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Neonatal Hypotonia Clinical Approach To Floppy Baby. Osama Naga, M.D., PGY2 7/23/09. Neonatal Hypotonia. Central Causes Cerebral palsy Hypoxic ischemic encephalopathy Intracranial hemorrhage Cerebral malformations Chromosomal abnormalities (e.g.Trisomy 21, Prader-Willi syndrome)

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neonatal hypotonia
Neonatal Hypotonia
  • Central Causes
    • Cerebral palsy
    • Hypoxic ischemic encephalopathy
    • Intracranial hemorrhage
    • Cerebral malformations
    • Chromosomal abnormalities (e.g.Trisomy 21, Prader-Willi syndrome)
    • Congenital infection TORCH
    • Acquired infections
    • Peroxisomal disorders
    • Drug effects (e.g. benzodiazepines)
neonatal hypotonia3
Neonatal Hypotonia
  • Spinal cord
    • Birth trauma (especially Breech delivery)
    • Syringomyelia
neonatal hypotonia4
Neonatal Hypotonia
  • Anterior Horn Cell
    • Spinal Muscular Atrophy
    • Traumatic myelopathy
neonatal hypotonia5
Neonatal Hypotonia
  • Neuromuscular junction
    • Congenital myasthenia gravis
    • Transient acquired neonatal myasthenia
    • Infantile botulism
neonatal hypotonia6
Neonatal Hypotonia
  • Muscle
    • Muscular dystrophies (congenital myotonic dystrophy)
    • Congenital myopathies (e.g. central core disease)
neonatal hypotonia7
Neonatal Hypotonia
  • Peripheral nerves
    • Hereditary sensory motor neuropathies
      • Charcot-Marie-Tooth disease
neonatal hypotonia8
Neonatal Hypotonia
  • Metabolic myopathies
    • Acid maltase deficiency
    • Carnitine deficiency
    • Cytochrome-c-oxidase deficiency
neonatal hypotonia9
Neonatal Hypotonia
  • History
    • Any significant family history
      • Affected parents
      • Siblings
      • Consanguinity
      • Stillbirths
      • Childhood deaths
neonatal hypotonia10
Neonatal Hypotonia
  • History
    • Maternal disease
      • Diabetes
      • Epilepsy
      • Myotonic dystrophy
    • Pregnancy and delivery history
      • Drug or teratogen exposure
      • Decreased fetal movements
      • Abnormal presentation
      • Polyhydramnios/ oligohydramnios
neonatal hypotonia11
Neonatal Hypotonia
  • History
    • Apgar scores
    • Resuscitation requirements
    • Cord gases
neonatal hypotonia12
Neonatal Hypotonia
  • History
    • History since delivery
      • Respiratory effort
      • Ability to feed
      • Level of alertness
      • Level of spontaneous activity
      • Character of cry
neonatal hypotonia13
Neonatal Hypotonia
  • Identification of hypotonia
    • Holding the infant under the arms
    • The legs will be extended
    • Decreased tone of the shoulder girdle allows the infant to slip through the examiner's hands
neonatal hypotonia14
Neonatal Hypotonia
  • Identification of hypotonia
    • Holding the infant in horizontal suspension
    • The back hangs over the examiner's hand, and the limbs and head hang loosely
    • Passive extension of the legs at the knees no resistance is met
    • Pulling the infant from the supine to sitting position the head lags and continues to lag when the sitting position is reached
neonatal hypotonia15
Neonatal Hypotonia
  • Physical Examination
    • Central
      • Normal strength
      • Normal or increased DTRs
      • May be Seizure
      • May be dysmorphic features
neonatal hypotonia16
Neonatal Hypotonia
  • Physical Examination
    • Anterior horn cells
      • Generalized weakness
      • Decreased/ absent DTRs
      • Fasciculations
      • Often described as alert
neonatal hypotonia17
Neonatal Hypotonia
  • Physical Examination
    • Nerve
      • Weakness, distal>proximal
      • Decreased/ Absent DTRs
      • +/- fasciculations
neonatal hypotonia18
Neonatal Hypotonia
  • Physical Examination
    • Neuromuscular Junction
      • Weakness, face/ eyes/ bulbar
      • Normal DTRs
      • No fasciculations
neonatal hypotonia19
Neonatal Hypotonia
  • Physical Examination
    • Muscles
      • Weakness, proximal>distal
      • Decreased DTRs
neonatal hypotonia20
Neonatal Hypotonia
  • Physical Examination
    • Clues and Pitfalls
      • Profound central hypotonia may have absent DTR
      • Absent DTR in the first few DOL would not rule out a central cause for the hypotonia
neonatal hypotonia21
Neonatal Hypotonia
  • Physical Examination
    • Clues and Pitfalls
      • Presence of profound weakness and hypotonia suggest:
        • Disorder of the lower motor neuron
          • A sign of this may be a weak cry
      • Weakness is uncommon in central hypotonia except in the acute stages
neonatal hypotonia22
Neonatal Hypotonia
  • Physical Examination
    • Clues and Pitfalls
      • Arthrogryposis (the fixation of joints at birth)
        • Associated with:
          • Neonatal hypotonia
          • More commonly with lower motor neuron unit
          • Multisystem abnormalities
neonatal hypotonia23
Neonatal Hypotonia
  • Physical Examination
    • Clues
      • Hepatosplenomegaly
        • Storage disorders
        • Congenital infections
      • Renal cysts
      • High forehead
      • Wide fontanelles
        • Zellweger’s syndrome
neonatal hypotonia24
Neonatal Hypotonia
  • Physical Examination
    • Clues
      • Abnormal odor
        • Metabolic disorders
      • Hypopigmentation, undesceded testes
        • Prader Willi
      • Hepatomegaly
      • Retinitis pigmentosa
        • Neonatal adrenoleukodystrophy
neonatal hypotonia25
Neonatal Hypotonia
  • Physical Examination
    • Clues
      • Examination of the mother
        • Congenital myotonic dystrophy
        • Myasthenia gravis
neonatal hypotonia26
Neonatal Hypotonia
  • Investigation
    • History and examination
      • Hypotonia and a degree of  strength
        • Central cause is most likely
      • Hypotonic and weak
        • Peripheral cause is possible
          • Early review by the neurology service is warranted
neonatal hypotonia27
Neonatal Hypotonia
  • Investigation
    • Central Causes
      • Neuroimaging
        • Ultrasound scan in the first instance
        • MRI for structural abnormality
        • EEG: if seizures suspected
neonatal hypotonia28
Neonatal Hypotonia
  • Investigation
    • Central Causes
      • Genetics review if any dysmorphic features present
      • Karyotype (if dysmorphic features)
      • TORCH screen
      • DNA methylation studies or FISH for Prader-Willi syndrome (if clinically indicated after a genetics review)
      • Metabolic work up
neonatal hypotonia29
Neonatal Hypotonia
  • Investigation
    • Peripheral causes
      • Neurology services review
      • Molecular genetics – CTG repeats, deletions in SMN gene
neonatal hypotonia30
Neonatal Hypotonia
  • Investigation
    • Peripheral causes
      • Creatine kinase: If elevated in an early sample, repeat after a few days.
      • Nerve conduction studies
      • Muscle biopsy
        • Depending on clinical situation, may be delayed until around 6 months of age as neonatal results are difficult to interpret
references
References
  • 1-Fenichel GM. Neonatal Neurology 3rd edition. Churchill Livingston Inc. 1990
  • 2-Paro-Panjan D, Neubauer D. Congenital hypotonia: is there an algorithm? Journal of Child Neurology; Jun2004, Vol.19 (6): 439-43
  • 3-Prasad AN, Prasad C. The floppy infant: contribution of genetic and metabolic disorders. Brain and Development; Oct 2003, Vol.25(7): 457-76