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NEUROLOGY 1976;26:607
© 1976 American Academy of Neurology

Alexander's disease

A report and reappraisal LOUIS S. RUSSO, JR., M.D., ALAN ARON, M.D. and PAUL J. ANDERSON, M.D.

From the Departments of Neurology (Dr. Russo), Pathology—Division of Neuropathology (Dr. Anderson), and Pediatrics—Division of Neurology (Dr. Aron) of the Mount Sinai School of Medicine of the City University of New York.

A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination. This may be the first report of a case with significant neuronal changes and inflammation within the brain stem in this disease. The clinical picture of this entity is varied and age-dependent. Alexander's disease may be a motor system specific entity secondary to astrocytic dysfunction.

All correspondence and requests for reprints should be addressed to Dr. Russo, 3599 University Boulevard, South, Suite 601, Jacksonville, FL 32216.

This investigation was supported in part by a training grant in neurology from the National Institute of Neurologic Disease and Stroke NS 05072 and by the Jodi Wollman Research Fund.

Received for publication September 24, 1975.




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