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n CME : Your SA Journal of CPD - Clinical approach to the floppy child : main topic

Volume 22, Issue 8
  • ISSN : 0256-2170
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Abstract

When evaluating a floppy infant, the first goal should be to distinguish whether the disorder is central or peripheral in origin. <br>Hypotonia with weakness suggests a lower motor neuron lesion, whereas weakness is uncommon in disorders affecting the upper motor neuron except during the acute stage. <br>Evaluation of tone and power should be delayed in the unwell, nutritionally compromised child. <br>Muscle enzymes are rarely helpful in the floppy child, with the exception of the congenital muscular dystrophies and some of the structural congenital myopathies. <br>EMG does not allow a definitive diagnosis but is indispensable in deciding whether there is weakness due to neuromuscular disease, or merely hypotonia from causes in other systems or parts of the nervous system. <br>The most common of the neuromuscular disorders, spinal muscular atrophy (SMA), is now diagnosable by molecular genetic analysis (PCR). <br>Where clinical evaluation suggests complex multisystem involvement (i.e. hypotonia plus) inborn errors of metabolism should be excluded. <br>Children with neuromuscular disorders deserve special attention when it comes to anaesthesia. <br>The term 'benign essential hypotonia' or 'hypotonia with a favourable outcome' should be used with caution and only after compliance with strict diagnostic criteria.

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/content/m_cme/22/8/EJC62834
2004-08-01
2016-12-10

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