Malnutrition Despite Adequate Nutrient Intake: A Clue For Diencephalic Syndrome

Yıl 2022, Cilt: 32 Sayı: 5, 621 - 623, 29.10.2022

Anna Carina Ergani , Ayşe Kartal , Fuat Buğrul , Mert Şahinoğlu , Meltem Gümüş , Hakan Karabağlı , Halil Haldun Emiroğlu

https://doi.org/10.54005/geneltip.1122008

Öz

Diencephalic syndrome is characterized by excessive weight loss and subcutaneous fatty tissue loss, hyperactivity, euphoria, and hypervigilance observed in early childhood. It usually accompanies space-occupying neoplastic lesions of the hypothalamic–optochiasmatic region. There is an inverse relationship between mass size and weight gain. In this case report, a patient aged 1 year and 2 months with hypothalamic–optochiasmatic glioma was presented. We aimed to draw attention to diencephalic syndrome, which is a rare cause of weight gain and vomiting.

Anahtar Kelimeler

Diencephalic Syndrome, Severe Malnutrition, Tuberosclerosis type 1

Kaynakça

• Chheda E, Vora S, Prasad M. Nutritional management of diencephalic syndrome: a case series. Neurol India. 2021;69(2):524-5.
• Işık U, Kuter Yılancıoğlu Ş, Eryılmaz S, Canpolat C. Aşırı Kilo Kaybının Nadir Bir Nedeni: Diensefalik Sendromlu Üç Olgu. 2017.
• Salmon MA. Russell's diencephalic syndrome of early childhood. J Neurol Neurosurg Psychiatry. 1972;35(2):196-201.
• Ly KI, Blakeley JO. The diagnosis and management of neurofibromatosis type 1. Medical Clinics. 2019;103(6):1035-54.
• Campen CJ, Gutmann DH. Optic pathway gliomas in neurofibromatosis type 1. Journal of Child Neurology. 2018;33(1):73-81.
• Cavicchiolo ME, Opocher E, Daverio M, Bendini M, Viscardi E, Bisogno G, et al. 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;29(10):1941-5.,
• 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;58(1):28-32.
• Gianotti L, Pincelli AI, Scacchi M, Rolla M, Bellitti D, Arvat E, et al. Effects of recombinant human insulin-like growth factor I administration on spontaneous and growth hormone (GH)-releasing hormone-stimulated GH secretion in anorexia nervosa. The Journal of Clinical Endocrinology & Metabolism. 2000;85(8):2805-9.
• Pimstone BL, Sobel J, Meyer E, Eale D. Secretion of growth hormone in the diencephalic syndrome of childhood. J Pediatr. 1970;76(6):886-9.
• Fleischman A, Brue C, Poussaint TY, Kieran M, Pomeroy SL, Goumnerova L, et al. Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance. Pediatrics. 2005;115(6):e742-8.