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August 1958

Pelizaeus-Merzbacher Disease: A Clinical Study

Author Affiliations


From the Neurological Unit of the Boston City Hospital, the Department of Neurology, Harvard Medical School, and the Pearl Memorial Unit, Peter Bent Brigham Hospital.

AMA Arch NeurPsych. 1958;80(2):162-169. doi:10.1001/archneurpsyc.1958.02340080032004

In 1885 Pelizaeus1 described a family affected by a slowly progressive hereditary form of cerebral diplegia characterized by the early development of nystagmus, ataxia, and spasticity. Merzbacher,2 in 1907, examined pathologically a brain from a relative of the same family who had suffered from the illness which Pelizaeus described. Spielmeyer,3 in 1913, and Liebers,4 in 1928, studied another brain from this family. There have been isolated reports of individual cases and families in which a similar neurological disorder has been found, but the family reported by Pelizaeus and Merzbacher has been the only one in which the disease has extended back over a number of generations. My associates and I have recently encountered another large family that we feel is worth while recording because of its size and similarity to that of the original report. The time of onset and the symptomatology, as well as the

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