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SPS Literature Review

We've curated a list of representative academic literature references related to SPS and summarized their contents below. The summaries explain what each paper is about. Some of the summaries point out helpful figures and tables that we recommend looking at. Links are provided for direct access to the articles.

The articles are sorted from the newest publication date to the oldest.

 

Additionally. the articles are labeled by reading difficulty.

  • “Easy” articles are written in plain language, making them easy for most people to read.

  • “Medium” reading difficulty articles contain scientific language that can be understood by some people.

  • “Hard” articles contain a lot of scientific vocabulary and abbreviations, making them difficult to read for anyone without a scientific or medical background. 

 

Most of the literature is accessible to the public, although there are a few articles that we felt we should still share to cover the breadth of information as it pertains to SPS. Papers that are not open-access will have links to abstracts (and sometimes snippets of the article) that summarize what the paper is about. Patients may request any articles posted on Elsevier. You can find instructions on how to do that here


Note: Throughout the literature, you will read about the terms “antibody” and “autoantibody” as they relate to proteins like GAD. Please note that in the context of GAD, “antibody” and “autoantibody” are interchangeable. GAD is a specific type of antibody called an “autoantibody.” Antibodies typically help you to fight against diseases, however autoantibodies like GAD work against you to cause disease.

Brain γ-Aminobutyric Acid Changes in Stiff-Person Syndrome

Topic: Symptoms

Sub-Topic: GABA Levels in Brain

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.

Lucien M. Levy, MD, PhD
Igor Levy-Reis, MD
Mavis Fujii, MD
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2023

Open Access: Yes

The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome

Topic: Symptoms
Autoantibodies
Immunogenetic Marker

Sub-Topic: Stiffness
Anti-GAD65
DRβ1 0301 allele

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.

Marinos C. Dalakas, MD, FAAN
Mavis Fujii, MD
Mian Li, MD
Beverly McElroy, CNRN

Reading Difficulty: Medium

Publication Year: 2023

Open Access: No

A double-blind, placebo-controlled study of rituximab in patients with stiff person syndrome

Topic: Treatments

Sub-Topic: medications
Rituximab

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

Marinos C. Dalakas, MD, FAAN
Goran Rakocevic, MD
James M. Dambrosia, PhD
Harry Alexopoulos, DPhil
Beverly McElroy, CNRN

Reading Difficulty: Easy

Publication Year: 2023

Open Access: No

Efficacy of Intravenous Immunoglobulin in Neurological Diseases

Topic: Treatments

Sub-Topic: IVIg

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.

Jan D. Lünemann
Isaak Quast
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2023

Open Access: Yes

Cognitive and Mood Profiles Among Patients With Stiff Person Syndrome Spectrum Disorders

Topic: Symptoms

Sub-Topic: Psychiatric

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.

Carol K. Chan
Daniela A. Pimentel Maldonado
Yujie Wang, MD
Danielle Obando
Abbey J. Hughes
Scott D. Newsome, DO

Reading Difficulty: Medium

Publication Year: 2023

Open Access: Yes

Anesthesia in a patient with Stiff Person Syndrome

Topic: Adverse Drug Reactions

Sub-Topic: anesthesia

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.

Ozgur Yagan
Kadir Özyilmaz
Ahmet Özmadena
Özgür Sayin
Volkan Hanci

Reading Difficulty: Easy

Publication Year: 2023

Open Access: Yes

Anti–Glutamic Acid Decarboxylase Antibodies in the Serum and Cerebrospinal Fluid of Patients With Stiff-Person Syndrome: Correlation With Clinical Severity

Topic: Autoantibodies

Sub-Topic: Anti-GAD65

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

Goran Rakocevic, MD
Raghavanpillai Raju, PhD
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2023

Open Access: Yes

Long-term Effectiveness of IVIg Maintenance Therapy in 36 Patients With GAD Antibody–Positive Stiff-Person Syndrome

Topic: Treatments

Sub-Topic: 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.

Marinos C. Dalakas, MD, FAAN
Jessica Yi

Reading Difficulty: Easy

Publication Year: 2022

Open Access: Yes

High definition profiling of autoantibodies to glutamic acid decarboxylases GAD65/GAD67 in stiff-person syndrome

Topic: Autoantibodies

Sub-Topic: Anti-GAD65
Anti-GAD67

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.

Peter D. Burbelo
Sandra Groot
Marinos C. Dalakas, MD, FAAN
Michael J. Iadarola

Reading Difficulty: Hard

Publication Year: 2022

Open Access: Yes

Anti-Neuronal Antibodies Within the IVIg Preparations: Importance in Clinical Practice

Topic: Autoantibodies

Sub-Topic: IVIg

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.

Maria M. Dimitriadou
Harry Alexopoulos, DPhil
Sofia Akrivou
Eleni Gola
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Hard

Publication Year: 2022

Open Access: No

Paraneoplastic Neurologic Disorders

Topic: Diagnostic Criteria
Autoantibodies

Sub-Topic: PERM

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.

Michael Gilligan
Christopher McGuigan
Andrew McKeon, MD

Reading Difficulty: Easy

Publication Year: 2022

Open Access: Yes

Stiff-person Syndrome and GAD Antibody-spectrum Disorders: GABAergic Neuronal Excitability, Immunopathogenesis and Update on Antibody Therapies

Topic: Biology
Autoantibodies
Treatments

Sub-Topic: Anti-GAD65

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.

Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Easy

Publication Year: 2022

Open Access: Yes

Autoimmunity to GABAA-receptor-associated protein in stiff-person syndrome

Topic: Autoantibodies

Sub-Topic: Anti-GABARAP

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.

Raghavanpillai Raju, PhD
Goran Rakocevic, MD
Ziwei Chen
Gerard Hoehn
Cristina Semino-Mora
Wei Shi
Richard Olsen
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Hard

Publication Year: 2022

Open Access: Yes

Partial Stiff Person Syndrome as a Stroke Mimic (5382)

Topic: Diagnostic Criteria
Symptoms

Sub-Topic: Stroke

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.

Duarte Machado
Kendall Hodges

Reading Difficulty: Easy

Publication Year: 2021

Open Access: Yes

Intravenous Immunoglobulin in Autoimmune Neuromuscular Diseases

Topic: Treatments

Sub-Topic: IVIg

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.

Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Hard

Publication Year: 2021

Open Access: Yes

Analysis of GAD65 Autoantibodies in Stiff-Person Syndrome Patients

Topic: Autoantibodies
Biology

Sub-Topic: Anti-GAD65

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.

Raghavanpillai Raju, PhD
Jefferson Foote
J. Paul Banga
Tyler R. Hall
Carolyn J. Padoa
Marinos C. Dalakas, MD, FAAN
Eva Ortqvist
Christiane S. Hampe

Reading Difficulty: Hard

Publication Year: 2021

Open Access: Yes

Stiff Person Syndrome Misdiagnosis: Clinical and Ancillary Testing Characteristics

Topic: Diagnostic Criteria

Sub-Topic:

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.

Nicholas H. Chia
Andrew McKeon, MD
Eoin P. Flanagan
Divyanshu Dubey
Nicholas L. Zalewski
Sean J. Pittock, MD
Anastasia Zekeridou

Reading Difficulty: Easy

Publication Year: 2021

Open Access: No

Therapies in Stiff-Person Syndrome

Topic: Treatments
Children
Pregnancy
Elderly

Sub-Topic: medications
IVIg
plasmapheresis
future treatments

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.

Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Hard

Publication Year: 2021

Open Access: Yes

Stiff-man syndrome and variants: clinical course, treatments, and outcomes

Topic: Diagnostic Criteria
Autoantibodies

Sub-Topic: Anti-GAD65

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.

Andrew McKeon, MD
Maisha T Robinson
Kathleen M McEvoy
Joseph Y. Matsumoto, MD
Vanda A. Lennon, MD, PhD
J. Eric Ahlskog, PhD, MD
Sean J. Pittock, MD

Reading Difficulty: Medium

Publication Year: 2020

Open Access: No

Quantitative clinical and autoimmune assessments in stiff person syndrome: evidence for a progressive disorder

Topic: Symptoms Autoantibodies

Sub-Topic:

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.

Goran Rakocevic, MD
Harry Alexopoulos, DPhil
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Easy

Publication Year: 2020

Open Access: Yes

Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

Topic: Symptoms
Treatments
Autoantibodies
Animals
Pregnancy
Children

Sub-Topic: medications
IVIg

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.

Benjamin Vlad
Yujie Wang, MD
Scott D. Newsome, DO
Bettina Balint

Reading Difficulty: Easy

Publication Year: 2020

Open Access: Yes

Serotonin-Norepinephrine Reuptake Inhibitors May Exacerbate Stiff-Person Syndrome

Topic: Adverse Drug Reactions

Sub-Topic: SNRI

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

David R. Beavides, MD, PhD
Scott D. Newsome, DO

Reading Difficulty: Easy

Publication Year: 2019

Open Access: Yes

Autoimmune Neurogenic Dysphagia

Topic: Symptoms

Sub-Topic: Dysphagia

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

Stathopoulos
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2019

Open Access: Yes

Successful Autologous Hematopoietic Stem Cell Transplant in a Case of Stiff Person Spectrum Disorder with a positive Glycine Receptor antibody (P4-5.019)

Topic: Treatments

Sub-Topic: aHSCT

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.

Sofia Celli
Richard Nash
Constance McMenamin
Madeline Garza
Gloria Von Geldern
George Georges
Amanda L. Piquet

Reading Difficulty: Easy

Publication Year: 2019

Open Access: Yes

Expanding clinical profiles and prognostic markers in stiff person syndrome spectrum disorders

Topic: Symptoms

Sub-Topic:

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.

Yujie Wang, MD
Chen Hu
Salman Aljarallah
Maria Reyes Mantilla
Loulwah Mukharesh
Alexandera Simpson
Shuvro Roy
Kimystian Harrison
Thomas Shoemaker
Michael Comisac
Alexandra Balshi
Danielle Obando
Daniela A. Pimentel Maldonado
Jacqueline Koshorek
Sarah Snoops
Kathryn C. Fitzgerald
Scott D. Newsome, DO

Reading Difficulty: Medium

Publication Year: 2018

Open Access: Yes

High-Dose Intravenous Immune Globulin for Stiff-Person Syndrome

Topic: Treatments

Sub-Topic: IVIg

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.

Marinos C. Dalakas, MD, FAAN
Mavis Fujii, MD
Mian Li, MD
Bashar Lutfi, MD
Joan Kyhos, BSN
Beverly McElroy, CNRN

Reading Difficulty: Medium

Publication Year: 2018

Open Access: Yes

A neuropsychological assessment of phobias in patients with stiff person syndrome

Topic: Symptoms

Sub-Topic: Anxiety
Phobia

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

Rezvan Ameli
Joseph Snow
Goran Rakocevic, MD
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Easy

Publication Year: 2017

Open Access: Yes

The Stiff-Person Syndrome: An Autoimmune Disorder Affecting Neurotransmission of γ-Aminobutyric Acid

Topic: Pathogenesis

Sub-Topic: GABA Levels

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

Lucien M. Levy, MD, PhD
Marinos C. Dalakas, MD, FAAN
Mary Kay Floeter, MD, PhD

Reading Difficulty: Medium

Publication Year: 2016

Open Access: Yes

Adverse Reactions Associated with Intravenous Immunoglobulin Administration in the Treatment of Neurological Disorders: A Systematic Review

Topic: Treatments

Sub-Topic: IVIg

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

Melinda Jiang
James Stuart Kimber
Aashray Gupta
Joshua Kovoor
Brandon Stretton
Janakan Ravindran
Pravin Hissaria
William Bernard Smith
Stephen Bacchi

Reading Difficulty: Medium

Publication Year: 2016

Open Access: Yes

Defining the Expanding Clinical Spectrum of Pediatric-Onset Stiff Person Syndrome

Topic: Diagnostic Criteria Children

Sub-Topic:

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.

Anusha K. Yeshokumar, MD
Lisa R. Sun, MD
Scott D. Newsome, DO

Reading Difficulty: Easy

Publication Year: 2016

Open Access: Yes

Stiff person syndrome spectrum disorders; more than meets the eye

Topic: Symptoms
Treaments
Autoantibodies
Diagnostic Criteria

Sub-Topic: medications
IVIg

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.

Scott D. Newsome, DO
Tory Johnson

Reading Difficulty: Medium

Publication Year: 2016

Open Access: Yes

Variant Stiff Person Syndrome or Multiple Sclerosis?

Topic: Diagnostic Criteria

Sub-Topic: multiple sclerosis

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

Yujie Wang, MD
Scott D. Newsome, DO

Reading Difficulty: Easy

Publication Year: 2013

Open Access: No

Late-onset stiff-person syndrome: challenges in diagnosis and management

Topic: Diagnostic Criteria

Sub-Topic: Late-Onset SPS (LOSPS)

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.

Marinos C. Dalakas
Jessica Yi

Reading Difficulty: Medium

Publication Year: 2012

Open Access: Yes

Multiple anesthetics for a patient with stiff-person syndrome

Topic: Adverse Drug Reactions

Sub-Topic: anesthesia

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.

Jessica M. Cassavaugh, MD, PhD
Todd M. Oravitz, MD

Reading Difficulty: Medium

Publication Year: 2008

Open Access: Yes

Clinical and Immunologic Investigations in Patients With Stiff-Person Spectrum Disorder

Topic: Diagnostic Criteria
Autoantibodies

Sub-Topic: Anti-GAD65
glycine receptor autoantibody
antibody negative

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.

Eugenia Martinez-Hernandez, MD, PhD
Helena Ariño, MD
Andrew McKeon, MD
Takahiro Iizuka, MD
Maarten J. Titulaer, MD, PhD
Mateus M. Simabukuro, MD
Eric Lancaster, MD, PhD
Mar Petit-Pedrol, MS
Jesús Planagumà, PhD
Yolando Blanco, MD, PhD
Robert J. Harvey, Bsc, PhD
Albert Saiz, MD, PhD
Franesc Graus, MD, PhD
Josep Dalmau, MD, PhD

Reading Difficulty: Medium

Publication Year: 2006

Open Access: Yes

GAD antibody-spectrum disorders: progress in clinical phenotypes, immunopathogenesis and therapeutic interventions

Topic: Autoantibodies
Treatments
Diagnostic Criteria

Sub-Topic: medications

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.

Popianna Tsiortou
Harry Alexopoulos, DPhil
Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2005

Open Access: No

Stiff-Person Syndrome and Psychiatric Comorbidities: A Systematic Review

Topic: Symptoms

Sub-Topic:

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.

Deirdre Caffrey, M.D.
Christine T. Finn, M.D.
Seoho Michael Song, D.Phil
Frederick Burton III
Cybele Arsan, M.D.

Reading Difficulty: Easy

Publication Year: 2005

Open Access: No

Stiff Person Syndrome With Anti-GAD65 Antibodies Within the National Veterans Affairs Health Administration

Topic: Diagnostic Criteria
Autoanitbodies
Treatment

Sub-Topic: Anti-GAD65
IVIg
medications

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.

Jonathan R. Galli, MD
Sharon D. Austin, PhD
John E. Greenlee, MD
Stacey L. Clardy, MD, PhD

Reading Difficulty: Medium

Publication Year: 2005

Open Access: Yes

GAD antibodies in neurological disorders — insights and challenges

Topic: Diagnostic Criteria
Symptoms
Autoantibodies

Sub-Topic: Anti-GAD65

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.

Franesc Graus, MD, PhD
Albert Saiz, MD, PhD
Josep Dalmau, MD, PhD

Reading Difficulty: Easy

Publication Year: 2004

Open Access: No

Update on Intravenous Immunoglobulin in Neurology: Modulating Neuro-autoimmunity, Evolving Factors on Efficacy and Dosing and Challenges on Stopping Chronic IVIg Therapy

Topic: Treatments

Sub-Topic: IVIg

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.

Marinos C. Dalakas, MD, FAAN

Reading Difficulty: Medium

Publication Year: 2004

Open Access: Yes

Autologous haematopoietic stem cell transplantation for refractory stif‐person syndrome: the UK experience

Topic: Treatments

Sub-Topic: auto-HSCT

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.

Lewis Kass-Iliyya
John A. Snowden
Alice Thorpe
Helen Jessop
Andrew D. Chantry
Ptolemaios G. Sarrigiannis
Marios Hadjivassiliou
Basil Sharrack

Reading Difficulty: Medium

Publication Year: 2001

Open Access: Yes

Glycine receptor modulating antibody predicting treatable stiff-person spectrum disorders

Topic: Autoantibodies

Sub-Topic: glycine receptor autoantibody

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

Shannon R. Hinson, PhD
A. Sebastian Lopez-Chiriboga, MD
James H. Bower, MD
Joseph Y. Matsumoto, MD
Anhar Hassan, MBBCh
Eati Basal, PhD
Vanda A. Lennon, MD, PhD
Sean J. Pittock, MD
Andrew McKeon, MD

Reading Difficulty: Medium

Publication Year: 2000

Open Access: Yes

Novel clinical features of glycine receptor antibody syndrome: A series of 17 cases

Topic: Autoantibodies

Sub-Topic: glycine receptor autoantibody

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

Amanda L. Piquet
Murtaza Khan
Judith E.A. Warner
Matthew P. Wicklund
Jeffrey L. Bennett
Maureen A. Leehey
Lauren Seeberger
Teri L. Schreiner
M. Mateo Paz Soldan
Stacy L. Clardy

Reading Difficulty: Medium

Publication Year: 1999

Open Access: Yes

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