[Diencephalic syndrome. Case report and review of literature].
We report the case of a twenty-three month old, normally eating child, affected by organic macies and severe dystrophy. After the negative response of blood and laboratory examinations let us exclude a malabsorption syndrome, only the performance of neuro-radiologic exams showed evidence of a subthalamic tumor as the cause of a "Diencephalic syndrome". Diencephalic syndrome or Russel's syndrome is a diencephalic tumor induced disease, which sets in the first three years of life. The disease clinical markers are a severe emaciation with appetite preservation and absence or very scarce evidence of any telltale neurological sign. the tumoral histo-pathology most frequently shows a low grade of malignancy astrocytoma, whose eradication is very often difficult because of the particular anatomic site. Choice treatment includes an excisional biopsy associated to chemotherapy and to radiotherapy.
Baracchini
A
A
Istituto di Clinica Pediatrica, Università degli Studi di Pisa.
Chiaravalloti
G
G
Del Pistoia
M
M
Ceccarelli
M
M
D001254:Astrocytoma / Q000175:diagnosis* / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology*; D004027:Diencephalon / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008286:Malabsorption Syndromes / Q000175:diagnosis*; D008297:Male; D011859:Radiography
Sindrome diencefalica. Segnalazione di un caso e revisione della letteratura.