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Neuropediatrics 2000; 31(2): 86-92
DOI: 10.1055/s-2000-7479
Original Article

Georg Thieme Verlag Stuttgart · New York

Alexander Disease - Classification Revisited and Isolation of a Neonatal Form

S. Springer1 , Rita Erlewein2 , T. Naegele3 , Ingrid Becker4 , Dorothee Auer5 , W. Grodd3 , Ingeborg Krägeloh-Mann2
  • 1 Children's Outpatient Clinic of the Ludwig-Maximilians-University, München, Germany
  • 2 Department of Child Neurology, University Children's Hospital Tübingen, Germany
  • 3 Department of Neuroradiology, University of Tübingen, Germany
  • 4 Department of Pathology, Klinikum Rechts der Isar, Technical University of München, Germany
  • 5 NMR Study Group, Max-Planck-Institute of Psychiatry, München, Germany
Further Information

Publication History

Publication Date:
31 December 2000 (online)

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Alexander disease is usually classified according to the age of onset, e.g. an infantile form with onset during the first two years of life, a juvenile form with onset in childhood, mainly school age. It has been recognized, however, that the clinical course can be very variable within these groups. Thus, this clinical classification is not a useful predictor of severity and progression of the disease.

This is demonstrated here on the basis of the history of seven own patients and a literature review.

Only an onset in very early infancy, during the neonatal period, seemed to be associated with a rather uniform pattern of disease course, often leading to early death. This neonatal form showed very stereotyped symptoms, in part different from later onset: Early, often intractable, generalized seizures; hydrocephalus with raised intracranial pressure due to aqueductal stenosis because of pathological astroglia proliferation; lack of developmental progression but without prominent spasticity or ataxia; elevated CSF protein content. This was associated with the well-established neuroradiological findings, e.g. severe white matter affection with fronto-temporal predominance, involvement of basal ganglia and periventricular enhancement as an obligatory symptom. The identification of this early onset form is especially important as seizures and signs of raised intracranial pressure may mislead the diagnosis.

Key words

Alexander disease - Neonatal onset - Magnetic resonance imaging - Hydrocephalus

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M. D. Prof. Dr. Ingeborg Krägeloh-Mann

Dept. of Child Neurology University Children's Hospital

Hoppe-Seyler-Str. 1

72076 Tübingen

Germany