Transcript Pediatric Painless EMG

EDX in Floppy
(Hypotonic) Infant
M. Mohammadi MD
Professor, Pediatric Neurology
(TUMS)
Children’s Medical Center Hospital
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February 2014
Types of Tone
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Phasic→ rapid contraction in response to a
high-intensity stretch (Deep Tendon Reflexes)
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Postural→ the prolonged contraction of
antigravity muscles in response to the lowintensity stretch of gravity
Phasic Tone
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
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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
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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
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Electrodiagnostic Studies
 EMG
 NCS
 RST
 F Wave
 H Reflex
 …..
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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
Posterior Tibialis Motor NCS
& Sensory NCS
Medial Plantar Sensory NCS
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Myotonia
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NMJ Disorders Myasthenia
Gravis
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Algorhitmic Approach
Floppy Infant
Central
Imaging
MRI/MRS
Normal-essential
hypotonia/delayed
myelination
Peripheral
Syndromic
Cerebral
dysgenesis
Chromosome
studies
EMG/NCS/CK
Dennervation
SMA
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Myopathic
RNSdecreamentcongenital
myastenia
Muscle Biopsy
AchR ,MuSK ab
THANKS FOR YOUR ATTENTION
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