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EnglishAlexander 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 |
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