Synonyms|Editors|Languages|Comments|Home|Sponsors|Health Information
 
  Septo-Optic Dysplasia 
Synonym(s):  de Morsier's Syndrome
Reviewed  07-01-2001  

Get Web page suited for printing
Email this to a friend or colleague
Studies with patients

Table of Contents (click to jump to sections)

What is Septo-optic Dysplasia?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations

What is Septo-optic Dysplasia?
Septo-optic dysplasia (SOD) is a rare disorder characterized by abnormal development of the optic disk, pituitary deficiencies, and often agenesis (absence) of the septum pellucidum (the part of the brain that separates the anterior horns or the lateral ventricles of the brain). Symptoms may include blindness in one or both eyes, pupil dilation in response to light, nystagmus (a rapid, involuntary to-and-fro movement of the eyes), inward and outward deviation of the eyes, hypotonia (low muscle tone), and hormonal problems. Seizures may also occur. In a few cases, jaundice (prolonged yellow skin discoloration) may occur at birth. Intellectual problems vary in severity among individuals. While some children with SOD have normal intelligence, others have learning disabilities and mental retardation. Most, however, are developmentally delayed due to vision impairment or neurological problems.

Is there any treatment?
Treatment for SOD is symptomatic. Hormone deficiencies may be treated with hormone replacement therapy. The optical problems associated with SOD are generally not treatable. Vision, physical, and occupational therapies may be required.

What is the prognosis?
The prognosis for individuals with SOD varies according to the presence and severity of symptoms.

What research is being done?
The NINDS supports and conducts neurogenetic research which focuses on identifying and studying the genes involved in normal brain development. The knowledge gained from these fundamental studies provides the foundation for understanding how this process can go awry and, thus, may eventually give clues to understanding disorders such as SOD.

Select this link to view a list of studies currently seeking patients.

 Organizations

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
http://www.marchofdimes.com
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

National Eye Institute (NEI)
National Institutes of Health
Bldg. 31, Rm. 6A32
Bethesda, MD 20892-2510
2020@b31.nei.nih.gov
http://www.nei.nih.gov
Tel: 301-496-5248 Professionals 800-869-2020

National Institute of Child Health and Human Development (NICHD)
National Institutes of Health
Bldg. 31, Rm. 2A32
Bethesda, MD 20892-2425
NICHDClearinghouse@mail.nih.gov
http://www.nichd.nih.gov
Tel: 301-496-5133 800-370-2943

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
National Institutes of Health
Bldg. 31, Rm. 9A04
Bethesda, MD 20892-2560
http://www.niddk.nih.gov
Tel: 301-496-3583

FOCUS Families
2453 Emerald Street
San Diego, CA 92109
support@focusfamilies.org
http://www.focusfamilies.org
Tel: 866-FOCUS 50 (866-362-8750)
Fax: 866-362-8750


NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.


Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892




Return to top


Home | About NINDS | Disorders | Funding | News & Events | Find People | Jobs & Training | Accessibility

H H S Logo - link to U. S. Department of Health and Human Services     N I H logo - link to U. S. National Institutes of Health    N I N D S logo - link to National Institute of Neurological Disorders and Stroke    FirstGov Logo - link To FirstGov