Cutis verticis gyrata

The underlying cause of cutis verticis gyrate (CVG) is not well understood. The condition may be divided into 3 forms, based on whether it is isolated or it's association with specific underlying conditions:

• Primary essential - refers to cases in which no other abnormality is present. In this form the cause is not known. While most of these cases appear to be sporadic, autosomal recessive and autosomal dominant inheritance have been described.
• Primary nonessential - can be associated with intellectual disability, cerebral palsy, epilepsy (seizures), schizophrenia, cranial abnormalities (microcephaly), deafness, eye or vision abnormalities, or a combination of these. In this form, the cause may have an endocrinologic basis (caused by abnormalities of the endocrine system).
• Secondary - associated with a wide variety of underlying diseases and treatments, which can be viewed here on Medscape's website. In these cases, the underlying cause depends on the associated disease or treatment.

Cutis verticis gyrata (CVG) refers to deep folds on the scalp that look similar to the folds of the brain. It occurs more commonly in males, and most commonly develops after puberty, but before age 30. It may occur alone (isolated CVG) or in association with a variety of underlying conditions or treatments, including neuropsychiatric disorders, eye abnormalities, or inflammatory conditions. While most isolated cases of unknown cause are sporadic, autosomal recessive and autosomal dominant inheritance with varying degrees of severity have been described. In cases associated with underlying conditions, the cause depends on the underlying condition. Management may include keeping areas within the folds clean, and/or surgery if requested for psychological or cosmetic reasons.

The National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Although no studies involving cutis verticis gyrata are listed at this time, you can check this site often for updates. To search for a study, use "cutis verticis gyrata" as your search term.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling 1-800-411-1222 to speak with a specialist, who can help you search for clinical trials.

Patient Recruitment and Public Liaison Office (PRPL)
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 1-800-411-1222
Fax: 301-480-9793
E-mail: prpl@mail.cc.nih.gov
Web site: http://clinicalcenter.nih.gov/

ClinicalTrials.gov provides a helpful guide for information about participating in a clinical trial. Resources for travel and lodging assistance are listed on the National Center for Advancing Translational Sciences Office of Rare Diseases Research (NCATS-ORDR) website, part of the National Institutes of Health (NIH).

We were unable to find information regarding an association between cutis verticis gyrata and epilepsy medication in our search.

The estimated prevalence of cutis verticis gyrata (with data from 1964) is up to 1 in 100,000 people in the general population. It has been estimated to occur in 0.5% (1 in 200) of people with intellectual disability in the United States.