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.
An unusual diagnosis in a child suffering from juvenile Alexander disease: a clinical and imaging report.
FRANZONI, EMILIO;ERRANI, ALESSANDRA;COLONNELLI, MARIA CHIARA;GARONE, CATERINA;BERNARDI, BRUNO
2006
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.File in questo prodotto:
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