Guillain-Barre & ALS & MADCOW

Question 1

• Also known as acute inflammatory demyelinating polyradiculoneuropathy
• An autoimmune disorder affecting the PNS
• Most common acute peripheral neuropathy
• Most common cause of flaccid paralysis

Answer 1

Guillain-Barré Syndrome (GBS)

Question 2

Highest incidence of Guillain-Barré Syndrome (GBS) in young adults & the elderly
Occurs in

Answer 2

Men than women

Question 3

Common preceding infections of Guillain-Barré Syndrome (GBS)

Answer 3

• Campylobacter jejuni
• CMV
  Mycoplasma pneumoniae
• HIV
• EBV
• influenza

Question 4

Pathogenesis of Guillain-Barré Syndrome (GBS)

There is an autoimmune attack on myelin of the peripheral sensory & motor nerves
predominantly mediated by ____; cytoplasmic processes of macrophages penetrate the basement membrane of ___ & extend between the myelin lamellae; ___ is stripped away from the axon

Answer 4

• lymphocytes or macrophages
• Schwann cells
• myelin sheath

Question 5

Clinical Presentation
of Guillain-Barré Syndrome (GBS)

= Rapidly progressive ascending motor weakness

• ___ ascending weakness
• begins distally in the ___, migrating towards the ___
• danger of respiratory muscle paralysis & death

= Depressed or absent deep tendon reflexes in the ___
= “glove & ___” paresthesia/anesthesia
Autonomic dysfunction can be seen in severe cases

Answer 5

• symmetric
• hands & feet
• trunk
• arms & legs
• stocking

Question 6

Suspect GBS if a patient presents with:

Answer 6

• Rapid onset of muscle paralysis
• Areflexia
• Absence of fever
• GI or respiratory tract infection in the past 30 days

Question 7

Diagnosis
of GBS

Answer 7

• Lumbar puncture

- Electromyography (EMG) or nerve conduction study (NCS)

Question 8

• increased protein concentration with (or without) increased glucose & cell count
• albumino-cytologic dissociation

Answer 8

Lumbar puncture

Question 9

Prolonged distal latencies & conduction slowing

Answer 9

EMG or NCS

Question 10

to filter WBC (antibodies) out of the blood system

Answer 10

Plasmapheresis

Question 11

to neutralize antibodies

Answer 11

IV immunoglobulin (IVIG)

Question 12

Treatment in severe respiratory muscle weakness in GBS

Answer 12

Mechanical ventilation

Question 13

Fatality of GBS in 5% to 10% of cases is due to

Answer 13

respiratory paralysis

Question 14

Is often called Lou Gehrig’s disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s.

Answer 14

Amyotrophic Lateral Sclerosis
(ALS)

Question 15

ALS affects nerve cells of brain and spinal cord, mostly __and ___ that needed voluntary muscle activity.

Answer 15

upper and lower “motor neurons”

Question 16

90-95%:
5-10%:
Etiology:
Free radicals
- Mutated or misfolded ____
Higher levels of ____ which is toxic to some nerve cells.

Answer 16

• sporadic
• hereditary
• Superoxide Dismutase 1 (SOD)
• glutamate

Question 17

SIGNS AND SYMPTOMS OF ALS:

Upper motor neuron signs

Answer 17

• Increased spasticity

- Babinski’s sign

Question 18

SIGNS AND SYMPTOMS OF ALS:

Lower motor neuron signs

Answer 18

• Increasing muscle weakness (atrophy)

- Paralysis of respiratory muscles

Question 19

Other Ssx of ALS

Answer 19

• Pseudobulbar affect – involuntary laughing and crying
• No sensory changes
• Preservation of bowel and bladder function.

Question 20

Demographics of ALS:
AGE:
GENDER:

Answer 20

• 40-60 y.o
• ## men >women

Question 21

ALS is diagnose by

Answer 21

• electromyography,
• nerve conduction studies and
• muscle biopsy.

Question 22

ALS has NO cure.
Supportive and symptomatic treatment ___ (glutamate antagonist) - aimed at symptomatic relief.

Answer 22

Riluzole

Question 23

aka Bovine Spongiform Encephalopathy (BSE)
a transmissible spongiform encephalopathy (TSE) that affects cattle

Answer 23

Madcow disease

Question 24

Madcow disease is caused by

Answer 24

prions

Question 25

Prions are derived from ___, is a disease-causing form of a normal protein called cellular prion protein (PrPC)
located on the surface of CNS cells and in other tissues of the body in mammals.

Answer 25

proteinacious infectious particle

Question 26

Prions have no nucliec acid so it is resistant to nucleic acid breakdown procedures and recplicate by ___ to refold into a form called PrP scrapie (PrPSc) – named afterscrapie, the first TSE discovered. NO immune response.

Answer 26

stimulating PrPC

Question 27

Prions disrupt the normal cell process of ___ which causes a buildup of faulty proteins. Accumulations in the form of plaques, or flat areas causes degeneration of brain tissue and eventually neuronal cell death

Answer 27

protein recycling

Question 28

The destruction of neural cells causes tiny holes in the brain tissue and a sponge-like appearance under the microscope, thus giving rise to the term

Answer 28

spongiform disease

Question 29

early and prominent theory was that BSE is derived from

Answer 29

scrapie

Question 30

TRANSMISSION to CATTLE
of BSE

Answer 30

feeding ruminant-derived MBM (meat bone meal) to cattle as a protein supplement was the mechanism by which BSE was transmitted among cattle

Question 31

Pathogenesis of BSE

Answer 31

• Ingestion of pathogenic prion infected material
• GIT-lymphoid system
• ANS
• CNS

Question 32

Diagnosis of BSE

Answer 32

• Elisa

- Western Blot/Immunoblot