scholarly journals Clinical and Electrophysiological Aspects of Charcot – Marie Tooth Disease- A Case Report of Two Patients

Author(s):  
Geetanjali Sharma

Aims/ Objectives: To study the importance of electrophysiological tests in diagnosing hereditary motor sensory neuropathy in absence of genetic studies. Study Design: Cross-sectional study. Place and Duration of Study: Department of Physiology, Pt. B.D. Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India. Methodology: The patients were referred from the Department of Medicine to the Department of Physiology for nerve conduction, F-wave, EMG, VEP & BERA studies. Results: On electrophysiological examination, there was symmetrical decreased motor conduction velocity of median nerve (less than 38 m/sec), ulnar, tibial and peroneal nerves except in the first patient where the left peroneal nerve conduction velocity was not recordable with decreased amplitude and increased distal motor latencies. Sensory conduction velocities for bilateral median nerves were also decreased with increased latency and decreased amplitude in both the patients. Sensory conduction velocity and amplitudes of bilateral sural nerves were decreased in the first patient with increased latencies. However, sensory conduction velocity wasn’t recordable for bilateral sural nerves in the other patient. EMG shows decrease in recruitment of motor unit potentials, amplitude in bilateral tibial, peroneous, abductor digiti minimi & 1st dorsal interosseus muscle in the first patient. In proximal upper & lower limb muscles, EMG showed features of denervation. In the second patient, EMG was not advised. VEP in one patient had increased latency of P100 wave & other had normal VEP. Brainstem auditory evoked potential was normal in both patients. Conclusion: The paper highlights the importance of electrophysiological studies in diagnosis of motor sensory neuropathy in absence of genetic studies. Marked slowing of conduction velocity is the hallmark of CMDT1 [demylinating type].

2020 ◽  
Vol 17 (4) ◽  
pp. 451-455
Author(s):  
Rekha Limbu ◽  
Dilip Thakur ◽  
Nirmala Limbu ◽  
Prakash Parajuli ◽  
Shivalal Sharma ◽  
...  

Background: Repetitive exposure to vibration has been shown to induce peripheral nerve dysfunction. Dentists are exposed to handheld vibrating tools in their daily clinical practice. Most of the studies are done in dentists who have symptoms such as paresthesia and numbness of the hands. Thus, we conducted the study to explore the effect of vibration on nerve conduction variables in apparently healthy asymptomatic dental residents. Methods: This cross-sectional study enrolled 22 dental residents and age matched 22 medical residents as controls. Nerve conduction study was performed in median and ulnar nerves of both hands. Results: Anthropometric and cardiorespiratory variables were comparable between the groups. There were no statistically significant differences between dental and medical residents in the sensory conduction variables (right median onset latency=2.05±0.27 vs 1.91±0.21, p value=0.07; right median amplitude =27.80±8.11 vs 29.55±7.04, p=0.45; right median conduction velocity = 59.54±7.05 vs 61.06±5.15, p= 0.42) and motor conduction variables (right median distal latency = 2.87±0.38 vs 2.87±0.38, p= 0.94; right median distal amplitude=10.71±2.19 vs 11.10±2.37, p=0.58; right median conduction velocity= 70.57±13.16 vs 68.53±7.73, p= 0.54) of median and ulnar nerves. Further, there was no significant difference between the dominant and non-dominant hands of dental residents. Conclusions: Hand held vibration tools did not alter nerve conduction study parameters of dental residents. Keywords: Dentists; nerve conduction study; vibration.


2017 ◽  
Author(s):  
Kate Goddard ◽  
Prashanth Vas ◽  
Alistair Purves ◽  
Viktoria McMillan ◽  
Thomas Langford ◽  
...  

BACKGROUND Various tests are used to detect diabetic peripheral neuropathy by assessing sense perception in the feet. Tests vary in terms of time and resources required. Simple tests are those that can be conducted quickly and easily in primary care without laboratory equipment. There are some limitations to these simple tests, an example being the variable amplitude of the 128 Hz tuning fork. A new test, VibraTip (McCallan Medical, UK), might be a valuable alternative as it emits a consistent amplitude and may offer improved diagnostic accuracy. OBJECTIVE The aims of this study are to estimate the diagnostic accuracy of the VibraTip device for diabetic peripheral neuropathy against the reference standard of sural nerve conduction velocity measurement, and to assess whether the VibraTip offers superior diagnostic accuracy to other routine tests based on vibration or touch. METHODS The study will prospectively recruit adults with type 2 diabetes who are due to attend a routine follow-up clinic. A cross-sectional study design will be employed to assess the diagnostic accuracy of 5 standard index tests for peripheral neuropathy, including VibraTip. The reference test will be sural nerve conduction velocity measurement. RESULTS Funding is being sought to conduct this research. The outcomes assessed will be the diagnostic accuracy of the 5 index tests against sural nerve conduction velocity measurement, including sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio, and negative likelihood ratio. Receiver operating characteristic curves will be constructed and compared for each test. CONCLUSIONS This study will be the first within-study comparison of 5 simple tests for screening diabetic peripheral neuropathy and will address uncertainties in the potential benefits of using VibraTip in comparison with the other tests.


2017 ◽  
Vol 08 (S 01) ◽  
pp. S083-S088
Author(s):  
Manjinder Singh ◽  
Sharat Gupta ◽  
Kamal Dev Singh ◽  
Avnish Kumar

ABSTRACT Background: Nerve conduction studies (NCSs) are essential for diagnosing various kinds of focal and diffuse neuropathies. Due to the paucity of local NCS data, electrodiagnostic laboratories in Punjab rely on values from Western and other Indian studies. Aim: This study was conducted to provide normative data for median nerve conduction parameters (motor and sensory) in Punjabi populace. Materials and Methods: A cross-sectional study was done on 290 participants (150 males and 140 females), aged 17–21 years, as per standardized protocol. The data were analyzed separately for both genders using SPSS version 20. It consisted of distal latencies and conduction velocities of motor and sensory divisions of median nerve. Student's unpaired t-test was used for statistical analysis. Results: There was no effect of gender on any of the median nerve conduction parameters. Height and weight had nonsignificant negative and positive correlation, respectively (P > 0.05), with conduction velocity in both motor and sensory median nerves. For median motor nerve, the values of distal latency and conduction velocity in males were 2.9 ± 0.16 ms and 60.25 ± 2.99 m/s, respectively, whereas, in females, they were 2.6 ± 0.43 ms and 59.83 ± 2.82 m/s. Similarly, for median sensory nerve, the latency and velocity values in males were 2.8 ± 0.56 ms and 54.81 ± 3.70 m/s, whereas, in females, they were 2.4 ± 0.33 ms and 54.56 ± 3.65 m/s, respectively. Conclusion: The data in this study compared favorably with already existing data. It would help the local electrodiagnostic laboratories in assessing the median nerve abnormalities with greater accuracy in this population subset.


2021 ◽  
Vol 9 (1) ◽  
pp. e001698
Author(s):  
Haifa Maalmi ◽  
Kristiaan Wouters ◽  
Hans H C M Savelberg ◽  
Jeroen H P M van der Velde ◽  
Jos P H Reulen ◽  
...  

IntroductionDistal sensorimotor polyneuropathy (DSPN) is common in people with diabetes but is also found in pre-diabetes. Peripheral nerve myelin damage, which can be assessed by reduced nerve conduction velocity (NCV), is an essential feature of DSPN. Emerging evidence indicates that the development of DSPN may involve the activation of the immune system. However, available studies have mainly investigated circulating immune mediators, whereas the role of immune cells remains unclear. Therefore, we aimed to test whether leukocyte subsets are associated with NCV.Research design and methodsThis cross-sectional study analyzed data from 850 individuals (of whom 252 and 118 had type 2 diabetes and pre-diabetes, respectively) of the Maastricht Study. NCV was measured in the peroneal and tibial motor nerves and the sural sensory nerve and summed to calculate a standardized NCV sum score. Associations between percentages of leukocyte subsets and NCV sum scores were estimated using linear regression models adjusted for demographic, lifestyle, metabolic and clinical covariates.ResultsAfter adjustment for covariates, higher percentages of basophils and CD4+ T cells were associated with lower NCV (p=0.014 and p=0.005, respectively). The percentage of CD8+ T cells was positively associated with NCV (p=0.022). These associations were not modified by glucose metabolism status (all pinteraction >0.05). No associations were found for monocytes, eosinophils, neutrophils, lymphocytes, total T cells, Treg cells and B cells.ConclusionsThe associations of basophils, CD4+ and CD8+ T cells with NCV suggest that cell types from both innate and adaptive immunity may be implicated in the development of DSPN.


2018 ◽  
Vol 59 (2) ◽  
pp. 187-193 ◽  
Author(s):  
Mustafa A. Kural ◽  
Signe T. Andersen ◽  
Niels T. Andersen ◽  
Henning Andersen ◽  
Morten Charles ◽  
...  

2019 ◽  
Vol 24 (01) ◽  
pp. e86-e92
Author(s):  
Bianca Simone Zeigelboim ◽  
Anylize Wachholz Vom Scheidt ◽  
Kairone Fernandes Kronbauer ◽  
Paulo Breno Noronha Liberalesso ◽  
Maria Renata José ◽  
...  

Abstract Introduction Spinocerebellar ataxia (SCA) is part of a genetic and clinical heterogeneous group of neurodegenerative diseases characterized by progressive cerebellar ataxia. Objective To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA). Methods A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests. Results The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral. Conclusion The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.


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