SPS 101

Medications and Treatments for SPS

Treatment is Focused on Relief from Symptoms

Treatment currently focuses on relief from difficult symptoms, usually muscle spasms and pain. Additionally, immunotherapies involve medications and other treatments to address specific autoimmune abnormalities.

Effectiveness of Treatments Vary

People with SPS have very different responses to treatments, so medications are carefully tried and evaluated by the patient and their physicians. It is not possible to reliably predict which medications will be effective for an individual, though some experts report that the types of SPS antibodies in a person might influence which immunotherapies are most likely to be effective.

There is No Cure for SPS

There is currently no cure for SPS. Medications and other treatments may help reduce stiffness, muscle spasms and pain in some individuals. Unfortunately, though, most patients with SPS still have at least some degree of disability.

The specific details of how each medication works is beyond the scope of this discussion, though general medication categories with some specific examples are listed below.

All of these medications have significant risks and side effects which may cause other problems or limit the use of any of these medications. These medications must be prescribed and monitored closely by a physician who has expertise in their use and management.

Symptomatic Relief for Muscle Spasms and Pain

Muscle Relaxants

Baclofen (Lioresal) – May be given orally or directly infused into the spine with an implantable pump which requires surgery.

Benzodiazepine group including diazepam (Valium), clonazepam (Klonopin)

Tizanidine (Zanaflex)

Methocarbamol (Robaxin)

Botox injections – paralyze muscles around the site of injection

Dantrolene (Dantrium) – first doses usually given intravenously in a hospital

Anti-seizure Medication (may help with pain management)

Gabapentin (Neurontin)

Pregabalin (Lyrica)

Tiagabine (Gabatril)

Valproate (Depakote)

*Other types of pain relievers including narcotics and anesthetic agents are infrequently used and have a limited role in pain management of SPS. Some of these medications can make treatment with the main drugs more difficult.*

Other therapies which may help with SPS muscle spasms or pain include:

Physical therapy

Myofascial release

Massage therapy

Heat therapy

Acupuncture

Acupressure

Yoga

Meditation

Aqua therapy

Management of Anxiety and Depression

Anxiety and depression are often part of SPS. Mental health therapy focused on addressing depression and anxiety, as well as pain and chronic illness and its complications, should be done with an experienced therapist. Treatment can include different types of talk therapy and medication. Cognitive behavioral therapy is often helpful.

Medication choices should be coordinated with an SPS specialist since some medications for anxiety or depression can make SPS symptoms worse. Medications in the SNRI category (serotonin and norepinephrine reuptake inhibitors) can exacerbate SPS symptoms. Medications in the SSRI category (selective serotonin reuptake inhibitors) are thought to be safe for people with SPS.

Immunotherapies used in Stiff Person Syndrome

Immunotherapy is the use of medications or other interventions to address specific abnormalities in the function of a person’s immune system.

An important part of the immune system are certain white blood cells called lymphocytes. Two types of lymphocytes are B cells and T cells. B cells make antibodies. T cells are vital in identifying and killing cells with infection and in regulating other aspects of the immune response.

Certain subsets of B cells and T cells can be the intended target of some immunotherapies for treating SPS.

“First Line” Immunotherapies

Intravenous immunoglobulins (IVIg) Immunoglobulins are antibodies that impact many components of the immune system activities. IVIg has purified immunoglobulins that have been obtained from pooled human donor sources.

Subcutaneous immunoglobulin (SCIG) SCIG is another route of giving immunoglobulins. It is delivered by a pump system with IV tubing, infusing the immunoglobulins into the subcutaneous layer of the skin. For people on high doses of immunoglobulins, this could involve multiple infusion sites. This treatment can be self-administered making it more convenient.

SPSRF Medical Advisory Board Member, Marinos Dalakas, discusses IVIg therapies in GAD+ SPS patients.

Intravenous methylprednisolone (Solu-Medrol) This immunotherapy is usually a short course of high dose intravenous steroids which are used for the management of some autoimmune disorders. It is sometimes followed by a more prolonged course of oral steroids (prednisolone or prednisone) that is often slowly tapered over weeks.

Plasma exchange (PLEX) or plasmapheresis A special catheter (central line) or port is sterilely placed into a large blood vessel. During the plasmapheresis treatment, blood comes from the patient, through the catheter to the plasmapheresis machine where it is treated and then returned to the patient. The process of plasma exchange involves “cleaning” the blood by removing some of its liquid plasma which contains antibodies. Then the rest of the patient’s blood is returned to the patient with either donated plasma or albumin to replace some of what was removed. A typical course of plasmapheresis might be 5 – 7 treatments done every other day. For those who respond, it may then be done less often as an ongoing maintenance therapy.

“Second Line” Immunotherapies

These are the immunotherapies with B cells and or T cells as the intended target.

Rituximab – Depletes B cells. It is given by intravenous infusion using different regimens. One typical course would be to give two doses, two weeks apart, repeating about every 6 months.

Cyclophosphamide (Cytoxan) – Targets B cells and T cells, given by intravenous infusion with various protocols.

Mycophenolate mofetil (CellCept, Myfortic) – Oral medication which has been used as additional immunotherapy to decrease B cell and T cell growth.

Azathioprine (Imuran) – Orally administered, decreases number of B and T cells.

Experimental

Autologous stem cell transplants Stem cells are normal cells which have the unique ability to form many other types of cells. Stem cell transplants have been used to treat some severe autoimmune diseases. These transplants are a multi-step, complicated process where the person with the autoimmune disease becomes their own stem cell donor (autologous). The process involves receiving medication to generate an increased number of normal bone marrow stem cells which will then be treated outside of the body and infused back into the patient to reset the immune system. There have been several small trials of autologous stem cell transplants in SPS patients who have already responded poorly to other therapies. Results have been mixed with some patients doing well for at least a few years. Some patients initially did well and then relapsed. Some patients have no improvement or change in their SPS symptoms.

There are many important questions requiring further research about which patients with SPS might have the best chance of a good and sustained response to a stem cell transplant. The procedure itself is complicated and considered high risk.

Read More about SPS

Disclaimer: The content of this Website is provided for informational and educational purposes only, and does not in any way intend to substitute for professional medical advice, diagnosis, or treatment. The content is not intended to be medical advice for any particular person or patient and should not be relied upon as medical advice. You should always seek the advice of a physician or other qualified healthcare provider for medical advice, including any questions you may have regarding diagnosis or treatment of any medical condition.

Contributors to this document include: Jim Weiss, MD; Vered Lewy-Weiss, MD; Tara Zier, DDS and SPS patient.