Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome

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Overview

Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome is an extremely rare genetic condition that affects multiple body systems. The name describes its main features: polyneuropathy (damage to nerves throughout the body, especially in the hands and feet), intellectual disability (difficulty with learning and thinking), acromicria (unusually small hands and feet), and premature menopause (early loss of menstrual periods and fertility in women, typically before age 40). This syndrome belongs to a group of conditions classified under multiple congenital anomalies. People with this condition may experience numbness, tingling, or pain in their hands and feet due to nerve damage. They may also have learning difficulties that range from mild to moderate. The small size of the hands and feet is usually noticeable from childhood. Women with this syndrome often experience early menopause, which can affect their ability to have children and may lead to other hormonal changes such as bone thinning. Because this syndrome is so rare, there is no specific cure or targeted treatment available. Management focuses on addressing each symptom individually. This may include medications for nerve pain, educational support for intellectual disability, hormone replacement therapy for premature menopause, and regular monitoring by a team of specialists. Early diagnosis and coordinated care can help improve quality of life for affected individuals.

Also known as:

Key symptoms:

Numbness or tingling in hands and feetPain in the hands and feetMuscle weakness in the arms and legsUnusually small handsUnusually small feetIntellectual disability or learning difficultiesEarly menopause in women (before age 40)Reduced fertilityDifficulty with balance or coordinationShort statureHot flashes and hormonal changes related to early menopauseDifficulty with fine motor tasks

Clinical phenotype terms (15)— hover any for plain English
Ulnar deviation of fingerHP:0009465Furrowed tongueHP:0000221
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 Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome.

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No actively recruiting trials found for Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome at this time.

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No specialists are currently listed for Polyneuropathy-intellectual disability-acromicria-premature menopause 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 Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What tests do we need to confirm this diagnosis?,Is genetic testing available to identify the exact cause in our family?,What treatments can help manage the nerve pain?,Should hormone replacement therapy be started, and what are the risks and benefits?,What educational or developmental support services should we look into?,How often should we schedule follow-up appointments with each specialist?,Are there any clinical trials or research studies we could participate in?

Common questions about Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome

What is Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome?

Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome is an extremely rare genetic condition that affects multiple body systems. The name describes its main features: polyneuropathy (damage to nerves throughout the body, especially in the hands and feet), intellectual disability (difficulty with learning and thinking), acromicria (unusually small hands and feet), and premature menopause (early loss of menstrual periods and fertility in women, typically before age 40). This syndrome belongs to a group of conditions classified under multiple congenital anomalies. People

How is Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome inherited?

Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome typically begin?

Typical onset of Polyneuropathy-intellectual disability-acromicria-premature menopause syndrome is childhood. Age of onset can vary across affected individuals.