Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis

2018 ◽  
Vol 129 (5) ◽  
pp. 974-980 ◽  
Author(s):  
Yu-ichi Noto ◽  
Neil G. Simon ◽  
Alexis Selby ◽  
Nidhi Garg ◽  
Kazumoto Shibuya ◽  
...  
2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Hua Wang ◽  
Bin Liu ◽  
Jiyou Tang

This study is aimed at investigating the features of fasciculation potentials (FPs) in amyotrophic lateral sclerosis (ALS) and peripheral nerve hyperexcitability syndromes (PNH). Needle electrophysiologic examination (EMG) was performed for 5-15 muscles in the ALS and PNH patients. The spontaneous activity of fasciculations and fibrillations/sharp-waves (fibs-sw) was recorded. The distribution, firing frequency, and waveform parameters of FPs in muscles were calculated and compared. In total, 361 muscles in ALS patients and 124 muscles in PNH patients were examined, with the FP detection rates of 45.1% and 53.2%. Moreover, the ALS patients with the upper limb onset had the highest FP detection rate. Fasciculations occurred more frequently in the upper limbs than in the lower limbs in ALS and PNH. The detection rate of fibs-sw in the bulbar muscle was relatively low, which could be elevated when combining fibs-sw and FPs. Benign FPs in PNH were of smaller amplitude, shorter duration, and fewer phases/turns, compared with malignant FPs in ALS. The FP area in PNH was significantly smaller than that in ALS. The incidence of polyphasic FPs in ALS was distinctly greater than that in PNH. The firing frequency of FPs in PNH was higher than that in ALS. There was no significant difference in the amplitude, duration, phases and turns, and area of FPs between groups with and without fibs-sw in the muscles of normal strength in ALS. Conclusively, it is necessary to detect the FPs in the thoracic and bulbar muscles of patients suspected having ALS. FP parameters in ALS are significantly different from PNH.


2011 ◽  
Vol 44 (3) ◽  
pp. 346-351 ◽  
Author(s):  
Michael S. Cartwright ◽  
Francis O. Walker ◽  
Leah P. Griffin ◽  
James B. Caress

2021 ◽  
Vol 132 (8) ◽  
pp. e118
Author(s):  
Toh Tsun Haw ◽  
Nur Adilah Abdul Aziz ◽  
Mohd Azly Yahaya ◽  
Ee Chin Loh ◽  
David Capelle ◽  
...  

2017 ◽  
Vol 57 (2) ◽  
pp. 273-278 ◽  
Author(s):  
Stefanie Schreiber ◽  
Grazyna Debska-Vielhaber ◽  
Susanne Abdulla ◽  
Judith Machts ◽  
Frank Schreiber ◽  
...  

2016 ◽  
Vol 54 (6) ◽  
pp. 1133-1135 ◽  
Author(s):  
Bas A. Jongbloed ◽  
Wieke Haakma ◽  
H. Stephan Goedee ◽  
Jeroen W. Bos ◽  
Clemens Bos ◽  
...  

2015 ◽  
Vol 51 (5) ◽  
pp. 669-675 ◽  
Author(s):  
Stefanie Schreiber ◽  
Susanne Abdulla ◽  
Grazyna Debska-Vielhaber ◽  
Judith Machts ◽  
Verena Dannhardt-Stieger ◽  
...  

1994 ◽  
Vol 52 (4) ◽  
pp. 493-500 ◽  
Author(s):  
A.S.B. Oliveira ◽  
E. Isozaki ◽  
D. Younger ◽  
A.A. Gabbai ◽  
A.P. Hays

To investigate the possibility of local antigen presentation within the peripheral nerve in amyotrophic lateral sclerosis (ALS), cryostat sections of 83 peripheral nerve biopsies were stained for the demonstration of HLA-DR using a monoclonal antibody. Forty samples showed increased expression of HLA-DR in endoneurium. The phenotypic characteristics of the HLA-DR positive cells are chiefly Schwann cells, using S-100 protein as a marker. We did not detect any co-expression between HLA-DR and NF (axons) and HLA-DR and myelin marker. We also detected co-expression between HLA-DR and NGFr in a majority of HLA-DR positive cells. Inflammatory cells were infrequent, being detected only in 11 cases, predominantly around epineurial blood vessels. Motor and sensory nerve biopsies performed simultaneously showed higher expression of HLA-DR in motor nerves in 2 out of 4 patients. The significance of these findings is not clear. The presence of endoneurial cells expressing HLA-DR suggests that an autoimmune mechanism may be involved in ALS having Schwann as the main target.


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