Synonyms|Editors|Languages|Comments|Home|Sponsors|Health Information
 
  Lissencephaly 
  
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 Lissencephaly?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations

What is Lissencephaly?
Lissencephaly, which literally means "smooth brain," is a rare brain formation disorder characterized by the lack of normal convolutions (folds) in the brain, and an abnormally small head (microcephaly). It is caused by defective neuronal migration, the process in which nerve cells move from their place of origin to their permanent location. Symptoms of the disorder may include unusual facial appearance, difficulty swallowing, failure to thrive, and severe psychomotor retardation. Anomalies of the hands, fingers, or toes, muscle spasms, and seizures may also occur. Lissencephaly may be associated with other diseases including isolated lissencephaly sequence, Miller-Dieker syndrome, and Walker-Warburg syndrome.

Is there any treatment?
The severe malformations of the brain in lissencephaly most likely will not respond to treatment. Normal supportive care may be needed to help with comfort and nursing needs. Seizures may be controlled with medication. Progressive hydrocephalus (an excessive accumulation of cerebrospinal fluid in the brain) may require shunting. If feeding becomes difficult, a gastrostomy tube may be considered.

What is the prognosis?
The prognosis for children with lissencephaly is variable depending on the degree of brain malformation. Many individuals show no significant development beyond a 3- to 5-month-old level. Some may have near-normal development and intelligence. Many will die before the age of 2. Aspiration and respiratory disease are the most common causes of death.

What research is being done?
The NINDS conducts and supports a wide range of studies that explore the complex systems of normal brain development, including neuronal migration. The knowledge gained from these studies provides the foundation for understanding how this process can go awry and, thus, offers hope for new means to treat and prevent neuronal migration disorders such as lissencephaly.

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

 Organizations

Lissencephaly Network
10408 Bitterroot Court
Ft. Wayne, IN 46804
lissnet@lissencephaly.org
http://www.lissencephaly.org
Tel: 219-432-4310
Fax: 219-432-4310

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


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