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Unusual Diagnosis in a Child Suffering From Juvenile Alexander Disease: Clinical and Imaging Report

Child Neuropsychiatry Unit Department of Pediatrics University of Bologna Bologna, Italy; franzoni{at}unibo.it

Marjo S. Van der Knaap, MD

Department of Child Neurology VU University Medical Center Amsterdam, the Netherlands

Alessandra Errani, MD

Maria Chiara Colonnelli, MD

Roberta Bracceschi, MD

Elisabetta Malaspina, MD

Filomena Caterina Moscano, MD

Caterina Garone, MD

Jasenka Sarajlija, MD

Child Neuropsychiatry Unit Department of Pediatrics University of Bologna Bologna, Italy

Robert A. Zimmerman, MD

Department of Radiology Children's Hospital Philadelphia, Pennsylvania

Gajja S. Salomons, PhD

Metabolic Unit Department of Clinical Chemistry VU University Medical Center Amsterdam, the Netherlands

Bruno Bernardi, MD

Department of Neuroradiology Bellaria Hospital, Bologna, Italy

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 T2-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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