Muscle pathology

Question 1

Thymic hyperplasia

Thymoma

Answer 1

associations of Myasthenia Gravis

Question 2

Pathology of Myasthenia gravis

Answer 2

autoantibodies attach post synaptic AChRs

Question 3

Diagnostics for Myasthenia Gravis

Answer 3

AChR antibody test
Edrophonium test (rapid improvement after administration of edrophonium

Question 4

Fluctuating weakness (worse on exertion)
vision problems
Bulbar muscle weakness
Proximal limb weakness
Respiratory muscle weakness
Normal reflexes

Answer 4

Clinical presentation of Myasthenia Gravis

Question 5

Neuroendocrine carcinoma of the lung
Thyroid Disease
Vitiligo

Answer 5

Associations of Lambert Eaton Syndrome

Question 6

Pathology of Lambert Eaton Syndrome

Answer 6

Autoantibodies attack presynaptic calcium channels

Question 7

Diagnostics for Lambert Eaton Syndrome

Answer 7

antibodies agianst voltage gated calcium channels

Question 8

Exercise improves strength
Autonomic Dysfunction
Extremity weakness
Reduced or absent reflexes

Answer 8

Clinical Presentation of Lambert Eaton Syndrome

Question 9

How does C. botulinum cause botulism

Answer 9

neurotoxin blocks the release of ACh from presynaptic nerve terminals by cleaving SNARE protein

Question 10

canned foods

honey

Answer 10

associations of Botulism

Question 11

flaccid descending paralysis
Mydriasis
Dysphagia
Dysarthria
Xerostomia
NVD then constipation

Answer 11

Clinical presentation of Botulism

Question 12

Major difference between Dermatomyositis and Polymyositis

Answer 12

Dermatomyositis has cutaneous involvement

Question 13

Pathology of Dermatomyositis

Answer 13

Deposition of CD4+ T cells and MAC complex in the capillary vessels of skeletal muscle

Question 14

Pathology of Polymyositis

Answer 14

CD8+ T cells attack skeletal muscle antigens, mostly endomysium

Question 15

Which diagnostic of dermatomyositis yields the worst prognosis?

Answer 15

Anti M2( helicase)

Question 16

Anti-M2
Anti-Jo1
Anti-P155/P140

Answer 16

Diagnostics suggesting Dermatomyositis

Question 17

Increased CK, Aldolase, myoglobin, ESR/CRP, Anti-Jo1

Presence of ANA

Answer 17

Diagnostics suggestive of Polymyositis

Question 18

Morphology of Dermatomyositis

Answer 18

perifascular atrophy of muscle fibers

perimyseal inflammation

Question 19

Morphology of Polymyositis

Answer 19

Endomysial inflammation with CD8+ T cells

Question 20

Pathologic difference in Duchenne vs Becker Muscular dystrophy

Answer 20

Duchenne= mutation causing total absence of dystrophin
Becker= mutation causing reduced dystrophin

Question 21

Diagnostic suggestive of Duchenne Muscular Dystrophy

Answer 21

elevated CK in first decade, then drops due to decrease in muscle mass

Question 22

Differentiate the onset of Duchenne Muscular Dystrophy vs Becker Muscular Dystrophy

Answer 22

Duchenne= normal at birth and appears to slow down with developlment
Becker= sx present in late childhood or adulthood

Question 23

Differentiate the life expectancy of Duchenne Muscular Dystrophy vs Becker Muscular Dystrophy

Answer 23

Duchenne= death before 30 y/o
Becker= Normal Life Expectancy

Question 24

Which muscular dysdtophy presents with cardiac abnormalities

Answer 24

Duchenne

Question 25

Inheritance of Muscular Dystrophy

Answer 25

X linked

Question 26

Mode of inheritance of Myotonic Dystrophy

Answer 26

Autosomal Recessive

Question 27

Pathology of Myotonic Dystrophy

Answer 27

Expansions of CTG triplet repeats at the 3' noncoding region of the DNPK gene on chromosome 19

Question 28

which set of fibers undergo atrophy in myotonic dystrophy

Answer 28

type 1

Question 29

Clinical features of myotonic dystrophy

Answer 29

``` muscle weakness/ wasting myalgias myotonia cataracts frontal balding testicular atrophy arrhythmias ```

Question 30

Pathology of Malignant Hyperthermia

Answer 30

inhales halogens cause RYR1 gene mutations, leading to increased efflux from SR

Question 31

What causes fibromyalgia

Answer 31

abnormal ascending and descending pain pathways lead to central amplification of pain signals causes widespread musculoskeletal pain without evidence of inflammation or increase in muscle enzymes

Question 32

what is used to diagnose fibromyalgia

Answer 32

11/18 tender points

Question 33

``` pain primarily where muscles and tendons attach to bones HA fatigue morning stiffness unrefreshing sleep cognitive dysfunction paresthesias ANS dysfunction ```

Answer 33

Clincal findings of fibromyalgia

Question 34

treatment for fibromyalgias

Answer 34

TCA | SNRI

Question 35

Northern european descent Giant Cell arteritis HLA-DR4

Answer 35

associations of Polymyalgia Rheumatica

Question 36

Diagnostics used to diagnose Polymyalgia Rheumatic

Answer 36

``` Elevated inflammatory markers Leukocytosis Normochormic anemia Normal CK Negative Rheumatoid Factor ```

Question 37

``` Fever Weight loss Night sweats Fatigue Malaise Morning stiffness in neck, shoulder, and pelvic girdle subjective weakness ```

Answer 37

Clinical findings of polymyalgia rheumatica

Question 38

Toxic myopathies with elevated CK

Answer 38

Statin use | Hypothyroidism

Question 39

Which toxic myopathy yields rhabdomyolysis, renal failure and myoglobinuria

Answer 39

EtOH

Question 40

Explain channelopathies

Answer 40

mutations in channel proteins cause decreased or increased excitability hyper/hypotonia leads to varied potassium levels and periodic paralysis