Correct!
5. All of the above
All of the above studies are appropriate. Bronchoscopy with bronchioloalveolar lavage, possibly transbronchial biopsy, is a reasonable procedure to perform given the lower lobe pulmonary infiltrative abnormalities. An enhanced neck CT is a reasonable study to obtain given the bilateral submandibular gland enlargement. Cardiac MRI is often performed for evaluation of abnormalities detected at echocardiography, particularly when left ventricular hypertrophy is present, raising the possibility of an infiltrative cardiomyopathy. Nerve conduction studies are reasonable to purse given the lower extremity weakness and abnormal reflexes detected at clinical examination.
The patient underwent enhanced neck CT which showed bilateral submandibular gland enlargement and mild tongue enlargement but no lymphadenopathy. Nerve conduction studies showed paraspinal thoracolumbar and florid distal lower extremity fibrillation potentials suggesting a radicular process. Cardiac MRI (Figure 5) was performed and showed findings characteristic of an infiltrative, restrictive cardiomyopathy.
Figure 5. 4-chamber (A) and short axis (B) steady-state free precession cardiac MR images (a “white blood sequence that allows visualization of flowing blood without the use of intravenous contrast) show left ventricular muscular hypertrophy evidenced by thickening of the inferior base-to-mid-cavity interventricular septum (line, B). 4-chamber (C) and short axis (D) late gadolinium enhancement cardiac MR images (a sequence performed 8 – 10 minutes following intravenous contrast administration) shows very poor enhancement of the cardiac blood pool (*) as well as intense subendocardial delayed enhancement (arrowheads). The thickened left ventricular muscle and subendocardial delayed enhancement suggests an infiltrative, restrictive cardiomyopathy. Normal 4-chamber (E) and short axis (F) late gadolinium enhancement cardiac MR images presented for comparison. Note how the myocardium is properly “nulled,” appearing black (arrowheads), and there is substantial contrast in the blood pool (*)- this appearance is normal for this sequence. No delayed enhancement is present. RV= right ventricle.
Which of the following represents appropriate confirmatory testing for the diagnosis in this patient? (Click on the correct answer to be directed to the eighth of nine pages)