Neuro20

Question 1

Autoimmune inflammation and demyelination of CNS (brain & spinal cord) disease.

Answer 1

Multiple Sclerosis

Question 2

What cells are affected in MS?

Answer 2

Oligodendrocytes

Question 3

What type of Hypersensitivity reaction is MS?

Answer 3

Type 4

Question 4

What HLA serotype is associated with MS?

Answer 4

HLA-DR2

Question 5

What is the patient presentation of MS?

Answer 5

White Women (20-30s) - most commonly
Sx:
 - Optic neuritis (sudden vision loss)
 - MLF syndrome (INO)
 - Hemiparesis
 - Hemisensory symptoms
 - Bladder/Bowel Incontinence

Question 6

What is Charcot’s classic triad of MS?

Answer 6

“SIN”
Scanning Speech
Intention Tremor
Nystagmus

*also Incontinence & INO

Question 7

What is the gold standard for diagnosing MS?

Answer 7

MRI

Question 8

What is seen in CSF of MS patients?

Answer 8

INcreased protein (IgG)
Oligoclonal bands (Diagnostic)

Question 9

What is seen on MRI in MS?

Answer 9

Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) w/ destruction of neurons

Question 10

What is the treatment for MS?

Answer 10

ß-interferon
Immunosuppression
Natalizumab

Question 11

What is the autoimmune condition that destroys Schwann cells?

Answer 11

Guillain-Barre syndrome

Question 12

What is the most common variant of Guillain-Barre syndrome?

Answer 12

Acute inflammatory demyelinating polyradiculopathy

Question 13

What are the signs and symptoms of Guillan-Barre?

Answer 13

• Symmetric Ascending muscle weakness/paralysis
  
  • Starts in lower extremities
• Facial paralysis (50% of cases)
• Autonomic function

*recovery in weeks to months

Question 14

What are the laboratory findings in Guillan-Barre?

Answer 14

INcreased CSF protein w/ Normal cell count

*INcreased protein -> Papilledema

Question 15

What is Guillain-Barre associated with?

Answer 15

Campylobactor jejuni & CMV

• Autoimmune attack of peripheral myelin due to Molecular Mimicry, inoculations, & stress
  
  • NO definitive link to pathogens

Question 16

What is the treatment for Guillain-Barre?

Answer 16

• Respiratory support is critical until recovery

- Plasmapharesis, IV immune globulins

Question 17

What is Progressive multifocal leukoencephalopathy (PML)?

Answer 17

Demyelination of CNS due to destruction of oligodendrocytes

*Rapidly progressive -> Fatal

Question 18

What is PML associated with?

Answer 18

JC virus

AIDS patients (2-4%) - reactivation of latent viral infection

Question 19

What is Acute Disseminated Encephalomyelitis (AKA Subacute Sclerosing Panencephalitis)?

Answer 19

Multifocal perivenular inflammation & Demyelination after infection or certain vaccinations

Question 20

What infections can cause Acute Disseminated Encephalomyelitis (AKA Subacute Sclerosing Panencephalitis)?

Answer 20

MEASLES!!! - 5-10 years later

VZV

Question 21

What vaccinations are associated with Acute Disseminated Encephalomyelitis (AKA Subacute Sclerosing Panencephalitis)?

Answer 21

Rabies

Smallpox

Question 22

What autosomal recessive lysosomal storage diseases lead to demyelinating diseases?

Answer 22

Metachromatic Leukodystrophy

Krabbe’s disease

Question 23

What is the deficiency in Metachromatic Leukodystrophy?

Answer 23

Arylsufatase A deficiency

Question 24

How does an Arylsufatase A deficiency lead to Metachromatic Leukodystrophy?

Answer 24

Myelin cannot be degraded - Pathoma

Build-up of Sulfatides leads to impaired production of myelin sheath - FA

Question 25

What is the deficiency in Krabbe’s disease?

Answer 25

Galactocerebrosidase

Question 26

How does Krabbe’s affect myelin?

Answer 26

Buildup of Galactocerebroside (inside macrophages) destroys myelin sheath

Question 27

What is Charcot-Marie-Tooth disease?

Answer 27

AKA - Hereditary Motor & Sensory Neuropathy

• Group of Progressive hereditary nerve disorders related to the DEFECTIVE PRODUCTION OF PROTEINS involved in the structure and function of peripheral nerves or the myelin sheath