scholarly journals Nerve Conduction Studies and Electromyography in Friedreich's Ataxia

Author(s):  
J.M. Peyronnard ◽  
L. Lapointe ◽  
J.P. Bouchard ◽  
A. Lamontagne ◽  
B. Lemieux ◽  
...  

SUMMARY:Twenty-six of 50 patients were investigated with nerve conduction studies and electromyography using a standard protocol and were compared to the findings in 50 normal control subjects. Almost all cases of typical Friedreich's ataxia had absent sensory action potentials (SAP) in the digital (92%) or sural (96%) nerves. The others had markedly decreased S.A.P's. In these same patients motor conduction auvelocities were either normal or only slightly decreased. In the second, atypical group of 9 patients, the motor conduction velocities were considerably decreased.Because of the absence of sensory action potentials in Friedreich's ataxia, and that the absence was noted in our very mild cases, it is proposed that this measure be used to facilitate early diagnosis.

Author(s):  
J.P. Bouchard ◽  
A. Barbeau ◽  
R. Bouchard ◽  
R.W. Bouchard

SummaryTwenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction.The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.


Author(s):  
J.M. Peyronnard ◽  
L. Charron ◽  
A. Barbeau

SummaryTwo female patients aged 30 and 40 years with the Charlevoix-Saguenay ataxia were studied. Both had absent sensory action potentials in upper and lower extremities but, unlike typical cases of Friedreich's ataxia, they displayed a marked slowing of motor conduction velocities. Sural nerve biopsies taken from calf and ankle revealed a severe loss of large my elina ted axons contrasting with a normal myelinated fiber density. Evidence for active axonal degeneration was scarce, with no indication of axonal regeneration.Teased myelinated fibers revealed an increased variability of internodal length but no evidence for myelin breakdown. These findings support, as a primary defect, a developmental abnormality of peripheral nerve, namely a lack of maturation of large myelinated axons and possibly a faulty myelination of nerve fibers. We think it is unlikely to represent a progressive axonal atrophie or dystrophic process, as suggested in Friedreich's ataxia.


2006 ◽  
Vol 117 ◽  
pp. 103-104
Author(s):  
S. Bhath ◽  
C. Harrison ◽  
S. Qabil ◽  
D. Rollings ◽  
P. Mocroft ◽  
...  

2013 ◽  
Vol 4 (3) ◽  
pp. 187 ◽  
Author(s):  
Dr. Neha Harjivanbhai Pandya ◽  
Dr. Kinnar Somabhai Desai ◽  
Dr. Toral Goswami ◽  
Dr. Vaishali Patel ◽  
Dr. Amita Mevada ◽  
...  

2019 ◽  
Vol 9 (6) ◽  
Author(s):  
Kawthar AL-Salmi ◽  
Farook S Wali ◽  
Ahmed SM Nadeem ◽  
Abdullah AL-Salti

Background: nerve conduction study (NCS) is a neurophysiologic medical diagnostic test used commonly to evaluate the function of the peripheral nerves. It is an extension to the clinical examination and extremely useful to diagnose and document a peripheral nerve disorder, localize the lesion, and to establish underlying pathophysiology. Objective: The aim of this study is to evaluate the influence of gender on nerve conduction study values and to generate reference nerve conduction studies (NCS) data for the commonly tested nerves among healthy adults in Oman. Subjects and methods: This study was conducted in the Neurology Department at the Royal Hospital, a tertiary care hospital in Muscat-Oman, for a period of four years (from March 2015 until May 2019) which included a total of 143 (80 females and 63 males) healthy Omani subjects. Sensory and motor nerve conduction studies were performed to the Median, Ulnar, Peroneal and Tibial nerves to establish the normative data. Statistical analysis was performed using Minitab comparing the mean values of all NCS parameters for both genders. Results: Gender comparison concluded that Omani males have longer distal motor latencies for all of the tested nerves and slower motor conduction velocities for the lower limbs’ nerves (p <0.005) than the females. While sensory latencies were significantly delayed in males as compared to Omani females for the upper limbs’ nerves and the ensory nerve potential amplitudes in the upper limbs were greater in females (p<0.0001) than males. A normative data for distal latencies, conduction velocities and responses amplitudes for motor and sensory nerves were sat up for the first time for normal adult Omani population. Conclusion: Gender has a significant influence on distal latencies and conduction velocities of some peripheral nerves in healthy Omani adult subjects, suggesting that different cut-off values for the two genders may be needed for interpreting such studies. These differences could be related to anatomical (height and limbs’ length gender differences) and/or physiological factors. Our normative data for nerve conduction study values are comparable to other published studies in the literature.


2016 ◽  
pp. 292-311
Author(s):  
Eric J. Sorenson

Sensory nerve action potentials (SNAPs) are an important component of an electrodiagnostic test of peripheral nerves and can localize lesions either proximal or distal to the dorsal root ganglia. They are among the most sensitive measures of peripheral nerve dysfunction, and can provide information about the pathophysiology of the neuropathy, suggesting demyelination or axonal loss in some cases. Because SNAPs are much smaller than compound muscle action potentials, they are technically more difficult to study. Temperature will have a greater impact on SNAPs than motor potentials, with cooler limb temperatures leading to longer latencies, slower conduction velocities, and larger amplitudes. Responses are commonly averaged 3 to 5 times to minimize the effect of the background noise on the waveforms. Distance from the electrical generator and inter-electrode distance will also impact the appearance and size of the nerve potential waveform. This chapter reviews the concepts and applications of sensory nerve action potentials and sensory nerve conduction studies.


Sangyo Igaku ◽  
1990 ◽  
Vol 32 (2) ◽  
pp. 136-137
Author(s):  
Hiroshi MITSUTAKE ◽  
Keiko TERAMOTO ◽  
Shun'ichi HORIGUCHI ◽  
Tadashi YAMAMOTO

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