Skip to main content

Abstract

Lipodystrophic disorders are a heterogeneous group of rare syndromes characterized by an abnormal physical appearance, due to a generalized or partial loss of subcutaneous adipose tissue. Due to the reduction of physiological sites of energy stores, triglycerides of exogenous and endogenous origin are accumulated in ectopic organs such as the liver, skeletal muscle, heart, kidneys, and pancreas, predisposing to the development of insulin resistance and its complications: diabetes mellitus, dyslipidemia, hepatosteatosis, acanthosis nigricans, and polycystic ovary syndrome.

Leptin is a hormone produced only by the adipose tissue, and in lipodystrophic patients there is a characteristic state of hypoleptinemia, which can be mild (as in some partial forms) or complete (as in generalized forms). Leptin is a fundamental hormone in the regulation of hunger, and hypoleptinemia is responsible for hyperphagia that characterizes the more severe forms of lipodystrophy.

Recently there has been a renewed interest in the study of psychiatric comorbidities in affected patients: lipodystrophic patients have an increased prevalence of mood, anxiety, pain, and eating disorders that must be effectively researched and treated. Indeed, both the European and American guidelines recommend the assessment of the psychopathological profile of lipodystrophic patients, to refer them to mental health professional for psychiatric and psychological support, if needed. Evaluating psychological distress, even when mild, appears to be very important, and improving it can indeed impact positively on the other comorbidities. Hypoleptinemia is likely an important driver for food craving and an important player in the pathogenesis of depression in lipodystrophic patients.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Garg A. Acquired and Inherited Lipodystrophies. N Engl J Med. 2004;350:1220–34.

    Article  CAS  PubMed  Google Scholar 

  2. Chiquette E, Oral EA, Garg A, Araujo-Vilar D, Dhankhar P. Estimating the prevalence of generalized and partial lipodystrophy: findings and challenges. Diabetes Metab Syndr Obes. 2017;10:375–83.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Garg A. Gender differences in the prevalence of metabolic complications in familial partial lipodystrophy (Dunnigan variety). J Clin Endocrinol Metab. 2000;85:1776–82.

    CAS  PubMed  Google Scholar 

  4. Brown RJ, Cheung PT, Dunger D, Garg A, Jack M, Mungai L, Oral EA, Patni N, Rother KI, von Schnurbein J, Sorkina E, Stanley T, Vigouroux C, Wabitsch M, Williams R, Yorifuji T. The diagnosis and management of lipodystrophy syndromes: a multi-society practice guideline. J Clin Endocrinol Metab. 2016;101:4500–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Hussain I, Garg A. Lipodystrophy Syndromes. Endocrinol Metab Clin North Am. 2016;45:783–97.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Florenza C, Chou S, Mantzoros S. Lipodystrophy: pathophysiology and advances in treatment. Nat Rev Endocrinol. 2011;7:137–50.

    Article  CAS  Google Scholar 

  7. Patni N, Garg A. Congenital generalized lipodystrophies-new insights into metabolic dysfunction. Nat Rev Endocrinol. 2015;11:522–34.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Berger S, Ceccarini G, Scabia G, Barone I, Pelosini C, Ferrari F, Magno S, Dattilo A, Chiovato L, Vitti P, Santini F, Maffei M. Lipodystrophy and obesity are associated with decreased number of T cells with regulatory function and pro-inflammatory macrophage phenotype. Int J Obes (Lond). 2017;11:1676–84.

    Article  CAS  Google Scholar 

  9. Araùjo-Vilar D, Santini F. Diagnosis and treatment of lipodystrophy: a step-by-step approach. J Endocrinol Invest. 2018;1:61–73.

    Google Scholar 

  10. Schlögl H, Müller K, Horstmann A, Miehle K, Püschel J, Villringer A, Pleger B, Stumvoll M, Fasshauer M. Leptin substitution in patients with lipodystrophy: neural correlates for long-term success in the normalization of eating behavior. Diabetes. 2016;65:2179–86.

    Article  PubMed  CAS  Google Scholar 

  11. Lightbourne M, Brown RJ. Genetics of lipodystrophy. Endocrinol Metab Clin North Am. 2017;2:539–54.

    Article  Google Scholar 

  12. Guillín-Amarelle C, Sánchez-Iglesias S, Castro-Pais A, Rodríguez-Cañete L, Ordóñez-Mayán L, Pazos M, González-Méndez B, Rodríguez-García S, Casanueva F, Fernández-Marmiesse A, Araújo-Vilar D. Type 1 familial partial lipodystrophy: understanding the Köbberling syndrome. Endocrine. 2016;2:411–21.

    Article  CAS  Google Scholar 

  13. https://www.ema.europa.eu/en/medicines/human/EPAR/myalepta

  14. Brown RJ, Valencia A, Startzell M. Metreleptin-mediated improvements in insulin sensitivity are independent of food intake in humans with lipodystrophy. J Clin Invest. 2018;8:3504–16.

    Article  Google Scholar 

  15. Warin RP, Ingram JT. Progressive lipodystrophy report of two cases. Lancet. 1950;6619:55–7.

    Article  Google Scholar 

  16. Fowler PBS. Lipodystrophia progressiva and temporary hydronephrosis. Br Med J. 1955;4924:1249–51.

    Article  Google Scholar 

  17. Gibson R. The occurrence of progressive lipodystrophy in mental defectives. Can Med Assoc J. 1957;3:217–9.

    Google Scholar 

  18. Shawky RM, Gamal R, Seifeldin NS. Berardinelli–Seip syndrome type 2—an Egyptian child. Egypt J Med Human Genet. 2015;16:189–93.

    Article  Google Scholar 

  19. Van Maldergem L. Berardinelli-Seip congenital lipodystrophy. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, LJH B, Stephens K, Amemiya A, editors. GeneReviews. Seattle: University of Washington; 2003. p. 1993–2020.

    Google Scholar 

  20. Ding L, Yang X, Tian H, Liang J, Zhang F, Wang G, Wang Y, Ding M, Shui G, Huang X. Seipin regulates lipid homeostasis by ensuring calcium-dependent mitochondrial metabolism. EMBO J. 2018;37:1–17.

    Article  CAS  Google Scholar 

  21. Van Maldergem L, Magré J, Khallouf TE, Gedde-Dahl T Jr, Delépine M, Trygstad O, Seemanova E, Stephenson T, Albott CS, Bonnici F, Panz VR, Medina JL, Bogalho P, Huet F, Savasta S, Verloes A, Robert JJ, Loret H, De Kerdanet M, Tubiana-Rufi N, Mégarbané A, Maassen J, Polak M, Lacombe D, Kahn CR, Silveira EL, D’Abronzo FH, Grigorescu F, Lathrop M, Capeau J, O’Rahilly S. Genotype-phenotype relationships in Berardinelli-Seip congenital lipodystrophy. PLoS One. 2016;7:e0158874.

    Google Scholar 

  22. Wei S, Soh SL, Qiu W, Yang W, Seah CJ, Guo J, Ong WY, Pang ZP, Han W. Seipin regulates excitatory synaptic transmission in cortical neurons. J Neurochem. 2013;4:478–89.

    Article  CAS  Google Scholar 

  23. Sánchez-Iglesias S, Unruh-Pinheiro A, Guillín-Amarelle C, González-Méndez B, Ruiz-Riquelme A, Rodríguez-Cañete BL, Rodríguez-García S, Guillén-Navarro E, Domingo-Jiménez R, Araújo-Vilar D. Skipped BSCL2 transcript in Celia’s encephalopathy (PELD): new insights on fatty acids involvement, senescence and adipogenesis. J Med Genet. 2002;10:722–33.

    Google Scholar 

  24. Patni N, Alves C, von Schnurbein J, Wabitsch M, Tannin G, Rakheja D, Garg A. A novel syndrome of generalized lipodystrophy associated with pilocytic astrocytoma. J Clin Endocrinol Metab. 2015;10:3603–6.

    Article  CAS  Google Scholar 

  25. Lockemer HE, Sumpter KM, Cope-Yokoyama S, Garg A. A novel paraneoplastic syndrome with acquired lipodystrophy and chronic inflammatory demyelinating polyneuropathy in an adolescent male with craniopharyngioma. J Pediatr Endocrinol Metab. 2017;4:479–83.

    Google Scholar 

  26. Anderson RJ, Clouse RE, Freedland KE, Lustman PJ. The prevalence of comorbid depression in adults with diabetes: a meta-analysis. Diabetes Care. 2011;6:1069–78.

    Google Scholar 

  27. Dawes AJ, Maggard-Gibbons M, Maher AR, Booth MJ, Miake-Lye I, Beroes JM, Shekelle PG. Mental health conditions among patients seeking and undergoing bariatric surgery: a meta-analysis. JAMA. 2016;2:150–63.

    Article  CAS  Google Scholar 

  28. Calabrò PF, Ceccarini G, Calderone A, Lippi C, Piaggi P, Ferrari F, Magno S, Pedrinelli R, Santini F. Psychopathological and psychiatric evaluation of patients affected by lipodystrophy. Eat Weight Disord. 2019; https://doi.org/10.1007/s40519-019-00716-6.

  29. Ajluni N, Meral R, Neidert AH, Brady GF, Buras E, McKenna B, DiPaola F, Chenevert TL, Horowitz JF, Buggs-Saxton C, Rupani AR, Thomas PE, Tayeh MK, Innis JW, Omary MB, Conjeevaram H, Oral EA. Spectrum of disease associated with partial lipodystrophy (PL): lessons from a trial cohort. Clin Endocrinol (Oxf). 2017;5:698–707.

    Article  CAS  Google Scholar 

  30. Adams C, Stears A, Savage D, Deaton C. “We’re stuck with what we’ve got”: the impact of lipodystrophy on body image. J Clin Nurs. 2018;9–10:1958–68.

    Article  Google Scholar 

  31. Katz J, Rosenbloom BN, Fascler S. Chronic pain, psychopathology, and DSM-5 somatic symptom disorder. Can J Psychiatry. 2015;4:160–7.

    Article  Google Scholar 

  32. Innamorati M, Imperatori C, Balsamo M. Food craving questionnaire-trait (FCQ-T) discriminates between obese and overweight patients with and without binge eating tendencies: the italian version of the FCQ-T. J Pers Assess. 2014;6:632–9.

    Article  Google Scholar 

  33. Farooqi IS, Bullmore E, Keogh J, Gillard J, O’Rahilly S, Fletcher PC. Leptin regulates striatal regions and human eating behavior. Science. 2007;5843:1–3.

    Google Scholar 

  34. Baicy K, London ED, Monterosso J, Wong ML, Delibasi T, Sharma A, Licinio J. Leptin replacement alters brain response to food cues in genetically leptin-deficient adults. Proc Natl Acad Sci U S A. 2007;46:18,276–9.

    Article  Google Scholar 

  35. Frank S, Heni M, Moss A, von Schnurbein J, Fritsche A, Häring HU, Farooqi S, Preissl H, Wabitsch M. Leptin therapy in a congenital leptin-deficient patient leads to acute and long-term changes in homeostatic, reward, and food-related brain areas. J Clin Endocrinol Metab. 2011;8:E1283–7.

    Article  CAS  Google Scholar 

  36. Frank S, Heni M, Moss A, von Schnurbein J, Farooqi S, Häring H-U, Fritsche A, Preissl H, Wabitsch M. Long-term stabilization effects of leptin on brain functions in a leptin-deficient patient. PLoS One. 2013;6:e65893.

    Article  CAS  Google Scholar 

  37. Farr OM, Florenza C, Papageorgiou P. Leptin therapy alters appetite and neural responses to food stimuli in brain areas of leptin-sensitive subjects without altering brain structure. J Clin Endocrinol Metab. 2014;12:E2529–38.

    Article  CAS  Google Scholar 

  38. Aotani D, Ebihara K, Sawamoto N, Kusakabe T, Aizawa-Abe M, Kataoka S, Sakai T, Iogawa H, Ebihara C, Fujikura J, Hosoda K, Fukuyama H, Nakao K. Functional magnetic resonance imaging analysis of food-related brain activity in patients with lipodystrophy undergoing leptin replacement therapy. J Clin Endocrinol Metab. 2012;10:3663–71.

    Article  CAS  Google Scholar 

  39. Zaraouna S, Wozniak G, Papachristou AI. Mood disorders: a potential link between ghrelin and leptin on human body? World J Exp Med. 2015;2:103–9.

    Article  Google Scholar 

  40. Lu XY. The leptin hypothesis of depression: a potential link between mood disorders and obesity? Curr Opin Pharmacol. 2007;6:648–52.

    Article  CAS  Google Scholar 

  41. Yildiz G, Senturk MB, Yildiz P, Cakmak Y, Budak MS, Cakar E. Serum serotonin, leptin and adiponectin changes in women with postpartum depression: controlled study. Arch Gynecol Obstet. 2017;4:853–8.

    Article  CAS  Google Scholar 

  42. Zou X, Zhong L, Zhu C, Zhao H, Zhao F, Cui R, Gao S, Li B. Role of leptin in mood disorder and neurodegenerative disease. Front Neurosci. 2019;378:1–8.

    Google Scholar 

  43. Cernea S, Both E, Huţanu A, Şular FL, Roiban AL. Correlation of serum leptin and leptin resistance with depression and anxiety in patient with type 2 diabetes. Psychiatry Clin Neurosci. 2019;12:745–53.

    Article  CAS  Google Scholar 

  44. Syk M, Ellström S, Mwinyi J, Schiöth HB, Ekselius L, Ramklint M, Cunningham JL. Plasma levels of leptin and adiponectin and depressive symptoms in young adults. Psychiatry Res. 2018;272:1–7.

    Article  PubMed  CAS  Google Scholar 

  45. Ceccarini G, Pelosini C, Ferrari F, Magno S, Vitti J, Salvetti G, Moretto C, Marioni A, Buccianti P, Piaggi P, Maffei M, Santini F. Serum IGF-binding protein 2 (IGFBP-2) concentrations change early after gastric bypass bariatric surgery revealing a possible marker of leptin sensitivity in obese subjects. Endocrine. 2019;65:86–93.

    Article  CAS  PubMed  Google Scholar 

  46. An JH, Jang EH, Kim AY, Fava M, Mischoulon D, Papakostas GI, Na EJ, Jang J, Yu HY, Hong JP, Jeon HJ. Ratio of plasma BDNF to leptin levels are associated with treatment response in major depressive disorder but not in panic disorder: a 12-week follow-up study. J Affect Disord. 2019;259:349–54.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ferruccio Santini .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Ferrari, F., Calabrò, P.F., Ceccarini, G., Santini, F. (2022). Eating Behavior and Psychopathology in Non-HIV Lipodystrophic Patients. In: Manzato, E., Cuzzolaro, M., Donini, L.M. (eds) Hidden and Lesser-known Disordered Eating Behaviors in Medical and Psychiatric Conditions . Springer, Cham. https://doi.org/10.1007/978-3-030-81174-7_35

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-81174-7_35

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-81173-0

  • Online ISBN: 978-3-030-81174-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics