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Klochkova I.S.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Astaf'eva L.I.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Konovalov A.N.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Kadashev B.A.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Kalinin P.L.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Sharipov O.I.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Kutin M.A.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Sidnevа Yu.G.

Clinical and Research Institute of Emergency Children’s Surgery and Traumatology, Moscow Health Department, Moscow, Russia

Shishkina L.V.

Burdenko Neurosurgery Institute, Moscow, Russia

Pronin I.N.

NII neĭrokhirurgii im. akad. N.N. Burdenko RAMN, Moskva

A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma

Authors:

Klochkova I.S., Astaf'eva L.I., Konovalov A.N., Kadashev B.A., Kalinin P.L., Sharipov O.I., Kutin M.A., Sidnevа Yu.G., Shishkina L.V., Pronin I.N.

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Journal: Burdenko's Journal of Neurosurgery. 2017;81(5): 84‑95

DOI: 10.17116/neiro201781584-95

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A RARE CASE OF DIENCEPHALIC CACHEXIA IN AN ADULT FEMALE WITH CRANIO-PHARYNGIOMA
A RARE CASE OF DIENCEPHALIC CACHEXIA IN AN ADULT FEMALE WITH CRANIO-PHARYNGIOMA

To cite this article:

Klochkova IS, Astaf'eva LI, Konovalov AN, et al. . A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma. Burdenko's Journal of Neurosurgery. 2017;81(5):84‑95. (In Russ., In Engl.)
https://doi.org/10.17116/neiro201781584-95

Подписка 2025 Журнал «Вопросы нейрохирургии» имени Н.Н. Бурденко (Burdenko's Journal of Neurosurgery)
МСФ на ЯМ
Использование инфографики авторами научных работ
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Abstract:

Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

Keywords:

craniopharyngiomа
diencephalic cachexia
Russel syndrome
hypothalamic tumor

Authors:

Klochkova I.S.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Astaf'eva L.I.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Konovalov A.N.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Kadashev B.A.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Kalinin P.L.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Sharipov O.I.

Burdenko Neurosurgical Institute, Moscow, Russian Federation

Kutin M.A.

NII neĭrokhirurgii im. N.N. Burdenko RAMN, Moskva

Sidnevа Yu.G.

Clinical and Research Institute of Emergency Children’s Surgery and Traumatology, Moscow Health Department, Moscow, Russia

Shishkina L.V.

Burdenko Neurosurgery Institute, Moscow, Russia

Pronin I.N.

NII neĭrokhirurgii im. akad. N.N. Burdenko RAMN, Moskva

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References:

  1. Russell AA. A diencephalic syndrome of emaciation in infancy and childhood. Arch Dis Child. 1951;26:274.
  2. Burr IM, Slonim AE, Danish RK, Gadoth N, Buttler IJ. Diencephalic syndrome revisited. J Pediatr. 1976;88(3):439-444. https://
  3. Addy DP, Hudson FP. Diencephalic syndrome of infantile emaciation: analysis of literature and report of a further 3 cases. Arch Dis Child. 1972;47(253):338-343. https://doi.org/10.1136/adc.47.253.338
  4. Poussaint TY, Barnes PD, Nichols K, Anthony DC, Cohen L, Tarbell NJ, Goumnerova L. Diencephalic syndrome: clinical features and imaging findings. Am J Neuroradiol. 1997;18:1499-1505.
  5. Kim A, Moon JS, Yang HR, Chang JY, Ko JS, Seo JK. Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants. Korean J Pediatr. 2015 Jan;58(1):28-32. https://doi.org/10.3345/kjp.2015.58.1.28
  6. Fleischman A, Brue C, Poussaint TY, Kieran M, Pomeroy SL, Goumnerova L, Scott RM, Laurie E, Cohen LE. Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance. Pediatrics. 2005 June;115(6). https://doi.org/10.1542/peds.2004-2237
  7. Mazerkina NA. Opukholi khiazmal’no-sellyarnoi oblasti i III zheludochka u detei: pozdnii diagnoz. Pediatriya. 2012;4:50-55 (In Russ.)
  8. Kilday JP, Bartels U, Huang A, Barron M, Shaqo M, Mistry M, Zhukova N, Laperriere N, Dirks P, Hawkins C, Bouffet E. Favorable survival and metabolic outcome for children with diencephalic syndrome using a radiation-sparing approach. J Neurooncol. 2014 Jan;116(1):195-204. https://doi.org/10.1007/s11060-013-1284-2
  9. Shuper A, Bloch I, Kornreich L, Horev G, Michowitz S, Zaizov R, Cohen I. Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence. Pediatr Hematol Oncol. 1996;13:443-449. https://doi.org/10.3109/08880019609030856
  10. Brauner R, Trivin C, Zerah M, Souberbielle JC, Doz F, Kalifa C, Sainte-Rose C. Diencephalic syndrome due to hypothalamic tumor: a model of the relationship between weight and puberty onset. J Clin Endocrinol Metab. 2006 Jul;91(7):2467-2473. https://doi.org/10.1210/jc.2006-0322
  11. Stival A, Lucchesi M, Farina S, Buccoliero AM, Castiqlione F, Genitori L, De Martino M, Sardi I. An infant with hyperalertness, hyperkinesis, and failure to thrive: a rare diencephalic syndrome due to hypothalamic anaplastic astrocytoma. BMC Cancer. 2015 Sep 4;15:616. https://doi.org/10.1186/s12885-015-1626-x
  12. Distelmaier F, Janssen G, Mayatepek E, Schaper J, Göbel U, Rosenbaum T. Disseminated pilocytic astrocytoma involving brain stem and diencephalon: a history of atypical eating disorder and diagnostic delay. J Neurooncol. 2006 Sep;79(2):197-201. https://doi.org/10.1007/s11060-006-9125-1
  13. Velasco P, Clemente M, Lorite R, Ventura MC, Gros L, S de Toledo J, Galleqo S. The role of leptin in diencephalic syndrome. Pediatrics. 2014 Jan;133(1). https://doi.org/10.1542/peds.2012-3196
  14. Cavicchiolo ME, Opocher E, Daverio M, Bendini M, Viscardi E, Bisoqno G, Perilongo G, Da Dalt L. Diencephalic syndrome as sign of tumor progression in a child with neurofibromatosis type 1 and optic pathway glioma: a case report. Childs Nerv Syst. 2013 Oct;29(10):1941-1945. https://doi.org/10.1007/s00381-013-2109-5
  15. Marec-Berard P, Szathmari A, Conter C, Mottolese C, Berlier P, Frappaz D. Improvement of diencephalic syndrome after partial surgery of optic chiasm glioma. Pediatr Blood Cancer. 2009 Sep;53(3):502-504. https://doi.org/10.1002/pbc.22085
  16. Patni N, Alves C, von Schnurbein J, Wabitsch M, Tannin G, Rakheja D, Garq A. A novel syndrome of generalized lipodystrophy associated with pilocytic astrocytoma. J Clin Endocrinol Metab. 2015 Oct;100(10):3603-3606. https://doi.org/10.1210/jc.2015-2476
  17. Mohan SM, Dharmalingam M, Prasanna Kumar KM, Verma RG, Balaji Pai S, Krishna KN, Dhandekar C. Suprasellar germ cell tumor presenting as diencephalic syndrome and precocious puberty. J Pediatr Endocrinol Metab. 2003 Mar;16(3):443-446. https://doi.org/10.1515/JPEM.2003.16.3.443
  18. Hoffmann A, Gebhardt U, Sterkenburg AS, Warmuth-Metz M, Müller HL. Diencephalic syndrome in childhood craniopharyngioma—results of German multicenter studies on 485 long-term survivors of childhood craniopharyngioma. J Clin Endocrinol Metab. 2014 Nov;99(11):3972-3977. https://doi.org/10.1210/jc.2014-1680
  19. Kohira I, Yoshimura Y, Ninomiya Y, Ikeda K, Iwata J. Pilocytic astrocytoma and diencephalic syndrome in an adult with neurofibromatosis type 1. Rinsho Shinkeigaku. 2003 Jun;43(6):327-329.
  20. Tanabe M, Watanabe T, Hori T. Von Recklinghausen’s disease with diencephalic syndrome in an adult. Case report. J Neurosurg. 1994 Mar;80(3):556-558. https://doi.org/10.3171/jns.1994.80.3.0556
  21. Sharma RR, Chandy MJ, Lad SD. Diencephalic syndrome of emaciation in an adult associated with a suprasellarcraniopharyngioma—a case report. Br J Neurosurg. 1990;4(1):77-80. https://doi.org/10.3109/02688699009000687
  22. Miyoshi Y, Yunoki M, Yano A, Nishimoto K. Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report. Neurosurgery. 2003 Jan;52(1):224-227. https://doi.org/10.1097/00006123-200301000-00029
  23. Gropman AL, Packer RJ, Nicholson HS, Vezina LG, Jakacki R, Geyer R, Olson JM, Phillips P, Needle M, Broxson EHJr, Reaman G, Finlay J. Treatment of diencephalic syndrome with chemotherapy: growth, tumor response, and long-term control. Cancer. 1998;83(1):166-172. https://doi.org/10.1002/(SICI)1097-0142(19980701)83:1
  24. Lainscak M, Filippatos GS, Gheorghiade M, Fonarow GC, Anker SD. Cachexia: common, deadly, with an urgent need for precise definition and new therapies. Am J Cardiol. 2008 Jun 2;101(11A). https://doi.org/10.1016/j.amjcard.2008.02.065
  25. Evans WJ, Morley JE, Argiles J, Bales C, Baracos V, Guttridgea D, Jatoi A, Kalantar-Zadeha K, Lochsa H, Mantovani G, Marks D, Mitch WE, Muscaritoli M, Najand A, Ponikowski P, Fanelli FR, Schambelan M, Schols A, Schuster M, Thomas D, Wolfe R, Anker SD. Cachexia: a new definition. Clin Nutr. 2008;27:793-799. https://doi.org/10.1016/j.clnu.2008.06.013
  26. Anker SD, Negassa А, Coats AJ, Afzal R, Poole-Wilson RA, Cohn JN, Yusuf S. Prognostic importance of weight loss in chronic heart failure and the effect of treatment with angiotensin-converting-enzyme inhibitors: an observational study. Lancet. 2003;361:1077-1083. https://doi.org/10.1016/S0140-6736(03)12892-9
  27. Tan BH, Fearon KC. Cachexia: prevalence and impact in medicine. Curr Opin Clin Nutr Metab Care. 2008;11:400-407. https://doi.org/10.1097/MCI.0b013e328315510b
  28. Diseases of the heart in the works of Hippokrates. Br Heart J. 1962;24:257-264.
  29. Turgenev IS. Polnoe sobranie sochinenii i pisem v tridtsati tomakh. T. 3. M.: Nauka. 1979. (In Russ.)
  30. Sorva R, Heiskanen O, Perheentupa J. Craniopharyngioma in adults. Ann Clin Res. 1987;19:339-343.
  31. Bunin GR, Surawicz TC, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998;89(4): 547-551. https://doi.org/10.3171/jns.1998.89.4.0547
  32. Samii M, Tatagiba M. Craniopharyngioma. In: Kaye AH, Laws ERJr. (eds): Brain tumors: an encyclopedic approach. New York: Churcill Livingstone. 1995;873-894. https://doi.org/10.1007/BF02742928
  33. Alen JF, Boto GR, Lagares A, dela Lama A, Gomez PA, Lobato RD. Intratumoural bleomycin as a treatment for recurrent cystic craniopharyngioma. Case report and review of the literature. Neurochirugia (Astur). 2002;13(6):479-485. https://doi.org/10.1016/S1130-1473(02)70576-9
  34. Semenova ZhB. Gistobiologiya kraniofaringiom i osobennosti techeniya zabolevaniya: Dis... d-ra med. nauk. M. 2000. (In Russ.)
  35. Muller HL, Emser A, Faldum A, Bruhnken G, Etavard-Gorris N, Gebhardt U, Oeverink R, Kolb R, Sorensen N. Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma. J Clin Endocrinol Metab. 2004; 89:3298-3305. https://doi.org/10.1210/jc/203-031751
  36. Hochberg I, Hochberg Z. Expanding the definition of hypothalamic obesity. Obes Rev. 2010;11:709-721. https://doi.org/10.1111/j/147-789X.2010.00727.x
  37. Müller HL, Gebhardt U, Teske C, Faldum A, Zwiener I, Warmuth-Metz M, Pietsch T, Pohl F, Sorensen N, Calaminus G. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial Kraniopharyngeom 2000 after 3-year follow-up. Eur J Endocrinol. 2011;165(1):17-24. https://doi.org/10.1530/EJE-11-0158
  38. Tolle V, Kadem M, Bluet-Pajot M-T, Frere D, Foulon C, Bossu C, Dardennes R, Mounier C, Zizzari P, Lang F, Epelbaum J, Estour B. Balance in ghrelin and leptin plasma levels in anorexia nervosa and constitutionally thin women. J Clin Endocrinol Metab. 2003;88:109-116. https://doi.org/10.1210/jc.2002-020645
  39. Konovalov AN, Kutin MA, Kadashev BA, Kalinin PL, Fomichev DV, Astaf’eva LI, Golanov AV, Trunin YuYu. Sovremennye printsipy lecheniya kraniofaringiom. Zhurnal Opukholi golovy i shei. 2012;3:7-16. (In Russ.)
  40. Kutin MA, Sidneva YuG, Konovalov AN, Pronin IN, Kadashev BA, Kalinin PL, Semenova ZhB, Fomichev DV, Sharipov OI, Shul’ts EI, Lubnin AYu, Popugaev KA, Savin IA, Astaf’eva LI, Klochkova IS, Mazerkina NA, Shishkina VL, Dobrovol’skii DV, Rotin DV, Savateev AN, Sidoruk EV. Gipotalamus. Sovremennye predstavleniya o prostranstvennoi i funktsional’noi organizatsii (obzor literatury). Vestnik nevrologii, psikhiatrii i neirokhirurgii. 2016;2:55-66. (In Russ.)
  41. Gorelyshev SK. Opukholi khiazmy i dna III zheludochka: Dis... d-ra med. nauk. M. 2001. (In Russ.)
  42. Choux M, Lena G. Craniopharyngioma (Surgery of Third Ventricle. Second Edition. Apuzzo MLJ. 1998;1143-1180.
  43. Apuzzo MLJ, Levy ML, Tung H. Surgical strategies and technical methodologies in optimal management of craniopharyngioma and masses affecting the third ventricular chamber. Acta Neurochir Suppl (Wien). 1991;53:77-88. https://doi.org/10.1007/978-3-7091-9183-5_14
  44. Gorelyshev S, Matuev K, Lubnin A, Laskov M, Lemeneva N, Mazerkina N, Khuhlaeva E. Brain tumors in infants: a report on 102 cases. J Neuro-Oncology (Toronto) — Ontario—Canada. 2012 June 24-2.
  45. Mazerkina NA, Konovalov AN, Gorelyshev SK, Semenova ZhB, Krasnova TS, Tenedieva VD. Endokrinnye narusheniya pri kraniofaringiomakh u detei: Zavisimost’ ot lokalizatsii opukholi. Zhurnal voprosy neirokhirurgii. 2008;23-29. (In Russ.)
  46. Kutin MA, Rotin DL, Shishkina LV, Kadashev BA. Current opinion on the craniopharyngioma biology. Zhurnal «Voprosy neirokhirurgii».2013; 1:53-61. (In Russ.)
  47. Duo D, Gasverde S, Benech F, Zenqa F, Giordana MT. MIB-1 immunoreactivity in craniopharyngiomas: a clinico-pathological analysis. Clin Neuropathol. 2003;22(5):229-234.
  48. Huber J, Sovinz P, Lackner H, Mokry M, Eder H, Urban C. Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive. Кlin Paediatr. 2007;219(2):91-94. https://doi.org/10.1055/s-2007-921559
  49. Chamberlain MC, Levin VA. Chemotherapeutic treatment of the diencephalic syndrome. A case report. Cancer. 1989; 63:1681-1684. https://doi.org/10.1002/1097-0142(19900501)
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