Cutis verticis gyrata

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Skin Diseases

Summary Summary

Cutis verticis gyrata (CVG) refers to deep folds on the scalp that look similar to the folds of the brain.^[1]^[2] 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.^[1]^[2] 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.^[1]

Last updated: 7/27/2017

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Nevus	Mole	0003764
Seizure		0001250
Thickened skin	Thick skin	0001072
30%-79% of people have these symptoms
Intellectual disability	Mental deficiency Mental retardation Mental retardation, nonspecific Mental-retardation [ more ]	0001249
Microcephaly	Abnormally small skull Decreased circumference of cranium Decreased size of skull Reduced head circumference Small head circumference [ more ]	0000252
5%-29% of people have these symptoms
Aplasia/Hypoplasia of the cerebellum	Absent/small cerebellum Absent/underdeveloped cerebellum [ more ]	0007360
Aplasia/Hypoplasia of the corpus callosum		0007370
Cataract	Clouding of the lens of the eye Cloudy lens [ more ]	0000518
Cerebral cortical atrophy	Decrease in size of the outer layer of the brain due to loss of brain cells	0002120
EEG abnormality		0002353
Nystagmus	Involuntary, rapid, rhythmic eye movements	0000639
Strabismus	Cross-eyed Squint Squint eyes [ more ]	0000486

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Last updated: 2/1/2021

Cause Cause

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:^[1]

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.

Last updated: 7/27/2017

Statistics Statistics

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.^[1]

Last updated: 7/27/2017

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
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.
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 Cutis verticis gyrata. Click on the link to view a sample search on this topic.

News & Events News & Events

News

Rare Disease Day at NIH 2021
March 1, 2021

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

Has there been any research into the reasons why persons with brain injury, seizures, schizophrenia would have cutis verticis gyrata? Has there been any reports of cutis verticis gyrata associated with long-term use of carbamazepine in young men? See answer

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