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.

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Presentation transcript:

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.

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.

Radiology Head CT Head MRI Electromyogram (EMG) Nerve Conduction Studies Serum electrolytes Serum Calcium Serum Glucose

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 SyndromeSpinal cord syndrome poliomyelitis EitiologyDelayed hypersesitivity. antibody mediated Trasverse myelitis,spinal cord abscess,TB Poliovirus type I,II,III HistoryGI or URTI, 5 to 14days preceding symptoms Rapid progression of symptoms Unimmunized, URTI or GI infection SymptomsSymmetric 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

SignsSymmetric 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 findingsNormal cell count with raised protiens 2nd week of illness Pleocytosis with raised protiens EMG/NCSSigns of denervation, NCS shows delayed conduction Normal initiallyNCS normal, EMG denervation later Course and prognosis Recovery in majority within 12 months Depends on eitiology Permanent disability in 1% cases