Sensory Nerve Action Potential Amplitude Research Articles

Glycemic variability is associated with sural nerve conduction velocity in outpatients with type2diabetes: Usefulness of a new point-of-care device for nerve conduction studies.

Although several studies have shown the association between continuous glucose monitoring (CGM)-derived glycemic variability (GV) and diabetic peripheral neuropathy, no studies have focused on outpatients or used NC-stat®/DPNCheck™, a new point-of-care device for nerve conduction study (NCS). We investigated the association between CGM-derived GV and NCS using DPNCheck™ in outpatients with type 2 diabetes, and further analyzed the difference in results between patients with and without well-controlled HbA1c levels. All outpatients with type 2 diabetes using the CGM device (FreeStyle Libre Pro®) between 2017 and 2022 were investigated. Sural nerve conduction was evaluated by sensory nerve action potential (SNAP) amplitude and sensory conduction velocity (SCV) using DPNCheck™. Associations of CGM-derived GV metrics with SNAP amplitude and SCV were investigated. In total, 304 outpatients with type 2 diabetes were included. In a linear regression model, most CGM-derived GV metrics except for the mean amplitude of glucose excursion and low blood glucose index were significantly associated with SCV, but not with SNAP amplitude. The significant associations of most CGM-derived GV metrics with SCV remained after adjustment for possible confounding factors, but not after adjustment for glycated hemoglobin (HbA1c). Most CGM-derived GV metrics were significantly associated with SCV after adjustment for HbA1c in patients with a HbA1c ≤ 6.9%, but not in those with a HbA1c ≥ 7.0%. In outpatients with type 2 diabetes, multiple CGM-derived GV metrics were significantly associated with SCV obtained by DPNCheck™. GVmay have independent impacts on peripheral nerve function, particularly in patients with well-controlled HbA1c levels.

Evaluation of hand-arm vibration syndrome owing to nerve conduction study and cumulative exposure index -A 2.5-year cohort study.

This study aims to clarify the relationship between peripheral neuropathy in hand-arm vibration syndrome and cumulative exposure index. 68 participants without symptoms were surveyed. The participants were divided into three groups based on past and current vibration exposure (VE). Comparison among groups according to past VE showed that the median and ulnar sensory nerve conduction velocities and median sensory nerve action potential (SNAP) amplitude were significantly lower in past high and low exposure groups than in the past non-exposure group. Comparison among groups according to current VE showed that the median and ulnar SNAP amplitudes were significantly lower in the current high exposure group than in the current low or non-exposure group. Vibration tool handlers have potential peripheral nerve lesions at a certain stage without subjective symptoms of the finger.

Reference values for nerve conduction studies of the peroneal, tibial, and sural nerve derived from a large population-based cohort: Associations with demographic and anthropometric characteristics-The Maastricht study.

Nerve conduction studies (NCSs) are widely used to support the clinical diagnosis of neuromuscular disorders. The aims of this study were to obtain reference values for peroneal, tibial, and sural NCSs and to examine the associations with demographic and anthropometric factors. In 5099 participants (aged 40-79 years) without type 2 diabetes of The Maastricht Study, NCSs of peroneal, tibial, and sural nerves were performed. Values for compound muscle action potential (CMAP) and sensory nerve action potential amplitude, nerve conduction velocity (NCV), and distal latency were acquired. The association of age, sex, body mass index (BMI), and height with NCS values was determined using uni- and multivariate linear regression analyses. Detailed reference values are reported per decade for men and women. Significantly lower NCVs and longer distal latencies were observed in all nerves in older and taller individuals as well as in men. In these groups, amplitudes of the tibial and sural nerves were significantly lower, whereas a lower peroneal nerve CMAP was only significantly associated with age. BMI showed a multidirectional association. After correction for anthropometric factors in the multivariate analysis, the association between sex and NCS values was less straightforward. These values from a population-based dataset could be used as a reference for generating normative values. Our findings show the association of NCS values with anthropometric factors. In clinical practice, these factors can be considered when interpreting NCS values.

Simplified electrophysiological approach combining a point-of-care nerve conduction device and an electrocardiogram produces an accurate diagnosis of diabetic polyneuropathy.

This study aimed to investigate the diagnostic potential of two simplified tests, a point-of-care nerve conduction device (DPNCheck™) and a coefficient of variation of R-R intervals (CVR-R ), as an alternative to traditional nerve conduction studies for the diagnosis of diabetic polyneuropathy (DPN) in patients with diabetes. Inpatients with type 1 or type 2 diabetes (n = 167) were enrolled. The study population consisted of 101 men, with a mean age of 60.8 ± 14.8 years. DPN severity was assessed using traditional nerve conduction studies, and differentiated based on Baba's classification (BC). To examine the explanatory potential of variables in DPNCheck™ and CVR-R regarding the severity of DPN according to BC, a multiple regression analysis was carried out, followed by a receiver operating characteristic analysis. Based on BC, 61 participants (36.5% of the total) were categorized as having DPN severity of stage 2 or more. The multiple regression analysis yielded a predictive formula with high predictive power for DPN diagnosis (estimated severity of DPN in BC = 2.258 - 0.026 × nerve conduction velocity [m/s] - 0.594 × ln[sensory nerve action potential amplitude (μV)] + 0.528In[age(years)] - 0.178 × ln[CVR-R ], r = 0.657). The area under the curve in receiver operating characteristic analysis was 0.880. Using the optimal cutoff value for DPN with severer than stage 2, the predictive formula showed good diagnostic efficacy: sensitivity of 83.6%, specificity of 79.2%, positive predictive value of 51.7% and negative predictive value of 76.1%. These findings suggest that DPN diagnosis using DPNCheck™ and CVR-R could improve diagnostic efficiency and accessibility for DPN assessment in patients with diabetes.

Values and diagnostic accuracy of sensory nerve action potentials in control participants and participants with diabetes with and without clinical diabetic neuropathy, based on neuropathy scale measurements.

The assessment of the normative values of sensory nerve action potentials (SNAP) and their diagnostic accuracies using validated neuropathy-assessment tools to classify participants into groups with and without neuropathy was not previously described in the literature. The Utah Early Neuropathy Scale (UENS), Michigan neuropathy-screening instrument, and nerve conduction data were collected prospectively. We described and compared the values of the sural, superficial peroneal sensory (SPS), and superficial radial SNAP amplitude in different age groups for three groups. Group 1 (G1)-control participants (UENS <5), group 2 (G2)-participants with diabetes without clinical diabetic neuropathy (UENS <5), and group 3 (G3)-participants with clinical diabetic neuropathy (UENS ≥5). We also described the diagnostic accuracy of single-nerve amplitude and a combined sensory polyneuropathy index (CSPNI) that consists of four total points (one point for each of the following nerves if their amplitude was <25% lower limit of normal: right sural, left sural, right SPS, and left SPS potentials). We assessed 135 participants, including 41, 37, and 57 participants in G1, G2, and G3, respectively, with age median (interquartile ranges) of 51 (45-56), 47 (38-56), and 54 (51-61) years, respectively, whereas 19 (46.3%), 18 (48.7%), and 32 (56.14%) of them were males, respectively. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) scores were 68.4%, 92.3%, 86.7%, and 80% for the sural amplitude; 86%, 58.3%, 62%, and 84% for the SPS amplitude; 66.7%, 94.4%, 90.5%, and 78.2% for the CSPNI of 3; and 54.4%, 98.6%, 96.9%, and 73.2% for the CSPNI of 4, respectively. Sural nerve had a high specificity for neuropathy; however, the CSPNI had the highest specificity and PPV, whereas the SPS had the highest sensitivity and NPV.

Effect of age on metabolomic changes in a model of paclitaxel-induced peripheral neurotoxicity.

Chemotherapy-induced peripheral neurotoxicity (CIPN) is one of the most common dose-limiting side effects of paclitaxel (PTX) treatment. Many age-related changes have been hypothesized to underlie susceptibility to damage or impaired regeneration/repair after nerve injury. The results of these studies, however, are inconclusive and other potential biomarkers of nerve impairment need to be investigated. Twenty-four young (2 months) and 24 adult (9 months) Wistar male rats were randomized to either PTX treatment (10 mg/kg i.v. once/week for 4 weeks) or vehicle administration. Neurophysiological and behavioral tests were performed at baseline, after 4 weeks of treatment and 2-week follow-up. Skin biopsies and nerve specimens collected from sacrificed animals were examined for intraepidermal nerve fiber (IENF) density assessment and nerve morphology/morphometry. Blood and liver samples were collected for targeted metabolomics analysis. At the end of treatment, the neurophysiological studies revealed a reduction in sensory nerve action potential amplitude (p < .05) in the caudal nerve of young PTX-animals, and in both the digital and caudal nerve of adult PTX-animals (p < .05). A significant decrease in the mechanical threshold was observed only in young PTX-animals (p < .001), but not in adult PTX-ones. Nevertheless, both young and adult PTX-rats had reduced IENF density (p < .0001), which persisted at the end of follow-up period. Targeted metabolomics analysis showed significant differences in the plasma metabolite profiles between PTX-animals developing peripheral neuropathy and age-matched controls, with triglycerides, diglycerides, acylcarnitines, carnosine, long chain ceramides, sphingolipids, and bile acids playing a major role in the response to PTX administration. Our study identifies for the first time multiple related metabolic axes involved in PTX-induced peripheral neurotoxicity, and suggests age-related differences in CIPN manifestations and in the metabolic profile.

Sensory nerve conduction studies in infants, children and teenagers – An update

ObjectiveNerve conduction studies (NCS) in children remain technically challenging and depend on the cooperation of the child. Motor NCS are not compromised by analgosedation but data for sensory NCS are lacking. Here, we ask whether sensory NCS is influenced by analgosedation. We also compare the present data with NCS studies from the 1990s regarding anthropometric acceleration of the contemporary paediatric population. MethodsSensory NCS of the median nerve and sural nerve were performed in 182 healthy subjects aged 1 to 18 years during general anaesthesia and in 47 of them without analgosedation. ResultsSensory NCS was not influenced by midazolam or propofol. The sensory nerve action potential (SNAP) amplitude and the nerve conduction velocity (NCV) of the sural nerve as well as the SNAP of the median nerve show no significant age dependence in age range 1–18 years. The sensory NCV of the median nerve increased age-dependent. ConclusionsIn clinical practice, analgosedation can be used for diagnostic NCS. Sensory NCS data show no relevant secular trend over the last 30 years. Differences due to technical inconsistency predominate. SignificanceAnalgosedation can improve diagnostic quality of sensory NCS in children. Additionally, we provide sensory NCS values from a large pediatric cohort.

Effect of metformin nanoemulsion through AMP-activated protein kinase (AMPK)-mediated autophagy on diabetic neuropathy

Metformin (MET) is the preferred first-line treatment for patients with type 2 diabetes. However, the mechanism of diabetic peripheral neuropathy (DPN) is still unclear. To improve the oral utilization of metformin, a metformin nanoemulsion (MET-NE) was prepared to investigate its effects on DPN and its underlying mechanism. In this study, a DPN model was established in Wistar rats induced by streptozotocin (STZ). The diabetic rats were randomly divided into four groups: the diabetic model group (DM group), the metformin tablet-treated group (MET group), the metformin nanoemulsion-treated group (MET-NE group), and a normal control group consisting of five normal Wistar rats. All groups were administered the treatment orally for a period of 10 weeks. The findings of the study demonstrated that both MET and MET-NE significantly reduced blood glucose levels, glycated serum protein levels, food intake, and water intake in DM rats. It was also observed that MET-NE was more effective than MET in reducing blood glucose levels. Additionally, both MET and MET-NE treatments significantly increased the motor nerve conduction velocity (MNCV), sensory nerve conduction velocity (SNCV), and sensory nerve action potential (SNAP) amplitude in DM rats. Furthermore, these treatments improved mechanical withdrawal threshold (MWT) and thermal withdrawal latency (TWL), resulting in a reduced sensitivity to pain stimuli. Moreover, both MET and MET-NE treatments promoted the phosphorylation of AMP-activated protein kinase (AMPK) and acetyl-CoA carboxylase (ACC) proteins, as well as increased the activity of autophagic proteins in the sciatic nerve. However, no significant differences were observed between MET and MET-NE treatments in terms of these effects. In conclusion, the MET-NE demonstrated a rapid decrease in blood glucose levels and improved glucose tolerance and metabolism, which was found to be superior to MET. Furthermore, MET-NE significantly improved the neurophysiological function and sciatic nerve pain threshold in DPN rats. These beneficial effects may be attributed to the regulation of AMPK-mediated autophagy by MET-NE.

Sensory nerve action potential in patients with functional neurological disorders with sensory manifestations.

Functional neurological disorder (FND) has various clinical manifestations. Even though diagnostic criteria for FND have been proposed, FND characteristics with sensory manifestations have not been elucidated. Therefore, we aimed to investigate the association between sensory nerve action potential (SNAP) amplitudes and FND with sensory manifestations. We included 76 outpatients with FND with sensory manifestations whose nerve conduction studies were performed retrospectively. Additionally, we defined 121 patients with other neurological diseases who did not have peripheral neuropathy as disease controls. The SNAP amplitudes were compared between the two groups. We also explored the relationship between SNAP amplitudes and FND-specific clinical symptoms in patients with FND. No differences were observed in SNAP amplitudes adjusted for age between patients with FND who had sensory manifestations and disease control patients. Additionally, no differences were observed between patients with FND who had and did not have FND-specific clinical symptoms. The SNAP amplitude in patients with FND who had sensory manifestations was equivalent to that in controls.

Association of Sensory Nerve Action Potential Amplitude and Velocity With Type 2 Diabetic Peripheral Neuropathy

Association of Sensory Nerve Action Potential Amplitude and Velocity With Type 2 Diabetic Peripheral Neuropathy

Electrophysiologic pattern of carpal tunnel syndrome among patients with and without diabetes mellitus

Background: Carpal tunnel syndrome (CTS) is the most common compression neuropathy in the upper limb in general population. Female gender, obesity, increase of age, diabetes mellitus (DM), hypothyroidism, pregnancy, rheumatoid arthritis, osteoarthritis and occupational factors like repetitive work are the main risk factors for CTS.  The objective of this study was to determine whether the nerve conduction study measurement of the median nerve (MN)  at the wrist differ between diabetic and non-diabetic CTS patients. Methods: This cross-sectional study was conducted at Electrodiagnostic Lab of BIRDEM General Hospital and Popular Medical College Hospital, Dhaka during the period of October 2017 to August 2019. Five hundred and seventeen hands from 377 patients were sampled according to inclusion criteria. Participants were assigned to two groups: Group 1 (CTS with DM) and Group 2 (CTS without DM). NCS study were performed using Nihon Kohden electrodiagnostic machine. Measurements at the MN was taken under consideration for study. Severity of CTS was graded according to Canterbury electrophysiologic grading scale and parameters were compared among diabetic and non-diabetic participants. Data were analyzed using Statistical Package for the Social Sciences SPSS version 23 (SPSS Inc., Chicago, IL, USA). Results:  The 377 participants comprised 144 and 233 in Group 1 and Group 2, respectively. Out of 377 patients, 334 (88.59%) were females and there was no statistically significant difference between two groups. The average age was 50.66±10.2 years in diabetic participants and 43.34±10.7 years in non- diabetic participants. In the study population moderate CTS (Grade 3) was the most frequent (46.4%) followed by very severe (Grade 5) CTS. There was no statistically significant difference in incidence between diabetic and non-diabetic patients. Analysis of electrophysologic parameters of MN showed statistically significant difference in mean MN  sensory nerve action potential (SNAP) amplitude (P 0.003),  compound muscle action potential (CMAP) amplitude (p 0.05, right) and CMAP latency (p 0.02, right and 0.05, left) between diabetic and non –diabetic patients. Inter-group comparison revealed that impaired glycaemic status and duration of diabetes in diabetic population had a minimal influence in the electrophysiological severity of CTS in either hands. Conclusion: Some nerve conduction study parameters of the MN differ significantly between diabetic and non-diabetic patients with CTS and may provide both diagnostic and predictive results across the entire spectrum of CTS. BIRDEM Med J 2023; 13(3): 136-142

Age-Related Changes in Neurologic Examination and Sensory Nerve Amplitude in the General Population: Aging of the Peripheral Nervous System.

Chronic axonal polyneuropathy is a common disease of the peripheral nervous system with increasing prevalence with age. Typical neurologic signs are present in patients with polyneuropathy but may also occur in individuals without disease. Owing to limited knowledge on normal aging of the peripheral nervous system, it can be difficult to distinguish peripheral nerve dysfunction due to disease from variations in normal aging. Therefore, we described the changes in neurologic examination and nerve conduction studies that accompany aging in the general population. In this cross-sectional population-based study, we screened participants for chronic polyneuropathy in a controlled environment using standardized methods including a symptom questionnaire, neurologic examination, and nerve conduction studies (NCS). Inclusion criteria were 40 years or older and living in a suburb of Rotterdam, the Netherlands. Participants not diagnosed with chronic polyneuropathy, based on the discussion of findings in the screening by an expert team, were included to determine the effect of age (range 41-96 years) on features of neurologic examination and NCS using frequency calculations and quantile regression analysis. In total, 4,179 participants (mean age 64.5 ± 12.7 years, 54.9% female) were included of whom 3,780 (90.5%) did not fulfil the criteria for polyneuropathy. In the population without polyneuropathy, the frequency of normal features at neurologic examination declined with age, most pronounced for vibration sense at the hallux (from 6.6 [SD ± 1.5] in 40-49 years to 3.6 [SD ± 3.1] in 80 years or older) and Achilles tendon reflexes (absent in 9% in 40-49 years up to 33% in 80 years or older). Superficial pain sensation and patellar tendon reflexes remained stable over time. Sural sensory nerve action potential (SNAP) amplitude declined with age from 11.2 μV in 40-49 years to 3.3 μV in 80 years or older. Nonrecordable SNAP amplitudes were found in 25.1% of the participants older than 80 years, more often in men (30.3%) than in women (21.0%). This study showed the effect of age on features of neurologic examination and sural nerve amplitude in the general population. These findings are helpful to distinguish features suggesting polyneuropathy from variations of normal aging of the peripheral nervous system.

Nerve conduction studies through landmark based recording and effects of hand length

As per existing guidelines, the distance between stimulator and recording electrodes in nerve conduction studies (NCS) should be the same (fixed) in all the subjects, i.e., it should not be based on anatomical landmarks. However, there are no studies comparing fixed distance recordings with landmark based NCS. We postulated that hand length can influence the NCS parameters in fixed distance recordings and this can be nullified using landmark based recordings. To test this theory, we performed NCS in 48 normal subjects as per standard guidelines (standard protocol) and then compared it to NCS with ulnar styloid as the landmark (modified protocol). NCS were performed on median and ulnar nerves of the right upper limb. Three motor NCS parameters including distal latency, compound muscle action potential (CMAP) amplitudes and nerve conduction velocities were measured. Sensory nerve action potential (SNAP) amplitudes and conduction velocities were the two sensory parameters measured. On analysis, ulnar motor conduction velocity was the only parameter affected by hand length in both standard and modified protocols. Modified protocol did not have any additional advantage to the standard protocol advised by NDTF. We conclude that the NDTF guidelines are therefore reasonable when considering the effects of hand length. Possible reasons for this result including anatomical and anthropometric explanations are discussed.

No Major Nerve Regeneration Seems to Occur during Recovery of Ulnar Neuropathy at the Elbow.

Introduction: There are three main potential mechanisms of recovery after nerve lesion: (1) resolution of conduction block, (2) collateral reinnervation, and (3) nerve regeneration. Their relative contributions in recovery after focal neuropathies are not well established. Methods: In a group of previously reported prospective cohort of patients with ulnar neuropathy at the elbow (UNE), I performed a post-hoc analysis of their clinical and electrodiagnostic findings. I compared amplitudes of the compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) on ulnar nerve stimulation, as well as qualitative concentric needle electromyography (EMG) findings in the abductor digiti minimi muscle on the initial and follow-up examinations several years later. Results: Altogether, 111 UNE patients (114 arms) were studied. During median follow-up period of 880 days (range: 385-1545 days), CMAP amplitude increased (p = 0.02), and conduction block in the elbow segment recovered (from median 17% to 7%; p < 0.001). By contrast, SNAP amplitude did not change (p = 0.89). On needle EMG, spontaneous denervation activity diminished (p < 0.001), motor unit potential (MUP) amplitude increased (p < 0.001), and MUP recruitment remained unchanged (p = 0.43). Conclusions: Findings of the present study indicate that nerve function in chronic focal compression/entrapment neuropathies seems to improve mainly due to the resolution of the conduction block and collateral reinnervation. Contribution of nerve regeneration seems to be minor; the majority of axons lost in chronic focal neuropathies probably never recover. Further studies using quantitative methods are needed to validate present findings.

MRI negative Varicella zoster virus (VZV) plexopathy in a young man with history of malignancy (P2-4.001)

<h3>Objective:</h3> To emphasize the importance of including VZV plexopathy in differential diagnosis in young individuals and to prevent the delay in diagnosis and treatment <h3>Background:</h3> VZV-associated plexopathy in mainly patients over 60 years old. Post herpetic neuralgia is a well-known complication of Herpes Zoster (HZ), however segmental zoster paresis secondary to HZ was reported in 1–20% of the cases in literature. MRI findings may be positive in up to 70% of the patients. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> 43 years-old man with history of grade 2 left frontal oligodendroglioma which was treated with 2 partial resections, radiation and adjuvant procarbazine/lomustine presented with left upper extremity pain and developed a blistering rash in dermatomal pattern in left proximal upper extremity 2 weeks after the initial symptoms. He was diagnosed with shingles and treated with steroids and acyclovir with minimal improvement. 6 weeks after presentation, physical exam revealed left deltoid, supraspinatus and infraspinatus weakness with normal muscle stretch reflexes and decreased sensation on C5 dermatome. EMG revealed absent left lateral antebrachial cutaneous sensory nerve action potentials (SNAP) amplitude, and a small left radial SNAP amplitude compared to the right side. Evidence of ongoing denervation with reinnervation was seen in the left upper trunk supplied muscles. MRI of the brachial plexus was negative for any abnormalities. Patient was diagnosed with VZV-associated plexopathy, improved with pregabalin and physical therapy. <h3>Conclusions:</h3> Our patient was significantly younger than expected in HZ group. MRI usually shows T2 hyperintensities and thickening of the nerve roots in patients with VZV-associated plexopathy. However, the presentation, onset of symptoms, characteristics of the rash and clinical course was diagnostic of HZ, and the weakness pattern, supported by the EMG findings were diagnostic for VZV-associated plexopathy. <b>Disclosure:</b> Dr. Gul has nothing to disclose. Ms. Ahmed has nothing to disclose. Dr. McGraw has nothing to disclose. Dr. Alshiekh Nasany has nothing to disclose.

Neuropathy due to bi-allelic SH3TC2 variants: genotype-phenotype correlation and natural history.

Recessive SH3TC2 variants cause Charcot-Marie-Tooth disease type 4C (CMT4C). CMT4C is typically a sensorimotor demyelinating polyneuropathy, marked by early onset spinal deformities, but its clinical characteristics and severity are quite variable. Clear relationships between pathogenic variants and the spectrum of disease manifestations are to date lacking. Gene replacement therapy has been shown to ameliorate the phenotype in a mouse model of CMT4C, emphasizing the need for natural history studies to inform clinical trial readiness. Data, including both genetic information and clinical characteristics, were compiled from the longitudinal, prospective dataset of the Inherited Neuropathy Consortium, a member of the Rare Diseases Clinical Research Network (INC-RDCRN). The Charcot Marie Tooth Neuropathy Score (CMTNS), Examination Score (CMTES) and the Rasch-weighted CMTES (CMTES-R) were used to describe symptoms, neurological examinations and neurophysiological characteristics. Standardized response means were calculated at yearly intervals and a mixed model for repeated measures was used to estimate the change in CMTES and CMTES-R over time. Fifty-six individuals (59% female), median age 27 years (range 2-67 years) with homozygous or compound heterozygous variants in SH3TC2 were identified, including 34 unique variants, 14 of which have not previously been published. Twenty-eight participants had longitudinal data available. While there was no significant difference in the CMTES in those with protein truncating versus non-protein truncating variants, there were significant differences in the mean ulnar nerve compound muscle action potential amplitude, the mean radial sensory nerve action potential amplitude, and in the prevalence of scoliosis, suggesting the possibility of a milder phenotype in individuals with one or two non-protein-truncating variants. Overall, the mean value of the CMTES was 13, reflecting moderate clinical severity. There was a high rate of scoliosis (81%), scoliosis surgery (36%), and walking difficulty (94%) among study participants. The CMTES and CMTES-R appeared moderately responsive to change over extended follow-up, demonstrating a standardized response mean of 0.81 standard deviation units or 0.71 standard deviation units, respectively, over 3 years. Our analysis represents the largest cross-sectional and only longitudinal study to date, of the clinical phenotype of both adults and children with CMT4C. With the promise of upcoming genetic treatments, these data will further define the natural history of the disease and inform study design in preparation for clinical trials.

Morphological and electrophysiological evaluation of median and ulnar nerve in complex regional pain syndrome type 1.

Complex regional pain syndrome (CRPS) can be distinguished as type I without and type II with electrophysiological evidence of major nerve lesion. The pathophysiology of both subgroups is still under investigation. The aim of this research is to demonstrate the nerve morphology and electrophysiology in CRPS type I patients. Bilateral median and ulnar nerve cross-sectional areas were evaluated with ultrasound and also median and ulnar nerve conduction studies of both hands were performed. Cross-sectional areas of median and ulnar nerves and nerve conduction studies in healthy controls were also obtained and compared with the patients. Twenty-five male patients and 11 healthy male controls were enrolled in the study. The mean age of the patients was 24.08 ± 5.50 years and controls was 23.18 ± 5.09 (p> 0.05). Compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes of the diseased side were found significantly lower than the healthy side (p< 0.05). Both median and ulnar nerve distal motor latency values were significantly higher in the patient group (p< 0.05). There was no significant difference in the median and ulnar nerve cross-sectional area when compared with the opposite extremity and healthy volunteers. The lower SNAP and CMAP amplitudes of the median and ulnar nerves compared to the healthy side and the prolongation of the affected side median and ulnar nerve distal motor latencies of the affected individuals may indicate axonal involvement in patients with CRPS type 1. Decreased CMAP amplitudes may also indicate muscle atrophy due to a decrease in the number of functional motor units.

Electrophysiology of ataxia-telangiectasia-like disorder 1.

Ataxia-telangiectasia-like disorder-1 (ATLD1, OMIM # 604391) is a very rare clinical condition, characterized by slowly progressive ataxia with onset in childhood, associated with oculomotor apraxia and dysarthria. Laboratory findings reveal increased susceptibility to radiation, with a defect in DNA repair. Patients with ATLD1 show no telangiectasia, have no immunodeficiency, and also have preserved cognition. Reflexes might be initially brisk and later becomes reduced associated with axonal sensorimotor neuropathy. Brain magnetic resonance imaging (MRI) detects cerebellar atrophy. The condition is caused by mutations in the meiotic recombination 11 (MRE11A) gene. The present study reports on the neurophysiologic finding in eight Saudi patients, belonging to three Saudi families, who have genetically confirmed ATLD1. All investigated patients had cerebellar atrophy on brain MRI (5/5). Electrophysiologic studies showed normal motor conduction velocity (MCV) of the median (8/8) and tibial (2/2) nerves, while 5/6 (83%) had normal peroneal nerve MCV. The distal motor latency (DML) for median, tibial, and peroneal nerves was within the normal range in all examined patients. The amplitude of compound muscle action potential (CMAP) of median and tibial nerves was also normal, while that of the peroneal nerve was normal in 3/6 (50%). Two of seven (29%) patients had reduced amplitude of median nerve sensory nerve action potential (SNAP) while 3/8 (38%) had a reduction in the amplitude of sural nerve SNAP. These findings favour an axonal type of neuropathy predominately affecting the sensory fibres (axonal sensorimotor neuropathy). The present study constitutes the largest cohort of ATLD1 patients worldwide who had electrophysiologic tests.

Sural nerve conduction: Amplitude and distal latency of sensory nerve action potential

Background: The sensory nerve action potential (SNAP) is an important factor in the electro diagnostic evaluation of a patient with suspected peripheral neuropathy. Sural nerve is the most frequently studied nerve in the electrophysiology. For proper diagnosis sural sensory conduction abnormalities, a reference data are required for each electrophysiology laboratory. Aims and Objectives: The aim of the study was to find out normative reference data of amplitude and distal latency (DL) of sural SNAP for Northern Kerala population. Materials and Methods: The study was designed as cross-sectional and observational study and analysis of values was done using SPSS version 16. A total of 250 healthy subjects of age 15–50 from Northern Kerala participated in the study which included equal number of males and females. Results: Normative reference data for sensory amplitude and DL were derived in the present study. Sensory amplitude was more and DL was found to be less in female population, indicating faster nerve conduction in female. Conclusion: The reference data derived here can be utilized to detect any abnormality sural nerve sensory conduction. The gender effect on sural nerve sensory conduction parameters can be attributed to the height difference, as nerve conduction velocity is inversely proportional to height.

Analysis of Electrodiagnostic Recovery after Carpal Tunnel Release: A Retrospective Study

Objective: We investigated the factors affecting electrodiagnostic (EDX) parameters after carpal tunnel release (CTR).Methods: Thirty-nine cases with clinically diagnosed carpal tunnel syndrome who received CTR and EDX studies before and after CTR were enrolled in this study. We analyzed EDX parameters such as distal onset latency and the amplitude of median compound motor action potentials (CMAPs) and sensory nerve action potentials (SNAPs).Results: Among 39 cases, 24 (61.5%) showed improvement of at least 1 grade, based on Bland’s scale, after CTR. Follow-up EDX studies were performed 6 to 36 months after CTR. Improvement on Bland’s scale was shown in 50% of patients who received follow-up EDX studies at 6 and 12 months after CTR and in all patients who received follow-up at 24 and 36 months. The EDX parameters showed significant recovery. Younger patients showed greater recovery of SNAP amplitude (p = 0.021, r = -0.369) after CTR. The preoperative severe group showed greater recovery of CMAP (both amplitude and latency) than the non-severe group (p = 0.011 and p = 0.038, respectively).Conclusion: We confirmed the effectiveness of CTR through EDX studies. Age and preoperative EDX severity can affect the recovery of EDX parameters after CTR.