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Neurology > Myopathies > Flashcards

Myopathies Flashcards

1
Q

What are myopathies characterised by?

A

Prominent symmetrical proximal weakness and absent sensory changes

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2
Q

What are good questions to assess proximal weakness?

A

Legs: climbing stairs, stand from sit
Arms: reach above head, wash hair

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3
Q

What are the common medications causing neuropathy?

A
  • Steroids
  • Statins
  • Anti-retrovirals
  • Thyroxine
  • Fibrates
  • Cyclosporine
  • Ipecac
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4
Q

Common drugs (i.e. non-prescription) causing myopathy?

A
  • Ethanol
  • Cocaine
  • Heroin
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5
Q

What are the inherited myopathies?

A 
DYSTROPHIES:
-Myotonic dystrophy
-Facio-scapulo-humeral dystrophy
-Duchenne's dystrophy
-Becker's dystrophy
-Limb-Girdle dystrophies
-PROMM
METABOLIC: 
-McArdle's
-CPT
ION CHANNEL MUTATIONS:
-Periodic paralyses (Hypo K+, Hyper K+, paramyotonia)
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6
Q

What are the acquired myopathies?

A
  • DRUG: EtOH, statins
  • ENDOCRINE/METABOLIC: hypothyroidism, hypokalemia
  • INFLAMM: polymyositis, dermatomyositis, inclusion body myositis
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7
Q

Genetic mutation in myotonic dystrophy?

A

Unstable trinucleotide repeat in DMK gene (protein kinase) on Chr 19

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8
Q

Myotonic dystrophy inheritance pattern?

A

AD

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9
Q

Genetic mutation in Duchenne’s muscular dystrophy?

A

Loss of dystrophin gene on X chromosome

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10
Q

Ix in myopathy assessment?

A
  • Electrophysiology (needle EMG)
  • CK
  • Genetic testing
  • Muscle biopsy
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11
Q

Muscle biopsy features of muscular dystrophies?

A
  • Varying muscle fibre size
  • Regenerating and degenerating fibres
  • Increased fibrosis
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12
Q

What are the special stains used to identify muscle membrane proteins in myopathy investigations?

A
  • Dystrophin: Duchenne’s, Becker’s
  • Dysferlin: limb girdle dystrophies
  • Sarcoglycans: Limb girdle dystrophies
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13
Q

Pathophysiolgy of PM/DM?

A

Inflammatory cell infiltrate:
PM: CD8 mediated muscle necrosis found in adults
DM: B cell and CD4 immune complex mediated peri-fascicular vascular abnormalities

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14
Q

What is idiopathic inflammatory myopathy?

A

Autoimmune disease characterised by proximal muscle weakness +/- pain

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15
Q

Pathophysiology of inflammatory myopathy?

A

Muscle becomes damaged by a non-suppurative lymphocytic inflammatory process

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16
Q

How is idiopathic inflammatory myopathy characterised?

A
  • Polymyositis and dermatomyositis

- Inclusion body myositis

17
Q

Features of inclusion body myositis?

A
  • Age >50y
  • Slowly progressive
  • Suspect when pt unresponsive to Rx
  • Distal + proximal weakness
18
Q

Biopsy features of inclusion body myositis?

A
  • Vacuoles in cells on biopsy

- Biopsy positive for inclusion bodies

19
Q

What are the criteria for PM/DM?

A

Probable if 3+, definite if 4+

  1. Symmetric proximal muscle weakness
  2. Elevated muscle enzymes
  3. EMG changes
  4. Muscle biopsy
  5. Typical rash of dermatomyositis
20
Q

Typical site involvement in DM/PM?

A

Typical involvement of shoulder girdle and hip girdle

21
Q

Which enzymes are elevated indicating PM/DM?

A

Increased CK, aldolase, LDH, AST, ALT

22
Q

EMG changes of PM/DM?

A

Short polyphasic motor units, high frequency repetitive discharge, insertional irritability

23
Q

Muscle biopsy features of PM/DM?

A
  • Segmental fibre necrosis
  • Basophilic regeneration
  • Perivascular inflammation (DM)
  • Enomysial inflammation (PM)
  • Atrophy
24
Q

Signs and symptoms of PM/DM?

A
  • Progressive symmetrical proximal muscle weakness over w–> m
  • Dermatological fx of DM
  • CV: dysrhythmias, CHF, conduction defect, ventricular hypertrophy, pericarditis
  • GIT: oropharyngeal and lower oesophageal dysphagia, reflux
  • Pulmonary: weakness of resp muscles, ILD, aspiration pneumonia
25
Q

Ix in PM/DM?

A
  • Bloods: CK, ANA, anti-Jo1 (DM), anti-MI2, anti SRP
  • EMG
  • Muscle biopsy
26
Q

Rx of PM/DM?

A
  1. Non-pharm: PT/OT
  2. Pharm:
    - High dose steroids w/ slow taper
    - Immunosuppressive agents (azathioprine, methotrexate, cyclosporine)
    - IV Ig if severe or refractory
    - Hydroxychloroquine for DM rash
27
Q

What are the dermatological manifestations of DM?

A
  • Gottron’s papules
  • Gottron’s sign
  • Heliotrope rash
  • Shawl sign
  • Mechanic’s hands
  • Perinungal oedema
28
Q

What are Gottron’s papules?

A

Pink-violaceous, flat-topped papules overlying the dorsal surface of the interphalangeal joints

29
Q

What is Gottron’s sign?

A

Erythematous, smooth or scaly patches over the dorsal IPs, MCPs, elbows, knees or medial malleoli

30
Q

What is a heliotrope rash?

A

Violaceous rash over the eyelids; usually with oedema

31
Q

What is the Shawl sign?

A

Erythematous rash over neck, upper chest and shoulders

32
Q

What are Mechanic’s hands?

A

Dark, dry, thick scale on palmar and lateral surface of digits

33
Q

Which malignancies are associated with DM?

A
  • Breast
  • Lung
  • Colon
  • Ovarian

Neurology (38 decks)

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