Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report

Abstract

Objective and importance: Diencephalic syndrome of emaciation (DS) is seen almost exclusively in infants and young children, and only two cases of DS in adults have been reported previously. We describe a case of DS associated with a third ventricle intrinsic craniopharyngioma in an adult patient.

Clinical presentation: A 54-year-old man presented with profound emaciation, disorientation, memory loss, and psychological disorders. Computed tomographic scanning and magnetic resonance imaging of his brain disclosed a tumor within the third ventricle. The preoperative endocrinological examination indicated an elevated growth hormone level and a decreased somatomedin C level.

Intervention: The patient underwent partial resection of the tumor, which was adherent to the floor of the third ventricle but not to the ventricle wall, by an interhemispheric-transcallosal approach. The histological examination revealed a squamous papillary-type craniopharyngioma. The patient received 50 Gy of radiotherapy, which resulted in 90 to 95% reduction of the tumor size within 6 months, as indicated by the magnetic resonance images presented. The psychological disorders, memory disturbance, and severe emaciation improved gradually thereafter.

Conclusion: Although extremely rare, DS can occur in an adult harboring a tumor in the anterior hypothalamus.