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
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
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
Phasic TonePhasic Tone
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
Needle electrodes used in Needle electrodes used in pediatric EMGpediatric EMG
Needle electrodes used in Needle electrodes used in pediatric EMGpediatric EMG
Motor NCS & Sensory NCSMotor NCS & Sensory NCS
Posterior Tibialis Motor NCS Medial Plantar Sensory NCS
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