Alopecia-hypogonadism-extrapyramidal syndrome

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Overview

Alopecia-hypogonadism-extrapyramidal syndrome is an extremely rare genetic condition that affects several body systems at once. The name describes its three main features: alopecia (hair loss), hypogonadism (reduced function of the sex glands, meaning the ovaries or testes do not produce enough hormones), and extrapyramidal symptoms (movement problems caused by issues in certain parts of the brain that help control movement). People with this condition typically experience progressive hair loss that can begin in childhood or adolescence, delayed or incomplete puberty due to low levels of sex hormones, and involuntary movements or difficulty coordinating movements similar to what is seen in conditions like Parkinson's disease or dystonia. Some affected individuals may also have intellectual disability or learning difficulties. Because this syndrome is so rare, with only a handful of cases described in the medical literature, there is limited information about the full range of symptoms and the best approaches to treatment. Management is currently focused on treating each symptom individually, such as hormone replacement therapy for hypogonadism and medications to help control movement problems. There is no cure at this time, and care typically involves a team of specialists working together.

Also known as:

Key symptoms:

Hair loss or thinning hair (alopecia)Delayed or absent pubertyLow levels of sex hormonesInvoluntary movements (such as tremors or jerky motions)Stiffness or rigidity of musclesDifficulty with coordination and balanceIntellectual disability or learning difficultiesReduced fertilitySlow or abnormal movementsPossible mental health changes

Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Alopecia-hypogonadism-extrapyramidal syndrome.

View clinical trials →

No actively recruiting trials found for Alopecia-hypogonadism-extrapyramidal syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Alopecia-hypogonadism-extrapyramidal syndrome community →

No specialists are currently listed for Alopecia-hypogonadism-extrapyramidal syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Alopecia-hypogonadism-extrapyramidal syndrome.

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Community

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Latest news about Alopecia-hypogonadism-extrapyramidal syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What specific tests should be done to confirm this diagnosis?,Should we pursue whole exome or whole genome sequencing to look for a genetic cause?,What hormone replacement options are available and when should they be started?,What medications can help with the movement problems, and what are their side effects?,Are there any clinical trials or research studies we could participate in?,How often should we schedule follow-up visits with each specialist?,What support services are available for emotional and educational needs?

Common questions about Alopecia-hypogonadism-extrapyramidal syndrome

What is Alopecia-hypogonadism-extrapyramidal syndrome?

Alopecia-hypogonadism-extrapyramidal syndrome is an extremely rare genetic condition that affects several body systems at once. The name describes its three main features: alopecia (hair loss), hypogonadism (reduced function of the sex glands, meaning the ovaries or testes do not produce enough hormones), and extrapyramidal symptoms (movement problems caused by issues in certain parts of the brain that help control movement). People with this condition typically experience progressive hair loss that can begin in childhood or adolescence, delayed or incomplete puberty due to low levels of sex h

How is Alopecia-hypogonadism-extrapyramidal syndrome inherited?

Alopecia-hypogonadism-extrapyramidal syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Alopecia-hypogonadism-extrapyramidal syndrome typically begin?

Typical onset of Alopecia-hypogonadism-extrapyramidal syndrome is childhood. Age of onset can vary across affected individuals.