Muscle pathology Flashcards

1
Q

What is the epimysium?

A

The fibrous sheath which surrounds the entire skeletal muscle

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2
Q

What is the endomysium?

A

The fibrous sheath which surrounds individual muscle fibres

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3
Q

What is the perimysium?

A

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

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4
Q

What are the indications for muscle biopsy?

A

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

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5
Q

What histological changes might be seen in muscular dystrophy?

A
Variability in size of muscle fibres
Endomysial fibrosis
Fatty infiltration
Increased central nuclei
Segmental necrosis
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6
Q

What are muscular dystrophies?

A

A group of inherited diseases that cause progressive weakening of muscles

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7
Q

What is the pathophysiology of muscular dystrophies?

A

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

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8
Q

When does Duchenne Muscular Dystrophy typically present?

A

2-4 years old

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9
Q

What is the pathophysiology of Duchenne muscular dystrophy?

A

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

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10
Q

What is myositis ossificans?

A

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

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11
Q

What are polymyositis and dermatomyositis?

A

Idiopathic inflammatory myopathies

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12
Q

Which gender do polymyositis and dermatomyositis affect?

A

Female:male 2:1

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13
Q

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

A

40-50

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14
Q

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

A

Malignancy, particularly dermatomyositis (15%)

Lung, breast, ovarian, stomach, colon and bladder

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15
Q

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

A

Men >45

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16
Q

What cutaneous signs may be present in dermatomyositis?

A

Gottrons sign (fingers and knuckles)
Heliotrope rash
Shawl sign

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17
Q

What other organs may be involved in polymyositis and dermatomyositis?

A

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

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18
Q

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

A

Fever
Weight loss
Raynaud’s
Non-erosive polyarthritis

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19
Q

What other diseases are polymyositis and dermatomyositis associated with?

A

Diabetes mellitus

Thyroid disease

20
Q

What drugs are development of polymyositis and dermatomyositis associated with?

A

Steroids

Statins

21
Q

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

A

Confrontational testing - direct testing of power

Isotonic testing - 30 second sit to stand test

22
Q

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

A

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

23
Q

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

A

ANA

Anti-Jo-1

24
Q

What investigations should be done to confirm polymyositis and dermatomyositis?

A

Electromyography (EMG)
Muscle biopsy
MRI

25
Q

What would be seen on electromyography in polymyositis and dermatomyositis?

A

Increased fibrillations
Abnormal motor potentials
Complex repetitive discharges

26
Q

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

A

Perivascular inflammation and muscle necrosis

27
Q

What would be seen on MRI of polymyositis and dermatomyositis?

A

Muscle inflammation, oedema, fibrosis and calcification

28
Q

What is the treatment for polymyositis and dermatomyositis?

A
Glucocorticoids
Azathioprine
Methotrexate
Ciclosporin
IV immunoglobulin
29
Q

What is inclusion body myositis?

A

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
Q

Which gender is inclusion body myositis most apparent in?

A

Males 3:1

31
Q

At what age are patients affected by inclusion body myositis?

A

> 50

32
Q

Which muscles are most commonly affected by inclusion body myositis?

A

Wrist and finger flexors in upper limbs

Quadriceps and anterior tibial muscles in legs

33
Q

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

A

Polymyositis

34
Q

What is polymyalgia rheumatica?

A

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

35
Q

At what age does polymyalgia rheumatica occur?

A

> 50

36
Q

What condition is polymyalgia rheumatica associated with?

A

Giant cell arteritis

37
Q

What are the symptoms of polymyalgia rheumatica?

A

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

38
Q

Is muscle strength affected in polymyalgia rheumatica?

A

No

39
Q

What is the treatment for polymyalgia rheumatica?

A

Low dose steroids

Gradual reduction at 18months - 2years

40
Q

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

A

Fibromyalgia

41
Q

What gender is fibromyalgia most common in?

A

Females 6:1

42
Q

What may trigger fibromyalgia?

A

Emotional or physical trauma

43
Q

Is fibromyalgia associated with inflammation?

A

No

44
Q

What are the clinical manifestations of fibromyalgia?

A

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
Q

What is the treatment for fibromyalgia?

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