Pelizaeus-Merzbacher Syndrome: Sudanophilic
leukodystrophy or leukoencephalopathy, which usually affects only males. It
is a chronic disease of the central nervous system with onset early in the infancy period,
and which might persist for decades. It is characterized by rotary nystagmus,
ataxia, intention tremor, spasticity, and dementia. Autosomal recessive
inheritance. There is a second, congenital, autosomal recessive form with
late onset (adulthood) associated with a faster course marked by
photosensitivity of the skin, dwarfism, cerebellar ataxia, corticospinal
signs, cataracts, deafness, and retinitis pigmentosa.