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 Syndrome1. 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 lesionsAnterior horn cell disease – werdnig Hoffman spinal muscular atrophy, poliomyelitis.

3. Peripheral nervousAcute polyneuropathy, familial dysautonomia, congenital sensory neuropathy.

4. Myoneural junctionNeonatal 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 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 of symptoms

Unimmunized, 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, NCS shows delayed conduction

Normal initially NCS normal, EMG denervation later

Course and prognosis

Recovery in majority within 12 months

Depends on eitiology

Permanent disability in 1% cases