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MUSCLAR DISEASE (MUSCLE (MITOCHONDRIAL MYOPATHIES (MELAS (muscle weakness,…

- - - - 5-10 / >50 YEARS
      - Proximal and symmetrical muscle weakness
      - Weakness progresses (W. TO M.)
      - heliotrope rash
      - Gottron's papules
    - - 20 YEARS
      - Proximal and symmetrical muscle weakness
      - Weakness progresses (W. TO M.)
      - NO SKIN
    - - 50 YEARS
      - Proximal and distal
        
        ASYMMETRICAL
      - WEAKNESS PROGRESSES (YEARS)
  - - - Infantile-onset
        
        muscle weaknes
        
        hypotonia
        
        motor delay
      - Late-onset
        
        delayed motor milestones
        
        muscle weakness
        
        hypotonia
        
        Intelligence is normal
      - adult-onset
        
        proximal muscle weakness
        
        orthopnea
        
        somnolence
        
        headaches
        
        Diminished deep tendon reflexes
  - - - exertion
    - - progressive ophthalmoplegia
      - LACTAT (SERUM, CSF)
    - - ophthalmoplegia
      - Retinitis pigmentosa
      - impaired electrical activity of the heart
    - - muscle weakness
      - tonic-clonic
      - dementia
      - STROKES
    - - myoclonic jerks
      - generalized seizures
      - cerebellar ataxia
      - dementia
  - - - distal muscles
      - Myopathic facies
    - - proximal muscles
  - - - triggers
        
        fasting
        
        rest
        
        K+
      - onset: <10
      - Proximal
      - myotonic lid lag
    - - before age 16 years
      - morning
      - exercise
      - carbohydrate meal
      - predominantly legs
      - Urinary output dec.
    - - 20-40 years
      - Hyperinsulinemia
      - carbohydrate
      - last hours to days