Hereditary Neuralgic Amyotrophy: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Hereditary Neuralgic Amyotrophy?
A primary care doctor is often the first doctor a person sees. Care usually also involves a neurologist, a doctor who specializes in nerves, muscles, and the brain. A genetic counselor can help families understand testing and inheritance. Physical therapists also play a large role in recovery.
What it is
Hereditary neuralgic amyotrophy is a rare condition that runs in families. It causes sudden, severe pain in the shoulder and arm. After the pain fades, some muscles in the arm can become weak and shrink from lack of use.
Symptoms
The first sign is usually sharp pain in one shoulder, neck, or arm. The pain often starts with no warning, can be very strong, and lasts from days to weeks. As the pain gets better, the muscles may feel weak, and some people notice numbness. Many people have repeated episodes, and each one can leave some lasting weakness.
Causes and who gets it
This condition is caused by a change in a gene called SEPT9, which can be passed from a parent to a child. Some people who carry the gene never have symptoms. Attacks are sometimes triggered by an infection, surgery, childbirth, or unusual exercise.
How doctors diagnose it
There is no single quick test. A doctor will ask about the pattern of pain and weakness, and about family members with similar problems. A physical exam checks muscle strength, feeling, and reflexes. Doctors often order electromyography to measure how well nerves and muscles work. A genetic blood test can confirm the diagnosis.
Treatment
There is no cure. During a painful attack, doctors usually focus on easing pain with medicines and rest. Physical therapy often helps people rebuild strength after the pain settles. Many people recover a good amount of strength over months, but some weakness can remain. Because attacks can repeat, doctors may suggest avoiding known triggers.
When to see a doctor
Anyone with sudden, severe shoulder or arm pain should see a doctor promptly, especially if the pain is followed by weakness or numbness. Anyone with a family history of this condition should mention it during visits.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance. A concierge doctor usually charges more and often works alongside insurance. They can manage pain medicines, follow recovery over time, and coordinate physical therapy. When the diagnosis is unclear, they will refer the patient to a neurologist.
Common questions
Is hereditary neuralgic amyotrophy the same as regular shoulder pain?
No. Regular shoulder pain often comes from an injury or joint wear. It usually does not cause lasting muscle weakness. This condition causes nerve damage that leads to weakness after severe pain.
Will my children inherit it?
If a parent carries the changed SEPT9 gene, each child has a chance of inheriting it. Not everyone who inherits the gene develops symptoms. A genetic counselor can explain the details for a specific family.
Can it go away on its own?
The pain of a single attack usually improves over days to weeks. Muscle weakness often takes months to improve. Some people keep some weakness after repeated attacks.
How is it different from a pinched nerve in the neck?
A pinched nerve usually affects one clear spot and often causes pain down a specific path. Hereditary neuralgic amyotrophy tends to affect several nerves around the shoulder at once. It can also run in families.
Sources
- MedlinePlus Genetics - Hereditary neuralgic amyotrophy
- NIH Genetic and Rare Diseases Information Center - Amyotrophic neuralgia
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
