Alexander disease is a rare, sporadic leukoencephalopathy characterized by white matter abnormalities with frontal predominance and clinically as a rule associated with megalencephaly, seizures, spasticity and psychomotor deterioration. We describe a boy who was diagnosed as affected by anorexia nervosa because his refusal to eat, progressive weight loss and psychological disturbances. The observation of a hyperintense lesion on T2-weighed MR images was initially explained as a pontine and extrapontine myelinolysis related to malnutrition. Following MRI and DNA analysis we diagnosed a juvenile type of Alexander disease.
Titolo: | An unusual diagnosis in a child suffering from juvenile Alexander disease: a clinical and imaging report. | |
Autore/i: | FRANZONI, EMILIO; Van der Knaap M. S; ERRANI, ALESSANDRA; COLONNELLI, MARIA CHIARA; Bracceschi R.; Malaspina E.; Moscano F. C.; GARONE, CATERINA; Sarajlija J.; Zimmerman R. A.; Salomons G. S.; BERNARDI, BRUNO | |
Autore/i Unibo: | ||
Anno: | 2006 | |
Rivista: | ||
Abstract: | Alexander disease is a rare, sporadic leukoencephalopathy characterized by white matter abnormalities with frontal predominance and clinically as a rule associated with megalencephaly, seizures, spasticity and psychomotor deterioration. We describe a boy who was diagnosed as affected by anorexia nervosa because his refusal to eat, progressive weight loss and psychological disturbances. The observation of a hyperintense lesion on T2-weighed MR images was initially explained as a pontine and extrapontine myelinolysis related to malnutrition. Following MRI and DNA analysis we diagnosed a juvenile type of Alexander disease. | |
Data prodotto definitivo in UGOV: | 2006-02-28 15:55:37 | |
Appare nelle tipologie: | 1.01 Articolo in rivista |