Hertz CIS

Question 1

muscle biopsy- what do we take for proximal muscle weakness?

Answer 1

quadriceps. Can get away with it, plus that’s what you use to get up out of a chair– a proximal muscle likely to show a myositis.

Look for inflammatory infiltrate

Save some for EM to check for inclusion diseases

Question 2

nerve biopsy, what do we take?

Answer 2

sural nerve; only sensory, not motor, so we won’t see motor deficits as a result

Question 3

see onion bulb on MRI. What does this mean?

Answer 3

evidence of demyelination, should biopsy

person could be complaining of foot drop, weakness –> suggestive of Guillain Barre

Question 4

what would we find on spinal tap of guillain barre guy?

Answer 4

glucose– this is normal
protein– this is abnormal (evidence of inflammation)
no pleocytosis– this is normal

Question 5

muscle biopsy shows loss of checkerboarding of muscle fibers

Answer 5

this is not an inflammation myopathy

Could be neuropathy or steroid myopathy

Question 6

pt comes in with neuropathy, skinny little calves and pes cavus (high arch)

Answer 6

Charcot Marie Tooth
can be very variable in its penetrance

Peroneal atrophy

Question 7

foot ulcers
peripheral neuropathy

what is this?

Answer 7

stocking glove pattern of neuropathy

DIABETES

Question 8

What causes a burning neuropathy?

Answer 8

vincristine

Question 9

about 40% of diabetics will show up in your office with this additional complaint

Answer 9

autonomic problems (bladder dysfunction, etc.)

Question 10

name a neurofibromatosis that causes carpal tunnel

Answer 10

Dupuytren’s

Question 11

guy comes in with super fat calves. What else might we find?

Answer 11

Gower’s sign- using arms to help get up from the ground

biopsy showing pseudohypertrophy (lots of fat instead of muscle)

This is Duchenne MS

Becker is the “light” version– you have some dystrophin, just not as much as usual

Question 12

What do most people with MS die of?

Answer 12

heart failure

Question 13

gal with periorbital redness. What is this?

Answer 13

heliotrope rash

she also has “mechanic’s hands–” Gottron papules

this is dermatomyositis. Muscle biopsy shows perifascicular inflammation (lymphocytes)- one focal area, spreads out from there

Question 14

gal comes in with ptosis. The longer we ask her to look up, the worse it is. After 10 second rest her eyes can’t look up at all. What is this?

Answer 14

Myasthenia gravis.

If the 10-second rest had improved symptoms, it would be Lambert-Eaton.

Give acetylcholinesterase inhibitor to the MG pt and it would get better

Question 15

what might we find on chest x-ray of myasthenia gravis pt?

Answer 15

thymoma

Question 16

what might we find on CXR of lambert eaton syndrome pt?

Answer 16

lung mass

small cell

Question 17

thymoma vs thymic hyperplasia in MG

Answer 17

take out the thymoma- see improvement

take out thymic hyperplasia– some better, some not. not so great.

Question 18

Lambert Eaton- take out the small cell cancer, do they improve?

Answer 18

sometimes

Question 19

a serum electrophoresis shows gamma spike. What is this?

Answer 19

monoclonal gammopathy

x-ray shows lesions all up and down the spine.–> take a biopsy and see plasma cells

= Multiple myeloma. a lytic lesion (punched out holes all over the place)

Question 20

Kid has weird x-ray (fuzzy in extremities), bowing of legs, funny looking teeth, blue sclera

Answer 20

Osteogenesis imperfecta

usually type I

3 and 4 don’t tend to have dentiginous imperfecta

Defect is type 1 collagen

Additional trouble with hearing loss

Question 21

dexa scan

Answer 21

osteopenia- -1.0 to - 2.5

osteoporosis- -2.5 and less

Question 22

what 3 scans do we do for bone loss?

Answer 22

spine, femoral head, and wrist

Question 23

guy has funny xray (homogeneity of the hip bones) and biopsy shows mosaic pattern. What’s up?

Answer 23

paget’s

Not usually a problem but can get osteosarcoma