Diabetic Amyotrophy

What is Diabetic Amyotrophy?

Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) is a condition that falls under the category of diabetic neuropathy – nerve damage caused by diabetes. It is also known by various other names such as diabetic amyotrophy, Bruns-Garland syndrome, and proximal diabetic neuropathy, to name a few. The multiple terms for this condition reflect different ideas about its exact location in the body and what causes it.

This condition was first identified in the late 19th century, with major contributions from researchers named Leyden, Auche and Bruns. However, it was not until the 1950s that the term “diabetic amyotrophy” was coined by Hugh Garland. Garland studied 12 patients who had shaky, steadily worsening pain in the lower part of their bodies, as well as muscle weakness. Other symptoms included muscle wastage, weight loss, and absence of reflexes. He found a clear connection between these symptoms and uncontrolled diabetes, and also discovered that managing blood sugar levels properly could almost completely resolve the condition.

We still don’t fully understand how diabetic amyotrophy works, but we do know it involves harm to various parts of the nervous system, including the peripheral nerves and lumbosacral plexus, which is a network of nerves in the lower back region. This nerve damage can cause a range of issues including axonal degeneration, which relates to the breakdown of nerve fibers, demyelination, (loss of insulative material around nerves), inflammation, reduced blood supply, and microvasculitis, a type of vascular inflammation often linked to immune system disorders.

What Causes Diabetic Amyotrophy?

There’s growing proof that a type of inflammation in the small blood vessels, or microvasculitis, is linked to diabetes. However, how it’s related to the development of the disease isn’t completely understood.

There are several risk factors for Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN):

It’s more often seen in patients with type 2 diabetes.
Rapid or tight control of blood sugar levels.

Other things that might increase the risk include:

Starting treatment for high blood sugar levels.
Getting vaccinations.
Physical injuries or trauma.
Infections.

There are also several conditions that cause lumbosacral plexopathies:

DLRNP (also known as diabetic amyotrophy).
DLRNP of unknown cause (idiopathic).
Inflammation of small blood vessels (microvasculitis).
Trauma or physical injury.
Bleeding into the space behind the abdominal organs (Retroperitoneal hematoma).
Cancer or its spread (metastasis).
Abnormal connections between arteries and veins (Dural arteriovenous fistulas).
Infections.
Radiation exposure.
A disease that causes areas of inflammation in different parts of the body (Sarcoidosis).

Risk Factors and Frequency for Diabetic Amyotrophy

DLRPN is a unique type of diabetic neuropathy that affects roughly 1% of all individuals with diabetes. It is more prevalent in men and people with type II diabetes compared to those with type I diabetes. Typically, DLRPN affects older diabetic individuals, generally aged over 50, but its median onset age is above 65 years.

The time period from when diabetes is initially diagnosed to when DLRPN sets in is usually around 4.1 years.

A study conducted in Olmsted County, MN, showed that the incidence of LRPN was around 4.16 for every 100,000 people per year, while DLRPN affected roughly 2.79 out of 100,000 individuals every year. However, these numbers could vary in the broader U.S. population, as the study involved a less racially diverse population.

Signs and Symptoms of Diabetic Amyotrophy

Diabetic lumbosacral radiculoplexus neuropathy (DLRPN), commonly seen in older adults (average age 65), is a condition that typically appears suddenly and lasts for a short duration, anywhere from a few months to 2 years. Initially, this nerve disease affects one side of the lower body like the thighs, buttocks, or hips. However, as it progresses, it tends to impact both sides of the body and can range from mild to severe symptoms.

People with DLRPN often experience intense nerve pain that worsens over time, muscle weakness, muscle wasting, and substantial weight loss, often more than 10 pounds. Even though the disease may eventually stabilize, recovery can be slow and often leaves some degree of lasting impairment. Interestingly, amongst individuals who have diabetes, those diagnosed with diabetic lumbosacral radiculoplexus neuropathy often have newly identified diabetes, less exposure to high blood sugar, better blood sugar control, and fewer other diabetes-related complications, such as eye disease and cardiovascular disease. They also tend to have a lower Body Mass Index (BMI) as compared to other people with diabetes.

Numbness
Tingling Sensation
Lack of Reflexes
Paralysis or reduced functioning of the arms and legs
Changes in Sweating
Difficulty lifting the front part of the foot (Foot Drop – a long-term complication)
Low blood pressure when standing up (Orthostatic Hypotension)
Diarrhea
Constipation

Testing for Diabetic Amyotrophy

Diagnosing this syndrome can be difficult because its symptoms can look like those of other serious conditions. It is diagnosed based on the characteristic features in a patient who has newly been identified as having diabetes. Doctors must stay alert and maintain a level of suspicion to accurately diagnose this condition and avoid any unnecessary tests or procedures.

Some blood tests may be required to assist in the diagnosis, such as for blood chemistry (like electrolytes and liver function tests), hemoglobin A1C levels, and inflammation markers. These tests would include checking for the erythrocyte sedimentation rate and C-reactive protein.

Analysing the cerebrospinal fluid (CSF) can reveal high protein levels and an increase in certain types of white blood cells, which would suggest inflammation.

Electrophysiological tests, such as a nerve conduction study and electromyography (EMG), can also provide valuable information. The nerve conduction study might show a decrease in sensory and motor action potential amplitude in the affected muscles. An EMG test might show varied nerve involvement, fibrillation potentials, and long-duration, high-amplitude motor unit potentials.

Magnetic Resonance Imaging (MRI) commonly shows an increase in the T2 signal, which can vary in severity. Sural nerve biopsy has been thoroughly explained to help diagnose this syndrome, but it is not a necessary step for its identification.

Laboratory studies, electrophysiological testing, biopsies, and imaging such as x-rays, CT scans, and MRI scans can be used to rule out other causes of neurological symptoms. However, it’s very important to understand that this condition is diagnosed primarily based on clinical observation and evaluation.

Treatment Options for Diabetic Amyotrophy

Diabetic lumbosacral radiculoplexus neuropathy is a condition that follows a limited disease course. There have been several small studies that have linked the use of immunosuppressant medicine, like steroids, immunoglobulin, and plasma exchange, to an improvement in patient symptoms. However, there is still a need for clear evidence. This is because there have been contradicting results on the effectiveness of these treatments.

There was a double-blind study that included 75 patients. This study compared results from high-dose steroids (given on a weekly basis) to a placebo. Even though there was a significant improvement in secondary outcomes (like reduced pain and weakness), the primary result, which was the time to improvement, was not met. Both the patients taking the steroid and the placebo showed improvements. The researchers proposed that if patients began taking steroids earlier, the outcome might be better. But, so far there is no conclusive evidence to support the use of immunomodulators.

Treatment mainly focuses on managing the symptoms such as pain, controlling high blood sugar, and improving mobility. Pain can be addressed with paracetamol and NSAIDS. Drugs like amitriptyline can be taken at night especially when the patient also has trouble sleeping. Selective serotonin receptor inhibitors can be used if the patient is dealing with depression or anxiety, and anticonvulsant agents can also be used. Other options like opioids or steroids may be considered if the disease is severe. In extreme cases, hospitalization or a consultation with a pain management specialist may be needed.

If total relief from pain is not possible, the goal switches to making the pain more tolerable. Patients should be given guidance about the long-term nature of the disease. They should be assured that some degree of improvement is possible, and they should be supported and encouraged because of the potential for disability. It may also be necessary to pay attention to issues like disability and safety at home. Also, managing diabetes might be recommended.

Doctors take into account several possible conditions when diagnosing an issue related to overall pain and discomfort in the pelvic and lower body area. These include:

Diabetic nerve damage or “neuropathy”
Pain caused by pressure on a nerve root, also known as “nerve root compression”
Muscle damage linked to diabetes, referred to as “diabetic muscle infarction”
Possible cancer in the pelvic area or “pelvic malignancy”
A condition where the protective covering of the nerves gets damaged, called “chronic inflammatory demyelinating polyradiculoneuropathy”
Medical disorders related to inflamed blood vessels, known as “vasculitides”
A disease which leads to inflammation of body tissues, named “sarcoidosis”
Certain infections such as HIV, Epstein-Barr virus, Cytomegalovirus, Chickenpox/Shingles Virus, and Syphilis
Conditions after radiation therapy, surgical procedures, and childbirth interventions

It’s crucial for doctors to thoroughly evaluate these possibilities and perform necessary tests to pinpoint the exact cause of the issue.

What to expect with Diabetic Amyotrophy

In general, people with diabetic amyotrophy can expect a good outcome as this condition typically resolves on its own. The disease usually gets worse over time, but eventually stabilizes and then fully recovers. However, some patients may have some remaining muscle weakness. This whole process often occurs over several months and can last up to two years from when it begins.

In rare cases, the condition has been known to progress to quadriparesis, a condition characterized by weakness in all four limbs. The recovery rate for these particular cases is uncertain.

Possible Complications When Diagnosed with Diabetic Amyotrophy

Some conditions or outcomes can include:

Paraplegia
Quadriplegia
Depression
Anxiety
Unnecessary spinal surgery

Preventing Diabetic Amyotrophy

Educating patients about the disease is crucial, especially in understanding the potential for it to progress to significant muscle weakness (such as paralysis of both legs or even all limbs). In some severe cases, relentless pain may also accompany this condition. Despite these serious symptoms, it’s important for patients to know that this disease condition usually has a specific timeframe and, in many cases, can either partially or totally disappear on its own. This knowledge can provide patients with reassurance, giving them the encouragement they need to cope with their condition.

Frequently asked questions

Diabetic Amyotrophy, also known as Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN), is a condition that falls under the category of diabetic neuropathy - nerve damage caused by diabetes. It involves harm to various parts of the nervous system, including the peripheral nerves and lumbosacral plexus, which is a network of nerves in the lower back region. This nerve damage can cause a range of issues including axonal degeneration, demyelination, inflammation, reduced blood supply, and microvasculitis.

DLRPN affects roughly 1% of all individuals with diabetes.

The signs and symptoms of Diabetic Amyotrophy, also known as Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN), include: - Numbness - Tingling Sensation - Lack of Reflexes - Paralysis or reduced functioning of the arms and legs - Changes in Sweating - Difficulty lifting the front part of the foot (Foot Drop - a long-term complication) - Low blood pressure when standing up (Orthostatic Hypotension) - Diarrhea - Constipation In addition to these symptoms, people with DLRPN often experience intense nerve pain that worsens over time, muscle weakness, muscle wasting, and substantial weight loss, often more than 10 pounds. It is important to note that while the disease may eventually stabilize, recovery can be slow and often leaves some degree of lasting impairment. Interestingly, individuals diagnosed with diabetic lumbosacral radiculoplexus neuropathy often have newly identified diabetes, less exposure to high blood sugar, better blood sugar control, and fewer other diabetes-related complications, such as eye disease and cardiovascular disease. They also tend to have a lower Body Mass Index (BMI) as compared to other people with diabetes.

DLRNP (Diabetic Amyotrophy) can be caused by several factors, including inflammation of small blood vessels (microvasculitis), trauma or physical injury, bleeding into the space behind the abdominal organs (Retroperitoneal hematoma), cancer or its spread (metastasis), abnormal connections between arteries and veins (Dural arteriovenous fistulas), infections, radiation exposure, and a disease that causes areas of inflammation in different parts of the body (Sarcoidosis).

The other conditions that a doctor needs to rule out when diagnosing Diabetic Amyotrophy are: - Diabetic nerve damage or "neuropathy" - Pain caused by pressure on a nerve root, also known as "nerve root compression" - Muscle damage linked to diabetes, referred to as "diabetic muscle infarction" - Possible cancer in the pelvic area or "pelvic malignancy" - A condition where the protective covering of the nerves gets damaged, called "chronic inflammatory demyelinating polyradiculoneuropathy" - Medical disorders related to inflamed blood vessels, known as "vasculitides" - A disease which leads to inflammation of body tissues, named "sarcoidosis" - Certain infections such as HIV, Epstein-Barr virus, Cytomegalovirus, Chickenpox/Shingles Virus, and Syphilis - Conditions after radiation therapy, surgical procedures, and childbirth interventions

The types of tests that may be needed to diagnose Diabetic Amyotrophy include: - Blood tests: These may include blood chemistry tests (electrolytes and liver function tests), hemoglobin A1C levels, and inflammation markers such as erythrocyte sedimentation rate and C-reactive protein. - Cerebrospinal fluid analysis: This can reveal high protein levels and an increase in certain types of white blood cells, indicating inflammation. - Electrophysiological tests: These may include a nerve conduction study and electromyography (EMG), which can provide valuable information about nerve involvement and muscle function. - Magnetic Resonance Imaging (MRI): This imaging technique can show an increase in the T2 signal, which can vary in severity. - Sural nerve biopsy: While not necessary for diagnosis, this biopsy can help confirm the condition. - Other tests: Laboratory studies, imaging such as x-rays, CT scans, and MRI scans, and biopsies may be used to rule out other causes of neurological symptoms. However, diagnosis is primarily based on clinical observation and evaluation.

Diabetic Amyotrophy is treated by managing the symptoms such as pain, controlling high blood sugar, and improving mobility. Pain can be addressed with paracetamol and NSAIDS. Drugs like amitriptyline can be taken at night especially when the patient also has trouble sleeping. Selective serotonin receptor inhibitors can be used if the patient is dealing with depression or anxiety, and anticonvulsant agents can also be used. Other options like opioids or steroids may be considered if the disease is severe. In extreme cases, hospitalization or a consultation with a pain management specialist may be needed. It is also important to provide guidance about the long-term nature of the disease, assure patients that some degree of improvement is possible, and support and encourage them due to the potential for disability. Managing diabetes might also be recommended.

When treating Diabetic Amyotrophy, the potential side effects can include: - Paraplegia - Quadriplegia - Depression - Anxiety - Unnecessary spinal surgery

The prognosis for Diabetic Amyotrophy is generally good as the condition typically resolves on its own. The disease usually worsens over time, stabilizes, and then fully recovers. However, some patients may have residual muscle weakness. In rare cases, the condition can progress to quadriparesis, and the recovery rate for these cases is uncertain.

A neurologist.

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