Introduction: Multiple symmetric lipomatosis (MSL) is a rare disease of unknown etiology characterized by multiple subcutaneous lipomas with a symmetrical distribution. One interesting aspect about MSL is a high incidence of sudden cardiac death despite a low incidence of metabolic syndrome and coronary arterial disease. Autonomic nervous system dysfunction may probably explain this feature of MSL. Case Report: A 74-year-old man was admitted with acute heart failure and atrial fibrillation. He had a morphotype suggestive of MLS. A 123I-metaiodobenzylguanidine myocardial scintigraphy was conducted for evaluation of cardiac autonomic nervous integrity, since atrial fibrillation precluded the classical approach. The heart-to-mediastinum ratio was 1.68 (normal >2.2). Ischemia was not detected in myocardial perfusion scintigraphy. Conclusion: We present the first reported case of MSL autonomic neuropathy detected by 123I- metaiodobenzylguanidine scintigraphy and suggest that this approach could play a role in MSL stratification by risk of sudden cardiac death and in exploring MSL disease mechanisms.

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