Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death. (ClinicalTrials.gov number, NCT00622453.)
Phrenic nerve function was evaluated by transcutaneous stimulation in the neck and recording the diaphragmatic potential from surface electrodes placed at the ipsilateral seventh intercostal space (7CS) and the xiphoid process (XP). Simultaneous recordings from 7CS and XP electrodes connected together (XP-7CS) and each connected to a remote reference (knee-7CS and knee-XP) disclosed that the 7CS electrode was always more active and showed electropositive activity, whereas the XP electrode, which was only minimally active, showed electronegative response. Out-of-phase summation of opposite polarity activity at the two electrodes resulted in a higher amplitude response in XP-7CS derivation. Phrenic nerve studies are useful in establishing phrenic nerve injury following cardiothoracic operation. They may also provide evidence of phrenic nerve or diaphragmatic involvement in demyelinative neuropathies, motor neuron disease, and muscular dystrophies.
This article provides a review of some of the muscular disorders that can arise from some of the commonly seen endocrinologic disturbances. Thyroid, parathyroid, and adrenal dysfunctions as they relate to neuromuscular symptoms are discussed. Common clinical presentations of the endocrine myopathies are highlighted, along with diagnostic evaluation and treatments.
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