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Dr. Arun Agrawal

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Approach to Floppy Infant Dr. Arun Agrawal MD, MNAMS, FIAP, FIAMS, MIUAT (Paris), FICMCH Consultant Pediatrician & Neonatologist, Ghaziabad National Chairperson ... – PowerPoint PPT presentation

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Title: Dr. Arun Agrawal


1
Approach to Floppy Infant
  • Dr. Arun Agrawal
  • MD, MNAMS, FIAP, FIAMS, MIUAT (Paris), FICMCH
  • Consultant Pediatrician Neonatologist,
    Ghaziabad
  • National Chairperson Neurology Chapter of IAP
  • Honorary Professor of Pediatrics ICMCH
  • National Convener Community Pediatrics,
    Chapter of IAP
  • National Vice President IAP 2004

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Floppy Infant
  • Floppy infant refers to those children presenting
    with generalized hypotonia, most often arising
    out of an insult incurred during fetal or
    neonatal period.

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Posture
  • The floppy infant assumes a frog legged position.
    On ventral suspension, the baby can not maintain
    limb posture against gravity and assumes the
    position of a rag doll.

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Movements
  • The muscles appear flabby. There is diminished
    resistance to passive movement of the limbs and
    the range of movement of the peripheral joints is
    increased.

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Scarf Sign
  • Put the child in a supine position and hold one
    of the infants hands. Try to put it around the
    neck as far as possible around the opposite
    shoulder. Observe how far the elbow goes across
    the body. In a floppy infant, the elbow easily
    crosses the midline.
  • Pull to sit
  • When pulled up from the supine to the sitting
    position, the head of the baby lags.

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Causes of Floppy Infant Syndrome
  • Central nervous system
  • Perinatal asphyxia, neonatal, encephalopathy,
    kernicterus, cerebral palsy (atonic type),
    intracranial hemorrhage, chromosomal anomalies
    including down syndrome and inborn errors of
    metabolism e.g., aminocidurias,
    mucopolysaccharidosis and cerebral lipidosis.
  • Spinal cord lesions
  • Anterior horn cell disease werdnig Hoffman
    spinal muscular atrophy, poliomyelitis.
  • Peripheral nervous
  • Acute polyneuropathy, familial dysautonomia,
    congenital sensory neuropathy.
  • Myoneural junction
  • Neonatal myasthenia gravis, infantile botulism,
    following antibiotic therapy.

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Causes of Floppy Infant Syndrome (Contd.)
  • Muscles
  • Muscular dystrophies, congenital myotonic
    dystrophies, congenital myopathies (including
    central core disease and nemalin myopathy),
    polymyositis, glycogen storage disease (pompes),
    and arthrogryposis multiplex congenital.
  • Miscellaneous
  • Protein energy malnutrition, rickets, prader
    willi syndrome, malabsorption syndromes,
    Ehler-Danlos syndrome, cutis laxa, cretinism.

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Differentiating Features of a Floppy Infant
according to Site of Involvement
Site of involvement Extent of weakness Extent of weakness Extent of weakness Proximal vs. distal weakness
Site of involvement Face Arms Legs Proximal vs. distal weakness
Central - gt or
Anterior horn cell gt or
Peripheral nerve - lt
Neuromuscular junction
Muscle Variable gt
16
Differentiating Features of a Floppy Infant
according to Site of Involvement (Contd.)
Site of involvement Deep tendon reflexes EMG Muscle biopsy
Central Normal or increased Normal Normal
Anterior horn cell Absent Fasciculation / fibrillation Denervation pattern
Peripheral nerve Decreased Fibrillation Denervation pattern
Neuromuscular junction Normal Decremental / incremental Normal
Muscle Decreased Short duration small amplitude potential Characteristic
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  • Signs Perform complete physical examination
  • Infant with decreased muscle tone
  • Exam distinguishes site of disorder
  • Upper motor neuron lesion
  • Lower motor neuron lesion
  • Radiology
  • Head CT
  • Head MRI
  • Diagnostic Studies
  • Electromyogram (EMG)
  • Nerve Conduction Studies
  • Labs Initial
  • Serum electrolytes
  • Serum Calcium
  • Serum Glucose

Look for Sepsis
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  • Creatine Phosphokinase (CPK)
  • Toxic scan
  • Blood Culture
  • Lumbar Puncture with Cerebrospinal Fluid
    Examination
  • Thyroid Function Tests
  • Labs Test as indicated
  • Toxicology screen
  • Serum Ammonia and Venous pH
  • Serum amino acids
  • Urine amino acids and organic acid
  • Karyotype
  • TORCH Virus Screening

Looks Like Sepsis without Sepsis
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Common causes of floppy infant
  • Cerebral Palsy
  • Many hypotonic children due to causes in central
    nervous system are mentally retarded. In atonic
    or hypotonic cerebral palsy, reflexes are brisk
    in spite of generalized flaccidity. Floppy infant
    due to cerebral causes is associated with
    lethargy, poor feeding, and lack of alertness,
    poor Moros reflex, and seizures during the
    neonatal period.

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Werdnig Hoffman disease
  • It is characterized by marked hypotonia, sluggish
    fetal movement, and fasciculation of tongue. The
    child is alert. Feeding behaviour and cry are
    poor. Deep tendon reflexes are absent. Muscle
    biopsy shows neurogenic type of atrophy or that
    the muscle spindles are atrophied in groups.
    Disease is inherited as an autosomal may be
    available. Death occurs by 2-4 years of age.

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Myasthenia gravis
  • Mmyasthenia gravis may occur in about 12 percent
    of the babies born to mothers with the disease.
    It is characterized by marked hypotonia, pooling
    of oral secretions, poor feeding, feeble cry and
    generalized muscle weakness appearing within 2-3
    days after the birth. Baby is alert. Facial
    weakness manifests by mark-like facies, open
    mouth and staring look. External opthalmoplegia
    and ptosis are rare. Deep tendon reflexes are
    normal. The prognosis is substantiated by
    improvement in the muscle functions following
    intramuscular injection of edrophonium chloride 1
    mg or neostigmine methyl sulfate 0.1 mg. the
    condition lasts for 3 to 4 weeks. The child is
    treated with neostigmine methyl sulphate 0.1 to
    0.5 mg IM 10 minutes before each feel for 1 or 2
    days followed by neostigmine bromide, 1 to 4 mg
    orally half an hour before each feed.

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Congenital myopathies
  • These are rare inherited disorders resulting in a
    benign congenital hypotonia, with generally good
    outlook for normal life span. Nemaline myopathy
    is the most common variant. Other disorders of
    this group include the central core disease,
    myotubular myopathy and congenital fiber type
    disproportion.

40
Others
  • In polyneuritis there is symmetrical weakness of
    the limbs with sensory changes. The diagnosis of
    Pompes disease is suspected when the child has
    macroglossia, cardiomegaly and generalized
    hypotonia. Babies with prader-willi syndrome are
    mentally retarded and obese deep tendon reflexes
    are diminished. Diabetes mellitus occurs later in
    life. Testes may be undescended. Ehlers-danlos
    syndrome is characterized by hyperelasticity of
    the skin, hyperflexibility of joints and extreme,
    fragility of skin. Wound healing is delayed and
    there are frelly movable subcutaneous nodules. In
    cutis laxa, the child has loose skin hanging in
    baggy folds.

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Profile of Floppy Patients (n 70) Prof. V.
Kalra et.al. 2001
Disorder No.
Spinal muscular atrophy Type I 13 18.6
Spinal muscular atrophy Type II 17 24.3
Spinal muscular atrophy Type III 7 10.0
Diaphragmatic SMA 1 1.4
Congenital myopathy 7 10.0
Congenital muscular dystrophy 5 7.1
Mitochondrial myopathy 4 5.7
Hereditary sensory motor neuropathy 3 4.3
Hereditary sensory autonomic neuropathy type IV 2 2.9
Unclassified 11 15.7
42
Key Messages of this Study
  • Spinal muscular atrophy emerged as the commonest
    cause of floppy children followed by congenital
    muscle disease.
  • 11 of the cases still remained unclassified
    despite sophisticated investigative techniques.
  • EMG was a good screening modality for floppy
    children.
  • A low gene deletion rate (50) was observed in
    our phenotype

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Key Messages in Approach to a Floppy Child
  • First ABC of resuscitation
  • Try to find out cause but again simple clinical
    examination is the first thing
  • Any sedative drug given during labour
  • Investigations
  • Only those investigations which are necessary
  • Sepsis
  • Sepsis without sepsis
  • Another sophisticated investigations
  • Improve the quality of life probably quantity can
    not be improve in most of the cases

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