,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Neonatal Hypotonia,Clinical Approach To Floppy Baby,Osama Naga,M.D.,PGY2,7/23/09,Neonatal HypotoniaClinical Ap,1,Neonatal Hypotonia,Central Causes,Cerebral palsy,Hypoxic ischemic encephalopathy,Intracranial hemorrhage,Cerebral malformations,Chromosomal abnormalities(e.g.Trisomy 21,Prader-Willi syndrome),Congenital infection TORCH,Acquired infections,Peroxisomal disorders,Drug effects(e.g.benzodiazepines),Neonatal HypotoniaCentral Caus,2,Neonatal Hypotonia,Spinal cord,Birth trauma(especially Breech delivery),Syringomyelia,Neonatal HypotoniaSpinal cord,3,Neonatal Hypotonia,Anterior Horn Cell,Spinal Muscular Atrophy,Traumatic myelopathy,Neonatal HypotoniaAnterior Hor,4,Neonatal Hypotonia,Neuromuscular junction,Congenital myasthenia gravis,Transient acquired neonatal myasthenia,Infantile botulism,Neonatal HypotoniaNeuromuscula,5,Neonatal Hypotonia,Muscle,Muscular dystrophies(congenital myotonic dystrophy),Congenital myopathies(e.g.central core disease),Neonatal HypotoniaMuscle,6,Neonatal Hypotonia,Peripheral nerves,Hereditary sensory motor neuropathies,Charcot-Marie-Tooth disease,Neonatal HypotoniaPeripheral n,7,Neonatal Hypotonia,Metabolic myopathies,Acid maltase deficiency,Carnitine deficiency,Cytochrome-,c,-oxidase deficiency,Neonatal HypotoniaMetabolic my,8,Neonatal Hypotonia,History,Any significant family history,Affected parents,Siblings,Consanguinity,Stillbirths,Childhood deaths,Neonatal HypotoniaHistory,9,Neonatal Hypotonia,History,Maternal disease,Diabetes,Epilepsy,Myotonic dystrophy,Pregnancy and delivery history,Drug or teratogen exposure,Decreased fetal movements,Abnormal presentation,Polyhydramnios/oligohydramnios,Neonatal HypotoniaHistory,10,Neonatal Hypotonia,History,Apgar scores,Resuscitation requirements,Cord gases,Neonatal HypotoniaHistory,11,Neonatal Hypotonia,History,History since delivery,Respiratory effort,Ability to feed,Level of alertness,Level of spontaneous activity,Character of cry,Neonatal HypotoniaHistory,12,Neonatal Hypotonia,Identification of hypotonia,Holding the infant under the arms,The legs will be extended,Decreased tone of the shoulder girdle allows the infant to slip through the examiners hands,Neonatal HypotoniaIdentificati,13,Neonatal Hypotonia,Identification of hypotonia,Holding the infant in horizontal suspension,The back hangs over the examiners hand,and the limbs and head hang loosely,Passive extension of the legs at the knees no resistance is met,Pulling the infant from the supine to sitting position the head lags and continues to lag when the sitting position is reached,Neonatal HypotoniaIdentificati,14,Neonatal Hypotonia,Physical Examination,Central,Normal strength,Normal or increased DTRs,May be Seizure,May be dysmorphic features,Neonatal HypotoniaPhysical Exa,15,Neonatal Hypotonia,Physical Examination,Anterior horn cells,Generalized weakness,Decreased/absent DTRs,Fasciculations,Often described as alert,Neonatal HypotoniaPhysical Exa,16,Neonatal Hypotonia,Physical Examination,Nerve,Weakness,distalproximal,Decreased/Absent DTRs,+/-fasciculations,Neonatal HypotoniaPhysical Exa,17,Neonatal Hypotonia,Physical Examination,Neuromuscular Junction,Weakness,face/eyes/bulbar,Normal DTRs,No fasciculations,Neonatal HypotoniaPhysical Exa,18,Neonatal Hypotonia,Physical Examination,Muscles,Weakness,proximaldistal,Decreased DTRs,Neonatal HypotoniaPhysical Exa,19,Neonatal Hypotonia,Physical Examination,Clues and Pitfalls,Profound central hypotonia may have absent DTR,Absent DTR in the first few DOL would not rule out a central cause for the hypotonia,Neonatal HypotoniaPhysical Exa,20,Neonatal Hypotonia,Physical Examination,Clues and Pitfalls,Presence of profound weakness and hypotonia suggest:,Disorder of the lower motor neuron,A sign of this may be a weak cry,Weakness is uncommon in central hypotonia except in the acute stages,Neonatal HypotoniaPhysical Exa,21,Neonatal Hypotonia,Physical Examination,Clues and Pitfalls,Arthrogryposis(the fixation of joints at birth),Associated with:,Neonatal hypotonia,More commonly with lower motor neuron unit,Multisystem abnormalities,Neonatal HypotoniaPhysical Exa,22,Neonatal Hypotonia,Physical Examination,Clues,Hepatosplenomegaly,Storage disorders,Congenital infections,Renal cysts,High forehead,Wide fontanelles,Zellwegers syndrome,Neonatal HypotoniaPhysical Exa,23,Neonatal Hypotonia,Physical Examination,Clues,Abnormal odor,Metabolic disorders,Hypopigmentation,undesceded testes,Prader Willi,Hepatomegaly,Retinitis pigmentosa,Neonatal adrenoleukodystrophy,Neonatal HypotoniaPhysical Exa,24,Neonatal Hypotonia,Physical Examination,Clues,Examination of the mother,Congenital myotonic dystrophy,Myasthenia gravis,Neonatal HypotoniaPhysical Exa,25,Neonatal Hypotonia,Investigation,History and examination,Hypotonia and a degree of strength,Central cause is most likely,Hypotonic and weak,Peripheral cause is possible,Early review by the neuro