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.

1.
Launois PE, Bensaude R: De l’adénolipomatose symétrique. Bull Mem Soc Med Hop Paris 1898;1:298–303.
2.
Enzi G, Busetto L, Ceschin E, et al: Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study. Int J Obes Relat Metab Disord 2002;26:253–261.
3.
Enzi G, Inelmen EM, Baritussio A, et al: Multiple symmetric lipomatosis: a defect in adrenergic-stimulated lipolysis. J Clin Invest 1977;60:1221–1229.
4.
Dorigo P, Prosdocimi M, Carpenedo F, et al: Multiple symmetric lipomatosis: a defect in adrenergic stimulated lipolysis II. Pharmacol Res Commun 1980;12:625–638.
5.
Nisoli E, Regianini L, Briscini L, et al: Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 2002;198:378–387.
6.
Haap M, Siewecke C, Thamer C, et al: Multiple symmetric lipomatosis: a paradigm of metabolically innocent obesity? Diabetes Care 2004;27:794–795.
7.
Enzi G, Angelini C, Negrin P, et al: Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis. Medicine (Baltimore) 1985;64:388–393.
8.
Fedele D, Bellavere F, Bosello G, et al: Impairment of cardiovascular autonomic reflexes in multiple symmetric lipomatosis. J Auton Nerv Syst 1982;11:181–188.
9.
Gerritsen J, Dekker JM, TenVoorde BJ, et al: Impaired autonomic function is associated with increased mortality, especially in subjects with diabetes, hypertension, or a history of cardiovascular disease. The Hoorn Study. Diabetes Care 2001;24:1793–1798.
10.
Busetto L, Sträter D, Enzi G, et al: Differential clinical expression of multiple symmetric lipomatosis in men and women. Int J Obes Relat Metab Disord 2003;27:1419–1422.
11.
Enzi G: Multiple symmetric lipomatosis: an updated clinical report. Medicine (Baltimore) 1984;63:56–64.
12.
Task Force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology: Heart rate variability: standards of measurement, physiological interpretation and clinical use. Circulation 1996;93:1043–1065.
13.
Patel AD, Iskandrian AE: MIBG imaging. J Nucl Cardiol 2002;9:75–94.
14.
Ji SY, Travin MI: Radionuclide imaging of cardiac autonomic innervations. J Nucl Cardiol 2010;17:655–666.
15.
Chirumamilla A, Travin MI: Cardiac applications of 123I-MIBG imaging. Semin Nucl Med 2011;41:374–387.
16.
Vinik AI, Ziegler D: Diabetic cardiovascular autonomic neuropathy. Circulation 2007;115:387–397.
17.
Hattori N, Tamaki N, Hayashi T, et al: Regional adnormality of iodine-123-MIBG in diabetic hearts. J Nucl Med 1996;37:1985–1990.
18.
Vinik AI, Maser RE, Mitchelli BD, Freeman R: Diabetic autonomic neuropathy. Diabetes Care 2003;26:1553–1579.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.