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Stiff person syndrome

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https://www.readbyqxmd.com/read/29984750/stiff-person-syndrome-with-waldenstr%C3%A3-m-macroglobulinemia-a-case-report
#1
Kazuhiro Horiuchi, Yasunori Maruo, Satomi Matsuoka
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. She showed improvement in muscle spasms post-chemotherapy, which was discontinued due to pancytopenia. Six months later, she was admitted to our hospital for repeated whole-body muscle spasms, at which point she was diagnosed with stiff-person syndrome. An anti-glutamic acid decarboxylase antibody text was negative...
July 6, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29858636/shear-wave-elastography-a-new-potential-method-to-diagnose-ulnar-neuropathy-at-the-elbow
#2
Łukasz Paluch, Bartłomiej Noszczyk, Żaneta Nitek, Jerzy Walecki, Katarzyna Osiak, Piotr Pietruski
OBJECTIVES: The primary aim of this study was to verify if shear-wave elastography (SWE) can be used to diagnose ulnar neuropathy at the elbow (UNE). The secondary objective was to compare the cross-sectional areas (CSA) of the ulnar nerve in the cubital tunnel and to determine a cut-off value for this parameter accurately identifying persons with UNE. METHODS: The study included 34 patients with UNE (mean age, 59.35 years) and 38 healthy controls (mean age, 57...
June 1, 2018: European Radiology
https://www.readbyqxmd.com/read/29801900/practical-issues-in-measuring-autoantibodies-to-neuronal-cell-surface-antigens-in-autoimmune-neurological-disorders-190-cases
#3
Juntaro Kaneko, Naomi Kanazawa, Naomi Tominaga, Atsushi Kaneko, Hiroki Suga, Ryo Usui, Daisuke Ishima, Eiji Kitamura, Tsugio Akutsu, Koji Yoshida, Kazutoshi Nishiyama, Takahiro Iizuka
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. In 190 patients, antibody-detection rate (ADR) and antibody-phenotype association were assessed...
July 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29735495/anaesthetic-management-of-a-patient-with-a-unique-combination-of-anti-n-methyl-d-aspartate-receptor-encephalitis-and-stiff-person-syndrome
#4
Mohammad Hadi Gharedaghi, Arjang Khorasani, Nebojsa Nick Knezevic, Farzad Ebrahimi
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. The patient was successfully treated with laparoscopic removal of the ovarian tumour under general anaesthesia...
May 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29705167/stiff-person-syndrome-with-evidence-of-nonspecific-focal-myositis-secondary-to-sustained-muscle-contraction-a-case-report
#5
Seung-Wook No, Il-Gyu Lim, Du Hwan Kim
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. We report the case of a 36-year-old woman with SPS in association with nonspecific focal myositis secondary to sustained muscle contraction. She presented with stiffness and pain in her extremities, and diazepam and baclofen were ineffective...
April 26, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/29561731/paraneoplastic-movement-disorders
#6
Karolina Popławska-Domaszewicz, Jolanta Florczak-Wyspiańska, Wojciech Kozubski, Sławomir Michalak
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. Two types of antibodies can be distinguished: antibodies against nuclear and cytoplasmic neuronal antigens (anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-CV2/CRMP5, anti-Gephrin, and anti-GABATRAP) and antibodies recently identified against cell surface and synaptic proteins (anti-NMDAR, anti-LGI1, and anti-Caspr2)...
March 21, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29532593/case-series-intrathecal-baclofen-therapy-in-stiff-person-syndrome
#7
Justin Ralph Abbatemarco, Mary Alissa Willis, Robert G Wilson, Sean J Nagel, Andre G Machado, Francois A Bethoux
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. MATERIALS AND METHODS: Patients were identified from an institutional review board-approved clinical registry of ITB therapy...
March 13, 2018: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/29464188/glycine-receptor-modulating-antibody-predicting-treatable-stiff-person-spectrum-disorders
#8
Shannon R Hinson, A Sebastian Lopez-Chiriboga, James H Bower, Joseph Y Matsumoto, Anhar Hassan, Eati Basal, Vanda A Lennon, Sean J Pittock, Andrew McKeon
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...
March 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29407421/common-variable-immunodeficiency-associated-with-stiff-person-syndrome
#9
Jason Schend, Dave McGarry, John Johnson, Robert Hostoffer
No abstract text is available yet for this article.
April 2018: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/29406902/autoimmune-and-paraneoplastic-movement-disorders-an-update
#10
REVIEW
José Fidel Baizabal-Carvallo, Joseph Jankovic
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. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29404645/-from-stiff-man-syndrome-to-stiff-person-spectrum-disorders
#11
H-M Meinck, B Balint
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...
February 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29379821/multiplex-family-with-gad65-abs-neurologic-syndromes
#12
Aude Belbezier, Bastien Joubert, Gonzalo Montero-Martin, Marcelo Fernandez-Vina, Nicole Fabien, Véronique Rogemond, Emmanuel Mignot, Jérôme Honnorat
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. Results: The proband had cerebellar ataxia and probable limbic encephalitis features, whereas her niece had stiff-person syndrome...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29349679/autoimmune-encephalitis-associated-with-glutamic-acid-decarboxylase-antibodies-a-case-series
#13
Faruk Incecik, Ozlem M Herguner, Seyda Besen, Mustafa Yılmaz
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. Three of them had extralimbic involvement and only one had limbic encephalitis.
January 18, 2018: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29338930/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
#14
Hideto Nakajima, Yoshitsugu Nakamura, Yuiko Inaba, Chiharu Tsutsumi, Kiichi Unoda, Takafumi Hosokawa, Fumiharu Kimura, Toshiaki Hanafusa, Masamichi Date, Haruko Kitaoka
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...
April 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29335314/teaching-video-neuroimages-electromyographic-variation-in-stiff-person-syndrome
#15
Xiao-Zhong Jing, Dan Zhu, Ya-Qian Zhang, Ming Dong
No abstract text is available yet for this article.
January 16, 2018: Neurology
https://www.readbyqxmd.com/read/29282340/-from-involuntary-movements-to-movement-disorders
#16
Toshio Fukutake
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...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29209545/difficult-to-treat-focal-stiff-person-syndrome-of-the-left-upper-extremity
#17
Nathan E Esplin, John W Stelzer, Timothy B Legare, Sayed K Ali
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. In rare cases it can be paraneoplastic which generally resolves on treatment of the underlying neoplasm. Case Report: A 46-year-old male with a history of Hodgkin's Lymphoma in remission presented with left upper extremity pain secondary to a diagnosis of Stiff Person Syndrome limited to his left upper extremity...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/29201327/stiff-person-case-misdiagnosed-as-conversion-disorder-a-case-report
#18
Saeed Razmeh, Amir Hasan Habibi, Farzad Sina, Elham Alizadeh, Monireh Eslami
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. The pathophysiology of the disease is caused by the decreased level of the glutamic acid decarboxylase (GAD) activity due to an autoantibody against GAD that decreases the level of gamma-aminobutyric acid (GABA). In this paper, we present a case of atypical presentation of SPS with lower limb stiffness misdiagnosed as conversion disorder...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/29200116/paraneoplastic-disorders
#19
Eric Lancaster
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. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29194871/are-you-better-a-multi-centre-study-of-patient-defined-recovery-from-complex-regional-pain-syndrome
#20
A Llewellyn, C S McCabe, Y Hibberd, P White, L Davies, J Marinus, R G S M Perez, I Thomassen, F Brunner, C Sontheim, F Birklein, T Schlereth, A Goebel, R Haigh, R Connett, C Maihöfner, L Knudsen, R N Harden, A Zyluk, D Shulman, H Small, F Gobeil, P Moskovitz
BACKGROUND: Complex Regional Pain Syndrome (CRPS) symptoms can significantly differ between patients, fluctuate over time, disappear or persist. This leads to problems in defining recovery and in evaluating the efficacy of therapeutic interventions. OBJECTIVES: To define recovery from the patients' perspective and better understand their priorities for treatment approaches. METHODS: Establishing an international consortium, we used a 2-Round Delphi-based study in eight countries across Europe and North America...
March 2018: European Journal of Pain: EJP
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