Oculocerebral syndrome with hypopigmentation

Oculocerebral syndrome with hypopigmentation is a rare disorder in which the skin and hair lack normal color or have characteristics of albinism. This syndrome also is referred to as Cross syndrome, Cross-McKusick-Breen syndrome, or Kramer syndrome. In addition, central nervous system (CNS) involvement specific to the eyes and oculocerebral areas is present. Vision problems associated with clouding (opacities) of the cornea, involuntary eye movements or nystagmus, or decreased size of one or both eyes maybe present (National Organization for Rare Disorders, 1996). As with other forms of albinism, sensitivity to light is a frequent problem as well.


 


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