MSK path II

Question 1

What is most common form paraneopkastic caused neuropathy

Answer 1

sensorimotor neuronopathy

Question 2

paraneoplastic sensorimotor neuronopathy is most commonly assoc with what tumor

Answer 2

SCC, oat cell of lung

Question 3

What tumors secrete IgM that could demyelinate nerves

Answer 3

waldenstrom macroglobulinemia

Question 4

what other condition secretes Ig that could damage nerves

Answer 4

multiple myeloma

Question 5

where are SCC located inlungs typically

Answer 5

central

Question 6

perinuclear clearing

Answer 6

waldenstrom macroglobulinemia

Question 7

what is it called when hurts between 2nd and 3rd digit toes

Answer 7

morton neuroma

traumatic nerve injury

Question 8

What is name for hereditary motor and sensory neuropathie

Answer 8

charcot-marie-tooth disease

Question 9

most common inherited peripheral neuropathy

Answer 9

CMT

Question 10

clincal signs CMT

Answer 10

distal mm atrophy
sensory loss– lots of bruises and scrapes
foot deformities– pes cavus foot

Question 11

damage to schwanna cells is called

Answer 11

demyelinating neuropathy

Question 12

damage to axons is called what

Answer 12

axonal neuropathy

Question 13

damage to central neurons is called what

Answer 13

neuronopathy

Question 14

painless weakness in eyes

Answer 14

myasthenia gravis

Question 15

autoAb in myasthenia

Answer 15

Ab against ACh R

Question 16

what tumor is myasthenia assoc with

Answer 16

thymoma

Question 17

hassels corpuscles

Answer 17

thymic gland

Question 18

young myasthenic patient with thyroid abrnomality

Answer 18

thymic hyperplasia> thymoma

Question 19

presentation myasthenia patient with Ab against muscle specific R tyrosine kinase

Answer 19

focal muscle involvement

neck, shoulder, facial, respiratory and bulbar

Question 20

Dx myasthenia

Answer 20

Hx
PE
autoAb
electrophysiologic studies

Question 21

Tx myasthenia

Answer 21

ACHE Inhibitors

Question 22

Tx thymoma in myasthenia patient

Answer 22

thymectomy

Question 23

Lambert Eaton Myasthenic Syndrome

Answer 23

autoimmune caused by Ab that block Ach release by inhibiting presynaptic Ca channel

Question 24

clinical difference myasthenia gravis and LEMS

Answer 24

LEMS gets better with time

myasthenia gets worse with time

Question 25

why does LEMS get better with time

Answer 25

because more stimulation will get enough Ca out to cause action

Question 26

LEMS assocaited with

Answer 26

neuroendocrine carcinoma of lung-- looks like ALL in child | usually SCC

Question 27

what microbe produces botulinum toxin

Answer 27

clostridium botulinum | gram +

Question 28

curare

Answer 28

blcok ACh R and cause flaccid paralysis

Question 29

Sx botulism

Answer 29

``` double vision, blurred vision, droopy eyelids slurred speech difficulty swallowing dry mouth muscle weakness ```

Question 30

genetic defects in NMJ lead to what

Answer 30

congenital myasthenic syndromes

Question 31

characteristics of type I skel mm fibers

Answer 31

slow, high lipid content, red color from myoglobin, | high oxidative capacity (low glycolytic)

Question 32

checkerboard pattern of skel mm fibers. darker are what type

Answer 32

II

Question 33

clusters of atrophic muscle fibers

Answer 33

neurogenic

Question 34

perifascicular atrophy in muscle fibers

Answer 34

dermatomyositis

Question 35

type II mm fiber atrophy | normal type I fibers

Answer 35

prolonged corticosteroid therapy or disuse

Question 36

disorders skel muscle happen how

Answer 36

myopathic injury or disrupting the muscle innervation

Question 37

increased CK MM in blood

Answer 37

degeneration releasing cytoplasmic enzymes (part of myofiber necrosis) segmental myofiber degeneration and regneration

Question 38

when is myofiber hypertophy seen

Answer 38

physiologic adaptation to exercise or in assoc with chronic myopathic conditions

Question 39

cytoplasmic inclusions in mm

Answer 39

vacuoles, aggregates protesins, clustered organelles | all suggest myopathy

Question 40

3 main primary inflammatory myopathies

Answer 40

polymyositis, dermatomyositis and inclusion body myositis

Question 41

what immune mediated disorders can cause myositis

Answer 41

SLE, systemic sclerosis, sarcoidosis

Question 42

muscle fiber surrounded by blue cells

Answer 42

lymphocytes = inflammatory myopathy

Question 43

dermatomyositis

Answer 43

systemic autoimmune disease presents with proximal muscle weakness and skin changes (swollen hands and feet)

Question 44

what occurs in dermatomyositis that leads to muscle injury

Answer 44

vasculitis small vessels

Question 45

anti Mi2 Ab

Answer 45

Gottron papules and heliotrope rash, dermatomyositis

Question 46

anti Jo1 Ab

Answer 46

interstitial lung disease, nonerosive arthritis and skin rash called "mechanics hands" Ab against histidyl t-RNA synthetase

Question 47

anti P155/P140 Ab

Answer 47

against transcriptional regulators | assoc with paraneoplastic and juvenile dermatomyositis

Question 48

myofiber atrophy accentuated at edges of fascicles

Answer 48

perifascicular atrophy

Question 49

infiltrate rich in CD4 and C'5-9 in muscle fascicles

Answer 49

dermatomyositis

Question 50

what movements are hardest in begining of dermatomyositis

Answer 50

getting up from chair and climbing because affect proximal muscles first

Question 51

heliotrope rash

Answer 51

lilac colored discoloration of upper eyelids with periorbital edema and scaling erythematous eruption dusky red patches over knucles, elbows and knees (gottron papules)

Question 52

associated conditions with dermatomyositis

Answer 52

dysphagia from esophageal mm | interstitial lung disease which can lead to death