Polyendocrine-polyneuropathy syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:453533OMIM:616113E34.8
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Polyendocrine-polyneuropathy syndrome is an extremely rare condition that affects multiple hormone-producing glands (the endocrine system) and the peripheral nerves (the nerves outside the brain and spinal cord). Because it involves both endocrine and nerve problems, patients may experience a wide range of symptoms. The endocrine component can lead to problems with glands such as the thyroid, adrenal glands, or pancreas, causing hormonal imbalances. These imbalances may result in fatigue, weight changes, blood sugar problems, or other metabolic issues. The polyneuropathy component means that nerves in the arms, legs, and sometimes other parts of the body do not work properly, which can cause numbness, tingling, pain, or weakness in the hands and feet. Because this syndrome is so rare, the medical understanding of it is still evolving. Treatment is generally focused on managing each individual symptom — replacing missing hormones, controlling pain, and supporting nerve function. A team of specialists is usually needed to provide comprehensive care. There is currently no single cure, and management is tailored to each patient's specific combination of endocrine and nerve problems.

Key symptoms:

Numbness or tingling in hands and feetMuscle weakness in the arms or legsFatigue and low energyUnexplained weight changesBlood sugar problems (too high or too low)Thyroid gland dysfunctionAdrenal gland insufficiencyBurning or shooting nerve painDifficulty with balance or coordinationSensitivity to cold or heatDigestive problemsDizziness upon standingSexual dysfunction or menstrual irregularities

Clinical phenotype terms (23)— hover any for plain English
Progressive hearing impairmentHP:0001730Demyelinating peripheral neuropathyHP:0007108Decreased circulating follicle stimulating hormone concentrationHP:0030341Decreased circulating luteinizing hormone levelHP:0030344Decreased serum testosterone concentrationHP:0040171EMG: slow motor conductionHP:0100287
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Polyendocrine-polyneuropathy syndrome.

View clinical trials →

No actively recruiting trials found for Polyendocrine-polyneuropathy syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Polyendocrine-polyneuropathy syndrome community →

No specialists are currently listed for Polyendocrine-polyneuropathy 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 Polyendocrine-polyneuropathy syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Polyendocrine-polyneuropathy syndromeForum →

No community posts yet. Be the first to share your experience with Polyendocrine-polyneuropathy syndrome.

Start the conversation →

Latest news about Polyendocrine-polyneuropathy syndrome

No recent news articles for Polyendocrine-polyneuropathy syndrome.

Follow this condition to be notified when news becomes available.

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.Which of my endocrine glands are affected, and what hormone replacements do I need?,How severe is my nerve damage, and is it likely to get worse over time?,What medications can help manage my nerve pain?,Should I undergo genetic testing to look for an underlying cause?,What are the warning signs of an adrenal crisis, and what should I do in an emergency?,How often should I have my hormone levels and nerve function checked?,Are there any clinical trials or research studies I could participate in?

Common questions about Polyendocrine-polyneuropathy syndrome

What is Polyendocrine-polyneuropathy syndrome?

Polyendocrine-polyneuropathy syndrome is an extremely rare condition that affects multiple hormone-producing glands (the endocrine system) and the peripheral nerves (the nerves outside the brain and spinal cord). Because it involves both endocrine and nerve problems, patients may experience a wide range of symptoms. The endocrine component can lead to problems with glands such as the thyroid, adrenal glands, or pancreas, causing hormonal imbalances. These imbalances may result in fatigue, weight changes, blood sugar problems, or other metabolic issues. The polyneuropathy component means that n

How is Polyendocrine-polyneuropathy syndrome inherited?

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