The Role of Leptin in Diencephalic Syndrome

Abstract

Diencephalic syndrome is a rare condition associated with central nervous system tumors. The most common presentation is secondary failure to thrive with proper caloric intake and no statural impairment. Despite the importance of this syndrome, little is known of its pathophysiology. Some reports have documented changes in human growth hormone and insulin levels at the onset, whereas others have described endocrine disorders of hypothalamic insufficiency resulting from surgery of the tumor. It has been suggested that the hormonal changes described, such as increased human growth hormone and ghrelin or decreased insulin and leptin levels, are related to a patient’s BMI. These findings support the role of these 4 hormones as indicators of the patient’s nutritional status but not as mediators or potential therapeutic targets of the disease. We report the case of an infant who initially presented with tumor progression and, after chemotherapy, progressive weight gain and reduced tumor size. Because he presented no hormonal deficiencies or obesity after therapy, we were able to analyze his hormonal status uninfluenced by effects of metabolic treatment or excess weight. Although ghrelin and leptin levels have been related to nutritional status, our patient’s leptin levels fell when tumor size decreased and weight increased: an extraordinary finding because leptin concentration is expected to increase with weight gain. This paradoxical response suggests that leptin may be dysregulated in diencephalic syndrome or that the diencephalic astrocytoma may have had an effect on leptin secretion.

Diencephalic syndrome in childhood, a challenging cause of failure to thrive: miniseries and literature review

“…Whether these changes are consequent to severe emaciation or responsible for weight loss is still matter of debate. Some authors suggest that metabolism-regulating hormones as leptin [ 27 ] and B-lipotropin [ 28 ] are directly produced by brain tumors, leading to decrease in subcutaneous fat and excess high release.…”

Síndrome diencefálico en un niño con desnutrición crónica e hiperactividad

“…El SD es una causa infrecuente de desnutrición, en el incremento de la lipólisis con consecuente pérdida del tejido adiposo. 4,5,7,9 Otro mecanismo descrito es la desregulación en la liberación de leptina y grelina, 3,7 ambas asociadas al mecanismo de apetito y saciedad. En lo que respecta a los síntomas neurológicos, al ingresar, el paciente presentaba un examen neurológico normal, coincidente con otros reportes de casos.…”

“…Los tumores más frecuentemente encontrados corresponden a astrocitomas pilocíticos grado I, 11 localizados en el área hipotálamo-quiasma óptico. 3,4,6,7 También se ha descrito su asociación con astrocitomas cerebelosos, ependimomas supraselares, espongioblastomas supraselares y tumores talámicos. 8 Por causas aún no establecidas, el SD solo afecta al 20% de los pacientes con tumores localizados en esta región.…”

“…La asociación con hiperactividad es muy frecuente. [3][4][5][6][7] Los signos neurológicos no suelen estar presentes en los primeros meses de evolución y, en caso de encontrarse, los más frecuentes son los oftalmológicos (50%) -nistagmus, alteración del campo visual y palidez de papila-, 6,8 seguidos por cefalea y vómitos. 4…”

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

“…Развитие ДК наблюдается при супраселлярных опухолях передних отделов гипоталамуса, а также хиазмы и зрительных нервов, сдавливающих дно III желудочка. Среди описанных в литературе [10][11][12][13][14][15][16] превалируют астроцитомы и другие виды https://doi.org/10.17116/neiro201781584-95…”