Approach To Floppy Infant

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
Neurology Chapter of IAP
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.

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.

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.

Scarf Sign
Put the child in a supine position and hold one
of the infant’s 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.

Causes of Floppy Infant Syndrome
1. 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.
2. Spinal cord lesions
Anterior horn cell disease – werdnig Hoffman spinal
muscular atrophy, poliomyelitis.
3. Peripheral nervous
Acute polyneuropathy, familial dysautonomia, congenital
sensory neuropathy.
4. Myoneural junction
Neonatal myasthenia gravis, infantile botulism, following
antibiotic therapy.
Causes of Floppy Infant Syndrome (Contd.)
5. Muscles
Muscular dystrophies, congenital myotonic dystrophies,
congenital myopathies (including central core disease and
nemalin myopathy), polymyositis, glycogen storage
disease (pompe’s), and arthrogryposis multiplex
congenital.
6. Miscellaneous
Protein energy malnutrition, rickets, prader willi syndrome,
malabsorption syndromes, Ehler-Danlos syndrome, cutis
laxa, cretinism.

Differentiating Features of a Floppy Infant
according to Site of Involvement
Extent of weakness
Proximal vs.
Site of involvement
Face Arms Legs distal weakness
Central - + + > or =
Anterior horn cell + ++++ ++++ > or =
Peripheral nerve - +++ +++ <
Neuromuscular +++ +++ +++ =

junction
Muscle Variable ++ + >

Differentiating Features of a Floppy Infant
according to Site of Involvement (Contd.)
Deep tendon
Site of involvement reflexes EMG Muscle biopsy
Central Normal or Normal Normal

increased
Anterior horn cell Absent Fasciculation / Denervation

fibrillation pattern
Peripheral nerve Decreased Fibrillation Denervation

pattern
Neuromuscular Normal Decremental / Normal

junction incremental
Muscle Decreased Short duration small Characteristic

amplitude potential
• 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
Look
• Electromyogram (EMG) for
• Nerve Conduction Studies
• Labs: Initial
Sepsis
• Serum electrolytes
• Serum Calcium
• Serum Glucose
• Creatine Phosphokinase (CPK)
• Toxic scan
• Blood Culture
• Lumbar Puncture with Cerebrospinal Fluid
Examination
• Thyroid Function Tests Looks
• Labs: Test as indicated Like
• Toxicology screen Sepsis
• Serum Ammonia and Venous pH without
– Serum amino acids
Sepsis
– Urine amino acids and organic acid
• Karyotype
• TORCH Virus Screening
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 Moro’s reflex, and
seizures during the neonatal period.
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.
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.
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.
Others
In polyneuritis there is symmetrical weakness of the
limbs with sensory changes. The diagnosis of Pompe’s
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.

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

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

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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Approach

• Consultant Pediatrician & Neonatologist, Ghaziabad

Floppy infant refers to those children

Neurology Chapter of IAP

The floppy infant assumes a frog legged

Neurology Chapter of IAP

The muscles appear flabby. There is

Neurology Chapter of IAP

Neurology Chapter of IAP

Neurology Chapter of IAP

Anterior horn cell + ++++ ++++ > or =

Peripheral nerve - +++ +++ <

Neuromuscular +++ +++ +++ =

Neurology Chapter of IAP

Central Normal or Normal Normal

Anterior horn cell Absent Fasciculation / Denervation

Peripheral nerve Decreased Fibrillation Denervation

Neuromuscular Normal Decremental / Normal

Muscle Decreased Short duration small Characteristic

Neurology Chapter of IAP

Neurology Chapter of IAP

• Spinal muscular atrophy emerged as the

Neurology Chapter of IAP

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