EDX in Floppy (Hypotonic) Infant

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EDX in Floppy (Hypotonic) Infant

• M. Mohammadi MD
• Professor, Pediatric Neurology (TUMS)
• Childrens Medical Center Hospital
• www.profmohammadi.net
• February 2014

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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

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Phasic Tone
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Postural Tone
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Postural Tone
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Gower Sign
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Fasciculations
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Waddling Gait
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Myotonia
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Myotonia
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Appearance of Hypotonia

• Frog leg
• Hand position
• Pectus excavatum
• Flattening loss of hair on occiput
• Head lag shoulder droop in sitting position
• Arthrogryposis (hypotonia in uterus)
• CDH
• Traction Suspension responses
  (vertical,Horizontal)

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Phenotypic Characteristics
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Phenotypic Characteristics
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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).

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EDX

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

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Surface electrodes used in pediatric EMG
Child recording electrode
Infant Recording electrode
Disposable self adhesive electrodes
Stimulating electrode
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Needle electrodes used in pediatric EMG
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Needle electrodes used in pediatric EMG
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Motor NCS Sensory NCS
Posterior Tibialis Motor NCS
Medial Plantar Sensory NCS
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Myotonia
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NMJ Disorders Myasthenia Gravis
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Algorhitmic Approach
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THANKS FOR YOUR ATTENTION