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


METHODS
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.


METHODS
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.


CONCLUSIONS
Although extremely rare, DS can occur in an adult harboring a tumor in the anterior hypothalamus.
Miyoshi
Yasuyuki
Y
Department of Neurological Surgery, Sumitomo Besshi Hospital, Niihama, Japan. ya-miyo@dokkyomed.ac.jp
Yunoki
Masatoshi
M
Yano
Akimasa
A
Nishimoto
Ken
K
D002551:Cerebral Ventricle Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000532:radiotherapy / Q000601:surgery; D003131:Combined Modality Therapy; D003397:Craniopharyngioma / Q000150:complications* / Q000175:diagnosis / Q000532:radiotherapy / Q000601:surgery; D003937:Diagnosis, Differential; D004614:Emaciation / Q000209:etiology*; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007027:Hypothalamic Diseases / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010911:Pituitary Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000532:radiotherapy / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D020542:Third Ventricle / Q000473:pathology / Q000601:surgery
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I