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Alexander's Disease: Clues to Diagnosis

Department of Paediatric Neurology, The Hospital for Sick Children, Great Ormond Street, London, England

Michael Baraitser, FRCP

Department of Clinical Genetics, The Hospital for Sick Children, Great Ormond Street, London, England

Brian Harding, DPhil, MRCPath

Department of Histopathology, The Hospital for Sick Children, Great Ormond Street, London, England

Stewart G. Boyd, MD

Department of Clinical Neurophysiology, The Hospital for Sick Childrne, Great Ormond Street, London, England

Brian Kendall, FRCR, FRCS, FRCP

Department of Radiology The Hospital for Sick Children, Great Ormond Street, London, England

Edward M. Brett, FRCP

Department of Paediatric Neurology, The Hospital for Sick Children, Great Ormond Street, London, England

The clinical, radiologic, neurophysiologic, and pathologic findings in 10 children with histologically proven Alexander's disease are described, and the presence of two broad clinical subgroups is confirmed. Macrocephaly, regression, and seizures are found in the infantile form, whereas bulbar signs predominate the so-called juvenile form. None of the clinical or neurophysiologic findings is pathognomonic, and radiologic features suggesting the diagnosis are not present in all cases. However, the combination of abnormalities may suggest the diagnosis and justify histologic confirmation. (J Child Neurol 1993;8:134-144).

Journal of Child Neurology, Vol. 8, No. 2, 134-144 (1993)
DOI: 10.1177/088307389300800205


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