Musculoskeletal Disorders - Ask Dr. P

What is Stiff Person Syndrome?

by Pain Doc | Dec 19, 2023 | Neuromusculoskeletal Disorders

The singer Celine Dion, whose glorious singing career spanned the last four decades, was recently diagnosed with a condition called Stiff Person Syndrome. Unfortunately, the condition is prevening her from performing, due to its debilitating effect on muscle control, including the vocal cord muscles.

Stiff Person Syndrome (SPS) is a rare, progressive neurological disorder characterized by significant muscle rigidity and spasms, often leading to debilitating physical and psychological symptoms. This article provides an overview of SPS, delving into its causes, symptoms, diagnosis, and current treatment options, as informed by medical research and trusted medical resources.

What is Stiff Person Syndrome?

SPS is an autoimmune neurological disorder primarily causing muscle stiffness and painful spasms. These spasms can worsen over time and vary in symptoms, including an unsteady gait, double vision, or slurred speech, depending on the SPS type. The condition can be severely disabling, often leading to hunched over postures and frequent falls due to impaired reflexes.

Causes of Stiff Person Syndrome

While the exact cause of SPS is unknown, it is believed to be an autoimmune reaction where the immune system attacks glutamic acid decarboxylase (GAD) in the brain and spinal cord. GAD is crucial for producing gamma-aminobutyric acid (GABA), a neurotransmitter controlling muscle movement. Neurotransmitters are protein molecules released from the ends of neurons, which then attach to other neurons causing them to continue the nerve impulse until it reaches the muscle. Disruption in GABA production can lead to continuous neuron firing, contributing to muscle rigidity and spasms seen in SPS. Low GABA levels are also associated with anxiety and depression. Interestingly, SPS often occurs alongside other autoimmune diseases like type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.

Symptoms of Stiff Person Syndrome

The primary symptoms of SPS include progressive muscle rigidity and painful spasms, often triggered by stimuli such as noise, touch, and emotional distress. Initial symptoms typically manifest between the ages of 30 and 60 and can vary in severity and progression. Common initial signs include muscle stiffness and pain, especially in the lower back and legs, potentially leading to difficulty in walking and performing daily activities. Severe cases may require wheelchair use, and there’s an increased risk of anxiety and depression.

Diagnosis of Stiff Person Syndrome

Diagnosing SPS is challenging due to its rarity and symptom overlap with other conditions like Parkinson’s disease, multiple sclerosis, and fibromyalgia. A definitive diagnosis is often made via a blood test measuring GAD antibodies. Most people with SPS show elevated GAD antibody levels. Electromyography (EMG) tests can also be employed to measure muscle electrical activity and assist in diagnosis and monitoring treatment response.

Treatment Options for Stiff Person Syndrome

While there is no cure for SPS, symptoms can be managed through personalized treatment plans focusing on pain management, muscle relaxation, and immune response suppression. Common medications include pain relievers, muscle relaxants, anti-seizure and anti-anxiety drugs, sedatives, and steroids. Non-medication treatments like physical therapy, heat therapy, red light therapy, and pulsed EMF are also beneficial. In cases where medications are ineffective, treatments like Botox, intravenous immunoglobulin (IVIg), and stem cell therapy have shown promise in symptom improvement.

Additionally, a narrative review of available medication treatments for SPS suggests starting with benzodiazepines as a first-line treatment, adding medications like levetiracetam or pregabalin if symptoms persist. For second-line therapy, oral baclofen is preferred over rituximab and tacrolimus. In cases of refractory symptoms, treatments like intrathecal baclofen, IVIG, or plasmapheresis can be effective, with intrathecal baclofen and IVIG being more effective than plasmapheresis.

Conclusion

Stiff Person Syndrome presents a complex clinical challenge due to its rarity, varied symptomatology, and the intricate interplay of autoimmune responses. Understanding its underlying causes, symptom patterns, and current treatment modalities is crucial for effective management. Ongoing research continues to shed light on this condition, offering hope for more effective treatments in the future. For individuals diagnosed with SPS, a collaborative approach involving neurologists, rheumatologists, and physical therapists, alongside personalized treatment strategies, is key to managing this condition and improving quality of life.

And lastly, when your body is struggling with disease, give it assistance by providing it with nutrients, water, sunlight and mild exercise when possible. Mind-body approaches including meditation, flotation therapy, biofeedback, yoga, tai-chi, deep breathing and so on, may provide some relief as well.

Sources:

Johns Hopkins Online

https://www.hopkinsmedicine.org/

National Institute of Neuromuscular Disorders and Stroke

https://www.ninds.nih.gov/

American Brain Foundation

https://www.americanbrainfoundation.org/

Two Modalities to Heal Low Back Pain in Half the Time

by Pain Doc | Oct 20, 2021 | Low Back Pain, Pulsed EMF, Red light therapy, Self Treatment

Hey, I know there are millions of pages on the internet on how to fix low back pain. It can be a dizzying experience searching through them. It’s information overload.

I began blogging on this site around 2010, but actually have been writing articles on things like exercises for low back pain, neck pain, sprains and strains and so forth, since about 1994 when the internet was in its infancy. Fast forward 28 years, and now there are tons of articles and videos online, including mine. Much of the online content for treating low back pain is good: well-written, easy to understand and follow, and backed by evidence. Others are mediocre; just a re-hash of old-school approaches to treating back pain (rest, ice, no heavy lifting, etc.).

If you know me, when it comes to teaching others how to self-treat pain, I like to write fresh, interesting and innovative content. I figure that there are more than enough good videos on stretching and exercising for low back pain. What I like to do is explain the etiology of pain and propose interventions to prevent that pain from developing or getting worse.

If you have acute (recent onset) low back pain, research shows that in most cases, it will go away on its own if you just take it easy for a couple of days. Sure, icing, applying hot packs and no heavy lifting are obviously recommended to prevent re-aggravating the condition. The problem is that most people can’t afford to wait that long, and don’t like being in pain. They have a job, they have responsibilities to other people, and, they want to have fun and do the things they want to do.

For these individuals, there are a couple of home therapies I recommend, to shorten the healing time.

When you have low back pain, muscles and ligaments in and around your spine are generating pain. Something was disrupted mechanically, and inflammation is going on – blood vessels are releasing histamine and heparin and the inflammatory cascade is active—heat, redness, swelling, pain. The inflammatory chemicals irritate sensory nerves, causing some of the pain; as well as the pressure from the swelling. The nerves themselves may be over-firing; generating a level of pain that is not really proportional to the amount of tissue injury.

So with that, my go-to home therapy is a combination of Pulsed EMF and Red Light. Pulsed EMF is an externally-applied, pulsed electromagnetic field. The field, which is similar in frequency to the body’s own natural EM fields, passes through your body and essentially energizes the membranes of cells.

Cell membranes let things in and out of the cell, especially synthesized proteins, nutrients, oxygen, and waste products. They do this via active and passive transport, which both rely on membrane potential—a weak voltage created by negatively charged ions on the outside of the cell, and positive ions on the inside. Like how a battery’s voltage can power a light bulb, a cell’s weak voltage along its membrane powers the exchange of molecules in and out of the cell.

When cells (in this case muscle, bone, nerve, blood vessel cells) are physically damaged or weakened, this exchange is hampered and the tissues are slow to recover and return to a normal, non-pain state. Pulsed EMF lends a boost to this energy, helping cells become more robust in their healing and recovery activities.

Red Light therapy also can energize weak cells, but via photobiomodulation. Cells absorb red light in the 660-720 nanometer wavelength (electromagnetic energy), due to their molecular composition. Photons strike the nucleus, mitochondria and membrane, which changes the oxidative state of the cell. When this happens, it triggers cell signaling pathways related to metabolism and energy production. The cells increase their ATP output, which gives them more energy to repair damaged sites and synthesize needed repair proteins.

Pulsed EMF devices for home use are very easy to operate. Usually, it’s a matter of just pressing the power button, and sometimes a Mode button and Timer button. One of the better models is the BioBalance. You can order it with a full body mat, or a pad. Simply find a comfortable place in your home such as your sofa; place the mat on it, and lie down so that your low back is directly over the mat. No need to remove clothing; the pulsed EMF field passes right through. Do it 3x day for 20-30 minutes/ day to help your body heal and recover.

Another option is the OMI full body PEMF mat. It is lower power than the BioWave, but sometimes that works just as well, as the EM fields are very subtle. You don’t want fields that are too strong. Remember, your body already produces weak magnetic fields; you just want to complement them with a boost of comparable energy.

Red Light therapy is also a great investment in your health. I recommend getting a red light LED wrap, or LED panel. The wrap is a flexible pad embedded with red light LEDs emitting red light and infrared light (660, 820 nm). The red light diodes create photobiomodulation while the infrared diodes provide deep penetrating heat to dilate blood vessels and increase oxygen delivery to cells.

Red Light panels come in different sizes. The small ones are popular for treating facial skin conditions and beautification. The larger panels are better for treating pain. You can mount or hang the panel on the wall, and position yourself so that you are exposed to the red light (usually requires standing up).

In summary, if you are prone to getting lower back pain or have chronic pain issues, Pulsed EMF and Red Light Therapy are two, powerful and safe modalities that can be used at home for self-treatment and are easy to operate. Best of all, they have a long history of medical research to support their use in treating pain and healing injury. It does require a modest investment, but what is more important to your health and well-being? Without this, nothing else matters.

Stenosing Tenosynovitis

by Pain Doc | Sep 22, 2010 | Hand and Wrist Pain

Image via Wikipedia

Stenosing tenosynovitis translates to “tendon inflammation due to narrowing passageways.” It is entrapment and inflammation of the extensor pollicus longus tendon, the muscle that moves the thumb up in a “hitchhiker sign.” It is also known as DeQuervain’s Syndrome.

The passageway in question is a tunnel formed by ligaments that hold the extensor pollicus longus tendon to the distal radius (lateral wrist), one of the two bones of the forearm.

The onset is gradual over a few days. There is a very sharp, unrelenting pain at the radial styloid, the bony prominence at the lateral wrist right below the wrist crease. There is no loss of muscle strength in the thumb. Bending the affected thumb inward towards the palm while bending the wrist sideways towards the ulna (in direction away from the pain) makes the pain much worse.

Causation is believed to be related to overusing the thumbs, such as BlackBerry use, or frequent bending of the wrist while carrying weight, such as in cradling a baby several times a day. However, there are documented cases of stenosing tenosynovitis occurring with no apparent trigger (idiopathic causation).

What is known is that the synovial sheath that covers the portion of the tendon in the tunnel gets inflamed and undergoes degenerative changes. It triggers a chronic, localized inflammatory response. Pain comes from tiny nerve endings in the synovial sheath that are irritated by rubbing against the ligament holding it to the distal radius, and perhaps also by exposure to the products of inflammation.

The condition is known to be self limiting, running its course for a few months and then resolving as the body regenerates the synovial sheath. However, during its acute stage it is quite unpleasant, despite the fact that only about an inch of tendon is involved.

TREATMENT:

Wrist immobilization (wrist brace with thumb splint) to limit movement of the adductor pollicus longus is recommended for 1-3 weeks. Wear most of the day and night; remove every hour or so to do ice therapy.

Ice massage is helpful in temporarily reducing the pain by numbing the area and constricting blood vessels, which tends to suppress production of inflammatory chemicals. A wrist ice wrap can also be effective. Apply ice for 10-20 minutes directly onto the painful area.

Topical ointments may be helpful in reducing the severity of the pain. Narayan oil and products containing capsaicin (don’t use together) are worth trying. Rub into inflamed tendon gently.

Chinese herbal patches may be helpful in temporarily relieving the pain as well.

Cortisone injection into the tunnel can be effective in neutralizing the pain; however it still may take a few weeks to months for the pain to go entirely away.

Exercises for stiff and achey shoulder joints

by Pain Doc | Sep 15, 2010 | Shoulder Pain

Dansk: Skulderled. Français : A. B. Acromion C. D. E. Tendon du biceps F. G. H. Processus coracoïde I. J. Clavicule K. Humérus. A = , B = Acromion, C , D , E = Tendon du biceps , F = , G = , H = Processus coracoïde , I = , J = Clavicule, K = Humérus (Photo credit: Wikipedia)

The shoulder is a complex body system tasked with moving the arms. It is comprised of the shoulder blade (scapula), collar bone (clavicle), humeral head (nearest end of the upper arm bone) and the breast bone (sternum), and associated muscles, ligaments and tendons that hold it together and move it.

When a patient complains of shoulder pain, it could mean pain in any of these areas. The doctor has to ask the patient to point to the precise area of pain, and ask the patient to move the arm and shoulder to get a better idea of what is causing the pain.

Today we’ll talk about general ache in the glenohumeral joint, comprised of the humeral head and glenoid fossa of the scapula (the shallow cup-shaped surface of the scapula).

First of all, realize that the glenohumeral joint has the widest range of motion of all the joints in the body. You can do all sorts of movements with your shoulder joint– raise your arm from the side, the front, the back; transcribe small and large circles, hug yourself, spread your arms far apart; throw a football, and throw an underhand pitch– that’s a lot of movement, compared to, say, the knee. In order to accomplish such a wide range of motion, there has to be a lot of moving parts (ligament and tendon attachments). When you have a lot of moving parts, there is a greater chance of something breaking down. And this is why shoulder problems are quite common in people.

A general ache in the glenohumeral joint can be the result of sleeping on your shoulder; an old injury, or simply over-using it. Tendons that slide over bony surfaces to move the shoulder joint in its many directions may be pinched in the narrow confines of the glenohumeral joint. It makes the shoulder feel stiff and achey.

NOTE: feeling a very deep and sharp, focal pain in the glenohumeral joint that is worse with a particular angle of arm movement is a different presentation and is not what we’re addressing here.

This is about general achiness and stiffness that does not cause any weakness or disability of the shoulder. If you have more of a sharp pain that doesn’t go away with rest or medications, refrain from doing the following exercise until you see a doctor who can properly diagnose your problem.

TREATMENT:

You will be doing gentle stretches to get the shoulder joint moving, eventually without the achey and stiff feeling.

First, let’s assume it’s your right shoulder that has the problem. Stand with your left foot about a foot forward of your right foot. Bend at the waist but keep your lower back straight, and rest your left hand on your left knee.

Let your right arm hang limp straight down. Sway your body in circles to get your limp right arm to transcribe a clockwise circle shape. Try not to use your right shoulder muscles themselves, let the rocking movement move the shoulder. Do ten circles, then reverse directions. Do 5-6 times a day. If you do it correctly, the mere weight of your right arm will traction the glenohumeral joint (slightly pull apart the surfaces) and the rotation movement will stretch the ligaments in all directions.

Try adding a small weight, in increments throughout the week (do not exceed ten pounds max) to increase the amount of traction. You should feel a gradual loosening of the shoulder joint, and less pain. Avoid sleeping on the affected shoulder for a few weeks.

The other exercise is more challenging, so only do it if the pain has gone down considerably. Take a bath towel and roll it length wise. Grab one end with your right hand, and raise that hand (90 degree elbow bend, upper arm at level of shoulder). Reach behind you with your left arm and grab the other end of the towel with your left hand. Now, extend your right elbow back and forth as though you are drying your back with the towel. Do for about a minute, and then switch hands. Repeat.

As always, if any of these exercises cause an increase in pain, stop immediately.

How to manage lower back strain

by Pain Doc | Sep 9, 2010 | Low Back Pain

Low back pain is said to be the most common cause of lost work days, after the common cold. Most everyone has experienced an episode of lower back pain at some point in their life; millions suffer from chronic (ongoing; unending) lower back pain of some form.

The low back, or lumbar spine, is comprised of the last five (5) vertebrae in the spine. These vertebrae are the largest as they support most of the body’s torso weight. Like the cervical spine; i.e. neck (the first 7 bones of the spine), there are no rib attachments in the lumbar spine to limit movement, making the lumbar spine more moveable than the thoracic spine (torso), but less flexible than the cervical spine due to its larger, heavier vertebrae.

This unique lumbar spine design offers advantages and disadvantges. The obvious advantage is flexibility–you can bend (flex) your low back forward, backward, sideways, and can rotate it a few degrees to either side. The disadvantage is that with more movement, there are more opportunities to stress the joints of the lumbar spine (more moving parts) and therefore more chance of injury and pain. This includes potential injury/ trauma to the surrounding ligaments, joint capsules, cartilage lining the small joint surfaces; small facet joints, intervertebral discs, and the lumbar vertebrae themselves.

Therefore, low back pain can originate in one of several structures in the lower back:

the discs (strong fibro-cartilage ligaments that hold vertebrae together)
the facet joints (the “rear” joints of a vertebra, opposite the vertebral
Image via Wikipedia

bodies)
the pars– the small extensions of bone to either side of the vertebrae that form the upper and lower borders of the intervertebral foramen, and end in the lumbar facet joints
the surrounding muscles
the surrounding fascia (muscle covering)
the nerve roots inside the spinal canal
the vertebrae itself (compression fractures; vertebral end-plate fractures)

Today we’ll address lower back pain due to muscle and fascia strain. I’ll refer to this a low back or lumbar strain. This is a common cause of lower back pain and is more manageable than pain due to deeper spinal structures.

In the many cases of lower back strain that I’ve treated over the years, the patient describes a sudden onset of pain after bending at the waist reaching for something. In other cases, the pain starts a day after doing something like weight lifting, running or rock climbing.

Medically speaking, a muscle sprain-strain occurs when muscle fibers tear during contraction and subsequently release inflammation.

The convention for naming soft tissue injuries is that strain refers to injury to a muscle and tendon while sprain refers to injury to ligaments, which connect bone to bone. Since muscles, ligaments and tendons typically get injured all at once in a typical injury due to their anatomical proximity to one another, doctors refer to these types of injuries as sprain-strain injuries.

Sprain-strain severity is described as Grade I, Grade II and Grade III, with Grade III being the most severe and refers to complete rupture of a tendon or ligament. Most lumbar strains are Grade I and II.

Inflammation is meant to contain/ quarantine an injury and is actually an important process in the healing phase (tissue regeneration). The problem is that it releases chemicals that irritate nerves and surrounding tissues, and stiffens adjacent muscles and joints. The inflammatory response can “overshoot” causing the patient to needlessly suffer.

If you go to your doctor complaining of lower back strain, and tests do not indicate damage to deeper structures (discs, facet joints, nerve roots, bone) then you will most likely be prescribed pain blockers (usually NSAIDs- non-steroidal anti-inflammatories), rest, and ice. Your doctor will likely put you on “temporary disability” which means no heavy lifting, bending at the waist, and other activities that put stress on the lower back. Sometimes muscle relaxants are prescribed, if there are complaints of spasm and stiffness. You will be told that it should resolve on its own, and most cases do.

TREATMENT:

At first onset of straining your back, apply ice for 20 minutes every two hours of the waking day. The easiest way in my opinion is to get a large freezer ziplock back, fill a third of it with ice cubes, put about a cup of water in the back and zip it closed (get as much air out as possible before closing shut).

Lie down so that your exposed (no clothing) lower back is directly on top of the bag. Bend your knees or put a pillow under them for comfort. This flattens the lower back and allows it to make good contact with the icepack. (Optional: put a neck roll under your neck for comfort). Do this for 1-2 days.

On the second day, you can introduce gentle stretches while you ice. After your 20 minutes of icing, try lifting your knees to your chest, pulling them gently towards you with your hands. Hold for 5 seconds; repeat five times. Then, keeping your feet together (you are still lying on your back), knees bent, let the knees fall to the right side, gently twisting the lower back; reverse sides. Do five times to each side. What these movements do is orient any scar tissue that develops, in the direction of contraction.

On the third day, assuming pain is still present, you can try using heat. I recommend an infrared lamp. This is radiant heat that penetrates deeper than a hotpack.

If you need to get pain free even sooner, I suggest using the 120 LED (Light Emitting Diode) wrap. This popular home therapy device used red light therapy + infrared heat, which goes beyond simply blood circulation increase. The red light diodes inhibit inflammation and increase cellular metabolism (energy production, waste removal) which means speedier tissue healing.

After a week, your lower back strain should be 60-90% better. If it is only 25% or so improved after a week, the injury is likely worse than originally thought; and deeper soft tissues may be involved. In this case, consider using Pulsed EMF to further enhance tissue healing.

Last thought: I believe that if someone strains his lower back by simply bending forward, it indicates that the back muscles, and probably core/abdominal muscles need better conditioning. It’s not all about strength, it’s also muscle coordination in response to varying loads; for example, picking up a piece of heavy luggage. Those with excellent muscle coordination (there are five major muscle groups that have to work together to move the lower back) are less likely to injure their backs like this. Interestingly, research shows that osteoarthritis, or degenerative joint disease in the lumbar spine, is related to poor lower back muscle coordination. And it makes sense– your muscles move and support your lumbar spine. Poor support and coordination between muscles (erector spinae group, abdominals, etc.) can cause your spinal joints to bear more stress than normal during every day movements.

So, the best strategy is to prevent getting lower back strain by strengthening AND conditioning (improve coordination of) your lower back muscles by doing functional exercises.