Pelizaeus-Merzbacher disease /pā′lētsā′o̅o̅s·merts′bä·kər/ [Friedrich Pelizaeus, German physician, 1850–1917; Ludwig Merzbacher, German physician, 1875–1942] , an X-linked leukoencephalopathy occurring in early life and running a slowly progressive course into adolescence or adulthood. It is marked by nystagmus, ataxia, tremor, choreoathetoid movements, parkinsonian facies, dysarthria, and mental deterioration. Pathologically, there is diffuse demyelination in the white substance of the brain that may involve the brainstem, cerebellum, and spinal cord. There is no cure. Treatment is symptomatic and supportive. Also called familial centrolobar sclerosis, Merzbacher-Pelizaeus disease, Pelizaeus-Merzbacher sclerosis.
