Acute Onset Infantile Hypotonia
Acute onset hypotonia in a previously normal infant should warrant an evaluation to rule out acute inflammatory demyelinating polyneuropathy (AIDP), infantile botulism, infantile polymyositis, an infantile form of myasthenia, a toxic process, or acute onset myelopathy.
Repetitive motor nerve stimulation studies should be performed under the following circumstances: 1) there is constipation, bulbar involvement, and/or respiratory distress; 2) an infant presents with ptosis or extraocular muscle weakness; 3) CMAP amplitudes are severely reduced; 4) “myopathic” MUAPs are present; 5) a repetitive CMAP is observed after single supramaximal stimulation on routine nerve conduction study, suggestive of a diagnosis of congenital myasthenia with congenital acetylcholinesterase (AChE) deficiency or classic slow channel syndrome.
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