A patient went to the ER for suspected stroke due to stiffening and spasm on one side of her body. It turned out that she actually had partial SPS.

Four patients demonstrated worsening symptoms possibly due to the use of duloxetine, a serotonin-norepinephrine reuptake inhibitor (SNRI) used to treat depression.

Autologous hematopoietic stem cell transplant (aHSCT) may be another possible form of treatment for SPS. This article is a case report of a patient who found relief after other treatments didn't work. The paper acknowledges that although there have been other successful treatments with aHSCT, a large study is needed to see if aHSCT is an appropriate treatment for SPS.

Although the GAD65 autoantibody often correlates with an SPS diagnosis, the amount of GAD65 autoantibodies does not indicate how severe someone's symptoms are. In fact, SPS patients may not have the GAD65 autoantibody at all. Figure 2 shows a great diagram of the other types of autoantibodies associated with SPS. Figure 1 shows the difference between normal muscle contraction vs muscle contraction seen in SPS. Table 1 shows a nice breakdown of what clinicians should test for when suspecting SPS in a patient. Table 3 lists the possible treatments for SPS.

Controlled trials indicate that 2 g/kg of IVIg may help alleviate symptoms of SPS, but more controlled studies need to study how appropriate IVIg is for long-term use, how worth the treatment is in the long run due to its expensive cost, and how quality of life is for a patient that may need long-term use of IVIg.

A decrease of GABA in the brain appears to be a characteristic of SPS. To note: there were no structural changes in the brains of the patients studied.

This paper describes the symptoms of patients who are positive for the autoantibody for glycine receptor alpha-1 subunit (GlyRα1).

This paper will be difficult to read for patients, but note that "Ag" stands for "antigen" and "Ab" stands for antibody. This paper discusses the biology of GAD65 and its association with SPS.

Although not about SPS specifically, this paper is important to share as it discusses the possible side effects of IVIg.

This paper shows the long term effects of using IVIg therapy to mitigate symptoms of SPS. 24 of the 36 patients in the study showed improvement over a 3.3 year period. 12 patients did not show any improvement over time, thus the paper concludes that more research is needed for more effective therapies.

SPS which begins in childhood is often not diagnosed until adulthood. Because of this, symptoms can be exacerabated over the years due to little treatment. This paper describes the symptoms of these patients in the hopes that clinicians may be able to spot the symptoms of SPS in children for faster treatment.

Although the paper talks about the effectiveness of IVIg on various diseases, its short section on page 2048 of the paper is still informative to SPS patients. Although IVIg is an effective treatment for many patients, the length of effectiveness varies. In addition, a long-term controlled study with IVIg for SPS patients has not been conducted yet.

A wide range of symptoms of SPS are summarized in this paper, including summaries of the several categories of the disease (classic SPS, SPS plus, PERM, and partial SPS). Of note, female sex and initial brainstem/cerebellar involvement may predict a worse outcome over time for ability to perform daily activities. In addition, the following factors may predict a greater need over time for an assistive walking device: older age at symptom onset, female sex, black race, and initial brainstem/cerebella involvement. Figure 1 maps out the general areas of the body where stiffness/spasms tend to be for each category of SPS.

20 GAD65 positive patients were studied to provide a way for clinicians to assess stiffness. Stiffness can be measured by documenting where and how frequently stiffness occurs in the body over time. Stiffness can also be measured by documenting the degree of heightened sensitivity over time. The study also looked at symptoms of each patient. Notably, all the patients had increased muscle tone in the paraspinal muscles. There is also a discussion of the frequency of the DRβ1 0301 allele depending on the race of the patient.

Of 16 patients, 11 patients were able to walk without assistance and perform household tasks again after receiving IVIg. Duration of benefit lasted from six weeks to one year. Although effective, IVIg is expensive.

Although this paper addresses several diseases, it's important to note that SPS can cause dysphagia (difficulty swallowing).

A medium-level reading difficulty paper with easy to read diagrams and tables. Page 354 has a nice summary of the paper under the table "Key Points." Figure 1 has a diagram which shows what autoimmune targets affect which symptom. Box 1 contains diagnostic criteria for SPS. Figure 2 shows how GAD65 antibody levels can be assessed via different techniques. Figure 3 shows a diagram of the disorders associated with GAD65 positive and negative patients. Figure 4 shows a flowchart in determining if whether symptoms in a patient have an autoimmune basis or not.

In this case report, a patient with SPS did not experience prolonged muscle weakness when using baclofen with volatile anesthetics. This paper discusses the conflicting evidence of the use of volatile anesthesia with neuromuscular blockers. More needs to be understood about how volatile anesthetics may interact with neuromuscular blockers to cause potential muscle weakness.

In this placebo-controlled randomized trial, rituximab did not show to be that effective against the placebo.

Although this paper isn't specifically about SPS, it describes what IVIg is, the possible mechanisms of how it works, and what diseases it's used to treat. Due to its efficacy in treating various diseases, the cost of IVIg is rising and supply is becoming limited. Alternatives to IVIg are being developed, but these treatments have not undergone clinical trials yet. While IVIg is beneficial for many SPS patients, it may not alleviate symptoms for all patients. There currently is no way to tell who will respond well to IVIg treatment before trying the treatment out.

This article discusses the symptoms of SPS, including possible changes to your eyes, gastrointestinal system, respiratory system, and mental health. Figures 1 and 2 show easy to understand diagrams of what different parts of the body are affected by SPS. Table 1 includes a summary of possible treatments. The paper goes into detail about when certain treatments can be implemented. The article also addresses the autoantibodies involved with SPS, SPS in animals, and considerations for SPS in pregnancy and childhood.

SPS is on a spectrum since there are many types of it. Therefore, the authors refer to SPS as stiff-person spectrum disorder, or SPSD. After studying data from 121 patients, patients with GAD antibodies tend to have worse outcomes than patients with glycine receptor antibodies or patients who had none of the antibodies associated with SPS. The authors also noted that the type of SPS didn't predict a patient's outcome.

In a study of 99 patients, the authors summarized the types of symptoms they had depending on the type of SPS the patients had. Table 1 breaks down the symptoms by type of SPS. Table 3 demonstrates how having another coexisting autoimmune disease.

An autoantibody profile was put together based on 40 patients. In addition to the typical observation of GAD65 autoantibodies, GAD67 and tyrosine hydroxylase autoantibodies were also observed.

The purpose of this paper is to make physicians aware that a patient's blood results could be influenced by IVIg. It's important to wait at least four weeks after IVIg treatment so as to not misinterpret the level of autoantibodies in a patient's blood since the infusion may contain some levels of these autoantibodies as well.

An abstract describing how patients were diagnosed with SPS or a different disease at the Mayo Clinic from 2016 to 2021. Misdiagnosis is common, with most alternative diagnoses were non-neurologic. The short results section describes the main symptoms used to diagnosis SPS.

SPS has never been observed to co-exist with multiple sclerosis.

The presence of the autoantibody targeting glycin receptor alpha-1 subunit (GlyRɑ1-IgG) helps in the diagnosis of SPS.

There can be late onset (after 60 years of age) of SPS. Delayed treatment occurs for these patients since they were misdiagnosed with other disease with similar symptoms. Clinicians need to be aware of the symptoms of SPS so that patients with late-onset SPS (LOSPS) can be treated faster. Table 1 shows a sumary of the patients examined, including their symptoms, misdiagnosis, and response to treatment. This paper can be very important for patients with suspected LOSPS to share with their doctor.

This article has easy to read tables which summarize: symptoms of each category of SPS (Table 1); treatments (Table 2); autoantibodies used to diagnose SPS (Table 3). The different categories of SPS may have different ways in which the disease manifests, so the paper discusses the gaps in knowledge that scientists need to look into in order to get a better understanding of what causes the disease for each category and how they each can be treated.

SPS can impact our mental health. The paper highlights the importance for doctors to do an assessment for any impairments in brain processing, including learning, recalling information, and speaking. Doctors should also assess the patient for depression or anxiety, which are common symptoms in SPS.

Through literature analysis, the authors of this review paper conclude that SPS patients are at higher risk for mental illnesses such as phobias and depression.

Neuromuscular blockers and volatile anesthetics may cause prolonged muscle weakness in SPS patients. It is not understood why. This paper discusses anesthesia techniques without the use of neuromuscular blockers for SPS patients to avoid muscle weakness.

A study of 15 SPS patients from the National Veterans Health Administration population showed that they all benefitted from antispasmodic medication. 10 of them benefitted from IVIg. Interestingly, all of them had normal cerebrospinal findings (obtained via lumbar puncture). Electrodiagnostic testing helped with diagnosis of SPS.

A few patients were treated using autologous haematopoietic stem cell transplantation (auto-HSCT) when other therapies were not effective. Table 1 summarizes the condition of each patient before treatment and Table 2 summarize the results of each patient after treatment.

This article provides a step-by-step, combination therapy plan for SPS. The therapies target two things: increasing GABA in the body and slowing down disease progression. Possible treatments for SPS patients include medications, IVIg, and plasmapheresis. The article also provides insight into what could happen with long-term use of these therapies and therapeutic challenges toward children, women planning pregnancy, and the elderly. The article also talks about possible future therapies.

In a small study (10 patients), anxiety and phobias seemed to be caused by SPS.

57 patients were studied for several years (Tables 1 and 2 summarize their symptoms and other diseases they had along with SPS). 46 patients showed progressive worsening of symptoms over the period of study.

While anti-GAD antibodies can indicate that a person has SPS, the level or absence of them do not correlate with disease severity.

Although the paper isn't focused just on SPS, it provides a general overview of paraneoplastic disorders, including a section on SPS. The most common type of SPS associated with glycine receptor is progressive encephalomyelitis with rigidty and myoclonus (PERM). Only 1-2% of SPS patients are of the paraneoplastic type.

This review paper covers the following topics: 1) biology of how autoantibodies cause muscle stiffness and spasms; 2. how having high GAD autoantibodies help doctors diagnose patients with SPS or other related disorders; 3) the difficulty of diagnosing patients with low GAD autoantibodies; 4) discussion of whether or not GAD causes SPS; 5) available treatments; 6) possible future treatments. Figure 4 has a diagram of the molecules related to SPS. Figure 5 shows a diagram of muscle contraction for better understanding of the mechanics of SPS. Figure 6 shows a comparison of what normal muscle contraction looks like vs muscle contraction in SPS.

A paper from 1999 which discusses the association of decreased levels of GABA with the development of spasms and stiffness.

Unfortunately, SPS is often misdiagnosed. This article offers a possible set of criteria to use for diagnosing SPS in Table 4.

High anti-GABARAP (GABA-A receptor associated protein) antibody levels may indicate that a patient's symptoms will be more severe than patients with lower levels of this antibody.