Obj. 6: Demyelinating, Myopathic, NM Disorders - SK

Question 1

A 35 year-old female Oregonian presents for EPISODES of fatigue, LE numbness/tingling, muscle spasms and weakness, diplopia, and urinary incontinence. After a few days, her symptoms will disappear and will not return for months. A few of the episodes correlated with viral infections. What do you suspect?

Answer 1

MS

Question 2

Briefly, what is the etiology of MS? / What age group does it most commonly affect? / Climate it is most common in?

Answer 2

Areas of demyelination/gliosis in the white matter, spinal cord, or optic nerve / UNDER age 55 (most commonly young adults) / Temperate

Question 3

In most MS pts, there is a period of months to yrs that separates the initial episode from the next relapse. What is the name for this pattern? / As relapses become more frequent and remission becomes less complete, what is the name for this pattern?

Answer 3

Relapsing-Remitting Pattern / Secondary Progressive

Question 4

In some MS patients, their initial symptoms become progressively worse (with NO remission) and disability develops early on in the disease course. What is this pattern called? / Is it typical of MS?

Answer 4

Primary progressive disease / NO - in most MS patients, the disease begins in relapsing-remitting pattern –> secondary progressive disease over time

Question 5

If an MS patient asks you how pregnancy will affect her symptoms, how do you respond?

Answer 5

MS relapses DECREASE during pregnancy, however, relapses INCREASE after delivery/during post-partum period

Question 6

For MS, what type of imaging do you want to use for diagnosis? / What must be present?

Answer 6

MRI / Lesions in 2 or more areas of central white matter.

Question 7

Despite the fact that there is nothing that can be done to stop the progression of MS, 10 years after diagnosis, ~_____% of MS patients are without severe disability.

Answer 7

50%

Question 8

What medication would you use to treat an acute MS relapse? / Would you use this long term?

Answer 8

Corticosteroid / NO

Question 9

What would you put someone on to reduce the frequency of MS exacerbations?

Answer 9

Beta-interferon

Question 10

A 55 YOM presents w/ R eyelid drooping, difficulty chewing & swallowing, double vision, trouble breathing, and extremity weakness. Pupillary responses, sensation & reflexes are intact. Sx intensity changes throughout the day. What are you thinking?

Answer 10

Myasthenia Gravis

Question 11

If the 55 YOM does have myasthenia gravis, based on his demographic, what else should you be considering?

Answer 11

Thymoma

Question 12

Besides a thymoma, what other conditions are often associated with myasthenia gravis? / for women, when might exacerbations occur?

Answer 12

Thyrotoxicosis, rheumatoid arthritis, lupus / before period & during or after pregnancy

Question 13

Etiology of myasthenia gravis?

Answer 13

Autoimmune antibodies bind to acetylcholine receptors, reducing the # of functioning receptors; cranial nerves often involved

Question 14

Course of myasthenia gravis?

Answer 14

Progressive disease that can be fatal d/t respiratory complications like aspiration pneumonia

Question 15

For myasthenia gravis - 1) What imaging should be done? / 2) What lab value might be elevated?

Answer 15

1) CT to look for thymoma / 2) Elevated acetylcholine receptor antibodies

Question 16

What med should be given to myasthenia gravis patients? / does it alter the dz course?

Answer 16

Anticholinesterase drugs / no - taken for symptoms

Question 17

What is the most common progressive motor neuron disease?

Answer 17

ALS - a mixed upper and lower motor neuron deficit

Question 18

Whereas MS typically progresses slowly / episodically, ALS causes death in _____ yrs

Answer 18

3-5

Question 19

Between MS and ALS, which more frequently causes incontinence?

Answer 19

MS; ALS usually spares sphincters.

Question 20

A 40 YOM pt presents with difficulty swallowing, chewing, coughing, breathing, and talking. The pt has also noticed limb weakness, stiffness, wasting, and fasciculations. Electromyography shows denervation in the cervical, thoracic, and lumbar spine. Acetylcholine receptor antibody levels are normal and the condition is not episodic. What do you think?

Answer 20

ALS.

Question 21

What med helps slow the progression of ALS? / What meds help symptoms?

Answer 21

Riluzole / Anticholinergic drugs

Question 22

Name the x-linked disorder that presents btw age 1 and 5 and leads to progressive muscle weakness and wasting. Mental impairments might be present. / What lab value is likely elevated?

Answer 22

Duchenne Muscular Dystrophy / Creatine Kinase

Question 23

With muscular dystrophy, weakness tends to present in a characteristic pattern. What is that pattern?

Answer 23

Pelvic –> Shoulder Girdle –> Limb/Respiratory

Question 24

Duchenne Muscular Dystrophy prognosis? / What med can you use to improve muscle strength?

Answer 24

Rapid progression; death in ~15 years of onset / Prednisone

Question 25

A 49 YOM presents w/ difficulty swallowing. On exam, you note a drooping palate, depressed gag reflex, saliva pooling in his pharynx, and a wasted/fasciculating tongue. What’s going on?

Answer 25

Progressive Bulbar Palsy

Question 26

What does Progressive Bulbar Palsy affect?

Answer 26

Motor nuclei of Cranial Nerves