Unilateral megalocornea in lamellar ichthyosis.

The case report of a 16-month-old girl with lamellar ichthyosis and unilateral megalocornea is presented. This is a previously unrecorded association and the first report of a unilateral megalocornea in a female.

Familial spinocerebellar degeneration with corneal dystrophy.

We report on two sisters born to normal but consanguineous parents, with the unusual combination of spinocerebellar degeneration and corneal dystrophy. Their manifestations include mental subnormality, bilateral corneal opacification starting in the second year of life and leading to severe visual impairment, and slowly progressive cerebellar abnormalities with variable dorsal column and upper motor neuron involvement. A third sister had only minor spinocerebellar signs but no eye findings, and three other sibs were completely normal.