What is Klumpke Palsy?
Klumpke palsy, named after Augusta Dejerine-Klumpke, is a condition that affects the nerves in the lower part of the network of nerves near the neck, arm, and hand, called the brachial plexus. This is different from the more common Erb–Duchenne palsy, which affects the upper part of this nerve network. The brachial plexus is a group of nerves that come out from between the muscles at the front and side of the neck. Even though these nerves usually come out between certain neck muscles, there can be differences, with the most common variance being that the nerves penetrate one of these muscles. Klumpke palsy often happens when there is a forceful pulling apart of the arm, which, depending on how severe it is, results in signs and symptoms that come with damage to the nerves.
What Causes Klumpke Palsy?
Klumpke palsy, a type of nerve damage, is commonly caused by an injury to the arm that involves an intense stretching of the lower part of the brachial plexus – a network of nerves that send signals from your spine to your shoulder, arm, and hand.
Sometimes, this type of nerve injury can occur during childbirth if there are strong forces pulling or stretching the baby’s upper body. A similar kind of symptoms can also happen if a growing lung tumor presses against the nerves in the neck or the nerve root at the points where the eighth cervical (C8) and first thoracic (T1) nerves come out of the spinal column.
If the pressure affects the part of the nervous system that controls sweating, blood pressure, and temperature (known as the sympathetic nervous system), it may lead to a condition called Horner syndrome.
It’s interesting to note that approximately half of all injuries to the brachial plexus occur among individuals aged 19 to 34.
Risk Factors and Frequency for Klumpke Palsy
In a study of 4538 patients with multiple injuries, 1.2% had injuries to the brachial plexus, a network of nerves that sends signals from your spine to your shoulder, arm, and hand. Of these injuries, 62% were above the collarbone (supraclavicular), and 38% were below the collarbone (infraclavicular, specifically between the 8th cervical and 1st thoracic vertebrae). The majority of these injuries resulted from motor vehicle accidents. Supraclavicular injuries appear to cause more severe nerve damage, according to the Sunderland classification, a system for assessing severity of nerve injuries. Meanwhile, infraclavicular injuries more commonly cause neurapraxia, a temporary loss of motor and sensory function due to blockage of nerve impulses.
In another study of 203 patients with similar injuries, 90% had supraclavicular injuries and 10% had infraclavicular injuries. An interesting observation was that 47% of these patients didn’t have any pain before the injury, while 28% did.
Signs and Symptoms of Klumpke Palsy
The patient’s personal account often suggests an injury caused by violent stretching or pulling, particularly of an arm that was raised at the time. Often, this injury results in a decrease in feeling along the inside of the arm, covering the areas connected to the C8 and T1 nerves. The patient may also show signs of muscle weakness, which can range from a decrease in muscle strength to muscle wasting and abnormal positional changes. For instance, if the nerves controlling muscles have been injured leading to muscle shrinking and stiffening, the patient’s hand might be in a “claw-like” position which involves bent fingers and wrist. The patient may also describe severe pain that begins at the neck and travels down the inside part of the arm.
A lower brachial plexus injury may also result in Horner syndrome. This situation can occur due to damage to the sympathetic nerve chain located near the T1 nerve root. If this happens, symptoms such as a droopy eyelid, inability to sweat, and a small pupil on the same side of the body, will develop.
Testing for Klumpke Palsy
If you’ve injured your upper extremity (limbs, shoulders, and upper back), the first step to diagnosis involves a detailed understanding of how the injury occurred and a physical examination by a health professional. Depending on the results of the physical exam, they might find it necessary to do further tests if they suspect there’s a problem with your nervous system.
There are different types of medical imaging that can be used. These include X-rays of your neck and upper back, or more detailed scans like Computed Tomography (CT) or Magnetic Resonance Imaging (MRI). These tools are helpful for ‘seeing’ what the problem might be. For example, an X-ray might rule out if a broken bone is pressing against nerve roots. In some non-trauma related cases, X-rays can help identify if a growth is occupying space it shouldn’t in your upper chest or at the base of your neck.
Both CT and MRI scans are excellent for more precise evaluation if a nerve injury is suspected. CT Myelography, another type of imaging, is more specific and sensitive, meaning it is better at pinpointing and identifying particular types of injuries, but it does expose you to more radiation than an MRI. Despite offering less radiation, MRI does have lower sensitivity and specificity compared to CT myelography, which means it might not be as accurate in some cases.
Beyond imaging, other tests such as a histamine injection and a nerve conduction velocity (NCV) test could be used. The histamine test helps determine if the nerve injury is above or below the dorsal root ganglion. This is a cluster of neurons (nerve cells) in your spinal cord. If an injection of histamine—a chemical your body makes when you’re having an allergic reaction—causes a strong skin reaction in a certain area, then the injury might be above the dorsal root ganglion.
An NCV test measures the speed of electrical signals through your nerves. It can help reveal the specific location of your injury. These tests provide your medical team with a detailed picture of your injury, helping them plan the most effective treatment approach.
Treatment Options for Klumpke Palsy
Klumpke palsy is a condition that can affect the axillary artery, which is located in your armpit. This can happen because of two types of injuries: a crush injury in the area or a stretch injury caused by pulling the arm up and away from the body too far. In severe cases, where blood flow is compromised, immediate surgery may be required. Fractures are often associated with Klumpke palsy and typically require surgical treatment.
However, if there aren’t any open wounds that penetrate through the skin or any signs of blood flow being blocked, there is a less severe approach to treatment. This approach relies on the natural healing process, which is when the body can repair its damage over time. This is possible due to your nerves’ ability to heal themselves. For Klumpke palsy, usually action may not be needed immediately. It’s important to note, though, that most injuries to the C8-T1 nerves – the nerves located between the base of your neck and your armpit – don’t typically heal on their own without some sort of treatment.
This at-home treatment involves simple stretching and strengthening exercises for the muscles that have been affected by the nerve injury. Practicing these exercises regularly helps maintain your range of movement and can make a big difference in a successful recovery. It’s common for mild cases to naturally get better by themselves.
However, if there’s no sign of improvement within three to six months, surgery may be needed. This surgery usually involves taking a healthy nerve from another part of the body and using it to replace the injured one, a procedure known as nerve grafting. For injuries higher up the arm, near the neck, a procedure known as neurotization may be recommended. This involves rerouting undamaged nerves closer to the injury site to take over the functions of the injured nerve.
What else can Klumpke Palsy be?
If someone has symptoms similar to Klumpke palsy, they could actually be suffering from a similar condition named Erb palsy. Erb palsy affects the upper part of a bundle of nerves called the brachial plexus which usually result in signs along the C5-C6 path. People with Erb palsy often have what’s known as the “waiter tip” presentation, where the forearm is fixed in a pronated position with the palm facing outward.
In addition, conditions like the distal nerve entrapment of the ulnar nerve at the medial epicondyle of Guyon’s tunnel could produce similar symptoms to Klumpke palsy. This is known as ulnar nerve entrapment. A notable difference between this and Klumpke palsy is that there won’t be any effects on the nerves above the point of the lesion. For instance, there won’t be any involvement of the pectoralis major muscle with true ulnar nerve entrapment.
Thoracic outlet syndrome (TOS) could also have symptoms similar to Klumpke palsy. TOS usually occurs due to injury to the brachial plexus from a rudimentary rib, first rib, or the clavicle on the same side. This could be due to an injury, poor posture, or even genetics. Unlike Klumpke palsy, TOS affects more than just the C8-T1 nerve roots. However, like Klumpke palsy, it can also affect the axillo-subclavian artery.
Here are some of the most common conditions that could be mistaken for Klumpke palsy:
- Distal ulnar nerve entrapment
- Thoracic outlet syndrome
- Apical lung tumor
- Neurofibroma
- Disc herniation
- Shoulder impingement
- Fracture of clavicle or vertebra
- Others
What to expect with Klumpke Palsy
A study in 2006 discovered that children with a brachial plexus injury (damage to the nerves that send signals from your spine to your shoulder, arm and hand) tend to have better outcomes than adults when they undergo a type of surgery called microsurgery. This could be due to a few reasons: the shorter distance that nerves need to regenerate in children, children’s higher potential for nerve regeneration, and their increased ability to mentally adapt to changes.
A different study found similar results, showing that microsurgery helped recover hand function in 75% of the cases over a period of 8 years. In cases where the injury is not too severe, a spontaneous recovery, or one without surgical intervention, is possible.
Zuckerman and his team found that two similar groups of individuals – one group that underwent surgery and another group that was past the point of needing surgery – had similar outcomes in terms of function after two years. But most experts will agree that for more severe injuries, the sooner you undergo surgery, the better the outcomes will generally be.
Recovery from Klumpke Palsy
If a baby is born with a condition known as brachial plexus birth palsy and there are no broken bones or dislocations, exercises that focus on stretching and strengthening the restricted muscles can help the baby recover. The main objectives of these rehabilitation exercises are to increase movement range, prevent muscle wasting (known as muscular atrophy), and manage pain.
These exercises work by stretching the tissues to avoid muscle wasting. Continuous stretching exercises have shown promising results in rats by limiting atrophy. Aside from stretching exercises, electrical stimulation might also be beneficial as a treatment. Previous studies on rats have shown that electrical stimulation can have positive results on denervated muscles (muscles that lost nerve supply), in this case, the soleus muscle in the leg. However, there were some limitations observed.
