CC5: Demyelinating Disorders

Question 1

diseases of mylein can be categorized:

Answer 1

myelinoclastic or dysmyelinating

Question 2

destruction of normal myelin

Answer 2

myelinoclastic

Question 3

metabolic abnormality,
breakdown abnormal myelin

Answer 3

dysmyelinating

Question 4

chronic, demyelinating, autoimmune disease of the brain and spinal cord, usually characterized by recurrent attacks and remission of neurologic dysfunction

Answer 4

multiple sclerosis

Question 5

MS gene pathogenesis:

Answer 5

HLA-DRB1

Question 6

MS virus pathogenesis:

Answer 6

EBV, HHV, chlamidia, measles

Question 7

MS metabolic pathogenesis:

Answer 7

vit d, obesity

Question 8

MS can cause:

Answer 8

axonal injury, focal demylinated lesions, phagocytic microglia, lymphocytes, gliolisis, wallerian degneration

Question 9

severe eye pain left eye, sees black spot

Answer 9

MS

Question 10

higher in females and white people

Answer 10

MS

Question 11

most common MS signs

Answer 11

optic neuritis
brainsten syndrome
cerebellar
transverse myelitis

Question 12

optic neuritis is common for

Answer 12

MS

Question 13

esions of the medial longitudinal fasciculus (MLF). Paresis of adduction on lateral gaze & associated nystagmus in the abducting eye

Answer 13

Internuclear ophthalmoplegia

Question 14

Patients present with unilateral intense paroxysmal pain of the electrical discharge type (“painful flashes”)

Answer 14

Trigeminal Neuralgia

Question 15

what are cerebellar syndromes?

Answer 15

coordination difficulty: ataxia, gait, tremor, dysarthia

Question 16

May be the initial presentation of MS:

Answer 16

transverse myelitis

Question 17

MS diagnosis is based on:

Answer 17

history and physical finding were there is history or evidence of at least two attacks separated in space and time of at least one month that can’t be explained by one single lesion

Question 18

5 Key principles in the diagnosis of MS

Answer 18

1. Identification of a syndrome “typical” of MS-related demyelination
2. Objective evidence of CNS involvement
3. Demonstration of dissemination in space
4. Demonstration of dissemination in time
5. “no better explanation” other than MS

Question 19

what is the McDonald criteria

Answer 19

2010-Diagnostic Criteria for MS

Question 20

what is Schumacher Criteria 1965

Answer 20

older 5 key principles of MS diagnosis

Question 21

expalin Mcdonald ceiteria

Answer 21

diagnosis MS-no better explanation
possible MS-not all criteria met
not MS-another better diagnosis

Question 22

where can you find lesions in MS mri?

Answer 22

T2 lesions around lateral ventirvle, brainstem and cerebellum

Question 23

Neurodegenerative lesions appear on mri as

Answer 23

black holes

Question 24

MS lab studies:

Answer 24

CSF
IgG, oligoclonal bands, BMP

Question 25

Measures, in time, the response of a sensory tract, nucleus and/or cortical area to an external stimuli

Answer 25

Evoked potentials

Question 26

Visual evoked potentials VEP is sensitive to

Answer 26

optic neuritis P100

Question 27

Worsening of a sign or symptom with exercise of increased temperature

Answer 27

Uthhoffs phenomenon

Question 28

Sudden sensation of electric shock down spine and arms when patient flexes neck

Answer 28

Lhermitte's sign

Question 29

can MS relapse?

Answer 29

yes. relapsing remmiting MS.

Question 30

Mediated by lymphocytes & stimulated by exposure to infectious agents.

Answer 30

adaptive immunity

Question 31

Protection that relays on mechanisms that exist before infection, capable of rapid response, react essentially the same way to repeated infections.

Answer 31

innate immunity

Question 32

what guide innate immunity

Answer 32

microglia, monocytes, dendritic cells

Question 33

what guides adaptive immunity

Answer 33

t cells, b cells

Question 34

Earliest expression of MS

Answer 34

CIS clinically isolated syndrome

Question 35

Earliest expression of MS

Answer 35

CIS clinically isolated syndrome

Question 36

the best choice to support the clinical diagnosis of MS

Answer 36

MRI DIS and DIT

Question 37

the most important diagnostic CSF study

Answer 37

CSF qualitative assessment for oligoclonal bands (OCBs)

Question 38

Once known as Devic’s Disease

Answer 38

Neuromyelitis Optica Spectrum Disorder (NMOSD)

Question 39

Characterized by a combination of bilateral ON and cervical myelopathy

Answer 39

Neuromyelitis Optica Spectrum Disorder (NMOSD)

Question 40

neuromyelitis optica antibodies:

Answer 40

astrocytes and oligodendroyctes IgG

Question 41

neuromylelitis opctica clinical presentation:

Answer 41

relapsing, area postrema vomiting, Optic Nerve and myelopathy occur, MRI lesions

Question 42

NMOSD mri findings

Answer 42

diencephalic lesions and cerebral syndrome

Question 43

- AQP-4-IgG diagnostic

Answer 43

2 or more clinical core characteristics * 1 must be ON, acute myelitis, or area postrema syndrom

Question 44

NMO vs. MS

Answer 44

NMO is more severe, MRI has longer bands bilateral ON medullary lesion-vomit CSF IgG-astrocyte

Question 45

Uniphasic syndrome occurring is association with an immunization or vaccination or systemic viral infection

Answer 45

Acute disseminated encephalomyelitis (ADEM)

Question 46

Usually develops after URTI, EBV, CMV, mycoplasma, HIV

Answer 46

ADEM

Question 47

ADEM is more common in

Answer 47

children

Question 48

ADEM can present in two forms:

Answer 48

encephalitic and myelitic

Question 49

ADEM features

Answer 49

preceding events of vaccine multifocal or diffuse CNS signs vessel damage

Question 50

can ADEN relapse

Answer 50

no, rapid recovery