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Flashcards in Dmyelinating Diseases Deck (20)

Who gets MS? What's interesting about the epidemiology? 

Normally people ages 25-35 (but can be older) and Females:Males 2:1

Interestingly, occurs further away from equator


What is the generally accepted idea behind pathophysiology of MS?

Sensitization occurs in the periphery with some nervous system antigen being mistaken as foreign

then Th1 and Th17 cells (that are normally kept in check) get out of whack and move in to BBB and shit goes crazay


Typically, immune cells enter from a venule and attack myelin but axons are spared


What are the 4 major clinical subtypes of MS?

1) Relapsing-Remitting: exacerbations of worsening diability then improvement and can accumulate disability with each episode

2) Secondary Progressive - disability accumulates over time, stop seeing evidence of remission 

3) Primary Progressive

4) Progressive-relapsing


What do you see on imaging for MS?

Use a T2 Weighted MRI and see Periventricular areas of inflammation

- perpenduclar to long axis of ventricles 

- "Dwson's Fingers" - perpendicular orientation of lesions as they appear coming off of the long axis of lateral ventricle 


Aso check the WM of corpus callosum, optic nerves, peduncles and SC


What genes involved in MS?

Immune response genes on chromsome 6



What viruses can also cause demyelinating disease?

Epstein-Barr Virus: higher risk for MS in those exposed later in life

PML- JC Virus reactivation: damages Myelin forming cells (oligodendrocytes) 

HIV-related Demyelination: early in infection, rare complication


What are the environmental RF for MS?

1) Vitamin D Deficiency - low levels associated with immune activation 

2) CIgarrete smoke - more rapid MS progression, increased risk and likelihood for treatment failure, more likely to develop Anti-IFNbeta and Anti-Natalizumab antibodies

3) Increased Sodium intake - immune actviator 


Presentation features of MS - list some

Optic nerve inflammation, poor balance and dizziness, weakness, double visions


Bladder and Bowel dysfunction, pain, sensory loss


FATIGUE!!!! spasticity

sexual dysfunction

congnitive impairment - word finding, memory and concentration 


What is the definition of "clinically isolated syndrome"?

First neurologic episode of MS defined by symptoms that:

-last at least 24 hours

-caused by inflammation and demyelination - MRI scan

-single or multiple sites


To diagnose MS with CIS - need more than 1 lesion, some older lesions or evidence that things are happening at different points in time


General treatment approaches  used in MS

STeroids, IVIG, Plasma cleansing approach (?)

Nutritional/Metabolic - low fat, high fish oil, Vitamin D 

Physical Therapy and Psychosocial support 


+ Symptoms management 


Immune Modulators used in MS and their side effects: 

IFN-Beta!!!!! - need high doses and so see increased side effects

Natalizumab and rituximab - PML

Alemtuzumab - ITP and Grave's Disease

Fingolimod- Cardiopulmonary effects and infectious

Mitoxantrone - increased incidence and cardiac side effects, *Acute Leukemia


What is Marburg's Variant?

Acute, Fulminant MS - can be fatal and treated aggressively


What is Balo's Concentric Sclerosis?

See lesions expanding in concentric, inuform circles


typically toxic or viral cuase 


What is a progressive demyelintating disease of kids? 

Schilder's Disease


What is Neuromyelitis Optica? How does it present and what happens in it?

Disease where you have recurrent and simultaneous inflammation and demyelination of the Optic Nerve and Spinal Cord

Antigen - Alpha Aquaportin-4 Antibody against the water protein channel reacts with astrocyte foot processes

Presentation: longitudinally extensive SC lesions, Optic nerve problems but brain may be normal!


What do you need to diagnose Neuromyeltis Optica?

At least 2 of the following:

-Brain MRI negative or non-diagnostic for MS

-SC lesion on MRI extending >3 SC segments - longitudinal extensive transverse myelitis

- NMO-IgG seropositivity

-CSF exam - incrased WBC, increased protein, Oligoclonal bands (antibody in CSF thats not in blood) 


How do you treat neuroptic myelitis?

High dose steroids or plasma exchange

Azathioprine and Mycophenolate

or Rituximab


What is Acute Disseminated Enephalomyelitis (ADEM)? Who gets it? How does it present? 

ADEM is an intense acute inflammation of the brain and SC and occasionally optic nerves as a result of exposure to exogenous antigen  (follows viral/bacterial infection) 


Most common in children and 60% of cases in boys! (unlike everything else) 

Monophasic - only one attack with fever, HA, confusion, vomiting



What do you see for Acute Disseminated Encephalomyelitis (ADEM) on MRI? How do you treat it? 

see Cotton-ball type appearing lesions all over and NOT ONLY IN WHITE MATTER!!! Basal Ganglia can be involved (which you don't see in MS) 

Treat with high dose steroids


What is Transverse Myelitis? Who gets it? What's the treatment?

Inflammatory degeneration of SC that occurs suddenly or gradually with a 1-4 week onset


may be related to MS, NMO or viral infection 


Cases appear more in FEMALES and between ages 10-19 or 30-39

Treatment - high dose steroids, plasma exchange, antivirals