835 results match your criteria Stiff Person Syndrome


Progressive Encephalomyelitis With Rigidity: Stiff Person Syndrome Variant Associated With Multiple Sclerosis.

Prim Care Companion CNS Disord 2019 Feb 14;21(1). Epub 2019 Feb 14.

University of Louisville, Department of Psychiatry, Louisville, Kentucky, USA.

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http://dx.doi.org/10.4088/PCC.18l02360DOI Listing
February 2019

Teaching NeuroImages: Severe spasms resembling status dystonicus as an unusual presentation of stiff-person syndrome.

Neurology 2019 Feb;92(7):e748

From the Department of Neurology, University of Miami Miller School of Medicine, FL.

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http://dx.doi.org/10.1212/WNL.0000000000006925DOI Listing
February 2019

Neurological profiles beyond the sleep disorder in patients with anti-IgLON5 disease.

Curr Opin Neurol 2019 Jan 28. Epub 2019 Jan 28.

Parkinson's Disease and Movement Disorders Unit, Neurology Service, Hospital Clínic de Barcelona Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) Institut de Neurociències Centro de Investigación Biomedica en Red Enfermedades Neurodegenerativas (CIBERNED).

Purpose Of Review: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000677DOI Listing
January 2019
1 Read

Anti-GAD Antibody-associated Syndrome Presenting with Limb Myoclonus.

Tremor Other Hyperkinet Mov (N Y) 2018 1;8:590. Epub 2018 Nov 1.

Department of Neurology, Instituto Hospital de Base do Distrito Federal, Brasília, BR.

Background: The clinical spectrum of anti-glutamic acid decarboxylase (GAD) antibody-associated neurologic syndromes is expanding, with focal, generalized, and atypical forms.

Case Report: We describe a 59-year-old female showing continuous right lower limb myoclonus and mild encephalopathy. These symptoms started 2 weeks prior to evaluation. Read More

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http://dx.doi.org/10.7916/D8NK4XVPDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6315060PMC
November 2018

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

BMC Neurol 2019 Jan 3;19(1). Epub 2019 Jan 3.

Department of Neurology, Thomas Jefferson University, Philadelphia, USA.

Background: Stiff Person Syndrome (SPS) is an under-diagnosed disorder that affects mobility and the quality of life of affected patients. The aim of the study is to describe the natural history of SPS, the extent of accumulated disability and the associated clinical and immunological features in patients followed for up to 8 years in a single center.

Methods: Our collective cohort included 57 SPS patients. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-018-1232-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317182PMC
January 2019
2 Reads

Successful Anesthetic Management of Patient with Stiff Person Syndrome.

J Coll Physicians Surg Pak 2018 Dec;28(12):967-969

Department of Anaesthesiology, The Aga Khan University Hospital, Karachi, Pakistan.

We herein describe the successful anesthetic management of a patient with stiff person syndrome undergoing right total hip replacement under spinal anesthesia. We also describe the problems associated with general anesthesia. The advantage of using regional anesthesia in these patients is the avoidance of muscle relaxants. Read More

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http://dx.doi.org/10.29271/jcpsp.2018.12.967DOI Listing
December 2018
1 Read
0.318 Impact Factor

Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.

Curr Oncol Rep 2018 Nov 10;20(11):92. Epub 2018 Nov 10.

Neurology Division, Hospital das Clínicas, University of São Paulo School of Medicine, Sao Paulo, Brazil.

The disorders of the central nervous system associated with cancer by remote immune-mediated mechanisms are a heterogeneous group. These disorders encompass the classic paraneoplastic disorders and the recently recognized autoimmune encephalitis associated with antibodies against neuronal cell surface or synaptic proteins that occur with or without cancer association. In the last decade, the new surge of interest in neuronal diseases associated with anti-neuronal antibodies led to the rapid discovery of new forms of disease that have different manifestations and were not previously suspected to be immune mediated. Read More

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http://link.springer.com/10.1007/s11912-018-0721-y
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http://dx.doi.org/10.1007/s11912-018-0721-yDOI Listing
November 2018
13 Reads

Pragmatic Treatment of Stiff Person Spectrum Disorders.

Mov Disord Clin Pract 2018 Jul-Aug;5(4):394-401. Epub 2018 Jul 19.

Department of Neurology University Hospital Heidelberg Germany.

Background: Stiff person spectrum disorders (SPSD) are a group of rare conditions clinically characterized by stiffness, spasms, and heightened stimulus sensitivity. They also share a spectrum of antibodies.

Methods: We reviewed the literature and our own experience with the aim of providing a practical approach to the treatment of SPSD. Read More

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http://doi.wiley.com/10.1002/mdc3.12629
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http://dx.doi.org/10.1002/mdc3.12629DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174384PMC
July 2018
18 Reads

Anti-GAD Antibodies and the Cerebellum: Where Do We Stand?

Cerebellum 2018 Oct 20. Epub 2018 Oct 20.

University of Washington, School of Medicine, Seattle, WA, 98109, USA.

Anti-GAD65 antibodies (anti-GAD65 Abs) are associated with cerebellar ataxia (CA). The significance of anti-GAD65 Abs has been a focus of debates. Since GAD65 is intracellularly located and associated with type 1 diabetes mellitus and different clinical neurological phenotypes such as CA, stiff-person syndrome, and epilepsy, some researchers have argued that anti-GAD65 Abs have no pathogenic roles. Read More

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http://link.springer.com/10.1007/s12311-018-0986-6
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http://dx.doi.org/10.1007/s12311-018-0986-6DOI Listing
October 2018
8 Reads

A case report of rigidity and recurrent lower limb myoclonus: progressive encephalomyelitis rigidity and myoclonus syndrome, a chameleon.

BMC Neurol 2018 Oct 18;18(1):173. Epub 2018 Oct 18.

Department of Neurology, CHU Ambroise Paré Hospital, Boulevard John Fitzgerald Kennedy 2, 7000, Mons, Belgium.

Background: Progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome is a rare neurological condition. Its clinical characteristics include axial and limb muscle rigidity, myoclonus, painful spasms and hyperekplexia. Diagnosis of this disease can be very challenging and optimal long-term treatment is unclear. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-018-1176-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6193294PMC
October 2018
18 Reads

Reduced display of conformational epitopes in the N-terminal truncated GAD65 isoform: relevance for people with stiff person syndrome or DQ8/8-positive Type 1 diabetes mellitus.

Diabet Med 2018 Sep 27. Epub 2018 Sep 27.

Department of Clinical Sciences, Lund University/CRC, Skåne University Hospital SUS, Malmo, Sweden.

Aims: To investigate whether the N-terminal truncated glutamic acid decarboxylase 65 (GAD65) isoform is as well recognized by people with stiff person syndrome as it is by people with Type 1 diabetes, and whether conformational GAD65 antibody epitopes are displayed properly by the isoform.

Methods: GAD65 antibody-positive healthy individuals (n=13), people with stiff-person syndrome (n=15) and children with new-onset Type 1 diabetes (n=654) were analysed to determine binding to full-length GAD65 and the N-terminal truncated GAD65 isoform in each of these settings. GAD65 autoantibody epitope specificity was correlated with binding ratios of full-length GAD65/N-terminal truncated GAD65. Read More

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http://doi.wiley.com/10.1111/dme.13827
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http://dx.doi.org/10.1111/dme.13827DOI Listing
September 2018
7 Reads

The clinical features, underlying immunology, and treatment of autoantibody-mediated movement disorders.

Mov Disord 2018 Sep 14;33(9):1376-1389. Epub 2018 Sep 14.

Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.

An increasing number of movement disorders are associated with autoantibodies. Many of these autoantibodies target the extracellular domain of neuronal surface proteins and associate with highly specific phenotypes, suggesting they have pathogenic potential. Below, we describe the phenotypes associated with some of these commoner autoantibody-mediated movement disorders, and outline increasingly well-established mechanisms of autoantibody pathogenicity which include antigen downregulation and complement fixation. Read More

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http://dx.doi.org/10.1002/mds.27446DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221172PMC
September 2018
3 Reads

Stiff person syndrome with Anti-GAD65 antibodies within the national veterans affairs health administration.

Muscle Nerve 2018 Dec 20;58(6):801-804. Epub 2018 Nov 20.

Department of Neurology, University of Utah, Imaging and Neurosciences Center, 729 Arapeen Drive, Salt Lake City, Utah, 84108, USA.

Introduction: Stiff person syndrome (SPS) is a neurological disorder characterized by muscle rigidity primarily in the truncal muscles, commonly associated with autoantibodies to the glutamic acid-decarboxylase 65 kD receptor (GAD65). There is limited epidemiological information on patients with SPS.

Methods: We performed a retrospective case review using the National United States Veterans Affairs Health Administration electronic medical record system. Read More

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http://dx.doi.org/10.1002/mus.26338DOI Listing
December 2018
2 Reads

Impaired Glycine Receptor Trafficking in Neurological Diseases.

Front Mol Neurosci 2018 21;11:291. Epub 2018 Aug 21.

Institute for Clinical Neurobiology, Julius-Maximilians-University Würzburg, Würzburg, Germany.

Ionotropic glycine receptors (GlyRs) enable fast synaptic neurotransmission in the adult spinal cord and brainstem. The inhibitory GlyR is a transmembrane glycine-gated chloride channel. The immature GlyR protein undergoes various processing steps, e. Read More

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http://dx.doi.org/10.3389/fnmol.2018.00291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6110938PMC
August 2018
9 Reads

Stiff person syndrome with elevated titers of antibodies against cardiolipin and β2 glycoprotein 1: a case report and literature review.

J Musculoskelet Neuronal Interact 2018 Sep;18(3):389-392

Department of neurology, PLA Army General Hospital, Beijing, People's Republic of China.

We reported a Stiff person syndrome (SPS) patient with elevated autoantibodies against cardiolipin and β2 glycoprotein 1 but without glutamic acid decarboxylase (GAD) antibodies. A 40-year male was admitted due to limited mouth opening for 1 week. His blood routine, biochemical, infectious diseases, tumor markers, radiographic examinations were all normal. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6146186PMC
September 2018

Paraneoplastic stiff person syndrome with small cell carcinoma of the bladder and anti-Ri antibodies.

Clin Neurol Neurosurg 2018 Oct 16;173:194-195. Epub 2018 Aug 16.

Hospital Ruber Internacional, Department of Neurology, La Masó Street, 38, CP 28034, Madrid, Spain. Electronic address:

The stiff person syndrome is a rare neurological disorder, difficult to diagnose and to treat. Paraneoplastic patients usually present amphiphysin antibodies but the association with anti-Ri antibodies is less known. We present a case report of paraneoplastic SPS, small cell carcinoma of the bladder and anti-Ri antibodies. Read More

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http://dx.doi.org/10.1016/j.clineuro.2018.08.020DOI Listing
October 2018
3 Reads

Gravity-Independent Upbeat Nystagmus in Syndrome of Anti-GAD Antibodies.

Cerebellum 2018 Aug 22. Epub 2018 Aug 22.

Department of Neurology, Cleveland VA Medical Center, University Hospitals, Cleveland, OH, 44022, USA.

An autoimmune disorder of the central nervous system, stiff person syndrome, frequently presents with increased titers of 65KD anti-glutamic acid decarboxylase (anti-GAD) antibodies. The clinical phenomenology of this syndrome includes stiffness, ataxia, vertigo due to horizontal gaze-evoked and downbeat vertical nystagmus, and dysmetria of saccades and reaching movements. Here, we describe a novel phenomenology of syndrome of anti-GAD antibody, non-position-dependent upbeat nystagmus and superimposed horizontal gaze-evoked nystagmus. Read More

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http://link.springer.com/10.1007/s12311-018-0972-z
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http://dx.doi.org/10.1007/s12311-018-0972-zDOI Listing
August 2018
22 Reads

Inpatient care for stiff person syndrome in the United States: a nationwide readmission study.

J Clin Mov Disord 2018 6;5. Epub 2018 Aug 6.

1Department of Neurology, University of Pennsylvania Perelman School of Medicine, Blockley Hall, 423 Guardian Drive, Office 829, Philadelphia, PA 19104 USA.

Background: Stiff person syndrome (SPS) is a progressive neurological disorder characterized by axial muscle rigidity and involuntary spasms. Autoimmune and neoplastic diseases are associated with SPS. Our study objectives were to describe inpatient care for SPS in the United States and characterize 30-day readmissions. Read More

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http://dx.doi.org/10.1186/s40734-018-0071-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6091149PMC
August 2018
2 Reads

Prognostic Factors in Anti-Neuronal Antibody Positive Patients.

Noro Psikiyatr Ars 2018 4;55(2):189-194. Epub 2018 May 4.

Department of Neurology, Istanbul University, Faculty of Medicine, İstanbul, Turkey.

Introduction: Anti-neuronal antibodies (ANA) are found in paraneoplastic neurological syndrome and autoimmune encephalitis patients. Our aim was to analyze prognostic factors related with ANA seropositivity.

Methods: Twenty-seven consecutive ANA seropositive patients were included in the study. Read More

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http://dx.doi.org/10.29399/npa.23033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6060648PMC
May 2018
18 Reads

Longitudinal gait assessment in a stiff person syndrome.

Int J Rehabil Res 2018 Dec;41(4):377-379

Department of Neurosciences, IRCCS Bambino Gesù Children Hospital.

Stiff person syndrome (SPS) is an autoimmune disorder with multiple clinical presentations, all characterized by generalized or focal muscular stiffness leading to abnormal postures and movements. To date, no standardized treatments are available; also, the outcome measures are mainly clinical based and unstandardized, limiting the reliability of clinical trials. In this case study, we used the eight-camera motion capture system for gait analysis (GA) to outline the gait features and track the clinical evolution of a young patient with SPS receiving a personalized multimodal therapy. Read More

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http://Insights.ovid.com/crossref?an=00004356-900000000-9948
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http://dx.doi.org/10.1097/MRR.0000000000000304DOI Listing
December 2018
24 Reads

Stiff-person Syndrome with Waldenström Macroglobulinemia.

Intern Med 2018 Nov 6;57(22):3313-3316. Epub 2018 Jul 6.

Department of Hematology, Hakodate City Hospital, Japan.

We herein report the case of stiff-person syndrome in a 73-year-old woman. She experienced episodes of painful muscle spasms and was admitted to another hospital. She was diagnosed with Waldenström macroglobulinemia. Read More

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http://dx.doi.org/10.2169/internalmedicine.1043-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287988PMC
November 2018
5 Reads

[A pedigree of hereditary hyperekplexia].

Rinsho Shinkeigaku 2018 Jul 30;58(7):435-439. Epub 2018 Jun 30.

Department of Neurology, TOYOTA Memorial Hospital.

A 31-year old women presented with excessive startle reflex and frequent falls. Her startle reflex is induced by slight stimuli which are not problematic in most people. Soon after her startle reflex is evoked, generalized muscle stiffness occurs. Read More

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http://dx.doi.org/10.5692/clinicalneurol.cn-001129DOI Listing
July 2018
24 Reads

Case report of a woman with anti amphiphysin positive stiff person syndrome.

Ideggyogy Sz 2017 May;70(5-6):213-216

Department of Functional Neurosurgery, National Institut of Clinical Neuroscience, Budapest, Hungary.

Stiff person syndrome is a rare neuroimmunological disease, characterized by severe, involuntary stiffness with superimposed painful muscle spasms, which are worsened by external stimuli. The classical form is associated with high levels of antibodies against glutamic acid decarboxylase. One of the variant forms is associated with antibodies against amphiphysin. Read More

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http://www.elitmed.hu/isz.70.0213
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http://dx.doi.org/10.18071/isz.70.0213DOI Listing
May 2017
10 Reads

Practical issues in measuring autoantibodies to neuronal cell-surface antigens in autoimmune neurological disorders: 190 cases.

J Neurol Sci 2018 Jul 8;390:26-32. Epub 2018 Apr 8.

Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan. Electronic address:

Objectives: To address practical issues in measuring autoantibodies to neuronal cell-surface antigens (NSAs) in various autoimmune neurological disorders (ANDs).

Methods: We retrospectively reviewed the clinical information of 221 patients with clinically suspected ANDs who underwent antibody testing for NSAs between January 2007 and September 2017. 31 were excluded. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X183017
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http://dx.doi.org/10.1016/j.jns.2018.04.009DOI Listing
July 2018
4 Reads

Anaesthetic management of a patient with a unique combination of anti-N-methyl-D-aspartate receptor encephalitis and stiff-person syndrome.

BMJ Case Rep 2018 May 7;2018. Epub 2018 May 7.

Department of Anesthesiology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA.

Stiff-person syndrome (SPS) and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are rare paraneoplastic syndromes caused by antibodies that target the central nervous system. Here, we describe a 26-year-old woman who presented with psychosis, amnesia, rigidity and fever. After extensive diagnostic and laboratory workup, she was diagnosed with an ovarian teratoma which was causing the symptoms of anti-NMDAR encephalitis and SPS. Read More

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http://dx.doi.org/10.1136/bcr-2017-223261DOI Listing
May 2018
6 Reads

Stiff Person Syndrome With Evidence of Nonspecific Focal Myositis Secondary to Sustained Muscle Contraction: A Case Report.

PM R 2018 Dec 26;10(12):1426-1430. Epub 2018 Apr 26.

Department of Rehabilitation Medicine, Dongsan Medical Center, School of Medicine, Keimyung University, 56 Dalseong-ro, Jung-gu, Daegu 41931, South Korea(‡). Electronic address:

Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive muscular rigidity and spasms that affect axial and limb muscles. There have been a few reports that patients with SPS had evidences of polymyositis. There have been no clear explanations about the characteristics of polymyositis in SPS. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S19341482183021
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http://dx.doi.org/10.1016/j.pmrj.2018.04.007DOI Listing
December 2018
5 Reads

Progressive encephalomyelitis with rigidity and myoclonus, a diagnostic challenge.

Rev Neurol (Paris) 2018 May 22;174(5):343-346. Epub 2018 Apr 22.

Département de Neurologie, Hôpital de Hautepierre, CHU de Strasbourg, Strasbourg, France; Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.

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https://linkinghub.elsevier.com/retrieve/pii/S00353787173059
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http://dx.doi.org/10.1016/j.neurol.2017.09.012DOI Listing
May 2018
1 Read

GAD65 autoantibody characteristics in patients with co-occurring type 1 diabetes and epilepsy may help identify underlying epilepsy etiologies.

Orphanet J Rare Dis 2018 Apr 10;13(1):55. Epub 2018 Apr 10.

Department of Medicine, School of Medicine, University of Washington, 850 Republican, Seattle, WA, 98109, USA.

Background: Autoantibodies against the smaller isoform of glutamate decarboxylase (GAD65Ab) reflect autoimmune etiologies in Type 1 diabetes (T1D) and several neurological disorders, including Stiff Person Syndrome (SPS). GAD65Ab are also reported in cases of epilepsy, indicating an autoimmune component. GAD65Ab in patients with co-occurring T1D, epilepsy or SPS may be part of either autoimmune pathogenesis. Read More

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http://dx.doi.org/10.1186/s13023-018-0787-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5892043PMC
April 2018
5 Reads

[Stiff-Person Syndrome and Related Autoantibodies].

Brain Nerve 2018 Apr;70(4):357-362

Department of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate School.

Stiff-person syndrome (SPS) is a disorder characterized by fluctuating muscle rigidity and painful spasms that occur spontaneously or are triggered by diverse stimuli. Partial or segmental forms of the disorder, such as stiff-limb syndrome (SLS) and a more severe disease called progressive encephalomyelitis with rigidity and myoclonus (PERM), are usually considered within the spectrum of SPS. SPS responds to immunotherapies, and several autoantigens have been identified. Read More

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http://dx.doi.org/10.11477/mf.1416201008DOI Listing
April 2018
3 Reads

Paraneoplastic movement disorders.

Rev Neurosci 2018 09;29(7):745-755

Department of Neurochemistry and Neuropathology, Poznan University of Medical Sciences, 49, Przybyszewskiego Str., 60355 Poznan, Poland.

Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Read More

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http://dx.doi.org/10.1515/revneuro-2017-0081DOI Listing
September 2018
27 Reads

Case Series: Intrathecal Baclofen Therapy in Stiff-Person Syndrome.

Neuromodulation 2018 Oct 13;21(7):655-659. Epub 2018 Mar 13.

Mellen Center for Multiple Sclerosis Treatment and Research, Neurological Institute, The Cleveland Clinic Foundation, Cleveland, OH, USA.

Objectives: Stiff-person syndrome (SPS) is associated with axial rigidity superimposed on sustained muscle spasms. These symptoms commonly interfere with the performance of activities of daily living including ambulation. This retrospective case series evaluates the outcomes of screening tests and chronic infusion of intrathecal baclofen (ITB) in patients diagnosed with SPS treated in our spasticity clinic. Read More

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http://dx.doi.org/10.1111/ner.12765DOI Listing
October 2018
3 Reads

Glycine receptor modulating antibody predicting treatable stiff-person spectrum disorders.

Neurol Neuroimmunol Neuroinflamm 2018 Mar 23;5(2):e438. Epub 2018 Jan 23.

Department of Laboratory Medicine and Pathology (S.R.H., E.B., V.A.L., S.J.P., A.M.), Department of Neurology (A.S.L.-C., J.H.B., J.Y.M., A.H., V.A.L., S.J.P., A.M.), and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.

Background: Glycine receptor alpha-1 subunit (GlyRα1)-immunoglobulin G (IgG) is diagnostic of stiff-person syndrome (SPS) spectrum but has been reported detectable in other neurologic diseases for which significance is less certain.

Methods: To assess GlyRα1-IgGs as biomarkers of SPS spectrum among patients and controls, specimens were tested using cell-based assays (binding [4°C] and modulating [antigen endocytosing, 37°C]). Medical records of seropositive patients were reviewed. Read More

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http://nn.neurology.org/lookup/doi/10.1212/NXI.0000000000000
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http://dx.doi.org/10.1212/NXI.0000000000000438DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5813079PMC
March 2018
4 Reads

Common variable immunodeficiency associated with stiff-person syndrome.

Ann Allergy Asthma Immunol 2018 Apr;120(4):444

University Hospitals, Cleveland Medical Center, Cleveland, Ohio; Allergy/Immunology Association, Inc, Mayfield Heights, Ohio.

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http://dx.doi.org/10.1016/j.anai.2018.01.002DOI Listing
April 2018
4 Reads

Autoimmune and paraneoplastic movement disorders: An update.

J Neurol Sci 2018 02 30;385:175-184. Epub 2017 Dec 30.

Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, USA. Electronic address:

Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. Read More

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http://dx.doi.org/10.1016/j.jns.2017.12.035DOI Listing
February 2018
16 Reads

[From stiff man syndrome to stiff person spectrum disorders].

Authors:
H-M Meinck B Balint

Nervenarzt 2018 Feb;89(2):207-218

UCL Institute of Neurology, Sobell Department of Motor Neuroscience and Movement Disorders, National Hospital of Neurology and Neurosurgery, Queen Square, London, UK.

The identification of new variants of the stiff man syndrome (SMS) and of new, probably pathogenic neuronal autoantibodies has led to the concept of stiff man (or person) spectrum disorders (SPSD). This is an expanding group of rare chronic autoimmune inflammatory diseases of the central nervous system (CNS) that have in common the main symptoms of fluctuating rigidity and spasms with pronounced stimulus sensitivity. These core symptoms are mandatory and can be accompanied by a wide variety of other neurological signs. Read More

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http://link.springer.com/10.1007/s00115-017-0480-2
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http://dx.doi.org/10.1007/s00115-017-0480-2DOI Listing
February 2018
8 Reads

Multiplex family with GAD65-Abs neurologic syndromes.

Neurol Neuroimmunol Neuroinflamm 2018 Jan 5;5(1):e416. Epub 2017 Dec 5.

French Reference Center on Paraneoplastic Neurological Syndrome (A.B., B.J., V.R., J.H.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310 (A.B., B.J., V.R., J.H.), Université de Lyon-Université Claude Bernard Lyon 1, France; Stanford Blood Center (G.M.-M., M.F.-V., E.M.), Histocompatibility, Immunogenetics & Disease Profiling Laboratory, Palo Alto, CA; Immunology, Hospices Civils de Lyon (N.F.), Hôpital Lyon-Sud, France; and Stanford University Center for Sleep Sciences and Medicine (E.M), Palo Alto, CA.

Objective: Neurologic autoimmune syndromes associated with anti-glutamate acid decarboxylase 65 antibodies (GAD65-Abs) are rare and mostly sporadic.

Methods: We describe a niece and her aunt with GAD65-Abs neurologic syndromes. High-resolution HLA typing of Class I and Class II alleles was performed using next-generation sequencing. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000416DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5778747PMC
January 2018
7 Reads

Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: a case series.

Acta Neurol Belg 2018 Sep 18;118(3):411-414. Epub 2018 Jan 18.

Department of Pediatric Immunology, Cukurova University Faculty of Medicine, Adana, Turkey.

Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients including stiff person syndrome, cerebellar ataxia, refractory epilepsy, limbic and extralimbic encephalitis. GAD antibodies-related limbic encephalitis cases are well described; reports of extralimbic involvement are limited. We describe four cases of GAD antibody-related autoimmune encephalitis. Read More

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http://link.springer.com/10.1007/s13760-018-0880-5
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http://dx.doi.org/10.1007/s13760-018-0880-5DOI Listing
September 2018
15 Reads

Neurologic disorders associated with anti-glutamic acid decarboxylase antibodies: A comparison of anti-GAD antibody titers and time-dependent changes between neurologic disease and type I diabetes mellitus.

J Neuroimmunol 2018 Apr 9;317:84-89. Epub 2018 Jan 9.

Department of Internal Medicine, Seikeikai Hospital, Sakai, Osaka 590-0064, Japan.

To determine clinical features of neurologic disorders associated with anti-glutamic acid decarboxylase antibodies (anti-GAD-Ab), we examined titers and time-dependent changes of anti-GAD-Ab. Six patients, stiff person syndrome (2), cerebellar ataxia (1), limbic encephalitis (1), epilepsy (1), brainstem encephalitis (1), were compared with 87 type I diabetes mellitus (T1DM) patients without neurologic disorders. Anti-GAD-Ab titers and index were higher in neurologic disorders than in T1DM, suggesting intrathecal antibody synthesis. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2018.01.007DOI Listing
April 2018
9 Reads

Teaching Video NeuroImages: Electromyographic variation in stiff-person syndrome.

Neurology 2018 Jan;90(3):e262

From the Department of Neurology and Neuroscience Center, the First Hospital of Jilin University, Changchun, China.

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http://dx.doi.org/10.1212/WNL.0000000000004831DOI Listing
January 2018
40 Reads

[From 'Involuntary Movements' to 'Movement Disorders'].

Authors:
Toshio Fukutake

Brain Nerve 2017 Dec;69(12):1359-1371

Department of Neurology Kameda Medical Center.

Apart from the term 'involuntary movements', the term 'movement disorders' encompasses not only classical hyperkinesias but also hypokinesias including catalepsy or apraxia. It enables us to understand abnormal movements by their phenomena instead of by their localization. To advance the grasping of movement disorders, we discuss the clinical and pathophysiological features of abnormal movements in catatonia/catalepsy, anti-NMDAR encephalitis, paroxysmal dyskinesias, stiff person/leg syndrome, corticobasal degeneration/syndrome, and hysteria. Read More

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http://dx.doi.org/10.11477/mf.1416200921DOI Listing
December 2017
3 Reads

Difficult to Treat Focal, Stiff Person Syndrome of the Left Upper Extremity.

Case Rep Neurol Med 2017 25;2017:2580620. Epub 2017 Oct 25.

Department of Internal Medicine, Orlando VA Medical Center, 13800 Veterans Way, Orlando, FL 32827, USA.

Background: Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle rigidity. It is a disorder of reduced GABA activity leading to increased muscle tone and often painful spasms. It generally presents in the axial musculature but rarely can involve only one limb, typically a lower extremity. Read More

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https://www.hindawi.com/journals/crinm/2017/2580620/
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http://dx.doi.org/10.1155/2017/2580620DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5676382PMC
October 2017
12 Reads

Stiff person case misdiagnosed as conversion disorder: A case report.

Caspian J Intern Med 2017 ;8(4):329-331

Department of Neurology, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.

Background: Stiff person syndrome (SPS) is a rare neurological disease resulting in stiffness and spasm of muscles. It initially affects the axial muscles and then spread to limb muscles. Emotional stress exacerbated the symptoms and signs of the disease. Read More

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http://caspjim.com/article-1-868-en.html
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http://dx.doi.org/10.22088/cjim.8.4.329DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5686315PMC
January 2017
9 Reads

Paraneoplastic Disorders.

Authors:
Eric Lancaster

Continuum (Minneap Minn) 2017 12;23(6, Neuro-oncology):1653-1679

Purpose Of Review: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. Read More

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http://Insights.ovid.com/crossref?an=00132979-201712000-0001
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http://dx.doi.org/10.1212/CON.0000000000000542DOI Listing
December 2017
7 Reads

Paraneoplastic Stiff Person Syndrome Secondary to Pancreatic Adenocarcinoma.

J Gastrointest Surg 2018 Jan 6;22(1):172-174. Epub 2017 Nov 6.

Department of Hepatopancreatobiliary and Transplant Surgery, Singapore General Hospital, 20 College Road, Singapore, 169856, Singapore.

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http://link.springer.com/10.1007/s11605-017-3611-y
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http://dx.doi.org/10.1007/s11605-017-3611-yDOI Listing
January 2018
11 Reads

Paraneoplastic autoimmune movement disorders.

Authors:
Thien Thien Lim

Parkinsonism Relat Disord 2017 Nov 13;44:106-109. Epub 2017 Oct 13.

Island Hospital, 308 Macalister Road, 10450 Penang, Malaysia. Electronic address:

Purpose Of Review: To provide an overview of paraneoplastic autoimmune disorders presenting with various movement disorders.

Recent Findings: The spectrum of paraneoplastic autoimmune disorders has been expanding with the discovery of new antibodies against cell surface and intracellular antigens. Many of these paraneoplastic autoimmune disorders manifest as a form of movement disorder. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2017.08.017DOI Listing
November 2017
25 Reads

Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology.

Brain 2018 Jan;141(1):13-36

Sobell Department of Motor Neuroscience and Movement Disorders UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Movement disorders are a prominent and common feature in many autoantibody-associated neurological diseases, a group of potentially treatable conditions that can mimic infectious, metabolic or neurodegenerative disease. Certain movement disorders are likely to associate with certain autoantibodies; for example, the characteristic dyskinesias, chorea and dystonia associated with NMDAR antibodies, stiff person spectrum disorders with GAD, glycine receptor, amphiphysin or DPPX antibodies, specific paroxysmal dystonias with LGI1 antibodies, and cerebellar ataxia with various anti-neuronal antibodies. There are also less-recognized movement disorder presentations of antibody-related disease, and a considerable overlap between the clinical phenotypes and the associated antibody spectra. Read More

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http://dx.doi.org/10.1093/brain/awx189DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5888977PMC
January 2018
39 Reads

[Differential Diagnosis of Immune-Mediated Encephalopathies: "Neurological Symptoms of Diffuse Brain Damage": A New Concept].

Brain Nerve 2017 Oct;69(10):1131-1141

Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences.

In recent years, incidence of autoimmune encephalopathies has increased. The diagnosis of the severe form of autoimmune encephalopathy is not difficult; however, milder forms can be misdiagnosed as general encephalopathies. We often treat Hashimoto's encephalopathy, which has diverse clinical symptoms and is often misdiagnosed as a psychosomatic disease. Read More

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https://webview.isho.jp/journal/detail/abs/10.11477/mf.14162
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http://dx.doi.org/10.11477/mf.1416200881DOI Listing
October 2017
8 Reads

Treatment and Resolution of Filamentary Keratitis in a Patient with Stiff Person Syndrome.

Cornea 2018 Feb;37(2):258-259

Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, ON, Canada.

Purpose: To report a rare case of filamentary keratitis associated with stiff person syndrome.

Methods: Case report.

Results: A 26-year-old woman with several autoimmune conditions was referred because of filamentary keratitis. Read More

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http://dx.doi.org/10.1097/ICO.0000000000001416DOI Listing
February 2018
27 Reads

Neuronal autoantibodies: differentiating clinically relevant and clinically irrelevant results.

J Neurol 2017 Nov 3;264(11):2284-2292. Epub 2017 Oct 3.

Neurology Department, Johns Hopkins University, Baltimore, MD, USA.

The aim of this study is to compare the rates of clinically relevant and clinically irrelevant neuronal autoantibodies among patients presenting with new neurological symptoms. We reviewed 401 neurological patients who were tested for the Mayo-Clinic paraneoplastic panel from January 2014 to December 2014 at the Johns Hopkins Hospital. We divided antibody-positive patients into two groups: clinically relevant (CR), in which a recognizable autoimmune or paraneoplastic syndrome was confirmed, and clinically irrelevant (CI), in which an autoimmune/paraneoplastic etiology was initially suspected but an alternative diagnosis was eventually found. Read More

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http://link.springer.com/10.1007/s00415-017-8627-4
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http://dx.doi.org/10.1007/s00415-017-8627-4DOI Listing
November 2017
31 Reads

Why It Is Not Always Anxiety: A Tough Diagnosis of Stiff Person Syndrome.

Case Rep Neurol Med 2017 14;2017:7431092. Epub 2017 Aug 14.

Department of Medicine, Aventura Hospital and Medical Center, Aventura, FL, USA.

Anxiety disorder is a commonly used diagnosis that may mask underlying conditions. Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive rigidity and painful muscle spasms affecting axial and lower extremity musculature. These episodes can be triggered by sudden movement, noise, or emotional stress, which may present as a psychiatric condition. Read More

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http://dx.doi.org/10.1155/2017/7431092DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5584351PMC
August 2017
12 Reads