Cervical hypertrichosis-peripheral neuropathy syndrome

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ORPHA:2218OMIM:239840G60.0
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Overview

Cervical hypertrichosis with peripheral neuropathy is a very rare inherited condition that combines two main features: unusual hair growth on the back of the neck and upper back (called cervical hypertrichosis), and damage to the nerves outside the brain and spinal cord (called peripheral neuropathy). This condition is also sometimes referred to as cervical hypertrichosis-peripheral neuropathy syndrome or hypertrichosis-neuropathy syndrome. The nerve damage in this condition affects the nerves that control movement and sensation in the arms and legs. Over time, people may notice weakness in their muscles, reduced reflexes, and changes in how they feel touch, pain, or temperature. The extra hair growth is usually present from birth or early childhood and appears as a thick patch of hair on the back of the neck and shoulders. Because this condition is so rare, treatment focuses on managing symptoms rather than curing the underlying cause. Physical therapy and supportive care can help maintain strength and function. A team of specialists is usually needed to monitor both the nerve and skin aspects of the condition. Early diagnosis is important so that the right support can be put in place as early as possible.

Key symptoms:

Thick or excessive hair growth on the back of the neck and upper backMuscle weakness, especially in the hands and feetReduced or absent reflexes (like the knee-jerk reflex)Numbness or tingling in the arms or legsDifficulty feeling touch, pain, or temperature normallyMuscle wasting (muscles becoming smaller over time)Difficulty walking or problems with balanceFoot deformities such as high arches

Clinical phenotype terms (6)— hover any for plain English
PeriostitisHP:0040165
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cervical hypertrichosis-peripheral neuropathy syndrome.

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No actively recruiting trials found for Cervical hypertrichosis-peripheral neuropathy syndrome at this time.

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No specialists are currently listed for Cervical hypertrichosis-peripheral neuropathy syndrome.

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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 Cervical hypertrichosis-peripheral neuropathy 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 genetic tests should we do to confirm the diagnosis and find the specific gene change?,How quickly might the nerve damage progress, and what signs should we watch for?,What therapies or exercises can help maintain strength and prevent complications?,Are there any clinical trials or research studies we could participate in?,Should other family members be tested for this condition?,What specialists should be part of our care team, and how often should we have check-ups?,Are there any support groups or patient communities for this condition?

Common questions about Cervical hypertrichosis-peripheral neuropathy syndrome

What is Cervical hypertrichosis-peripheral neuropathy syndrome?

Cervical hypertrichosis with peripheral neuropathy is a very rare inherited condition that combines two main features: unusual hair growth on the back of the neck and upper back (called cervical hypertrichosis), and damage to the nerves outside the brain and spinal cord (called peripheral neuropathy). This condition is also sometimes referred to as cervical hypertrichosis-peripheral neuropathy syndrome or hypertrichosis-neuropathy syndrome. The nerve damage in this condition affects the nerves that control movement and sensation in the arms and legs. Over time, people may notice weakness in t

How is Cervical hypertrichosis-peripheral neuropathy syndrome inherited?

Cervical hypertrichosis-peripheral neuropathy syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cervical hypertrichosis-peripheral neuropathy syndrome typically begin?

Typical onset of Cervical hypertrichosis-peripheral neuropathy syndrome is childhood. Age of onset can vary across affected individuals.