Behr's syndrome

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Behr's syndrome (a.k.a. Behr's disease) is a hereditary disorder named after Carl Behr, who first described it in 1909. It has an autosomal recessive inheritance, although heterozygotes may still manifest much attenuated symptoms.

Behr's syndrome 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), ataxia, 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.

See also

• List of systemic diseases with ocular manifestations

fi:Behrin oireyhtymä

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .