Myasthenia gravis and Inflammatory myopathy

Question 1

Myasthenia gravis clinical presentation?

Answer 1

muscles commonly affected are extraocular and bulbar (mouth and throat, swallow) muscles
-neck and head, facial expression, altered speech or dysphagia and may progress into the limbs

muscle function improves with rest, worse with stress

Question 2

Myasthenia gravis thymus?

Answer 2

hyperplasia and thyoma may be seen with this

Question 3

Mysathenia gravis auto-antibody?

Answer 3

acetylcholinereceptors

Question 4

Lambert-Eaton syndrome?

Answer 4

muscle weakness associated with cancer
symptoms start with proximal muscle weakness of leg
autoimmune attacks prevent presynaptic release of acetylcholne as antibiotics attack the voltage gated calcium channels

Question 5

Cancer associated with Lambert-Eaton?

Answer 5

Small cell lung cancer (SCLC)

often have a history of smoking

Question 6

What else may Lambert-Eaton affect?

Answer 6

Autonomics
-cause dry mouth, constipation, etc
thymus appears normal

Question 7

Treat Lambert-Eaton?

Answer 7

Plasmaphoresis, Prednisone, azathiaprine, IVIG and others

must attend to the underlying malignancy too

Question 8

Symptoms of Lamber-Eaton?

Answer 8

weakness or loss of movement that can be more or less severe, including:
-difficulty chew, climb stairs, lift objects, talking, drooping head, need to used hands to get up
-swallowing difficult, gagging, choking
blury vision, double vision, problems with steady gaze

Question 9

Polymyositis/ Dermatomyositis?

Answer 9

idiopathic inflammatory myopathies
lymphocyctic infiltrate of skeletal muscle
patients present with symmetrical weakness in the proximal muscles of the upper and lower extremities

Question 10

Immunologic features of Polymyositis/ Dermatomyositis?

Answer 10

Antinuclear antibody (ANA) and other autoantibody
CD8+ T cells and macrophage infiltrates
production of proinflammatory and cytotoxic cytokines
immune complexes and complement may contribute

Question 11

Idiopathic inflammatory myopathies?

Answer 11

are rare diseases, polymorphism in HLA genes may contribute
subset of patients whose disease is triggered by infectious agents
associated/specific autoantibody, may include ANA

Question 12

most common myositis-specific autoantibody?

Answer 12

antisynthetase autoantibody, Jo-1

only 20-30% patients have, if have is conclusive

Question 13

Grotton’s papules?

Answer 13

violet colored inflammation over the nuckles in Polymyositis/ Dermatomyositis

Question 14

Dematomyositis rash?

Answer 14

violaceous rash, more often seen in women

on the face, different from SLE

Question 15

other characteristic skin chanes with dermatomyositis?

Answer 15

linear erythema
scalp rash
V-like sign
shawl sign
Mechanic's hands

Question 16

Mechanic’s hands?

Answer 16

skin changes on lateral side of fingers

scaling, fishers on the radial side`

Question 17

Lung probles with PM/DM? (Polymyositis/ Dermatomyositis?)

Answer 17

intersitial lung disease, a symmetric non-erosive arthritis of the hands and feet, associated with malignancy

Question 18

Diagnosis of Polymyositis/ Dermatomyositis?

Answer 18

Proximal muscle weakness
evidence of myositis from biopsy
elevated enzymes in blood (ck, ast, alt, aldolase, LDH)
EMG findings of myopathy
charcteristic rash for DM

Question 19

Treatment of DM?

Answer 19

corticosteroids

Question 20

Associated cancer with Idiopathic inflammatory myopathies Polymyositis/ Dermatomyositis?

Answer 20

Lymphoma or solid tumors may occur 20-25%

Question 21

Treatment of Myasthenia gravis?

Answer 21

Anti-cholinesterase, steroids, plasmapheresis

Question 22

Proximal muscle weakness with ptosis, altered speech, dysphagia, improves with rest?

Answer 22

Myasthenia gravis

Question 23

Proximal muscle weakness, dry mouth, cancer (SCLC) associated with smokeing?

Answer 23

Lambert-Eaton

Question 24

Symmetrical proximal muscle weakness, difficult walking upstairs, standing, skin disease in DM?

Answer 24

Idiopathic Inflammatory Myopathies