Diabetes-Related Amyotrophy

What is diabetes-related amyotrophy?

Diabetes-related amyotrophy is a rare neuromuscular condition that causes widespread pain and weakness in your hip and leg. After symptoms start, they typically get worse and then gradually improve over months or years.

Diabetes-related amyotrophy mainly affects people with Type 2 diabetes over the age of 50. On average, it develops about four years after a diabetes diagnosis.

Researchers and healthcare providers are still studying this complex condition and don’t know the exact cause. Providers may use several other names for diabetes-related amyotrophy, including:

• Diabetes-related lumbosacral radiculoplexus neuropathy (DLRPN).
• Bruns-Garland syndrome.
• Proximal diabetes-related neuropathy.
• Diabetes-related polyradiculopathy.
• Multifocal diabetes-related neuropathy.
• Femoral-sciatic neuropathy of diabetes.
• Diabetes-related myelopathy.
• Diabetes-related motor neuropathy.
• Diabetes-related mononeuritis multiplex.

How common is diabetes-related amyotrophy?

Diabetes-related amyotrophy is rare. It affects about 1% of all people with diabetes, but it more often affects people with Type 2 diabetes specifically.

What are the symptoms of diabetes-related amyotrophy?

Symptoms of diabetes-related amyotrophy mainly affect your hip, buttock and/or thigh. They include:

• Severe neuropathic pain (typically the first symptom).
• Muscle weakness.
• Muscle atrophy (loss).
• Lack of reflexes (areflexia).

These symptoms typically start suddenly in just one side of your body but can eventually spread to the other side. The initial pain can last for weeks to months. Weakness usually continues to progress for several months after the pain has improved. Diabetes-related amyotrophy can worsen for up to 18 months. In the worst phase, it’s often difficult to walk without assistance.

The symptoms then stabilize and gradually improve. Recovery can take several months.

People with diabetes-related amyotrophy often have unexplained weight loss as well — typically, over 10 pounds. Some people also experience pain and muscle weakness in their trunk or arms.

What causes diabetes-related amyotrophy?

Researchers don’t know the exact cause of diabetes-related amyotrophy, but they’re actively studying it.

There’s increasing evidence that it’s a type of microvasculitis triggered by immune system-related inflammation. Microvasculitis is inflammation of small blood vessels. Researchers think that an over-reaction of your immune system causes inflammation that damages blood vessels that support the nerves in your hip and leg region.

Studies show that diabetes-related amyotrophy typically involves damage to your:

• Nerve roots: The nerves that emerge from your spinal cord.
• Peripheral nerves: The nerves that branch off nerve roots.
• Lumbosacral plexus: This is a network of nerves that supplies sensation and muscle function to your leg.

What are the risk factors for diabetes-related amyotrophy?

Diabetes-related amyotrophy tends to affect people with diabetes, but researchers don’t know why exactly. There are rare cases of the same symptoms affecting people without diabetes.

Risk factors for diabetes-related amyotrophy include:

• Type 2 diabetes.
• Rapid blood sugar management (like going from having an A1C of 11% to 7% over a month, for example).
• Tight blood sugar management (this typically means having an A1C that’s less than 7%).

Other possible risk factors, or triggers, include:

• Starting diabetes treatment.
• Trauma.
• Infections.
• Immunizations.

How is diabetes-related amyotrophy diagnosed?

There’s no single test that can confirm a diabetes-related amyotrophy diagnosis. Healthcare providers come to the diagnosis through a process of elimination. In other words, your provider will have to rule out other possible causes of your symptoms before determining it’s diabetes-related amyotrophy. Examples of conditions they may aim to rule out include:

• Nerve root compression.
• Pelvic tumors.
• Chronic inflammatory demyelinating polyneuropathy (CIDP).
• Sarcoidosis.
• Infections, like HIV, Epstein-Barr virus, cytomegalovirus and syphilis.

To start, your provider will ask about your symptoms and medical history. They’ll then do a physical exam and a neurological exam.

They may recommend the following tests:

• Blood tests, like a complete blood count, A1C, liver function tests, calcium blood test and others.
• MRI scan of your lumbosacral spine.
• CT scan of your pelvis.
• Neurophysiological tests, which assess how your nervous system is functioning, especially its electrical activity.

What is the treatment for diabetes-related amyotrophy?

Nearly all people with diabetes-related amyotrophy have some recovery of function without medical treatment. But a lack of treatment may make the healing process slower and/or incomplete.

Researchers are still studying effective treatments for diabetes-related amyotrophy. You may be able to qualify for a clinical trial. Ask your provider if this is an option.

Otherwise, providers mainly recommend treatments for symptom management. Treatments may include:

• Pain management: Your provider may recommend medications like NSAIDs to help manage pain. Amitriptyline at night can help with pain and insomnia. If the pain is severe, your provider may offer stronger pain medicines, or you may need to stay in a hospital.
• Physical therapy: A physical therapist will develop a customized set of exercises and movements to help you use the affected parts of your body. These exercises can help manage pain and help you regain muscle mass.
• Occupational therapy: An occupational therapist will help you improve your ability to do daily tasks that are affected by diabetes-related amyotrophy. They may recommend assistive or mobility devices, like a walker or wheelchair, to help make tasks easier.

It’s also important to continue managing your blood sugar levels well to help prevent other types of diabetes complications.

Can I prevent diabetes-related amyotrophy?

As researchers don’t know the exact cause of diabetes-related amyotrophy, you can’t do anything to prevent the condition.

What is the prognosis for diabetes-related amyotrophy?

Diabetes-related amyotrophy is a self-contained condition, which means you’ll eventually experience improvement in your symptoms. But recovery can be prolonged and unpredictable.

Around 10% of people require the use of a wheelchair two years after the start of symptoms. Many people experience some degree of permanent weakness.

Even though it’s a self-contained condition, it’s important to seek medical care for diabetes-related amyotrophy. Physical therapy may help speed up your recovery time.

How long does diabetes-related amyotrophy last?

The symptoms of diabetes-related amyotrophy can last for a few months to up to two years. Recovery can then take months to years. Many people experience lasting symptoms (like weakness), though they’re much milder than when the condition is at its worst.

How do I take care of myself if I have diabetes-related amyotrophy?

Aside from getting medical care, other ways you can take care of yourself include:

• Leaning on loved ones: Diabetes-related amyotrophy may make it difficult to care for yourself like you used to. Ask loved ones for help as you recover.
• Seeking mental health support: Diabetes-related amyotrophy can affect your mental health, causing depression and/or anxiety. If the condition is causing distress or social isolation, reach out to a mental health professional, like a therapist or psychologist, for help.

When should I see my healthcare provider?

You’ll likely see your healthcare provider regularly if you have diabetes-related amyotrophy to monitor its progress and to make sure your treatment plan is helping. If your symptoms get worse or change, talk to your provider.