Pelizaeus-Merzbacher Disease

Abstract
In 1885 Pelizaeus1described a family affected by a slowly progressive hereditary form of cerebral diplegia characterized by the early development of nystagmus, ataxia, and spasticity. Merzbacher,2in 1907, examined pathologically a brain from a relative of the same family who had suffered from the illness which Pelizaeus described. Spielmeyer,3in 1913, and Liebers,4in 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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