scholarly journals A selective remarkably low M-wave amplitude in tibial nerve is characteristic in motor nerve conduction in spinal muscular atrophy type 2 and 3

2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Jun Jiang ◽  
Sun Ruidi
Keyword(s):  
Spinal Muscular Atrophy ◽  
Nerve Conduction ◽  
Wave Amplitude ◽  
Tibial Nerve ◽  
Muscular Atrophy ◽  
Motor Nerve ◽  
Motor Nerve Conduction ◽  
M Wave ◽  
Spinal Muscular Atrophy Type

Motor nerve conduction studies on children with spinal muscular atrophy

1996 ◽  
Vol 38 (6) ◽  
pp. 576-579 ◽  
Author(s):  
YURI MIYANOMAE ◽  
YOSHIHIRO TAKEUCHI ◽  
AKIRA NISHIMURA ◽  
SYOUJI KAWASE ◽  
KIYOSHI HIRAI ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Nerve Conduction ◽  
Muscular Atrophy ◽  
Motor Nerve ◽  
Nerve Conduction Studies ◽  
Motor Nerve Conduction

Very Early Neurophysiological Study in Guillain-Barre Syndrome

2018 ◽  
Vol 80 (1-2) ◽  
pp. 100-105 ◽  
Author(s):  
Jiaoting Jin ◽  
Fangfang Hu ◽  
Xing Qin ◽  
Xuan Liu ◽  
Min Li ◽  
...  
Keyword(s):  
Nerve Conduction ◽  
Nerve Conduction Velocity ◽  
Tibial Nerve ◽  
Early Stage ◽  
Motor Nerve ◽  
Sensory Nerve ◽  
Sensory Nerves ◽  
Motor Nerve Conduction ◽  
Abnormal Rate ◽  
Motor Nerves

Purpose: The diagnosis of Guillain-Barre syndrome (GBS) in the very early stage may be challenging. Our aim was to report the neurophysiological abnormalities in GBS within 4 days of clinical onset. We expected that GBS will be diagnosed by the assistance of neurophysiological study in the very early stage. Methods: We prospectively recruited patients with a diagnosis of GBS discharged from First Affiliated Hospital of Xi’an Jiaotong University and Xi Jing Hospital. Patients were classified into 3 groups according to the onset of symptoms to electromyography examination interval (OEI). The neurophysiological findings were carried out using standard procedures. All patients were examined by the same experienced neurophysiologist. Results: There were not significant group differences in abnormal rate, distal motor latency (DML), motor nerve conduction velocity (MNCV), F response (FR), compound muscle action potential (CMAP), conduction block (CB), sensory nerve action potential (SNAP), and sensory nerve conduction velocity among OEI ≤4 days, 4< OEI ≤10 days, and OEI > 10 days groups. Motor nerves were more affected than sensory nerves in neurophysiological presentation in very early stage patients. The difference of motor nerves and sensory nerves was statistically significant in lower limbs, but was not in upper limbs. In motor nerve conduction studies, the abnormal rate of DML, MNCV, FR, CB was more common seen in ulnar and peroneal nerve than median and tibial nerve, the abnormal rate of CMAP was the same in ulnar, median, peroneal and tibial nerve. In sensory nerve conduction studies, the abnormal rate of ulnar nerve and median nerve was higher than the superficial peroneal nerve and sural nerve. The OEI was not correlated with the SNAP decrease rate of median (r = 0.10, p = 0.23) and ulnar (r = 0.26, p = 0.06) but was statistically correlated with sural SNAP decrease rate (r = 0.29, p = 0.04). The sural-sparing pattern phenomenon was the most commonly discovered phenomenon in very early stage patients (OEI ≤4 days), followed by patients with 4< OEI ≤10 days, ultimately found in patients with OEI > 10 days. Conclusions: We suggest performing neurophysiological examination as soon as possible for suspected GBS patients, particularly focusing on multi-spots inspection of ulnar and peroneal nerves, and paying close attention to sural-sparing patterns.

scholarly journals Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series

Children ◽  
2021 ◽  
Vol 8 (11) ◽  
pp. 1039
Author(s):  
Minsu Gu ◽  
Hyun-Ho Kong
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Daily Living ◽  
Muscular Atrophy ◽  
Case Series ◽  
Manual Dexterity ◽  
Gross Motor ◽  
Spinal Muscular Atrophy Type ◽  
Gross Motor Function

Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA type 2. A total of five patients with SMA type 2 were included. The Hammersmith Functional Motor Scale (expanded version) (HFMSE) and Purdue Pegboard (PP) tests were used to evaluate gross motor function and fine manual dexterity, respectively, until 18 months after nusinersen administration. HFMSE scores improved by 3–10 points (+13–53%) in all patients following nusinersen administration. PP scores also improved in all patients, from 4 to 9 points (+80–225%) in the preferred hand and from 3 to 7 points (+60–500%) in the non-preferred hand. These results suggest that nusinersen treatment improved both gross motor function and fine manual dexterity in children with SMA type 2. Addition of the PP test may aid in evaluating the fine manual dexterity essential for activities of daily living in these patients.

Nusinersen improved respiratory function in spinal muscular atrophy type 2

2021 ◽  
Author(s):  
Ryuta Tanaka ◽  
Fujiko Fukushima ◽  
Keiichi Motoyama ◽  
Chie Kobayashi ◽  
Isho Izumi
Keyword(s):  
Spinal Muscular Atrophy ◽  
Respiratory Function ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type

P.226Longitudinal study of the natural history of spinal muscular atrophy type 2 and 3

2019 ◽  
Vol 29 ◽  
pp. S132
Author(s):  
J. Exposito ◽  
D. Natera-de Benito ◽  
L. Carrera ◽  
A. Frongia ◽  
M. Alarcón ◽  
...  
Keyword(s):  
Natural History ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
History Of
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