Neuromuscular Diseases-Weakness

Question 1

potassium imblanance levles

Answer 1

less than 2.5 or greater than 7

Question 2

calcium imbalances

Answer 2

less htan 7, greater than 12

Question 3

what is usually abnormal in myasthenia gravis patients

Answer 3

thymus

Question 4

myasthenia gravis is associated with what other diseases

Answer 4

thyroid disease
lupus
pernicious anemia
rheumatoid arthritis

Question 5

in addition to MG testing what should you also look for

Answer 5

thymoma

Question 6

differential for myasthenia gravis includes…

Answer 6

ALS
muscular dystrophy
Labert Eaton
Botulism 
organophosphate poisoning

Question 7

treatment for MG

Answer 7

pyridostigmine, neostigmine, plasmapheresis, IV IgG, surgery and immunosuppresive drugs

Question 8

what are the immunosuppresive drugs used for treatment of MG

Answer 8

steroids
axathioprine
cyclophosphamide
cyclosporin

Question 9

neonates with myasthenia present with

Answer 9

weak cry, suck, respiratory insuffiency

Question 10

neonatal myasthenia is due to

Answer 10

maternal antibodies

Question 11

are mothers affected in congenital myasthenia

Answer 11

no

Question 12

patients with congenital myasthenia presen with

Answer 12

opthalmoplegia
recessive inheritance suggested
no antibodies
synaptic dysfunction

Question 13

drugs associated with drug induced myasthenia gravis

Answer 13

D penicillamine
phenytoic
trimethadione
streptomycin
kanamycin
colistin
tetracycline
neomycin

Question 14

is associated with cancers especially small cell lung, breast and lymphoma

Answer 14

labert eaton syndrome

Question 15

myasthenic syndrome

Answer 15

presynpatic inhibition (voltage dependent calcium channel

Question 16

features of myasthetic syndrome

Answer 16

areflexia
weakness
dry mouth
metallic taste
autonomic signs

Question 17

how do you diagnose lambert eaton syndrome

Answer 17

H and P and EMG

Question 18

treatment of Lambert Eaton syndrome

Answer 18

IV IgG
3,4 diaminopyridine
guanidine
treat the tumor if it is present

Question 19

presynaptic inhibition of acetylcholine release

Answer 19

botulism

Question 20

what are the features of botulism

Answer 20

progresses 2-4 days, weakness, ophthalmoplegia, respiratory paralysis, facial diplegia, pupillary paralysis and dry mouth

Question 21

differential diagnosis of botulism

Answer 21

myasthenia gravis
Guianne barre
diptheria

Question 22

how do you treat botulism

Answer 22

antitoxin but usually it is just supportive because you run out of time

Question 23

potassium associated paralysis is precipitated by

Answer 23

hunger
food
cold

Question 24

featues of myopathy

Answer 24

proximal weakness of upper and lower extremities
difficulty arising out of a chair
difficulty climbing stairs
difficulty lifting arms over head

Question 25

type if genetics DMD

Answer 25

x linked recessive

Question 26

symptoms usually begin before the age of three

Answer 26

DMD

Question 27

toe walking and waddling gait

Answer 27

DMD

Question 28

gowers sign

Answer 28

DMD

Question 29

are there sphincter distrubances in DMD

Answer 29

NO

Question 30

pseudohypertroph of gactrocnemius, triceps and biceps muscles

Answer 30

DMD

Question 31

diagnosis of DMD

Answer 31

H and P Labs- CPK/LDH muscle biopsy EMG

Question 32

facial and shoulder muscle weakness that appears in adolescne

Answer 32

facioscapulohumeral muscular dystropy

Question 33

beevor sign

Answer 33

facioscapulohumeral muscular dystrophy

Question 34

increased lumbar lordosis

Answer 34

facioscapulohumeral muscular dystrophy

Question 35

winged scapula

Answer 35

FCH MD

Question 36

steinert disease associated with

Answer 36

chromosome 19

Question 37

what is the defect in steinert disease

Answer 37

chromosome 19

Question 38

stiffness in cold

Answer 38

Myotonic dystrophy

Question 39

hypersomnolecen, cataracts, gonadal atrophy, frontal balding and myotonia,

Answer 39

MD

Question 40

hatchet face and wasting of hands

Answer 40

MD

Question 41

how do you treat the myotonia

Answer 41

phenytoic quinidine procainamide

Question 42

myotonic congenita

Answer 42

autosomal dominant or recessive; generalized myotonia which is more severe than myotonic dystrophy and patients have muscular apperance

Question 43

mitochondrial cytopathies are characterized by

Answer 43

lactic acidosis ragged red fibers lipid droplets

Question 44

spasms due to spontaneous firing of peripheral nerves that causes muscle contraction

Answer 44

tetany

Question 45

____is associated with hypocalcemia, hypomagnesemia, and alkalosis

Answer 45

tetany

Question 46

what are the two "signs" asscoiated with tetany

Answer 46

troussaeu and chvyostek signs

Question 47

prolonged spasms of the limb muscles, seizure, curcumoral paresthesias, and laryngospasm

Answer 47

tetany

Question 48

sudden forceful, often painful muscle contractions

Answer 48

cramps

Question 49

causes of cramps

Answer 49

``` vigours exercise pregnancy LMN hypothyroidism uremia myotonic disorders sweatiing diarrrhea McArdle Disease Hemodialysis ```

Question 50

treatment of cramps

Answer 50

``` stretching quinine phenytoic diazepam procainamide carbamazepine ```

Question 51

begins with a rash of face, eyelids, extensor surfaces, knucles, elbows and knees and proximal weakness

Answer 51

dermatomyositis

Question 52

10% of dermatomyositis over the age of 40 are associated with

Answer 52

lung | breast and GI cancers

Question 53

when diagnosing dermatomyositis what should you carefully look fore

Answer 53

tumor!

Question 54

inflammatory myopathy from any cause

Answer 54

polymyositis

Question 55

polymositis usually occurs when

Answer 55

after age of 35

Question 56

are the EOM involved in polymyositis

Answer 56

NO!!!

Question 57

treatment for polymyositis

Answer 57

steroids and supportive

Question 58

what is a very common cause of myopathy

Answer 58

cholesterol agents

Question 59

what are some common causes for myopathy

Answer 59

``` collagen vascular disease HIV L tyrptophan Niacin Cholesteral agents ethanol endorcine (thyroid) ```

Question 60

increased CPK is associated with hypothyroid or hyperthyroid

Answer 60

hypothyroid

Question 61

type II glycogen storage disease

Answer 61

acid maltase deficiency, cardiomyopathy, myopathy, and infancy

Question 62

type V-McArdle's disease

Answer 62

myophosphorylase deficiency, cramps, intolerance, to intense exercise and myoglobuinuria