edx in floppy (hypotonic) infant
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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) - PowerPoint PPT PresentationTRANSCRIPT
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EDX in Floppy EDX in Floppy (Hypotonic) Infant(Hypotonic) Infant
M. Mohammadi MDM. Mohammadi MDProfessor, Pediatric Neurology Professor, Pediatric Neurology (TUMS)(TUMS)Children’s Medical Center HospitalChildren’s Medical Center Hospitalwww.profmohammadi.net February 2014February 2014
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Types of ToneTypes of Tone
PhasicPhasic→ → rapid contraction in response to a rapid contraction in response to a high-intensity stretch (Deep Tendon Reflexes)high-intensity stretch (Deep Tendon Reflexes)
PosturalPostural→ → the prolonged contraction of the prolonged contraction of antigravity muscles in response to the low-antigravity muscles in response to the low-intensity stretch of gravityintensity stretch of gravity
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Phasic TonePhasic Tone
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Postural TonePostural Tone
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Postural TonePostural Tone
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Gower SignGower Sign
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FasciculationsFasciculations
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Waddling GaitWaddling Gait
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MyotoniaMyotonia
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MyotoniaMyotonia
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Appearance of HypotoniaAppearance of Hypotonia
Frog legFrog leg Hand positionHand position Pectus excavatumPectus excavatum Flattening & loss of hair on occiputFlattening & loss of hair on occiput Head lag & shoulder droop in sitting positionHead lag & shoulder droop in sitting position Arthrogryposis (hypotonia in uterus)Arthrogryposis (hypotonia in uterus) CDHCDH Traction & Suspension responses Traction & Suspension responses
(vertical,Horizontal) (vertical,Horizontal)
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Phenotypic CharacteristicsPhenotypic Characteristics
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Phenotypic CharacteristicsPhenotypic Characteristics
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Some TermsSome Terms
A primary disorder of the anterior horn cell A primary disorder of the anterior horn cell body is a “body is a “neuronopathy”.neuronopathy”.
A primary disorder of the axon or its myelin A primary disorder of the axon or its myelin covering is a “covering is a “neuropathyneuropathy”.”.
A primary disorder of the muscle fiber is a A primary disorder of the muscle fiber is a ““myopathymyopathy”.”.
In infancy and childhood, cerebral disorders In infancy and childhood, cerebral disorders are far are far more common more common than motor unit than motor unit disorders (disorders (cerebral hypotoniacerebral hypotonia).).
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EDXEDX
Electrodiagnostic StudiesElectrodiagnostic Studies EMGEMG NCSNCS RSTRST F WaveF Wave H ReflexH Reflex ……....
Useful mean to screen hypotoniaUseful mean to screen hypotonia Trained, expert handsTrained, expert hands Highly Sensitive, but non-specificHighly Sensitive, but non-specific
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Surface electrodes used in Surface electrodes used in pediatric EMGpediatric EMG
Stimulating electrode
Infant Recording electrode
Child recording electrode
Disposable self adhesive electrodes
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Motor NCS & Sensory NCSMotor NCS & Sensory NCS
Posterior Tibialis Motor NCS Medial Plantar Sensory NCS
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MyotoniaMyotonia
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NMJ Disorders Myasthenia NMJ Disorders Myasthenia GravisGravis
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Algorhitmic ApproachAlgorhitmic Approach
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THANKS FOR YOUR ATTENTIONTHANKS FOR YOUR ATTENTION