MS/MG & ALS

Question 1

Define MS

Answer 1

1. inflammatory demyelinating disease of CNS: Brain, SC, and optic nerve
2. White and Grey matter

Question 2

What gender is MS MC in? Age range?

Answer 2

Females

20-40 y.o.

Question 3

MS etiology

Answer 3

1. Autoimmune: HLA alleles
2. Vitamin D deficiency
3. Identical twins association

Question 4

Define optic neuritis

Answer 4

• Loss of vision in affected eye and pain with eye movement

- Phosphenes: Circular “stars”

Question 5

Define acute transverse myelitis

Answer 5

Acute partial loss of:

1. Motor
2. Sensory
3. Autonomic
4. Reflex

Question 6

Lhermittes Sign

Answer 6

“Barber shop” test

Neck flexion causes lightning-shock type pain radiation from spine down leg

Question 7

What are the MC MS sx’s?

Answer 7

1. Paresthesia, Trigeminal neuralgia
2. Optic neuritis
3. Acute Transverse myelitis
4. Fatigue
5. Autonomic SC sx: Bowel (constipation), bladder dysfunction
6. Heat intolerance
7. Ataxia

Question 8

Uhthoff’s phenomen

Answer 8

Worsening symptoms with heat intolerance

Question 9

Neuro PEx findings in MS

Answer 9

1. Spasticity and Positive Babinski. (toes move up)

2. Ataxic gait

Question 10

Eye PEx findings in MS

Answer 10

1. Interneuclear Ophthlmoplegia

2. Nystagmus

Question 11

What is the imaging of choice to help confirm and monitor the disease progression of MS?

Answer 11

MRI of brain and SC WITH Gadolinum

Question 12

LP findings in CSF suggestive of MS

Answer 12

1. Oligoclonal bodies

2. Immunoglobulin G (IgG)

Question 13

MS diagnostic criteria

Answer 13

Dissemination in time and space

Question 14

Management of acute MS exacerbations?

Answer 14

High dose corticosteroids (IV Methylprednisone)

Question 15

First line treatment for RRMS to reduce frequency and severity of relapse?

Answer 15

B-Interferon or Glatiramer acetate

Question 16

What helps with fatigue in MS?

Answer 16

Amantadine and Modafinil

Question 17

Define Myasthenia Gravis

Answer 17

Rare autoimmune disorder=antibodies against Ach postsynaptic receptor @ neuromuscular junction= Decreased Ach receptors= Progressive SKELETAL MUSCLE weakness

Question 18

Who is MG MC in? Age?

Answer 18

Young Females= 30’s

Question 19

MG Clinical Manifestations

Answer 19

Fluctuating, fatiguable muscle weakness

1. Ocular weakness: Diplopia and ptosis
2. Generalized muscle weakness: Bulbar and respiratory

Question 20

What is NORMAL in MG that. helps differentiate it from other diseases?

Answer 20

1. Normal Sensory

2. Normal DTRs

Question 21

MG PEx

Answer 21

1. Myasthenic snarl: Bilateral facial muscle weakness

2. Recovery after rest

Question 22

What is the most reliable test for diagnosing MG?

Answer 22

anti-acetylcholine receptor antibody test

Question 23

Why would you want to order a CT scan in MG?

Answer 23

Rule out Thymoma or thymus abnormality

Question 24

What is first line treatment in MG?

Answer 24

Anti-cholinesterase inhibitors

Question 25

List Anti-cholinesterase inhibitors

Answer 25

1. Pyridostigmine | 2. Neostigmine

Question 26

List immunosuppression therapy in MG and indication

Answer 26

1. IV Immune Globulin (IVIg): in myasthenia crisis for rapid response 2. Plasmapheresis 3. Prednisone: Short and Long term benefit

Question 27

What is the major complication of myasthenia gravis

Answer 27

Myasthenia crisis= respiratory failure

Question 28

Define ALS

Answer 28

Mixed UMN and LMN neurodegenerative disease: Progressive motor degeneration

Question 29

What is another name for ALS?

Answer 29

Lou Gehrig's disease

Question 30

UMN sx's

Answer 30

1. Spasticity in LE 2. Stiffness 3. Hyperreflexia

Question 31

LMN sx's

Answer 31

1. Progressive bilateral fasciculations 2. Muscle atrophy 3. Muscle weakness 4. Hyporeflexia

Question 32

What dysfunction do we not see in ALS?

Answer 32

No dysfunction of: 1. Cognitive/behavioral 2. Sensory 3. Autonomic

Question 33

What movements are spared in ALS?

Answer 33

1. Urinary sphincter | 2. Voluntary eye movements

Question 34

What are common first sx's?

Answer 34

1. Drop foot 2. Walking 3. Speech 4. Dexterity

Question 35

Bulbar sx's in ALS

Answer 35

1. Dysphagia | 2. Dysarthria

Question 36

Later stages in ALS

Answer 36

1. Swallowing-Aspiration pneumonia | 2. Intercostal-Poor lung function

Question 37

ALS treatment

Answer 37

Riluzole- Reduce progression by 2-3 months

Question 38

Riluzole MOA

Answer 38

Inhibits presynaptic release of Glutamate