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EDX in Floppy (Hypotonic) Infant

EDX in Floppy (Hypotonic) Infant. M. Mohammadi MD Professor, Pediatric Neurology (TUMS) Children’s Medical Center Hospital www.profmohammadi.net February 2014. Types of Tone. Phasic → rapid contraction in response to a high-intensity stretch (Deep Tendon Reflexes)

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EDX in Floppy (Hypotonic) Infant

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  1. EDX in Floppy (Hypotonic) Infant M. Mohammadi MD Professor, Pediatric Neurology (TUMS) Children’s Medical Center Hospital www.profmohammadi.net February 2014

  2. Types of Tone Phasic→ rapid contraction in response to a high-intensity stretch (Deep Tendon Reflexes) Postural→ the prolonged contraction of antigravity muscles in response to the low-intensity stretch of gravity

  3. Phasic Tone

  4. Postural Tone www.profmohammadi.net

  5. Postural Tone www.profmohammadi.net

  6. Gower Sign www.profmohammadi.net

  7. Fasciculations www.profmohammadi.net

  8. Waddling Gait www.profmohammadi.net

  9. Myotonia www.profmohammadi.net

  10. Myotonia www.profmohammadi.net

  11. Appearance of Hypotonia • Frog leg • Hand position • Pectusexcavatum • Flattening & loss of hair on occiput • Head lag & shoulder droop in sitting position • Arthrogryposis (hypotonia in uterus) • CDH • Traction & Suspension responses (vertical,Horizontal) www.profmohammadi.net

  12. Phenotypic Characteristics www.profmohammadi.net

  13. Phenotypic Characteristics www.profmohammadi.net

  14. Some Terms • A primary disorder of the anterior horn cell body is a “neuronopathy”. • A primary disorder of the axon or its myelin covering is a “neuropathy”. • A primary disorder of the muscle fiber is a “myopathy”. • In infancy and childhood, cerebral disorders are far more common than motor unit disorders (cerebral hypotonia). www.profmohammadi.net

  15. EDX • Electrodiagnostic Studies • EMG • NCS • RST • F Wave • H Reflex • ….. • Useful mean to screen hypotonia • Trained, expert hands • Highly Sensitive, but non-specific www.profmohammadi.net

  16. Surface electrodes used in pediatric EMG Child recording electrode Infant Recording electrode Disposable self adhesive electrodes Stimulating electrode mohamadi@sina.tums.ac.ir

  17. Needle electrodes used in pediatric EMG mohamadi@sina.tums.ac.ir

  18. Needle electrodes used in pediatric EMG mohamadi@sina.tums.ac.ir

  19. Motor NCS & Sensory NCS Posterior Tibialis Motor NCS Medial Plantar Sensory NCS mohamadi@sina.tums.ac.ir

  20. mohamadi@sina.tums.ac.ir

  21. Myotonia www.profmohammadi.net

  22. NMJ Disorders Myasthenia Gravis www.profmohammadi.net

  23. Algorhitmic Approach www.profmohammadi.net

  24. THANKS FOR YOUR ATTENTION www.profmohammadi.net

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