Role of imaging studies in the evaluation of sinus arrest and syncope in a patient with neurofibromatosis.

摘要

There is little published data on the occurrence of arrhythmias and conduction abnormalities in patients with neurofibromatosis. Here, we report a rare case of recurrent seizures and syncope in a patient with neurofibromatosis. A 50-year-old male was admitted to the hospital with sepsis secondary to aspiration pneumonia. His medical problems included neurofibromatosis type I, seizure disorder of unclear etiology and recurrent syncope. He was not on any medications that could affect the heart rate. There was no history of alcohol abuse, and no family history of seizure disorder or arrhythmia. Physical examination was significant for multiple neurofibromas in the neck extending onto the anterior aspect of the chest. The patient's baseline electrocardiogram varied between sinus tachycardia, sinus rhythm and ectopic atrial rhythm. In addition to intermittent junctional escape rhythm noted on telemetry, there were several episodes of sinus arrest lasting up to nine seconds. As part of further diagnostic work up, imaging studies of the neck were obtained to look for extrinsic compression of the vagus nerve by the neurofibromas in the neck. The imaging studies confirmed the presence of multiple neurofibromas along the natural course of the vagus nerve (Figs. 1 and 2).