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Myopathies with Proximal and Distal Weakness

Myopathy with ringed muscle fibers

• Clinical features
  • Sporadic
  • Onset: 3rd decade
  • Weakness: Proximal (quadriceps) + Ankle dorsiflexion
  • Progression: Moderate 1 to 5 years
• Lab
  • Serum CK: Normal
  • EMG: Irritable myopathy
• Muscle Pathology
  • Myopathic: Fiber size variation; Increased connective tissue; Central nuclei
  • Rings in fibers: Outer sarcoplasmic pad; Middle annular myofibrils; Center normal

Syndromes with Neuropathy & Myopathy

• Paraneoplastic (with weight loss > 15%)
  • Neuropathy: Distal; Sensory > Motor
  • Myopathy: Proximal with type II muscle fiber atrophy
• Sarcoid
• HIV Infection
  • Myopathy: Inflammatory;
  • Several types of neuropathy
• Mitochondrial: MNGIE Syndrome
• Inclusion Body Myositis
• Uremia
• Collagen vascular disorders
• Drugs & Toxins
  • Amiodarone
  • Chloroquine
  • Clofibrate: Risk factor - renal failure
  • Colchicine: Risk factor - renal failure
  • Doxorubicin
  • Eosinophilia-myalgia syndrome
  • Ethanol: Risk factor - fasting
  • Hydroxychloroquine
  • Organophosphates
  • Perhexiline
  • Vincristine