Cns Demyelinat Diseases; Path Of Demyelin. Disease

Front 1

most common disease affecting central myelin

Back 1

multiple sclerosis

Front 2

primary dyemyelinating disease: defn

Back 2

where primary pathologic defect is in the myelin itself

Front 3

secondary dyemyelinating disease: defn

Back 3

a disease where myelin is destroyed as part of a more widespread pathological condition, eg., hypoxia

Front 4

dysmyelinating disease: defn

Back 4

myelin is improperly formed

Front 5

multiple sclerosis: symptoms

Back 5

weakness, incoordination, visual loss, sensation loss, bladder dysfunction

Front 6

MS: age of onset

Back 6

20s-30s

Front 7

4 subtypes of MS

Back 7

1) Relapsing - remitting
2) Primary Progressive
3) Secondary Progressive
4) Progressive relapsing

Front 8

Relapsing - remitting: defn

Back 8

relapses occur sporadically, from which patient may/not recover completely, with stability between relapses. most common.

Front 9

Most common MS subtype

Back 9

relapsing-remitting

Front 10

primary progressive ms: defn

Back 10

progression from onset

Front 11

secondary progressive ms: defn

Back 11

relapsing patients develop slow progression between relapses

Front 12

progressive-relapsing ms: defn

Back 12

progression from onset punctuated by relapses

Front 13

MRI in MS: what will be seen?

Back 13

shows areas of demyelination or scarring in brain

Front 14

Evoked potentials: what do they measure?

Back 14

measure the speed at which impulses move from periphery to cerebral cortex.

Front 15

CSF studies in MS: what is seen?

Back 15

Elevation of IgG in CSF with normal values in serum.

Electrophoresis of CSF proteins showing "oligoclonal bands" in IgG region.

Front 16

relapsing-remitting MS: treatment

Back 16

beta-interferon (1a, 1b)

glaterimer acetate

natalizumab

Front 17

secondary progressive MS: treatment

Back 17

mitozantrone - chemo drug for leukemia and prostate cancer that has been shown to decrease progression in secondary MS.

Front 18

Steroids: what do they do in MS?

Back 18

shorten and exacerbation

Front 19

Bladder spasticity in MS: treatment

Back 19

anticholinergics

Front 20

Acute transverse myelitis—defn

Back 20

acute spinal cord inflammatory demyelination

Front 21

leukodystrophy is an example of

Back 21

dysmyelination (abnormal formation of myelin)

Front 22

most common cause of non-traumatic neurologic disability in young adults

Back 22

multiple sclerosis

Front 23

Experimental allergic encephalomyelitis : defn

Back 23

animal MS model

Front 24

During pregnancy, risk of MS exarcerbation (increases, decreases)

Back 24

Decreases

Front 25

Immediately after pregnancy, risk of MS exarcerbation (increases, decreases)

Back 25

increases

Front 26

MS: pathology (general)

Back 26

CNS demyelination with axonal sparing

Front 27

Dawson’s fingers: defn

Back 27

name for the multiple sclerosis lesions around the ventricle-based brain veins

Front 28

What causes the conduction delay in MS?

Back 28

The inflammatory demyelination and edema.

Can improve as edema resolves and remyelination occurs

Front 29

MS is diagnosed using the ______ criteria

Back 29

McDonald

Front 30

Clincally Isolated Syndrome: defn

Back 30

1 attack; objective clinical evidence of 1 lesion

Front 31

What is needed to satisfy McDonald criteria for MS dx?

Back 31

Dissemination in space and time (two or more lesions in different areas at different times)

Front 32

Optic neuritis: symptoms

Back 32

Typically monocular visual loss, developing over hours to days.

Pain with eye movement.


Associated with loss of color perception and visual field (scotoma)
Afferent pupillary defect (Marcus Gunn pupil)

Front 33

MS: Ocular motor syndromes

Back 33

Internuclear ophthalmoplegia (INO)


Nystagmus
Impaired pursuit
Ocular dysmetria

Front 34

Bilateral weakness in MS suggests ___

Back 34

myelitis (spinal cord inflam)

Front 35

Lhermitte’s phenomenon: defn

Back 35

electric tingling sensation down the back or into the arms and legs, precipitated by neck flexing, and indicative of cervical spinal cord disease

Front 36

Cognition changes in MS

Back 36

50% show cognitive impairment

Attention, memory, concentration, processing.

Dementia is uncommon.

Front 37

Psych changes in MS

Back 37

Depression is common.


10% have pseudobulbar affect: inappropriate laughing or weeping
Bipolar disorder is more common in MS

Front 38

pseudobulbar affect: defn

Back 38

inappropriate laughing or weeping/emotional incontinence

Front 39

Probably most common symptom in MS

Back 39

Fatigue

Front 40

Fatigue in MS (worsens, improves) with heat.

Back 40

Worsens.

Front 41

Bowel, Bladder, Sexual Dysfunction in MS

Back 41

Bowel : constipation

Bladder : incontinence or retention

Sexual : occurs in 50-75%

Front 42

Neuromyelitis optica (Devic’s disease) : defn

Back 42

variant of MS where there's optic neuritis and transverse myelitis, sparing the brain

Front 43

Acute disseminated encephalomyelitis (ADEM)

Back 43

immune mediated demyelinating disease of brain. occurs most often in children. usually occurs post-infection.

Front 44

“Tumefactive” MS : defn

Back 44

very large plaque resembling tumor

Front 45

MS: characteristic MRI findings

Back 45

Hyperintense T2/FLAIR white matter foci

Front 46

Mitoxantrone: MOA

Back 46

Inhibition of B-cell, T-cell, and macrophage proliferation

Front 47

Natalizumab : MOA

Back 47

Prevention of migration of immune cells across BBB

Front 48

Fingolomod : MOA

Back 48

Sphingosine -1-phosphate (S1P) receptor modulator

Front 49

Rituximab : MOA

Back 49

chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells. Rituximab destroys B cells, and is therefore used to treat diseases which are characterized by having too many B cells, overactive B cells or dysfunctional B cells. This includes many lymphomas, leukemias, transplant rejection and some autoimmune disorders (MS)

Front 50

(Primary, Secondary) demyelinating diseases are associated with axonal preservation.

Back 50

Primary. Secondary are a consequence of axonal damage.

Front 51

First degree relatives of MS have ____ increased chance of getting it.

Back 51

15-20x

Front 52

oligoclonal IgG bands are seen in CSF in what disease?

Back 52

MS

Front 53

Is a severe demyelinating disease characterized by selective involvement of the spinal cord and optic nerves and is considered to frequently spare the brain

Back 53

neuromyelitis optica

Front 54

PML on imaging

Back 54

Bilateral lesion in subcortical white matter, may be asymmetrical

Front 55

Subacute sclerosing panencephalitis (SSPE): symptoms

Back 55

Insidious onset of behavioral changes and mental retardation followed by myoclonus, ataxia and seizures…stupor-death.

Front 56

Central pontine myelinolysis
: pathophys

Back 56

rapid correction of hyponatremia

Front 57

Central pontine myelinolysis: pathologic changes seen

Back 57

-Loss of myelin
-Intamyelinic edema
-Some axonal loss

Front 58

Subacute combined degeneration of the spinal cord
: pathophys

Back 58

B12 deficiency (pernicious anemia)

Front 59

HIV associated vacuolar myelopathy: what is iseen?

Back 59

Pathology in anterior, lateral, posterior white matter columns: vacuolition,demyelination and macrophages. HIV infects mononuclear cells, astrocytes and neurons.

Front 60

HIV associated vacuolar myelopathy: symptoms

Back 60

Painless leg weakness ,stiffness,sensory loss,imbalance,spinctor dysfunction

Front 61

Canavan’s disease : defn

Back 61

autosomal recessive[2] degenerative disorder that causes progressive damage to nerve cells in the brain. Canavan disease is also one of the most common degenerative cerebreal diseases of infancy. This disease is one of a group of genetic disorders called leukodystrophies.

Front 62

Canavan’s disease : what is seen on histology?

Back 62

Demyelination, Alzheimer type II cells, gliosis, vacuolization, which involves arcuate fibers

spongy degeneration

Front 63

Wernicke's encephalopathy: what pathological changes are seen grossly?

Back 63

Brown discoloration of mammillary bodies and petechiae

Front 64

CADASIL(cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy): what is seen histology?

Back 64

PAS+ material in arterioles and arteries
EM(GOM)-granular osmophilic material

Front 65

CADASIL: symptoms

Back 65

Migraine
-repeated subcortical ischemic strokes
-dementia
-myelin loss