Unusual diagnosis in a child suffering from juvenile Alexander disease: clinical and imaging report |
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Authors: | Franzoni Emilio Van der Knaap Marjo S Errani Alessandra Colonnelli Maria Chiara Bracceschi Roberta Malaspina Elisabetta Moscano Filomena Caterina Garone Caterina Sarajlija Jasenka Zimmerman Robert A Salomons Gajja S Bernardi Bruno |
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Institution: | Child Neuropsychiatry Unit, Department of Pediatrics, University of Bologna, Bologna, Italy. emilio.franzoni@unibo.it |
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Abstract: | Alexander disease is a rare, sporadic leukoencephalopathy characterized by white-matter abnormalities with frontal predominance and, as a rule, clinically associated with megalencephaly, seizures, spasticity, and psychomotor deterioration. We describe a boy who was diagnosed as affected by anorexia nervosa because of his refusal to eat, progressive weight loss, and psychologic disturbances. The observation of a hyperintense lesion on T(2)-weighed magnetic resonance images (MRIs) 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. Therefore, we can affirm the importance of the history and clinical examination to look for brainstem dysfunction in patients presenting with atypical anorexia nervosa. |
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