Nb3 Myelin Disorders Path

Front 1

Name a disease that
affects CNS myelin
affects PNS myelin
affects both

Back 1

Multiple Sclerosis- CNS only
Guillain-Barre - PNS only
Leukodystrophy-both

Front 2

Oligodendrocytes are in CNS/PNS and form one or many internodes?

Back 2

Oligodendrocytes in CNS form many internodes

Front 3

Name 2 major consequences of demyelination

Back 3

Conduction block which causes signal dispersion
Prolongation of refractory period causing failure of transmission

Front 4

Demyelinated axons have increased or decreased sensitivity?

Back 4

Increased sensitivity, they are hyperexcitable

Front 5

Name 3 acquired disorders of myelin that are immune-mediated

Back 5

Multiple Sclerosis
Acute disseminated encephalomyelitis (ADEM)
Acute hemorrhagic leukoencephalitis

Front 6

Describe areas of demyelination in MS

Back 6

multiple areas, lesions seperated in space and time

Front 7

MS begins in what age group and usually presents with

Back 7

Begins in young adults, can start with transient loss of vision

Front 8

Geographically where is MS high?

Back 8

At higher latitudes

Front 9

MS is more common in whites or blacks, men or women?

Back 9

More common in whites and women

Front 10

What is the Uhthoff's hot bath test?

Back 10

A test for Multiple Sclerosis, heat (increased body temp) exacerbates MS

Front 11

In the acute stage of MS what immune cells are seen?

Back 11

CD4+, Th1, Th17, macrophages

Front 12

In MS astrocytes may ___

Back 12

Abnormally present Class II molecules for antigen presentation to T cells

Front 13

In MS the level of ____ in the CSF may correlate with disease activity because this substance is toxic to oligodendroglia and myelin

Back 13

Tumore Necrosis Factor

Front 14

Almost all MS patients experience__

Back 14

motor weakness and spasticity

Front 15

CSF profile of MS
WBC
Protein
Glucose
2 abnormal findings

Back 15

WBC- normal or slightly high with lymphocytes
Protein-mildly elevated
Glucose- normal
Oligocolonal bands- IgGs
Myelin basic Protein- may be present during acute relapse

Front 16

Chronic Multiple Sclerosis (Charcot variant) shows these on histology

Back 16

Well-demarcated WM lesions called plaques in the periventricular region

Front 17

Acute MS (Marburg variant0 is likely to show more ___ than chronic MS

Back 17

active inflammation
see inflammation and edema on imaging

Front 18

Do you see plaques in acute MS?

Back 18

Yes but they are less well-cicumscribed than chronic MS and more pink-white and swollen

Front 19

Neuromyelitis optica (Devic's syndrome) describes

Back 19

A variant of MS characterized by optic neuritis (bilateral)
Spinal cord lesions are often severe and necrotizing

Front 20

In MS vascular deposition of immunoglobulin and complement are evidence for

Back 20

humoral immune mechanisms

Front 21

Histologically ADEM resembles ___ with-

Back 21

Acute MS, perivenous lymphocytes and macrophages with acute demyelination

Front 22

ADEM presents with ___

Back 22

an abrupt onset of neurological symptoms 2-30 days after infection

Front 23

Common findings in ADEM include

Back 23

Depressed consciousness, unilateral or bilateral long tract signs, acute hemiparesis, ataxia

Front 24

Name some ways to distinguish ADEM from the first attack of MS

Back 24

Prodromal febrile illness, headache, altered mental status, transient or absent oligoclonal bands, Basal ganglia involvement, Monophasic

Front 25

Acute hemorrhagic leukoencephalitis usually follows ___
how dangerous is it?

Back 25

Respiratory infection like mycoplasma
usually fatal

Front 26

Name 3 viral acquired disorders of myelin

Back 26

Progressive multifocal Leukoencephalopathy (PML)
Subacute sclerosing panencephalitis (SSPE)
HIV Leukoencephalopahty

Front 27

PML is associated with what kind of virus?

Back 27

Polyomavirus (JC), a double stranded DNA virus that usually infects immunocompromised

Front 28

Is there a treatment for PML?

Back 28

No, HIV patients do better with anti-retroviral therapy but there is no remyelination and deficits are permanent

Front 29

Name 2 biological drugs associated with development of PML

Back 29

Natalizumab for MS treatment and retuximab monoclonal antibody for lymphoma and rehumatological disease

Front 30

Microglial nodules are characteristic of

Back 30

Viral infections

Front 31

Bizzare astrocytes are specific for

Back 31

PML

Front 32

SSPE is characterized by-
and related to what disease?

Back 32

dementia, spasticity and seizures
Measles

Front 33

How does measles cause disease in SSPE

Back 33

the paramyxovirus infects oligodendroglia and allows cell fusion but not budding of infected cells, there is a slow progressive infection

Front 34

Describe histology of SSPE

Back 34

Perivascular inflammation accompanies intracelular viral inclusions in oligodendrocytes

Front 35

Name 2 toxic/metabolic acquired disorders of myelin

Back 35

Central Pontine Myelinolysis
Vitamin B12 deficiency

Front 36

Central Pontine Myelinolysis CPM is usually due to

Back 36

rapid correction of chronic hypoatremia

Front 37

Symptoms of CPM include

Back 37

corticobulbar and corticospinal dysfunction
quadriparesis
pseudobulbar palsy
locked-in syndrome

Front 38

Is CPM inflammatory? progressive?

Back 38

No it is myelin loss without inflammation, monophasic

Front 39

Who is at particular risk for CPM

Back 39

chronic alcoholics/ liver transplant
thiamine deficiency, electrolyte abnormalities

Front 40

Describe locked-in syndrome

Back 40

lesion in ventral pons that involves corticobulbar and corticospinal spare sensory
patient is paralyzed but conscious, can sometimes move eyes

Front 41

Subacute combined degeneration of the Spinal Cord is due to__

Back 41

Vitamin B12 deficiency

Front 42

What is B12's role in the nervous system?

Back 42

Cofactor for methione synthetase,
Methionine makes SAM which is the only methyl group donor of the nervous sys

Front 43

Lack of B12 and subsequent instability of lamellar myelin is clinicaly apparent in tracts-

Back 43

containg large myelinated axons
posterior columns
lateral corticospinal tracts

Front 44

Diagnosis of SCD is confirmed by finding

Back 44

Megaloblastic anemia
low levels of serum B12
high levels of methylmalonic acid and homocysteine

Front 45

Neruological conditions caused by B12 deficiency

Back 45

Spinal Cord degeneration
Psychiatric symptoms
Optic neuropathy
Peripheral neuropathy

Front 46

Name 3 conditions that resemble SCD

Back 46

HIV associated Vacuolar mylopathy
Nitrous oxide (prolonged exposure to anesthetic)
Copper deficiency

Front 47

Arteriolosclerosis is what kind of disorder

Back 47

Acquired vascular disorder

Front 48

Leukoaraiosis is a term used in imaging studies and describes

Back 48

rarefaction of white matter

Front 49

Binswanger Disease is associated with and includes these findings

Back 49

Dementia, arteriolosclerosis with small infarcts in white matter

Front 50

Leukodystrophies are what kind of disease?
they usually spare---
Involve PNS or CNS?

Back 50

inherited metabolic disease
Spare the subcortical U-fibers
Involve both (acquired diseases usually only involve CNS)

Front 51

Genetics of the following leukodystrophy
Krabbes
MLD
ALD

Back 51

Krabbes- ecessive lysosomal, galactocerebrosidase

MLD- recessive lysosomal, arysulfatase A

ALD- X linked ABCD1 transporter

Front 52

Histologically Krabbe Disease shows

Back 52

accumulation of galactocerebroside in macrophages, and accumulation of toxic psychosine

Front 53

Histologically MLD shows

Back 53

large cells with sulfatide

Front 54

In ALD there is preferential involvement

Back 54

of the more posterior white matter

Front 55

Define Leukodystrophy

Back 55

Dysfunction or death of myelin forming cells due to accumulation of toxic material