + 3 or more T2 MRI lesions --> ______ %+ likelihood of developing MS withint 7-10 yrs

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80%

Front 60

MS mean age of onset

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30.6

Front 61

MS avg # of attacks in 1st yr

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1.6

Front 62

MS total # of attacks

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3.5

Front 63

MS avg time to progressive dx

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18.1 yrs

Front 64

Relapsing Remitting MS: normal duration of relapse is ______ with ____ intervals

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6-8 wks with 15 month intervals

Front 65

Predictors of rapidly progressive MS course (4)

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- Older than 40 yrs
- Cerebellar signs (ataxia)
- Short 1st attack interval
- High # of attacks in 1st yr

Front 66

Visual deficit in MS

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Scotoma = field of vision with blind spot

Front 67

Cerebral impairment in MS causes (2)

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- Mood alteration
- Dementia

Front 68

Pyramidal sx in MS may progress to ______

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Quadriplegia

Front 69

With cebral atrophy in MS get decreased _____

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Cortical Thickness

Front 70

Expanded disability status scale of MS
1.0 =

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No disability

Front 71

Expanded disability status scale in MS
9.0 = _______

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Helpless and confined to bed

Front 72

Expanded disability scale in MS
10.0 =

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Death

Front 73

MS
- Atypical reactive astrogliosis

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Creutzfeld astrocytes

Front 74

MS = get collar = ___ --> in acute phase of demyelinization

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Perivascular lymphocytes

Front 75

Malignant monophasic MS (1 area affected rather than multiple plaques)

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Marburg's disease

Front 76

- Monophasic illness
- Kids
- 3-21 days after MMR or varicella

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Acute disseminated encephalomyelitis

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White matter dz
- Sx: stupor, meningismus, paraplegia, incontinence

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Acute disseminated Encephalomyelitis

Front 78

- In kids
- MRI shows affected area: periventricular, symmetrical or asymmetrical

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Acute disseminated encephalomyelitis

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- Adults
- Post respiratory tract infection

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Acute hemorrhagic leukoencephalitis

Front 80

- Fulminant course
- CSF: PMNs + RBCs
- Path: vasculitis with hemorrhage and demyelination

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Acute hemorrhagic leukoencephalitis

Front 81

Demyelinating dz from:
- Rapid correction of hyponatremia
- Alcohol abuse

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Central Pontine myelinolysis

Front 82

Demyelinating dz
- Causes paraparesis, quadriparesis, dysarthria, disorder of conjugate eye mvmt, locked-in syndrome

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Central Pontine Myelinolysis

Front 83

Injury to white matter from toxic meds

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Toxic leukoencephalopathy

Front 84

Caused by:
- Antineoplastic: methotrexate
- Immunosuppressive: cyclosporin
- Antimicrobial - amphotericin B
- Abuse - alcohol, touene, MDMA, cocaine
- Others: CO, carbon tet, arsenic

Back 84

Toxic leukoencephalopathy

Front 85

- From alcohol abuse
- Demyelination and hemorrhage around 3rd ventricle
- Atrophy of mamillary bodies (thiamine def)

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Wernicke's encephalopathy

Front 86

- From alcohol abuse
- Ataxia
- Confusion Opthalmoplegia
- Korsakoff - short term memory loss with fabrications

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Wernicke's encephalopathy

Front 87

- From alcohol abuse
- Demyelinization of corpus callosum - dementia, spasticity, dysarthria

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Marchiafava-Bignami

Front 88

Polyneuropathy with weakness and paresthesias can be from ______ neurotoxicity

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alcohol

Front 89

Imaging in chronic alcoholism: dec gray matter __-

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2-5%

Front 90

Imaging in chronic alcoholism: dec white matter _____

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6-17%

Front 91

Imaging of _________ --> T2 b/l periventricular isolated or confluent attenuation similar to those found in MS

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Chronic alcoholism

Front 92

- Deficiency of peroxisomal enzymes impairs oxidation of very long chain FA's --> accum in adrenal glands and white matter

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X linked adrenal leukodystrophy

Front 93

X linked adrenal leukodystrophy is associated with the inability to metabolize ________

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Very long chain FA's

Front 94

30% onset 4-8yrs
- ADD with rapid regression of cognitive fx

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X linked adrenal leukodystrophy

Front 95

40% of those with X linked adrenal leukodystrophy have this
- progressive paraparesis and sphicter disturbance --> cerebral involvement in almost 1/2

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Adrenomyeloneuropathy

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30% _______ w/o neuro dz in X linked adrenal leukodystrophy

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Addison's