Menke's Kinky Hair Syndrome

Many neurodegenerative disorders involving either white matter or grey matter have ocular manifestations in the form of optic atrophy or retinal degeneration. Menkes disease is a progressive neurodegenerative condition inherited as a X-linked recessive trait with disordered copper metabolism. Ocular involvement in Menkes disease is not only neuronal, it also involves blood vessels,anterior segment and vitreous. We present an infant with Menkes disease with such an ocular involvement.