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cAS3 > Demyelinating Disorders > Flashcards

Demyelinating Disorders Flashcards

1
Q

An autoimmune disease characterized by Chronic Inflammation, Demyelination, Gliosis(plaque or scarring) and Neuronal loss?

A

Multiple Scerosis

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2
Q

Diagnosis of Multiple Sclerosis

A

Clinical History
MRI
CSF analysis (Oligoclonal Bands)

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3
Q

Target cells of Multiple Sclerosis

A

Oligodendrocytes

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4
Q

What is the first manifestation of Multiple Sclerosis?

A

Impaired Vision

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5
Q

Genetic Predisposition of MS is associated with?

A

HLA-DRB1

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6
Q

Periventricular demyelinated sclerotic plaque (Dawson’s fingers) (FLARE MRI)

A

MRI of Multiple Sclerosis

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7
Q

Periventricular Inflammation Infiltrate

A

Histology of MS

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8
Q

IgG oligoclonal bands in the CSF on gel electrophoresis

A

Lab findings of MS

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9
Q

Cause: Reactivation of Latent JC virus
Risk Factors: long term treatment with monoclonal antibodies (Natalizumab) in MS, HIV patients

A

Progressive multi focal Leukoencephalopathy

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10
Q

Overly aggressive correction of hyponatremia

A

Osmotic demyelination syndrome

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11
Q

Disease of White Matter in CNS ———-and disease of White matter in PNS —————

A

Multiple sclerosis; Guillain Barre syndrome

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12
Q

Course: Chronic Relapsing (classical)
Episodic neurologic signs and symptoms: Disseminated in time and space
Peak age: 20-40yrs more common in women.
Attack followed by complete or partial remission

A

Multiple Sclerosis

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13
Q

Types of Multiple Sclerosis

A
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14
Q

What are the environmental factors of MS?

A

Vitamin D deficiency
Smoking
EBV- Epstein-Barr Virus

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15
Q

What are the immunologic Pathogenesis of MS?

A

For specific reason unknown, some T-cell escape the BBB activating Microglial function with cytokines infiltration aka Periventricular infiltrate

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16
Q

Clinical Presentation and Lab Findings of MS

A

Female pt (20-40y/o) presents with (optical neuritis) PERIORBITAL PAIN preceded with visual loss; worsens by exercise or a HOT BATH/ feel very HOT (Uhthoff Phenomenon); weakness of the limbs- upper motor Neuron type; Diplopia (double vision), May be Atexia; Vertigo; LHERMITTE’s symptom (neck flexroom may cause an electric shock-like sensation radiates down the back of legs; Bladder dysfunction (>90% of MS patients); Sensory: Paresthesia and Hypesthesia

17
Q

What triggers Perivenular cuffing in MS

A

Some T cells comes in brain parenchyma, identify myelin as a foreign antigen, this is expressed and they start secreting cytokines which damages tight junctions causing Periventricular infiltrate, predominantly T-cells and macrophages

18
Q

MRI of areas of demyelination in MS

A
19
Q

Cells targeted in MS ————— cell targeted in Gulian Barre Syndrome

A

Oligodendrocyte (CNS); Schwann Cells (PNS)

20
Q

Treatment of Acute Attack of MS?

A

Corticosteroids - High dose for Short term or intravenous Methylprednisolone

21
Q

Treatment for Remitting and Relapsing MS

A

Natalizumab
Rituximab
Mitroxantrone
Cladribine

22
Q

Drug MOA for MS: humanized monoclonal antibody against Alpha -4 ( a-4) integrity
Reduce the transmission of immune cells into the CNS by interfering with the alpha4Beta 1-integrins receptor molecules in the surfaces of cells

This drug increases the risk of progressive Multifocal Leukoencephalopathy

A

Natalizumab

23
Q

Acts against CD 20

A

Rituximab

24
Q

MOA of MS drug: Anti-tumor antibiotic acts by suppressing the activity of T-cells, B-cells, and macrophages that lead the attack on the Myelin sheath

A

Mitoxanthrone (Drug of last result for secondary MS)

25
Q

MOA of MS drug: Depletes B Cells

A

Cladribine

26
Q

MOA for MS drugs: composed of 4 amino acids and bare similar structure to myelin proteins; acts as decoy to T-cells; shifts the population of T-cells from pro inflammatory Th1 T-cell to regulatory Th2 T-cells that suppresses the inflammatory response

A

Glatiramer

27
Q

MOA of MS drugs: increases the cytotoxicity of natural killer cells and increases the phagocytosis activity of macrophages

A

Beta interferon: Interferon Beta -1a

28
Q

MOA of MS treatment: Selective sphingosine-1-phosphate receptor modulator, which sequesters lymphocytes in lymph nodes, preventing them from contributing to an autoimmune reaction

A

Fingolimod/Siponimod

29
Q
A

Methylprednisolone

30
Q

Multifocal areas of demyelination of varying size, throughout the brain, sparing SPINAL CORD AND OPTIC NERVES, caused by Reactivation of LATENT JC VIRUS

A

Progressive Multifocal Leukoencephalopathy (PML)

31
Q

An MS patient on Natalizomuab what kind of condition can they develop?

A

PML- progressive Multifocal Leukoencephalopathy

5% of AIDS pt develops PML

32
Q

Another name for Osmosis demyelination syndrome

A

Central Pontine Myelinolysis

33
Q

Demyelination WITHOUT inflammation in the base of the Pons, with relative sparing of axons and nerve cells.
Bilateral corticospinal and corticobulbar tracts are affected

A

Osmotic demyelination syndrome or Central Pontine myelinolysis (if it’s in the pons)

34
Q

Cause of Osmotic Demyelination Syndrome (ODS)

A

Overly rapid correction of hyponatremia

35
Q

Clinical features: Quadriparesis, psuedobulbar palsy (dysphagia, dysphonia, dysarthria (diff. Speaking); Lock-in-syndrome - absent motor function but maintain INTACT Sensation and Cognition

A

Osmotic Demyelination Syndrome

36
Q

Chronic alcoholic patient (develops hyponatremia) was corrected what is the pathophysiology

A

Osmotic demyelination syndrome (ODS) due to abrupt correction to chronic hyponatremia by MASSIVE ASTROCYTIC DEATH leading to Demyelination

37
Q

Pathophysiology of Osmotic Demyelination Syndrome

A
38
Q

What is the main cell targeted in Osmotic Demyelination Syndrome?

A

Astrocytes - forms the BBB

cAS3 (17 decks)

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