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Floppy (Hypotonic) Infant

Floppy (Hypotonic) Infant. M. Mohammadi MD Professor, Pediatric Neurology (TUMS) Children Medical Center Hospital www.profmohammadi.net January 2013. Types of Tone. Phasic → rapid contraction in response to a high-intensity stretch (Deep Tendon Reflexes)

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Floppy (Hypotonic) Infant

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  1. Floppy (Hypotonic) Infant M. Mohammadi MD Professor, Pediatric Neurology (TUMS) Children Medical Center Hospital www.profmohammadi.net January 2013

  2. 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

  3. Phasic Tone

  4. Postural Tone www.profmohammadi.net

  5. Postural Tone www.profmohammadi.net

  6. www.profmohammadi.net

  7. Hypotonia • Hypotonia is a common symptom of neurological dysfunction and occurs in diseases of the brain, spinal cord, nerves, and muscles. • One anterior horn cell and all the muscle fibers that it innervates make up a motor unit. The motor unit is the unit of force. www.profmohammadi.net

  8. 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). www.profmohammadi.net

  9. Appearance of Hypotonia • Frog leg • Hand position • Pectusexcavatum • Flattening & loss of hair on occiput • Head lag & shoulder droop in sitting position • Arthrogryposis (hypotonia in uterus) • CDH • Traction & Suspension responses (vertical,Horizontal) www.profmohammadi.net

  10. Traction Response www.profmohammadi.net

  11. Vertical Suspension www.profmohammadi.net

  12. Horizontal Suspension www.profmohammadi.net

  13. Clues to Cerebral Hypotonia • Abnormalities of other brain functions • Dysmorphic features • Fisting of the hands • Malformations of other organs • Movement through postural reflexes • Normal or brisk tendon reflexes • Scissoring on vertical suspension www.profmohammadi.net

  14. Approach to Diagnosis • The first step, determining the disease location is in the brain, spine, or motor u • More than one site may be involved. • The brain and the peripheral nerves are concomitantly involved in some lysosomal & mitochondrial disorders. • Both brain and skeletal muscles are abnormal in infants with acid maltase deficiency & congenital myotonic dystrophy. www.profmohammadi.net

  15. Combined Cerebral and MotorUnit Hypotonia • Acid maltase deficiency • Familial dysautonomia • Giant axonal neuropathy • Hypoxic-ischemic encephalomyopathy • Infantile neuronal degeneration • Lipid storage diseases • Mitochondrial (respiratory chain) disorders • Neonatal myotonic dystrophy • Perinatal asphyxia secondary to motor unit disease www.profmohammadi.net

  16. Motor Unit Disorders withPerinatal Respiratory Distress www.profmohammadi.net • Acute infantile spinal muscular atrophy • Congenital hypomyelinating neuropathy • Congenital myotonic dystrophy • Familial infantile myasthenia • Neurogenic arthrogryposis • X-linked myotubular myopathy

  17. Clues to Motor Unit Disorders • Absent or depressed tendon reflexes • Failure of movement on postural reflexes • Fasciculations • Muscle atrophy • No abnormalities of other organs www.profmohammadi.net

  18. Fasciculations www.profmohammadi.net

  19. Muscle Atrophy www.profmohammadi.net

  20. Differential Dx of Hypotonia www.profmohammadi.net

  21. Evaluation of Motor UnitDisorders • DNA-based testing • Edrophonium chloride (Tensilon test) • Electrodiagnosis • Electromyography • Nerve conduction studies • Repetitive stimulation • Muscle biopsy • Nerve biopsy • Serum creatine kinase www.profmohammadi.net

  22. Gower Sign www.profmohammadi.net

  23. Waddling Gait www.profmohammadi.net

  24. Myotonia www.profmohammadi.net

  25. Myotonia www.profmohammadi.net

  26. Myotonia www.profmohammadi.net

  27. Phenotypic Characteristics www.profmohammadi.net

  28. Phenotypic Characteristics www.profmohammadi.net

  29. Some Words About EDX • Electrodiagnostic Studies • EMG • NCS • RST • F Wave • H Reflex • ….. • Useful mean to screen hypotonia • Trained, expert hands • Highly Sensitive, but non-specific www.profmohammadi.net

  30. Surface electrodes used in pediatric EMG Child recording electrode Infant Recording electrode Disposable self adhesive electrodes Stimulating electrode mohamadi@sina.tums.ac.ir

  31. Needle electrodes used in pediatric EMG mohamadi@sina.tums.ac.ir

  32. Needle electrodes used in pediatric EMG mohamadi@sina.tums.ac.ir

  33. Motor NCS & Sensory NCS Posterior Tibialis Motor NCS Medial Plantar Sensory NCS mohamadi@sina.tums.ac.ir

  34. mohamadi@sina.tums.ac.ir

  35. Motor studies mohamadi@sina.tums.ac.ir

  36. Algorhitmic Approach www.profmohammadi.net

  37. THANKS FOR YOUR ATTENTION www.profmohammadi.net

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