Transcript m5zn_81e1eeb9cfd8dc7x

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
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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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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.
Miscellaneous
Protein energy malnutrition, rickets, prader willi
syndrome, malabsorption syndromes, Ehler-Danlos
syndrome, cutis laxa, cretinism.
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Radiology
Head CT
Head MRI
Electromyogram (EMG)
Nerve Conduction Studies
Serum electrolytes
Serum Calcium
Serum Glucose
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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
Duchenne Muscular Dystrophy
INVOLVEMENT
• Anterior horn cells
• Poliomyelitis
• Acute transverse myelitis
• Nerve fibres
• Postinfectious polyneuropathy (GBS syndrome)
• Toxins----diphtheria, porphyria
INVOLVEMENT
• Neuromuscular junction
• Tick toxin
• Botulinum toxin
• Metabolic causes
• Periodic paralysis
• Muscular disease
• Myositis
GB Syndrome
Spinal cord
syndrome
poliomyelitis
Eitiology
Delayed
hypersesitivity.
antibody mediated
Trasverse
myelitis,spinal
cord abscess,TB
Poliovirus type
I,II,III
History
GI or URTI, 5 to
14days preceding
symptoms
Rapid progression Unimmunized,
of symptoms
URTI or GI
infection
Symptoms
Symmetric weakness
in LL gradually
ascending with
parasthesias. normal
bowel & bladder
function
Back pain.
Sensory loss
below the level of
lesion, sphincter
problems
Fever,.
meningism,
muscle
tenderness,
asymmetric
weakness
Signs
Symmetric flaccid
weakness, sensations
intact, gradually
ascending
Symmetric
areflexia,sensory
loss below the
level of lesion.,
pain, bowel and
bladder
dysfunction
Assymmetric
flaccid weakness,
sensations intact,
muscle wasting
CSF findings
Normal cell count
with raised protiens
2nd week of illness
Pleocytosis with
raised protiens
Pleocytosis with
raised protiens
EMG/NCS
Signs of denervation, Normal initially
NCS shows delayed
conduction
NCS normal,
EMG denervation
later
Course and
prognosis
Recovery in majority Depends on
within 12 months
eitiology
Permanent
disability in 1%
cases