An infant with hyperalertness, hyperkinesis, and failure to thrive: a rare diencephalic syndrome due to hypothalamic anaplastic astrocytoma.
BACKGROUND
Diencephalic Syndrome is a rare clinical condition of failure to thrive despite a normal caloric intake, hyperalertness, hyperkinesis, and euphoria usually associated with low-grade hypothalamic astrocytomas.


METHODS
We reported an unusual case of diencephalic cachexia due to hypothalamic anaplastic astrocytoma (WHO-grade III). Baseline endocrine function evaluation was performed in this patient before surgery. After histological diagnosis, he enrolled to a chemotherapy program with sequential high-dose chemotherapy followed by hematopoietic stem cell rescue. The last MRI evaluation showed a good response. The patient is still alive with good visual function 21 months after starting chemotherapy.


CONCLUSIONS
Diencephalic cachexia can rarely be due to high-grade hypothalamic astrocytoma. We suggest that a nutritional support with chemotherapy given to high doses without radiotherapy could be an effective strategy for treatment of a poor-prognosis disease.
Neuro-oncology Unit, Department of Paediatric Medicine, Anna Meyer Children's University Hospital and Department of Health Sciences, University of Florence, Florence, Italy. alessia.stival@gmail.com.
Alessia
A
Stival
Neuro-oncology Unit, Department of Paediatric Medicine, Anna Meyer Children's University Hospital and Department of Health Sciences, University of Florence, Florence, Italy. maurizio.lucchesi@tin.it.
Maurizio
M
Lucchesi
Neuro-oncology Unit, Department of Paediatric Medicine, Anna Meyer Children's University Hospital and Department of Health Sciences, University of Florence, Florence, Italy. silvia.farina@meyer.it.
Silvia
S
Farina
Pathology Unit, Anna Meyer Children's University Hospital, Florence, Italy. annamaria.buccoliero@unifi.it.
Anna Maria
AM
Buccoliero
Pathology Unit, Anna Meyer Children's University Hospital, Florence, Italy. francesca.castiglione@unifi.it.
Francesca
F
Castiglione
Neurosurgery Unit, Department of Neurosciences, Anna Meyer Children's University Hospital, Florence, Italy. lorenzo.genitori@meyer.it.
Lorenzo
L
Genitori
Neuro-oncology Unit, Department of Paediatric Medicine, Anna Meyer Children's University Hospital and Department of Health Sciences, University of Florence, Florence, Italy. maurizio.demartino@unifi.it.
Maurizio
M
de Martino
Neuro-oncology Unit, Department of Paediatric Medicine, Anna Meyer Children's University Hospital and Department of Health Sciences, University of Florence, Florence, Italy. iacopo.sardi@meyer.it.
Iacopo
I
Sardi
D001254:Astrocytoma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D002100:Cachexia / Q000209:etiology*; D005183:Failure to Thrive / Q000209:etiology*; D006801:Humans; D006948:Hyperkinesis / Q000209:etiology*; D007029:Hypothalamic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D007223:Infant; D008297:Male