Diencephalic syndrome: a rare and easily overlooked cause of failure to thrive.

BACKGROUND: Diencephalic syndrome (DS) is an uncommon cause of failure to thrive in infants and young children. The major manifestations are emaciation, hyperkinesia, and euphoria. Most patients have a tumor in the hypothalamic-optic chiasma region. CASE REPORT: Two children, aged 14 months and 5 years 9 months, who presented with classic features of DS at an onset of 2 and 3 months respectively, were reported. Neurologic examination was normal, except for papilledema in the second child. Imaging of the brain showed a suprasellar mass, identified as pilocytic astrocytoma in both cases. The first case was lost to follow up. The latter underwent partial resection of the tumor and received radiotherapy postoperatively. He gradually gained in weight and height. CONCLUSION: DS should be a differential diagnosis in any children with emaciation despite adequate caloric intake and an inappropriately euphoric mood. Awareness of this syndrome, careful history taking, general detail as well as neurological examination including fundoscopic examination and appropriated investigations are crucial.

Síndrome de Russell o emaciación diencefálica, primer caso reportado en la literatura dominicana / Russellïs Syndrome or diencephalic emaciation: first case reported in the Dominican literature

Se reporta un caso de emaciación diencefálica debido a un glioma en el area supraselar que obstruía los agujeros de Monro en una niña de 6 años que se presentó con un cuadro de pérdida de peso, concomitantemente con una pérdida de la visión. El tumor fue removido parcialmente y la niña falleció tres semanas después de complicaciones pulmonares. En nuestra revisión de la literatura nacional, no hemos encontrado ningún caso reportado de esta patología

Síndrome diencefálico con emaciación infantil: informe de un caso / Diencephalic syndrome with infantile emaciation: informe of a case

Se describe el curso clínico de una preescolar de tres años con signos típicos de síndrome de emanciación quien era portadora de un astrocitoma fibrilar de ubicación intra y supra sellar

Síndrome de Russell: relato de dois casos / Russell's syndrome: report of 2 cases

Säo apresentados dois pacients portadores de tumores diencefálicos (teratoma indiferenciado e glioma pilocistico) que vieron a desenvolver síndrome de Rossell. os diagnósticos histológicos foram confirmados por biópsia estereotomográfica