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Cerebral White-Matter Hypoplasia
Amrik S. Chattha, MD;
Edward P. Richardson, Jr, MD
Arch Neurol. 1977;34(3):137-141.
Abstract
Twelve patients, including three sisters, with lifelong severe intellectual impairment and neurologic defects were found to have generalized hypoplasia of the cerebral white matter, with corresponding enlargement of the ventricular system. Gray-matter structures were remarkably intact. Reactive cellular changes (gliosis, phagocytosis, inflammation) were absent. To our knowledge, this particular syndrome has not previously been delineated in the extensive literature on cerebral palsy. The abnormalities in these cases could result from the effects of a selectively acting tissuedamaging factor, such as has been postulated in perinatal telencephalic leukoencephalopathy, but the factors underlying the disorder are still unknown.
Author Affiliations
From the Charles S. Kubik Laboratory for Neuropathology of the James Homer Wright Pathology Laboratories, Massachusetts General Hospital, and the Departments of Neurology-Neuropathology and Pathology, Harvard Medical School, Boston.
Footnotes
Accepted for publication Nov 5, 1976.
Reprint requests to Neuropathology Laboratory, Massachusetts General Hospital, Boston, MA 02114 (Dr Richardson).
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