*<unk>  Type I, the "classic" form, is characterized by normal fetal growth with the onset of abnormalities in the first two years of life. Vision and hearing gradually decline. The central and peripheral nervous systems progressively degenerate until death in the first or second decade of life as a result of serious neurological degradation. Cortical atrophy is less severe in<unk>  Type I. *<unk>  Type II is present from birth (congenital) and is much more severe than<unk>  Type 1. It involves very little neurological development after birth. Death usually occurs by age seven. This specific type has also been designated as cerebro-oculo-facio-skeletal (COFS) syndrome or Pena-Shokeir syndrome Type II. COFS syndrome is named so due to the effects it has on the brain, eyes, face, and skeletal system, as the disease frequently causes brain atrophy, cataracts, loss of fat in the face, and osteoporosis. COFS syndrome can be further subdivided into several conditions (COFS types 1, 2, 3 (associated with xeroderma pigmentosum) and 4). Typically patients with this early-onset form of the disorder show more severe brain damage, including reduced myelination of white matter, and more widespread calcifications, including in the cortex and basal ganglia. *<unk>  Type III, characterized by late-onset, is typically milder than Types I and II. Often patients with Type III will live into adulthood. * Xeroderma pigmentosum-Cockayne syndrome (XP-CS) occurs when an individual also suffers from xeroderma pigmentosum, another DNA repair disease. Some symptoms of each disease are expressed. For instance, freckling and pigment abnormalities characteristic of XP are present. The neurological disorder, spasticity, and underdevelopment of sexual organs characteristic of<unk>  are seen. However, hypomyelination and the facial features of typical<unk>  patients are not present.
Cockayne syndrome
Xeroderma pigmentosum
ATR-X syndrome
ATR-16 syndrome
Cornelia de Lange syndrome
MEDNIK syndrome
Treacher Collins syndrome
LIG4 syndrome