Other Names for this Disease
- Optic atrophy, infantile hereditary, Behr complicated form of
- Optic atrophy in early childhood, associated with ataxia, spasticity, mental retardation, and posterior column sensory loss
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optic atrophy, ataxia, and spasticity. Other signs and symptoms may be present and vary from person to person. Although the exact cause is unknown, the syndrome is believed to be genetic and inherited in an autosomal recessive fashion, in most cases. Autosomal dominant inheritance has been reported in one family. Treatment depends on the specific signs and symptoms seen in the patient.Behr syndrome is a disorder mainly characterized by early-onset
Last updated: 7/15/2010
- Marzan KAB, Barron TF. MRI Abnormalities in Behr Syndrome. Pediatr Neurol. 1994;
- Schramm P, Scheihing M, Rasche D, Tronnier VM. Behr syndrome variant with tremor treated by VIM stimulation. Acta Neurochir. 2005;
- Copeliovitch L, Katz K, Arbel N, Harries N, Bar-On E, Soudry M. Musculoskeletal Deformities in Behr Syndrome. Journal of Pediatric Orthopaedics. 2001;
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- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Behr syndrome. Click on the link to view a sample search on this topic.