Behr's syndrome

From Wikipedia, the free encyclopedia

Behr's syndrome, also known as Behr's disease, is a genetic disorder that results in a spectrum of optic and neurological complications for both sexes. The disorder begins from early childhood with disturbance to vision, and loss or reduction in body control and co-ordination.

It includes a partial and increasing loss of vision and/or blind spot in previously normal sight; eyesight degeneration is particularly prevalent in males. Symptoms can include rapid involuntary eye movements (nystagmus), progressive damage to nerves, nerve inflammation and unusual foot reflexes when the sole is stimulated (positive Babinski sign).

The syndrome is named after Carl Behr, who first identified it. The syndrome is recessive and is not linked to sex chromosomes.

[edit] See also

• List of systemic diseases with ocular manifestations