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February 1968

Demyelinating Leukodystrophy With Total Cortical Cerebellar Atrophy

Author Affiliations

From Children's Hospital (Dr. Thulin); Department of Pathology, General Rose Memorial Hospital (Drs. McTaggart and Neubuerger); and University of Colorado School of Medicine (Drs. Thulin and Neubuerger), Denver.

Arch Neurol. 1968;18(2):113-122. doi:10.1001/archneur.1968.00470320015001

THE classification of leukodystrophy in childhood is still complicated and unsettled. This report will attempt to contribute to the clinical and pathological classification and to describe an unusual combination of diffuse sclerosis of the white matter with total cerebellar atrophy.

Report of a Case  Family History.—The patient, a boy, was born on May 8, 1962, after an uneventful nine-month gestation. This was his mother's second pregnancy, her first pregnancy having resulted in an abortion at six weeks gestation. The mother was 23 years old and the father, 27 years of age. Both parents are of Anglo-Saxon ancestry and have no siblings. There is no history of a similar disorder in the family. There have been no additional pregnancies.Clinical Course.—The patient was delivered after ten hours of spontaneous labor. Membranes ruptured two to three hours before delivery. The mother was not awake for the actual delivery. The

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