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stiff person syndrome

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https://www.readbyqxmd.com/read/27905901/presynaptic-neuromuscular-transmission-defect-in-the-stiff-person-syndrome
#1
Y L Lo, Y E Tan
BACKGROUND: The stiff person syndrome (SPS) is a rare disorder characterized by muscular rigidity and stiffness. CASE PRESENTATIONS: We describe an SPS patient presenting with longstanding fatigue and electrophysiological evidence of presynaptic neuromuscular transmission defect, who responded to administration of pyridostigmine. In contrast, no electrophysiolgical evidence of neuromuscular transmission defect was demonstrated in 2 other SPS patients without fatigue symptoms...
December 1, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27861223/gastrointestinal-and-urologic-sphincter-dysfunction-in-stiff-person-syndrome
#2
Oana M Dumitrascu, Evgeny I Tsimerinov, Richard A Lewis
OBJECTIVES: Stiff person syndrome is a neurologic disorder characterized by axial rigidity leading to progressive disability, with broad clinical spectrum. METHODS: We report 2 cases with unique clinical presentation. RESULTS: Two young men suffered progressive urinary retention requiring bladder catheterization, anorectal spasms and constipation, complicated subsequently with lower extremity trigger-induced spasms, and gait instability. Associated symptoms revealed brainstem involvement (vertigo, diplopia, and cranial neuropathies) and dysautonomia (abnormal sweating and orthostatic hypotension)...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27761484/erratum-serotonin-norepinephrine-reuptake-inhibitors-may-exacerbate-stiff-person-syndrome
#3
(no author information available yet)
[This corrects the article on p. e281 in vol. 3, PMID: 27606356.].
December 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27759100/the-astrocytic-transporter-slc7a10-asc-1-mediates-glycinergic-inhibition-of-spinal-cord-motor-neurons
#4
Jeffrey T Ehmsen, Yong Liu, Yue Wang, Nikhil Paladugu, Anna E Johnson, Jeffrey D Rothstein, Sascha du Lac, Mark P Mattson, Ahmet Höke
SLC7A10 (Asc-1) is a sodium-independent amino acid transporter known to facilitate transport of a number of amino acids including glycine, L-serine, L-alanine, and L-cysteine, as well as their D-enantiomers. It has been described as a neuronal transporter with a primary role related to modulation of excitatory glutamatergic neurotransmission. We find that SLC7A10 is substantially enriched in a subset of astrocytes of the caudal brain and spinal cord in a distribution corresponding with high densities of glycinergic inhibitory synapses...
October 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27756410/stiff-person-syndrome-in-south-asia
#5
Thashi Chang, Bethan Lang, Angela Vincent
BACKGROUND: Stiff person syndrome is a highly disabling, progressive autoimmune disorder of the central nervous system characterized by muscle rigidity and spasms. Stiff person syndrome is rare, but is believed to be under diagnosed with only 14 cases been reported among a 1.7 billion population in South Asia. We report the first authenticated case from Sri Lanka. CASE PRESENTATION: A 55-year-old Sri Lankan female presented with difficulty in walking and recurrent falls due to progressive muscular rigidity in her lower limbs and trunk with superimposed muscle spasms that occurred in response to unexpected noise, startle or emotional upset...
October 18, 2016: BMC Research Notes
https://www.readbyqxmd.com/read/27734022/amphiphysin-positive-paraneoplastic-myelitis-and-stiff-person-syndrome
#6
Simon Faissner, Carsten Lukas, Anke Reinacher-Schick, Andrea Tannapfel, Ralf Gold, Ingo Kleiter
No abstract text is available yet for this article.
December 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27719837/assessment-of-patients-with-functional-neurologic-disorders
#7
A Carson, M Hallett, J Stone
We describe an overall approach and structure to the clinical assessment of the patient with a functional neurologic disorder. Whilst the primary purpose of the assessment is to make a diagnosis and develop a treatment plan, we believe the assessment also plays a key role in treatment in its own right, as it sets a tone and context for future clinical interactions. We aim to set up an atmosphere of collaboration based on taking the patient's problems seriously, and emphasizing that all facets of the patient's presentation - physical, psychologic, and social - are of importance...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27692358/stiff-person-syndrome-masquerading-as-acute-coronary-syndrome
#8
Ashish Sharma, Myat Han Soe, Jagdeep Singh, Scott D Newsome
Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by severe progressive muscle stiffness in axial and lower extremity musculature with superimposed painful muscle spasms. Although chest pain is a common reason for SPS patients presenting to the emergency room, this disorder is overlooked and not part of the differential diagnosis of chest pain. Herein, we report on a middle age male presenting with classic symptoms of SPS; however, due to the rarity of this disease, he was initially thought to have acute coronary syndrome...
2016: Journal of the National Medical Association
https://www.readbyqxmd.com/read/27677907/mechanics-in-human-fibroblasts-and-progeria-lamin-a-mutation-e145k-results-in-stiffening-of-nuclei
#9
Ketaki Apte, Reimer Stick, Manfred Radmacher
The lamina is a filamentous meshwork beneath the inner nuclear membrane that confers mechanical stability to nuclei. The E145K mutation in lamin A causes Hutchinson-Gilford progeria syndrome (HGPS). It affects lamin filament assembly and induces profound changes in the nuclear architecture. Expression of wild-type and E145K lamin A in Xenopus oocytes followed by atomic force microscopy (AFM) probing of isolated oocyte nuclei has shown significant changes in the mechanical properties of the lamina. Nuclei of oocytes expressing E145K lamin A are stiffer than those expressing wild-type lamin A...
September 28, 2016: Journal of Molecular Recognition: JMR
https://www.readbyqxmd.com/read/27673113/poster-360-acute-rehabilitation-of-a-patient-with-stiff-person-syndrome-sps-and-gad-antibody-cerebellar-ataxia-a-case-report
#10
Nicole A Strong, Nicholas F Love, Kristen M Brusky, Sara Salim
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27673015/poster-258-a-novel-approach-to-the-treatment-of-stiff-person-syndrome-with-botulinum-toxin-a-case-report
#11
Chirag D Shah, Daniel Bunzol
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27672995/poster-237-bilateral-hip-fracture-during-hospitalization-for-spasm-exacerbation-in-an-adult-with-stiff-person-syndrome-a-case-report
#12
Tomasz K Podobinski, Paolo C Mimbella, Michael J Irvine, Monica Verduzco-Gutierrez
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27606356/serotonin-norepinephrine-reuptake-inhibitors-may-exacerbate-stiff-person-syndrome
#13
David R Benavides, Scott D Newsome
No abstract text is available yet for this article.
October 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27591471/anesthesia-in-a-patient-with-stiff-person-syndrome
#14
Ozgur Yagan, Kadir Özyilmaz, Ahmet Özmaden, Özgür Sayin, Volkan Hanci
Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage...
September 2016: Brazilian Journal of Anesthesiology
https://www.readbyqxmd.com/read/27501653/anesthetic-considerations-of-stiff-person-syndrome-a-case-report
#15
Kristi Hylan, An-Duyen Nguyen Vu, Katherine Stammen
Stiff-person syndrome (SPS) is a neurologic disorder characterized by painful involuntary episodes of severe muscle rigidity affecting the axial muscles and extremities. Although the etiology of SPS is unknown, it is suspected to involve the synthesis of γ-aminobutyric acid (GABA). Symptoms of SPS are precipitated by sudden unexpected movements, noises, and stress. Additionally, SPS has been linked with various autoimmune disorders, including diabetes mellitus, thyroid disease, pernicious anemia, and certain cancers...
June 2016: AANA Journal
https://www.readbyqxmd.com/read/27430873/acute-respiratory-failure-in-a-patient-with-stiff-person-syndrome
#16
Vincent Jachiet, Laurent Laine, Thierry Gendre, Carole Henry, Daniel Da Silva, Etienne de Montmollin
BACKGROUND: Stiff-person syndrome (SPS) is a rare disorder characterized by progressive muscle stiffness, rigidity, and spasms involving the axial muscles. Acute respiratory distress has rarely been reported in this condition. METHODS: We report a case of a 49-year-old woman with autoimmune SPS diagnosed during an episode of acute respiratory failure secondary to repetitive episodes of apnea, requiring intensive care. RESULTS: Acute respiratory failure manifesting with apneic episodes is a life-threatening and unpredictable complication of SPS...
July 18, 2016: Neurocritical Care
https://www.readbyqxmd.com/read/27378710/anesthetic-management-of-a-parturient-with-stiff-person-syndrome-for-urgent-cesarean-delivery
#17
B T Boettcher, M Muravyea, C Kuo, C Drexler, P S Pagel
Stiff person syndrome is a rare neurologic disorder with an estimated incidence of 1:1000000. The underlying pathophysiology is truncal and proximal limb muscle stiffness resulting from continuous co-contracture of agonist and antagonist muscle groups concomitant with superimposed episodic muscle spasms. Loss of gamma-aminobutyric acid-mediated inhibition creates chronic excitation manifested by tonic agonist-antagonist muscle contraction. To date, only three case reports referred indirectly to the anesthetic management of parturients with Stiff person syndrome...
August 2016: International Journal of Obstetric Anesthesia
https://www.readbyqxmd.com/read/27356651/anti-glutamic-acid-decarboxylase-antibody-positive-neurological-syndromes
#18
Hassaan Tohid
A rare kind of antibody, known as anti-glutamic acid decarboxylase (GAD) autoantibody, is found in some patients. The antibody works against the GAD enzyme, which is essential in the formation of gamma aminobutyric acid (GABA), an inhibitory neurotransmitter found in the brain. Patients found with this antibody present with motor and cognitive problems due to low levels or lack of GABA, because in the absence or low levels of GABA patients exhibit motor and cognitive symptoms. The anti-GAD antibody is found in some neurological syndromes, including stiff-person syndrome, paraneoplastic stiff-person syndrome, Miller Fisher syndrome (MFS), limbic encephalopathy, cerebellar ataxia, eye movement disorders, and epilepsy...
July 2016: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/27262149/stiff-person-syndrome-and-other-immune-mediated-movement-disorders-new-insights
#19
Bettina Balint, Kailash P Bhatia
PURPOSE OF REVIEW: This review highlights the recent developments in immune-mediated movement disorders and how they reflect on clinical practice and our understanding of the underlying pathophysiological mechanisms. RECENT FINDINGS: The antibody spectrum associated with stiff person syndrome and related disorders (SPSD) has broadened and, apart from the classic glutamic acid decarboxylase (GAD)- and amphiphysin-antibodies, includes now also antibodies against dipeptidyl-peptidase-like protein-6 (DPPX), gamma-aminobutyric acid type A receptor (GABAAR), glycine receptor (GlyR) and glycine transporter 2 (GlyT2)...
August 2016: Current Opinion in Neurology
https://www.readbyqxmd.com/read/27242441/anti-gad65-containing-cerebrospinal-fluid-does-not-alter-gabaergic-transmission
#20
Jana K Hackert, Lorenz Müller, Marco Rohde, Christian G Bien, Rüdiger Köhling, Timo Kirschstein
Glutamic acid decarboxylase of 65 kDa (GAD65) antibodies have been reported in a variety of neurological disorders such as stiff-person syndrome (SPS), sporadic ataxia and some cases of epilepsy. Since the target is believed to be the cytoplasmic enzyme GAD65, the key enzyme of γ-aminobutyric acid (GABA) synthesis, the pathophysiological role of these antibodies is poorly understood. Here, we stereotactically injected human cerebrospinal fluid (CSF) containing GAD65-antibodies into the hippocampus of rats in vivo and then prepared hippocampal slices 1-2 days after post-operative recovery...
2016: Frontiers in Cellular Neuroscience
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