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https://www.readbyqxmd.com/read/29150079/mechanism-hypotheses-for-the-electrophysiological-manifestations-of-two-cases-of-endplate-acetylcholinesterase-deficiency-related-congenital-myasthenic-syndrome
#1
Qingyun Ding, Dongchao Shen, Yi Dai, Youfang Hu, Yuzhou Guan, Mingsheng Liu, Liying Cui
OBJECTIVE: To summarize the electrophysiological characteristics of two cases of endplate acetylcholinesterase deficiency (EAD) related congenital myasthenic syndrome (CMS) caused by COLQ mutation and to discuss the possible mechanism of these electrophysiological phenomena. METHODS: Electrophysiological examinations were conducted including nerve conduction studies, routine electromyography (EMG), repetitive nerve stimulation (RNS) and single fiber EMG (SFEMG)...
November 14, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29128986/longitudinal-neurophysiological-assessment-of-intramuscular-type-a-botulin-toxin-in-healthy-humans
#2
L Lispi, L Leonardi, A Petrucci
The aim of this study is to assess the neurophysiological abnormalities of type A botulin toxin-infiltrated human muscle, and their evolution over time. Seried cMAP measurements, 3 and 20 Hz repetitive nerve stimulation, EMG, SFEMG over 3 months from toxin injection. Our findings consist in lack of decrement with 3 Hz repetitive nerve stimulation and facilitation with 20 Hz repetitive nerve stimulation; progressive increasing of jitter; early appearance of fibrillations; small and short motor unit action potential in the first 3 weeks, followed by increasing of MUAP amplitude and duration, with polyphasic morphology...
November 11, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29121441/influence-on-jitter-measurement-of-components-from-distant-fibers-in-potential-recorded-by-single-fiber-electrode
#3
EDITORIAL
Ewa Zalewska, Malgorzata Gawel
Single-fiber electromyography (SFEMG) is one of the most important diagnostic tools for evaluating neuromuscular transmission and jitter as well as fiber density.(1) When a motor axon is depolarized, the action potential travels distally and activates all the muscle fibers belonging to the same motor unit at more or less the same time. The variations in the time interval between firing of adjacent single muscle fibers from the same motor unit are called neuromuscular jitter and reflect abnormalities in the transmission time in axonal derivations and synapses...
November 9, 2017: Acta Physiologica
https://www.readbyqxmd.com/read/28940165/an-investigation-on-the-effects-of-carbamazepine-and-sodium-valproate-on-neuromuscular-transmission
#4
Halil Ay, Özlem Ethemoğlu
The aim of this study was to investigate the effects of sodium valproate (SV) and carbamazepine (CBZ) on neuromuscular transmission using single-fibre electromyography (SFEMG) in patients with epilepsy. We performed SFEMG during the voluntary contraction of extensor digitorum communis muscle. 30 epileptic patients taking SV, 25 epileptic patients taking CBZ, and 25 age-matched healthy volunteers were included in the study. Mean jitter values (MCD) of subjects taking SV and CBZ were compared with normal controls...
September 22, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28650411/recording-fewer-than-20-potential-pairs-with-sfemg-may-suffice-for-the-diagnosis-of-myasthenia-gravis
#5
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Vera Bril
PURPOSE: Our aim in the current study was to determine the minimum number of SFEMG potential pairs required to confirm neuromuscular junction impairment and relate this number to disease severity. METHODS: Ninety-four patients with myasthenia gravis (MG) attending the neuromuscular clinic from February 2013 to November 2015 were included. The SFEMG sensitivity was determined for each number of recorded pairs up to 20. In addition, we compared clinical and electrophysiologic characteristics between patients with abnormality within the first 3, 4 to 7, and ≥8 recorded pairs to determine whether this number is associated with disease severity...
June 20, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28592233/single-fiber-electromyography-in-the-orbicularis-oculi-muscle-in-patients-with-ocular-myasthenia-gravis-symptoms-does-abnormal-jitter-predict-response-to-treatment
#6
Goran Rakocevic, Mark Moster, Mary Kay Floeter
BACKGROUND: Seronegative ocular myasthenia gravis (OMG) is diagnosed by ocular symptoms with supporting SFEMG, typically of frontalis or extensor digitorum muscles. We aimed to determine the sensitivity and specificity of orbicularis oculi SFEMG to diagnose and exclude myasthenia gravis and predict response to therapy. METHODS: Orbicularis oculi SFEMG studies were conducted in 142 consecutive patients with symptoms and/or findings of OMG and negative AChR antibody during the period of 5 years...
June 7, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28431604/a-reappraisal-of-diagnostic-tests-for-myasthenia-gravis-in-a-large-asian-cohort
#7
Yew Long Lo, Raymond P Najjar, Kelvin Y Teo, Sharon L Tow, Jing Liang Loo, Dan Milea
BACKGROUND: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by weakness of bodily skeletal muscles. Office-based diagnostic tests such as repetitive nerve stimulation (RNS), single fiber electromyography (SFEMG), and the ice test, are used to refine the differential clinical diagnosis of this disease. Evaluating the clinical sensitivity and specificity of these tests, however, may be confounded by lack of a gold standard, non-blinding, incorporation bias, use of non-representative populations and retrospective data...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28268468/detection-of-myasthenia-gravis-using-electrooculography-signals
#8
T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28226641/detection-of-myasthenia-gravis-using-electrooculography-signals
#9
T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy, T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy, M I Boulos, K Umapathy, H Katzberg, S Krishnan, T Liang, B J Murray
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/27935068/single-fiber-emg-with-concentric-electrodes-in-lambert-eaton-myasthenia
#10
Yuzhou Guan, Qingyun Ding, Mingsheng Liu, Jingwen Niu, Liying Cui
INTRODUCTION: We analyzed jitter recordings made with concentric needle electrode (CNE) single-fiber electromyography (SFEMG) in Lambert-Eaton myasthenia (LEM). METHODS: Fifteen subjects diagnosed with LEM were studied using CNE-SFEMG in the extensor digitorum (ED) and tibialis anterior (TA) muscles. CNE-SFEMG in the ED and TA was also used to evaluate 12 and 10 healthy controls (HCs), respectively. RESULTS: Ten men and 5 women were diagnosed with LEM based on an increase of 100% in compound muscle action potential amplitude during 50 Hz repetitive nerve stimulation...
August 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/27759891/does-change-in-neuromuscular-jitter-predict-or-correlate-with-clinical-change-in-mg
#11
Donald B Sanders, Janice M Massey
INTRODUCTION: The objective of this study was to determine if single-fiber electromyography (SFEMG) jitter accurately reflects change in severity in myasthenia gravis (MG). METHODS: We reviewed jitter and outcome data from all MG patients in our clinic who had at least 2 jitter measurements in the extensor digitorum or frontalis muscle. RESULTS: Change in all parameters of jitter measured with SFEMG electrodes predicted clinical change with acceptable accuracy...
July 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/27749461/diagnostic-accuracy-of-single-fiber-electromyography-for-myasthenia-gravis-in-patients-followed-longitudinally
#12
John A Morren, Kerry H Levin, Robert W Shields
INTRODUCTION: The literature lacks data on accuracy of single fiber electromyography (SFEMG) for myasthenia gravis (MG) patients followed longitudinally. METHODS: We included patients with a clinical suspicion of MG who received SFEMG and follow-up at our institution between 2003 and 2013. Data collected included demographics, symptom details, clinical deficits, other diagnostic testing results, MG medication regimen, duration on treatment, response to therapy, and ultimate diagnosis after follow-up...
October 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27648598/diagnostic-accuracy-of-single-fiber-emg-for-myasthenia-gravis-in-patients-followed-longitudinally
#13
John A Morren, Kerry H Levin, Robert W Shields
INTRODUCTION: The literature lacks data on accuracy of single fiber electromyography (SFEMG) for myasthenia gravis (MG) patients followed longitudinally. METHODS: We included patients with a clinical suspicion of MG who received SFEMG and follow-up at our institution between 2003-2013. Data collected included demographics, symptom details, clinical deficits, other diagnostic testing results, MG medication regimen, duration on treatment, response to therapy and ultimate diagnosis after follow-up...
March 31, 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27611986/neuromuscular-transmission-abnormalities-in-myotonic-dystrophy-type-1-a-neurophysiological-study
#14
Francesco Bombelli, Ludovico Lispi, Sandro Costanzi Porrini, Manlio Giacanelli, Chiara Terracciano, Roberto Massa, Antonio Petrucci
OBJECTIVES: Weakness and fatigue are frequent symptoms in myotonic dystrophy type 1 (DM1), mainly as a result of muscle impairment. However, neuromuscular junction (NMJ) abnormalities could play an additional role in determining these manifestations. We aimed to document the possible NMJ involvement in DM1. PATIENTS AND METHODS: In order to substantiate this hypothesis we performed low rate repetitive nerve stimulation (RNS) and single fiber electromyography (SFEMG), in 14 DM1 subjects...
November 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/27144873/single-fiber-emg-as-a-prognostic-tool-in-myasthenia-gravis
#15
Mateja Baruca, Lea Leonardis, Simon Podnar, Tanja Hojs-Fabjan, Anton Grad, Aleš Jerin, Rok Blagus, Saša Šega-Jazbec
INTRODUCTION: Single fiber electromyography (SFEMG) is the most sensitive diagnostic tool for diagnosis of myasthenia gravis (MG). Its prognostic value is not known. METHODS: We retrospectively analyzed the clinical course of 232 MG patients who presented with only mild symptoms and had SFEMG of the orbicularis oculi muscle. We correlated their SFEMG results with the severity of their later clinical course. RESULTS: During the observation period 39 patients (17%) developed severe disease exacerbations, and 193 (83%) remained stable...
December 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27098787/quantitating-changes-in-jitter-and-spike-number-using-concentric-needle-electrodes-in-amyotrophic-lateral-sclerosis-patients
#16
Ming-Sheng Liu, Jing-Wen Niu, Yi Li, Yu-Zhou Guan, Li-Ying Cui
BACKGROUND: Single-fiber electromyography (SFEMG) has been suggested as a quantitative method for supporting chronic partial denervation in amyotrophic lateral sclerosis (ALS) by the revised EI Escorial criteria. Although concentric needle (CN) electrodes have been used to assess jitter in myasthenia gravis patients and healthy controls, there are few reports using CN electrodes to assess motor unit instability and denervation in neurogenic diseases. The aim of this study was to determine whether quantitative changes in jitter and spike number using CN electrodes could be used for ALS studies...
May 5, 2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/26923566/neurotoxicity-in-russell-s-viper-daboia-russelii-envenoming-in-sri-lanka-a-clinical-and-neurophysiological-study
#17
Anjana Silva, Kalana Maduwage, Michael Sedgwick, Senaka Pilapitiya, Prasanna Weerawansa, Niroshana J Dahanayaka, Nicholas A Buckley, Sisira Siribaddana, Geoffrey K Isbister
CONTEXT: Russell's viper is more medically important than any other Asian snake, due to number of envenoming's and fatalities. Russell's viper populations in South India and Sri Lanka (Daboia russelii) cause unique neuromuscular paralysis not seen in other Russell's vipers. OBJECTIVE: To investigate the time course and severity of neuromuscular dysfunction in definite Russell's viper bites, including antivenom response. METHODOLOGY: We prospectively enrolled all patients (>16 years) presenting with Russell's viper bites over 14 months...
June 2016: Clinical Toxicology
https://www.readbyqxmd.com/read/26829229/neuromuscular-effects-of-common-krait-bungarus-caeruleus-envenoming-in-sri-lanka
#18
Anjana Silva, Kalana Maduwage, Michael Sedgwick, Senaka Pilapitiya, Prasanna Weerawansa, Niroshana J Dahanayaka, Nicholas A Buckley, Christopher Johnston, Sisira Siribaddana, Geoffrey K Isbister
OBJECTIVE: We aimed to investigate neurophysiological and clinical effects of common krait envenoming, including the time course and treatment response. METHODOLOGY: Patients with definite common krait (Bungarus caeruleus) bites were recruited from a Sri Lankan hospital. All patients had serial neurological examinations and stimulated concentric needle single-fibre electromyography (sfEMG) of orbicularis oculi in hospital at 6 wk and 6-9 mth post-bite. PRINCIPAL FINDINGS: There were 33 patients enrolled (median age 35 y; 24 males)...
February 2016: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/26770586/determination-of-the-normative-values-of-the-masseter-muscle-by-single-fiber-electromyography-in-myasthenia-gravis-patients
#19
Lei Shi, Heng-Fang Liu, Min Zhang, Ya-Pei Guo, Bo Song, Chang-Dong Song, Dan-Dan Song, Yu-Ming Xu
The purpose of this study is to obtain normative values of the masseter muscle of myasthenia gravis (MG) patients and healthy volunteers by single-fiber electromyography (SFEMG). Stimulation of SFEMG in the masseter muscle was studied in 15 healthy volunteers (men 8, women 7; mean age 40.2, range 21-77) and 30 patients affected by MG (men 16, women 14; mean age 42.8, range 12-75). The mean consecutive difference (MCD) of the individual fiber and the mean MCD per study were determined in the normal group. We recommend the upper normal limit for the individual fibers of jitter and the mean MCD per study in the healthy Chinese adults of 33 µs and 22 µs respectively...
2015: International Journal of Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/26481746/single-fiber-electromyography-in-the-extensor-digitorum-communis-for-the-predictive-prognosis-of-ocular-myasthenia-gravis-a-retrospective-study-of-102-cases
#20
Yu-Zhou Guan, Li-Ying Cui, Ming-Sheng Liu, Jing-Wen Niu
BACKGROUND: Single-fiber electromyography (SFEMG) abnormality in the extensor digitorum communis (EDC) was reported in ocular myasthenia gravis (OMG), which indicated subclinical involvement beyond extraocular muscles in OMG patients. The relationship between the abnormal findings of SFEMG in EDC and the probability for OMG to develop generalized myasthenia gravis (GMG) is unknown. This retrospective study aimed to determine the predictive value of abnormality of SFEMG in EDC of OMG patients...
October 20, 2015: Chinese Medical Journal
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