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Flashcards in Muscle pathology Deck (45):
1

What is the epimysium?

The fibrous sheath which surrounds the entire skeletal muscle

2

What is the endomysium?

The fibrous sheath which surrounds individual muscle fibres

3

What is the perimysium?

The fibrous sheath which surrounds groups of muscle fibres to create a fascicle

4

What are the indications for muscle biopsy?

Evidence of muscle disease - weakness, atrophy, raised CK
Presence of neuropathy
Presence of vascular disorder

5

What histological changes might be seen in muscular dystrophy?

Variability in size of muscle fibres
Endomysial fibrosis
Fatty infiltration
Increased central nuclei
Segmental necrosis

6

What are muscular dystrophies?

A group of inherited diseases that cause progressive weakening of muscles

7

What is the pathophysiology of muscular dystrophies?

There is destruction of single muscle fibres in the muscles, which are then regenerated
This causes fibrosis of the muscles

8

When does Duchenne Muscular Dystrophy typically present?

2-4 years old

9

What is the pathophysiology of Duchenne muscular dystrophy?

There is a mutation of the dystrophin gene on the long arm of chromosome X
Actin fibres don't adhere properly to the basement membrane, leaving fibres liable to tearing
There is uncontrolled Ca2+ entry into cells

10

What is myositis ossificans?

A condition where heterotopic ossification (bone forming outside the skeleton) occurs in muscles usually after an injury

11

What are polymyositis and dermatomyositis?

Idiopathic inflammatory myopathies

12

Which gender do polymyositis and dermatomyositis affect?

Female:male 2:1

13

What is the peak age of incidence in polymyositis and dermatomyositis?

40-50

14

What are patients with polymyositis and dermatomyositis at increased risk of?

Malignancy, particularly dermatomyositis (15%)
Lung, breast, ovarian, stomach, colon and bladder

15

Which patients with polymyositis and dermatomyositis in particular are at risk of malignancy?

Men >45

16

What cutaneous signs may be present in dermatomyositis?

Gottrons sign (fingers and knuckles)
Heliotrope rash
Shawl sign

17

What other organs may be involved in polymyositis and dermatomyositis?

Lungs: respiratory muscle weakness and interstitial lung disease, breathlessness, cough
Heart: myocarditis
Oesophagus: dysphagia

18

What general systemic symptoms may be present in polymyositis and dermatomyositis?

Fever
Weight loss
Raynaud's
Non-erosive polyarthritis

19

What other diseases are polymyositis and dermatomyositis associated with?

Diabetes mellitus
Thyroid disease

20

What drugs are development of polymyositis and dermatomyositis associated with?

Steroids
Statins

21

What tests on examination can be done to investigate polymyositis and dermatomyositis?

Confrontational testing - direct testing of power
Isotonic testing - 30 second sit to stand test

22

What blood tests should be done to investigate polymyositis and dermatomyositis?

Creatine kinase
Inflammatory markers
Electrolytes, calcium, PTH, TSH to exclude other causes

23

What auto-antibodies may be present in polymyositis and dermatomyositis?

ANA
Anti-Jo-1

24

What investigations should be done to confirm polymyositis and dermatomyositis?

Electromyography (EMG)
Muscle biopsy
MRI

25

What would be seen on electromyography in polymyositis and dermatomyositis?

Increased fibrillations
Abnormal motor potentials
Complex repetitive discharges

26

What would be seen in muscle biopsy in polymyositis and dermatomyositis?

Perivascular inflammation and muscle necrosis

27

What would be seen on MRI of polymyositis and dermatomyositis?

Muscle inflammation, oedema, fibrosis and calcification

28

What is the treatment for polymyositis and dermatomyositis?

Glucocorticoids
Azathioprine
Methotrexate
Ciclosporin
IV immunoglobulin

29

What is inclusion body myositis?

An inflammatory muscle disease, characterized by slowly progressive weakness and wasting of mostly distal, but some proximal muscles, most apparent in the muscles of the arms and legs

30

Which gender is inclusion body myositis most apparent in?

Males 3:1

31

At what age are patients affected by inclusion body myositis?

>50

32

Which muscles are most commonly affected by inclusion body myositis?

Wrist and finger flexors in upper limbs
Quadriceps and anterior tibial muscles in legs

33

Which has higher levels of CK: polymyositis or inclusion body myositis?

Polymyositis

34

What is polymyalgia rheumatica?

Polymyalgia rheumatica (PMR) is a condition that causes pain, stiffness and inflammation in the muscles around the shoulders, neck and hips

35

At what age does polymyalgia rheumatica occur?

>50

36

What condition is polymyalgia rheumatica associated with?

Giant cell arteritis

37

What are the symptoms of polymyalgia rheumatica?

Ache in shoulder and hip girdle
Morning stiffness
Fatigue, anorexia, weight loss and fever
Reduced movement of shoulders, neck and hips

38

Is muscle strength affected in polymyalgia rheumatica?

No

39

What is the treatment for polymyalgia rheumatica?

Low dose steroids
Gradual reduction at 18months - 2years

40

What is the commonest cause of musculoskeletal pain in women aged 22-50 years?

Fibromyalgia

41

What gender is fibromyalgia most common in?

Females 6:1

42

What may trigger fibromyalgia?

Emotional or physical trauma

43

Is fibromyalgia associated with inflammation?

No

44

What are the clinical manifestations of fibromyalgia?

Chronic pain: neck, shoulders, lower back, chest wall
Varies in intensity
Symptoms worse with exertion, fatigue and stress
Sensation of swelling
Fatigue and poor, unrefreshing sleep
Pins and needles/tingling, headaches, depression, abdominal pain (IBS), poor concentration and memory

45

What is the treatment for fibromyalgia?

Patient education
Graded exercise programme
Cognitive behavioural therapy
Complementary medicine eg. acupuncture
Antidepressants
Analgesia e.g. tramadol
Gabapentin and pregabalin