Miyastenia gravis prof. Dr. Aytekin Akyüz


Polimiyozit Second most common myopathy in adults



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Polimiyozit

  • Second most common myopathy in adults

  • Chronic inflammatory condition of striated muscle

  • Skin involved 50% of time – In this case its called Dermatomyositis

  • Insidious onset

  • Moderately progressive

  • Clinical signs:

    • Muscle weakness
    • Fatigue
    • Flexors more than extensors
    • Difficulty swallowing
    • Joint pain
    • Mild fever
    • Weight loss
    • Very diffuse erythema of face and neck


Müsküler Distrofi

  • Largest group of the myopathies

  • Group of inherited diseases

  • Characterized by:

  • Progressive muscle weakness



Müsküler Distrofi

  • Pseudohypertrophic Muscular Dystrophy (Duchenne’s)

  • – Becker-type Muscular Dystrophy

  • – Facioscapulohumeral Muscular Dystrophy

  • – Limb-girdle Muscular Dystrophy



Duchenne’s Muscular Dystrophy

  • Also known as Pseudohypertrophic Muscular Dystrophy

  • Most common and most devastating dystrophy

  • X-linked

  • Progressive

  • Rare for patients to live to the age of 30



Duchenne’s MD

  • Clinical signs and symptoms:

    • Marked elevation in serum Creatine Kinase
    • Psuedohypertrophy of the calves
    • Tightness of the achilles
    • Hyperlordosis in the low back
    • Progressive atrophy and weakness of the pelvis and LEs
    • Gover’s sign




Becker-type Muscular Dystrophy

  • More benign form of Duchenne’s

  • Found more in older children

  • Progresses much slower

  • Children live to reach adulthood



Emery Dreifuss MD



Facioscapulohumeral Muscular Dystrophy

  • Autosomal dominant

  • Involves teenagers

  • Pattern of muscle weakness in face and shoulder girdle



FSHD



Limb-girdle Muscular Dystrophy

  • Autosomal recessive

  • Affects ages 20 – 30

  • Pattern of muscle weakness of the proximal pelvic muscles and shoulder girdle



Neuromuscular Junction Disorders

  • Defect in the transmission of the neurochemical signal across the neuromuscular junction

  • Defect can take form of a problem in:

    • Release of acetylcholine (Ach)
    • Uptake of Ach,
    • Insufficient receptor sites at the junction


Neuromuscular Junction Disorders

  • Categorized as pre-synaptic or post-synaptic problem

  • Examples:

    • Myasthenia Gravis
      • Post-synaptic receptor site disorder
    • Myasthenia Syndrome
    • Lambert-Eaton Syndrome
    • Botulism
      • Toxin prevents pre-synaptic release of Ach


Clinical picture:

  • Clinical picture:

  • Pronounced, rapid fatigue

  • Associated muscle weakness





    • DEFINITION disorders caused by cancer but not due to metastatic disease or non-metastatic mechanisms
    • IMPORTANCE
    • early detection of cancer
  • save $$ in work-up







  • DIAGNOSIS

      • MAY DEVELOP BEFORE TUMOR Dx
      • HIGH INDEX OF SUSPICION
  • Dx OF EXCLUSION

      • SPECIFIC TESTS MAY BE HELPFUL
      • NEUROIMAGING
      • CSF STUDIES
      • ELECTROPHYSIOLOGY
      • PET SCAN




  • PNS OF THE NERVOUS SYSTEM

  • CENTRAL NERVOUS SYSTEM

  • PERIPHERAL NERVOUS SYSTEM

  • NEUROMUSCULAR JUNCTION & MUSCLE

















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