LO2 - mostly Inflammatory demyelinating

Question 1

85% of MS are which type?

Answer 1

Relapsing remitting (RRMS)

• comes and goes over time
• Relapse = Attack = Exacerbation

Question 2

15% of MS presents as which type?

Answer 2

primary progressive (PPMS)

• progressive disease from outset, w/o relapses

Question 3

Secondary progressive MS

Answer 3

RRMS converts to a Progressive MS

Question 4

Most common cause of CNS inflammatory disease

Answer 4

MS

Guillan berret: is peripheral demyelination

Question 5

The majority of pts with MS have which types of lesions?

Answer 5

Both brain and spinal lesions.

Question 6

Is EBV exposure risk for MS?

Answer 6

Yes
20x less risk if not exposed

• so is Vit D, smoking, obesity, high salt diet

Question 7

McDonald Criteria of MS

Answer 7

multiple lesions of CNS disseminated in TIME and SPACE

1. TIME:
   - 2/m symptoms lasting at least 24 hours, 30+ days apart for RRMS
   - or 12 month progression of sx for PPMS
2. Space:
   - 2 separate locations in brain/spinal cord

Question 8

Lhermitte’s is a classic finding in which disorder?

Answer 8

MS

paresthesias down spine with neck flexion

Question 9

Neuro exam for MS

Answer 9

UMN
“pyramidal tract signs”
- weakness, spasticity, + babkinski sign

• decreased visual acuity
• optic atrophy
• afferent pupillary defect
• abnormal eye movement
• sensory loss
• cerebellar signs
• cognitive dysfxn

Question 10

Lab findings in pts with MS

Answer 10

MRI (w or w/o contrast), FLAIR

1. atrophy, especially gray matter
2. high burden of T1 holes
3. high burden of T2 lesions
4. POSTERIOR FOSSA Lesions

Question 11

CSF analysis in MS

Answer 11

NI protein (may be mildly elevated)

NI WBC (may be mildly elevated)

NI glucose (always!)

ABNORMAL IgG

Question 12

In order to dx pts with MS

Answer 12

primarily dx clinically

but
brain MRI with criteria help define

Question 13

Is the progression and disability of MS related to inflammation?

Answer 13

No

Even as inflammation goes down, MS course still worsens - unknown why

Question 14

dawson’s fingers

Answer 14

MS

Demyelinating white fingers from corpus callosum - along medullary veins

Question 15

Effects of OH on CNS

Answer 15

metabolic disorder WITH morphological correlates

Question 16

Methanol can cause which changes in the brain?

Answer 16

Putamen hemorrhagic necrosis

Question 17

Ethyl Alcohol can cause which changes in the brain?

Answer 17

Acute:
- Severe cerebral edema

Chronic:

• Meningeal fibrosis
• Cerebral cortex WM vol loss (neuronal/dendritic reduction)
• Cerebellar Vermis degeneration

Question 18

Fetal alcohol syndrome

Answer 18

Hyperactivity
Poor motor skills
learning difficulties
mental retardation (if really bad)

Brain damage due to liver disease/cirrhosis

Question 19

Hepatic Encephalopathy

Answer 19

Usually too much OH –> cirrhosis

Aterixis (flapping tremor)
Stupor
Coma

Question 20

Wernicke encephalopathy triad

Answer 20

1. ataxia
2. confusion
3. opthalmoplegia (or nystagmus)

(treat with Thiamine B1 - Note that Wernicke is due to lack of thiamine, not direct effects of alcohol)

Question 21

Cobalamin deficiency

Answer 21

Vit B12 def
(very strict vegans, or indiv with pernicious anemia)

Degeneration:
- paresthesias, ataxia, decreased vibration and proprioception

Dementia: psychosis and mood disturbances

Question 22

Central Pontine Myelinolysis (CPM)

Answer 22

Severe damage of myelin sheath in brain stem/pons due to EXCESSIVE, RAPID CORRECTION/OVERCORRECTION OF HYPONATREMIA.

“lock in syndrome” if severe
- look for pts with cancer, liver disease, sepsis, burns

Question 23

Wilson’s Disease

Answer 23

AR disorder of Copper metabolism --> 
accumul. of copper in the lentiform nucleus (putamen/glob) --> 
basal ganglia degeneration --> 
movement disorder
- flapping tremor/spasticity

(usually in kids/young adults)

Question 24

tx for Wilsons disease

Answer 24

penicillamine to remove copper

Question 25

Areas of brain damaged by thiamine def

Answer 25

``` Mamillary bodies walls of 3rd ventricles Floor of 4th ventricle Inferior colliculi Thalamus ```

Question 26

Korsakoff syndrome

Answer 26

Chronic memory disorder caused by B1 (thiamine) defic. - usually caused by alcohol misuse

Question 27

How does pernicious anemia result in Cobalamine (B12 def?)

Answer 27

It is an immunologically mediated atrophic gastritis that causes failure of Intrinsic factor --> cant bind to B12 prior to abs from ileum --> megaloblastic anemia