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MIYOPATILER

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All show a loss of muscle fibers. Proximal more than distal ... Biceps. Triceps. Quadriceps, and. Gastrocnemius muscles Child appears to be a 'Tiny Hercules' ... – PowerPoint PPT presentation

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Title: MIYOPATILER


1
MIYOPATILER
  • Prof.Dr.Aytekin Akyüz
  • CÜ Tip Fak Nöroloji AD

2
Miyopatiler
  • Largest group of neuromuscular diseases
  • Most diverse group
  • All show a loss of muscle fibers
  • Proximal more than distal
  • No involvement of the anterior horn cell, nerve
    axon, or neuromuscular junction

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Miyopatiler
  • Includes 6 subcategories
  • 1. Endocrine Disorders
  • 2. Metabolic Disorders
  • 3. Myotonias
  • 4. Periodic Paralysis
  • 5. Polymyositis
  • 6. Muscular Dystrophy

6
Endocrine Disorders
  • Myopathies caused by some malfunction of the
    endocrine system
  • Chronic
  • Examples Addisons Disease Cushings
    Syndrome Thyrotoxic Myopathy
  • Respond to drug therapy Consists primarily of
    replacing the deficient hormones

7
Metabolic Disorders
  • Myopathies characterized by a deficiency of a
    specific enzyme resulting in muscle weakness
  • Examples
  • McArdles Disease Deficiency of the muscle
    enzyme myophosphorylase
  • Pompes Disease deficiency in Acid Maltase

8
Miyotoniler
  • Hereditary myopathies
  • Result of a chromosomal mutation
  • Characterized by Inability to relax a
    previously contracted muscle
  • Elicited by either voluntary contractions or
    some external stimuli such as percussion
  • Worsened by cold
  • Lessened by light exercise
  • Examples Myotonic Congenita (Thomsens Disease)
    Myotonia Atrophic

9
Miyotonik Distrofi (Steinerts Disease)
  • Most frequent neuromuscular disease although it
    is relatively rare
  • Clinical signs and symptoms
  • Atrophy
  • Weakness
  • Involvement of the more distal muscles such as
  • Face Neck Tongue Intrinsics of hands
    and feet

10
Miyotonik Distrofi
11
Myotonic Congenita (Thomsens Disease)
  • Children develop a characteristic hypertrophy of
    the
  • Neck
  • Deltoid
  • Biceps
  • Triceps
  • Quadriceps, and
  • Gastrocnemius muscles Child appears to be a
    Tiny Hercules

12
Miyotonia Congenita
13
Periodic Paralysis
  • Relatively rare myopathy
  • Hereditary Autosomal dominant
  • Characterized by
  • Transient flaccid paralysis or paresis affecting
    primarily the muscle of the proximal limbs
  • Attacks of weakness may last from a few seconds
    to several weeks
  • Involvement of a disruption in the serum K
    balance
  • Examples
  • Hyperkalemic Form
  • Hypokalemic Form

14
Hiperkalemik Form
  • Has increased serum K
  • Triggered by
  • Stress
  • Fasting
  • Cold
  • Rest following intensive or prolonged muscular
    exercise
  • Attacks minimized by
  • Light exercise
  • Ingestion of carbohydrates

15
Hipokalemik Form
  • Has decreased serum K
  • Affects men more than women
  • Triggered by
  • Stress
  • Fasting
  • Cold
  • Rest following intensive or prolonged muscular
    exercise
  • Alcohol consumption
  • High carbohydrate diets

16
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

17
Müsküler Distrofi
  • Largest group of the myopathies
  • Group of inherited diseases
  • Characterized by
  • Progressive muscle weakness

18
Müsküler Distrofi
  • Pseudohypertrophic Muscular Dystrophy
    (Duchennes)
  • Becker-type Muscular Dystrophy
  • Facioscapulohumeral Muscular Dystrophy
  • Limb-girdle Muscular Dystrophy

19
Duchennes Muscular Dystrophy
  • Also known as Pseudohypertrophic Muscular
    Dystrophy
  • Most common and most devastating dystrophy
  • X-linked
  • Therefore, only affects males
  • Progressive
  • Rare for patients to live to the age of 30

20
Duchennes 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
  • Govers sign

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Becker-type Muscular Dystrophy
  • More benign form of Duchennes
  • Found more in older children
  • Progresses much slower
  • Children live to reach adulthood

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Emery Dreifuss MD
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Facioscapulohumeral Muscular Dystrophy
  • Autosomal dominant
  • Involves teenagers
  • Pattern of muscle weakness in face and shoulder
    girdle

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FSHD
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Limb-girdle Muscular Dystrophy
  • Autosomal recessive
  • Affects ages 20 30
  • Pattern of muscle weakness of the proximal
    pelvic muscles and shoulder girdle

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