EDX in Floppy (Hypotonic) Infant

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

www.profmohammadi.net

Gower SignGower Sign


FasciculationsFasciculations


Waddling GaitWaddling Gait


MyotoniaMyotonia


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)


Phenotypic CharacteristicsPhenotypic Characteristics


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).).


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


[email protected]

Surface electrodes used in Surface electrodes used in pediatric EMGpediatric EMG

Stimulating electrode

Infant Recording electrode

Child recording electrode

Disposable self adhesive electrodes

[email protected]

Needle electrodes used in Needle electrodes used in pediatric EMGpediatric EMG

[email protected]

Motor NCS & Sensory NCSMotor NCS & Sensory NCS

Posterior Tibialis Motor NCS Medial Plantar Sensory NCS

[email protected]

MyotoniaMyotonia


NMJ Disorders Myasthenia NMJ Disorders Myasthenia GravisGravis


Algorhitmic ApproachAlgorhitmic Approach



THANKS FOR YOUR ATTENTIONTHANKS FOR YOUR ATTENTION

EDX in Floppy (Hypotonic) Infant

Documents

Transcript of EDX in Floppy (Hypotonic) Infant