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Neurology Chapter of IAP Dr. Arun Agrawal MD, MNAMS, FIAP, FIAMS, MIUAT (Paris), FICMCH Consultant Pediatrician & Neonatologist, Ghaziabad National Chairperson.

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Presentation on theme: "Neurology Chapter of IAP Dr. Arun Agrawal MD, MNAMS, FIAP, FIAMS, MIUAT (Paris), FICMCH Consultant Pediatrician & Neonatologist, Ghaziabad National Chairperson."— Presentation transcript:

1 Neurology Chapter of IAP 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 Approach to Floppy Infant

2 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.

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4 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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6 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.

7 Neurology Chapter of IAP 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.

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9 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.

10 Neurology Chapter of IAP 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.

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

16 Neurology Chapter of IAP Differentiating Features of a Floppy Infant according to Site of Involvement (Contd.) Site of involvement Deep tendon reflexes EMGMuscle biopsy CentralNormal or increased Normal Anterior horn cellAbsentFasciculation / fibrillation Denervation pattern Peripheral nerveDecreasedFibrillationDenervation pattern Neuromuscular junction NormalDecremental / incremental Normal MuscleDecreasedShort duration small amplitude potential Characteristic

17 Neurology Chapter of IAP 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

18 Neurology Chapter of IAP 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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36 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.

37 Neurology Chapter of IAP 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.

38 Neurology Chapter of IAP 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.

39 Neurology Chapter of IAP 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 Neurology Chapter of IAP 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.

41 Neurology Chapter of IAP Profile of Floppy Patients (n = 70) Prof. V. Kalra et.al DisorderNo.% Spinal muscular atrophy – Type I Spinal muscular atrophy – Type II Spinal muscular atrophy – Type III710.0 Diaphragmatic SMA11.4 Congenital myopathy710.0 Congenital muscular dystrophy57.1 Mitochondrial myopathy45.7 Hereditary sensory motor neuropathy34.3 Hereditary sensory autonomic neuropathy type IV22.9 Unclassified1115.7

42 Neurology Chapter of IAP 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

43 Neurology Chapter of IAP 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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