Large Group 3 -Clark

Question 1

What are some tests you can do to help dx Myasthenia Gravis?

Answer 1

• ACh receptor antibody test
• Tensillon test
• Ice pack test (ptosis resolves w/ice pack if MG)
• EMG

Question 2

What should you check for and how in a pt w/MG?

Answer 2

Thymoma using a chest CT

Question 3

What is the tx for a myasthenia crisis?

Answer 3

• ABCs
• IVIG or plasma exchange
• ACh esterase inhibitors
• +/- steroids

Question 4

What is the tx for maintenance of MG?

Answer 4

• ACh esterase inhibitors
• Steroids
• Thymectomy

Question 5

64 yo male presents w/soft and dysarthric speech, left leg and B/L UE atrophy, scattered muscle weakness, hyperreflexia, positive Babinski, normal sensation, normal CNs and inappropriate affect. What does he have?

Answer 5

Amyotrophic Lateral Sclerosis (ALS)

Question 6

What is a fasciculation?

Answer 6

Small, involuntary muscle twitch

Question 7

What is the tx for symptoms of ALS?

Answer 7

• Riluzole (glutamate transmission modulator; delays progression by ~ 2 months) to block the presynaptic release of glutamate
• dextromethorphan and quinidine for the pseudo bulbar affect
• Supportive/symptomatic care

Question 8

What other neurologic condition can be associated w/ALS?

Answer 8

Frontotemporal dementia (hence pseudo bulbar affect)

Question 9

38 yo male presents to clinic for evaluation for diplopia. Presents w/nystagmus, clumsiness of left arm. States that 2 weeks ago his right leg felt week, but it resolved after a few weeks. What does he have?

Answer 9

Multiple Sclerosis (MS)

Question 10

What is the tx for MS?

Answer 10

1000 mg IV Solumedrol for 3 days +/- Prednisone taper

Question 11

Impaired removal of what from synapses plays a role in ALS?

Answer 11

Glutamate

causes excitotoxic injury

Question 12

What is Myasthenia Gravis?

Answer 12

Autoimmune disorder with a postjunctional defect of Acetylcholine receptor

Question 13

Who is at risk for MG?

Answer 13

Young women and old men

Question 14

What are some sxs of MG?

Answer 14

Fluctuating weakness, fatigue in cranial, limb or trunk muscles (usually involve the eye, ptosis, diplopia, blurred vision)

Question 15

What is Lambert-Eaton Myasthenic Syndrome (LEMS)?

Answer 15

Rare paraneoplastic or autoimmune disease resulting from antibodies directed against presynaptic junction

Question 16

What are some sxs of LEMS?

Answer 16

Chronic fluctuating weakness of limbs, especially the legs (cranial nerve muscles spared)

Question 17

What is the tx for LEMS?

Answer 17

• Sxs may resolve after cancer (if there is one) is treated

- Pyridostigmine may improve sx

Question 18

What are some sxs of Botulism?

Answer 18

• Rapid onset of ocular sxs (diplopia, ptosis, blurred vision) and bulbar sx (dysarthria, dysphagia)
• Descending paralysis

Question 19

What is the most common motor neuron disease in adults?

Answer 19

ALS

Question 20

What are the UMN and LMN signs seen in ALS?

Answer 20

UMN: stiffness, spasticity, clumsiness, hyperactive reflexes, + Babinski

LMN: weakness, wasting, fasciculations, decreased or absent reflexes

NO loss of EOM or B/B function

Question 21

What will an EMG with repetitive stimulation of motor nerve show in MG?

LEMS?

Answer 21

MG: decremental response

LEMS: incremental response