Guillain-Barre syndrome of acute motor axonal neuropathy (AMAN) type associated with herpes zoster: a case report.

Guillain Barre syndrome (GBS) following Varicella zoster is a rare presentation and has only been reported in a few cases around the world. Of the reported cases, the type of GBS is not specified in the majority, and where specified is of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) type. We report a case of acute motor axonal neuropathy (AMAN) type GBS following herpes zoster in a 27-year-old male who presented with bilateral lower limb weakness and left sided lower motor neuron type facial nerve palsy a week after herpes zoster infection.

Computational models of compound nerve action potentials: Efficient filter-based methods to quantify effects of tissue conductivities, conduction distance, and nerve fiber parameters.

BACKGROUND: Peripheral nerve recordings can enhance the efficacy of neurostimulation therapies by providing a feedback signal to adjust stimulation settings for greater efficacy or reduced side effects. Computational models can accelerate the development of interfaces with high signal-to-noise ratio and selective recording. However, validation and tuning of model outputs against in vivo recordings remains computationally prohibitive due to the large number of fibers in a nerve. METHODS: We designed and implemented highly efficient modeling methods for simulating electrically evoked compound nerve action potential (CNAP) signals. The method simulated a subset of fiber diameters present in the nerve using NEURON, interpolated action potential templates across fiber diameters, and filtered the templates with a weighting function derived from fiber-specific conduction velocity and electromagnetic reciprocity outputs of a volume conductor model. We applied the methods to simulate CNAPs from rat cervical vagus nerve. RESULTS: Brute force simulation of a rat vagal CNAP with all 1,759 myelinated and 13,283 unmyelinated fibers in NEURON required 286 and 15,860 CPU hours, respectively, while filtering interpolated templates required 30 and 38 seconds on a desktop computer while maintaining accuracy. Modeled CNAP amplitude could vary by over two orders of magnitude depending on tissue conductivities and cuff opening within experimentally relevant ranges. Conduction distance and fiber diameter distribution also strongly influenced the modeled CNAP amplitude, shape, and latency. Modeled and in vivo signals had comparable shape, amplitude, and latency for myelinated fibers but not for unmyelinated fibers. CONCLUSIONS: Highly efficient methods of modeling neural recordings quantified the large impact that tissue properties, conduction distance, and nerve fiber parameters have on CNAPs. These methods expand the computational accessibility of neural recording models, enable efficient model tuning for validation, and facilitate the design of novel recording interfaces for neurostimulation feedback and understanding physiological systems.

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.

INTRODUCTION/AIMS: 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. METHODS: 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. RESULTS: 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. DISCUSSION: 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.

[Neuropathy in n-hexane poisoning]. / Neiropatiya pri otravlenii n-geksanom.

N-Hexane is a solvent widely used in manufacturing as a cleaner, degreaser and component of rubber cement. Chronic exposure to n-hexane either through contact with unprotected skin or inhalation can lead to the development of clinical symptoms and electrophysiological changes similar to those of inflammatory demyelinating polyneuropathy which requires careful differential diagnosis. This article presents three cases of severe predominantly motor polyneuropathy with demyelinating features in 15- and 16-year-old adolescents. The results of laboratory tests were within normal limits; electroneuromyography revealed symmetrical involvement of sensory and motor fibers of the nerves of the legs and arms with a decrease in the speed of propagation of excitation and conduction blocks. Sural nerve biopsy revealed intraneural and perineural swelling without any signs of inflammation or fibrosis confirming the genesis of the neuropathy. Despite a relatively favorable prognosis there is no specific therapy for hexane poisoning and the recovery period can last up to several years.

Nodal Conduction Block and Internodal Conduction Block in Nodopathy.

OBJECTIVES: In 2015, a new term "nodopathy" was introduced to represent a group of neuropathy because of autoantibodies at the node of Ranvier and paranodal area. This review was conducted to highlight the electrophysiologic characteristics of acute and chronic nodopathies by the newly introduced term: "nodal conduction block (CB); CB without temporal dispersion or slow nerve conduction velocity" and by introducing a new term: "internodal CB; CB with temporal dispersion or/and slow nerve conduction velocity". METHODS: Through PubMed searches, 23 cases of acute (<4 weeks of neuropathy) nodopathy and 12 cases of chronic (>4 weeks of neuropathy) nodopathy are identified. Two other required inclusion criteria are positive nodal antibody test and detailed nerve conduction data with or without figure. All existing data were analyzed to see whether these cases had nodal or internodal CB. RESULTS: Among 23 cases of acute nodopathy, 11 had nodal CB, 9 internodal CB, and 3 mixed CB. Thus, nodal CB was observed in 61% of acute nodopathy cases and internodal CB in 52% of acute nodopathy cases. Among 12 cases of chronic nodopathy, all 12 had internodal CB. CONCLUSIONS: Nodal CB is the nerve conduction characteristic of acute nodopathy, but internodal CB does not rule out acute nodopathy. Internodal CB is the nerve conduction characteristic of chronic nodopathy.

Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database.

BACKGROUND AND PURPOSE: This study aimed to assess the diagnostic criteria, ancillary investigations and treatment response using real-life data in multifocal motor neuropathy (MMN) patients. METHODS: Clinical and laboratory data were collected from 110 patients enrolled in the Italian MMN database through a structured questionnaire. Twenty-six patients were excluded due to the unavailability of nerve conduction studies or the presence of clinical signs and symptoms and electrodiagnostic abnormalities inconsistent with the MMN diagnosis. Analyses were conducted on 73 patients with a confirmed MMN diagnosis and 11 patients who did not meet the diagnostic criteria. RESULTS: The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria were variably applied. AUTHOR: When applying the American Association of Electrodiagnostic Medicine criteria, an additional 17% of patients fulfilled the criteria for probable/definite diagnosis whilst a further 9.5% missed the diagnosis. In 17% of the patients only compound muscle action potential amplitude, but not area, was measured and subsequently recorded in the database by the treating physician. Additional investigations, including anti-GM1 immunoglobulin M antibodies, cerebrospinal fluid analysis, nerve ultrasound and magnetic resonance imaging, supported the diagnosis in 46%-83% of the patients. Anti-GM1 immunoglobulin M antibodies and nerve ultrasound demonstrated the highest sensitivity. Additional tests were frequently performed outside the EFNS/PNS guideline recommendations. CONCLUSIONS: This study provides insights into the real-world diagnostic and management strategies for MMN, highlighting the challenges in applying diagnostic criteria.

Intermediate conduction velocity in two cases of Charcot-Marie-Tooth disease type 1A.

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease type 1A (CMT1A) is the most prevalent hereditary neuropathy worldwide and classically has slow nerve conduction velocity (NCV), in most cases below 38 m/s. Two unrelated patients with motor NCVs in the upper limbs above 38 m/s are reported. METHOD: Case report. RESULTS: Two genetically confirmed CMT1A patients are presented, from two unrelated families (one of British origin and the other of Brazilian origin). Both individuals had upper limb motor NCVs above 38 m/s, with values ranging from 41.9 to 45 m/s in the median nerve and from 42 to 42.3 m/s in the ulnar nerve. They presented with a very mild phenotype, with CMT Neuropathy Score version 2 (CMTNSv2) of 6 and 5, respectively. In contrast, affected family members within both kinships exhibited a classical phenotype with more severe disease manifestation (CMTNSv2 ranging from 12 to 20) and motor NCVs below 30 m/s. CONCLUSION: These cases, although very rare, highlight the importance of testing PMP22 duplication in patients with intermediate conduction velocities.

Typical CIDP, distal variant CIDP, and anti-MAG antibody neuropathy: An ultra-high frequency ultrasound comparison of nerve structure.

To date, little is known about the usefulness of ultra-high frequency ultrasound (UHF-US, 50-70 MHz) in clinical practice for the diagnosis of dysimmune neuropathies. We present a prospective study aimed at comparing UHF-US alterations of nerves and fascicles in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), distal CIDP (d-CIDP) and anti-MAG neuropathy and their relationships with clinical and electrodiagnostic (EDX) features. 28 patients were included (twelve CIDP, 6 d-CIDP and 10 anti-MAG) and ten healthy controls. Each patient underwent neurological examination, EDX and UHF-US study of median and ulnar nerves bilaterally. UHF-US was reliable in differentiating immune neuropathies from controls when using mean and/or segmental nerve and/or fascicle cross-sectional area (CSA); furthermore, fascicle ratio (fascicle/nerve CSA) was a reliable factor for differentiating d-CIDP from other types of polyneuropathies. The fascicle CSA appears to be more increased in CIDP and its variant than in anti-MAG neuropathy. UHF-US offers information beyond simple nerve CSA and allows for a better characterization of the different forms of dysimmune neuropathies.

Normal Values of Central, Peripheral, and Root Motor Conduction Times in a Healthy Korean Population.

PURPOSE: Central, peripheral, and root motor conduction times (CMCTs, PMCTs, and RMCTs, respectively) are valuable diagnostic tools for spinal cord and motor nerve root lesions. We investigated the normal values and the effects of age and height on each motor conduction time. METHODS: This study included 190 healthy Korean subjects who underwent magnetic stimulation of the cortex and spinous processes at the C7 and L1 levels. Recording muscles were abductor pollicis brevis and abductor digiti minimi in the unilateral upper limb and extensor digitorum brevis and abductor hallucis in the contralateral lower limb. F-wave and compound motor nerve action potentials were also recorded. Central motor conduction time was evaluated as the difference between cortical motor evoked potential onset latency and PMCT using calculation and spinal stimulation methods. Root motor conduction time was computed as the difference between spinal stimulated and calculated CMCTs. RESULTS: The average age and height of the participants were 41.21 ± 14.39 years and 164.64 ± 8.27 cm, respectively; 39.5% (75/190) patients were men. In the linear regression analyses, upper limb CMCTs showed a significant and weak positive relationship with height. Lower limb CMCTs demonstrated a significant and weak positive relationship with age and height. Peripheral motor conduction times were significantly and positively correlated with age and height. Root motor conduction times showed no significant relationship with age and height, except for abductor pollicis brevis-RMCT, which had a weak negative correlation with height. CONCLUSIONS: This study provides normal values of CMCTs, PMCTs, and RCMTs, which have potential clinical applications. When interpreting CMCTs, age and height should be considered.

Simulating the Effects of Partial Neural Conduction Delays in the Visual Evoked Potential.

Purpose: The purpose of this study was to understand the double peaks or broadening of P100 observed in some cases of optic neuritis by inducing conduction delays in healthy eyes through stimulus luminance manipulation in analogy to the perceptual Pulfrich effect. Methods: Checkerboard pattern reversal visual evoked potentials (VEPs) with check sizes of 0.8 degrees, 0.4 degrees, and 0.2 degrees were recorded in healthy participants using two experiment variants. Variant (1) involved binocular stimulation with inter-ocular luminance difference achieved by a 1.8 neutral density (ND) filter, along with monocular control conditions. Variant (2) included monocular stimulation with hemifields having a luminance difference (half of monitor with ND filter), along with single-hemifield control conditions. In both variants, VEP curves under mixed stimulation were compared to synthesized VEPs computed from offline summation of curves from the relevant control conditions, followed by assessing P100 characteristics. Results: Despite considerable variability between participants, the binocular variant demonstrated marked differences between VEPs from mixed recordings and synthesized curves, whereas in the hemifield variant, agreement was strong. The anticipated double peak or broadened deflection pattern was observed to varying extents in participants, often contingent on check size, with nominal peak time frequently failing to indicate partial conduction delays. Conclusions: The present findings corroborate the hypothesis that nominal peak time does not always reflect conduction delays if only a subset of fiber bundles is affected. Peak shape might provide additional diagnostic evidence of a partial conduction delay. Translational Relevance: Enhancing the understanding of VEP waveform changes associated with partial conduction delays could offer diagnostic insights for optic neuritis.

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.

BACKGROUND: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants.

BACKGROUND AND PURPOSE: There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. METHODS: We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. RESULTS: According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. CONCLUSIONS: The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.

Neuromuscular ultrasound in combination with nerve conduction studies helps identify inflammatory motor neuropathies from lower motor neuron syndromes.

BACKGROUND AND PURPOSE: Identifying patients with inflammatory motor neuropathies (IMNs) is warranted since effective treatments are available and the prognosis of these patients differs from that of amyotrophic lateral sclerosis patients. METHODS: Between January 2019 and May 2022, 102 consecutive treatment-naïve lower motor neuron syndrome (LMNS) patients were recruited; these patients were suspected of having multifocal motor neuropathy, pure motor chronic inflammatory demyelinating polyneuropathy or amyotrophic lateral sclerosis with initial lower motor neuron presentation. Neuromuscular ultrasound (US) and nerve conduction studies (NCSs) were conducted at baseline. Relevant diagnostic investigations were performed if clinically warranted. The proposed US evidence of IMN was as follows: (i) nerve enlargement at ≥1 of the predetermined sites or (ii) absence of high intensity fasciculations in predefined muscle groups. Final diagnoses were made by experienced physicians after a prolonged follow-up period (≥12 months). IMN patients were defined as LMNS patients who experienced convincing improvements in response to immunotherapies. IMN patients without electrodiagnostic demyelinating features were diagnosed with treatment-responsive LMNS (TR-LMNS). RESULTS: In total, 16 patients were classified as IMN, including nine chronic inflammatory demyelinating polyneuropathy/multifocal motor neuropathy patients and seven TR-LMNS patients. Six TR-LMNS patients were identified by neuromuscular US. The sensitivity and specificity of NCSs, nerve US and muscle US were 56.3% and 100%, 43.8% and 90.7% and 68.8% and 97.7%, respectively. When these three modalities were combined, the sensitivity and specificity were 93.8% and 88.4%, respectively. CONCLUSION: Neuromuscular US studies are supplementary modalities to NCSs, and the combined use of these techniques might improve the identification of IMNs in LMNS patients.

Upper and lower limb tremor in inflammatory neuropathies.

OBJECTIVE: The mechanisms underlying neuropathic tremor remain incompletely understood and a distinction has not been drawn between proximal and distal neuropathies. Lower limb tremor contributes to imbalance in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but this is unexplored in other neuropathies. We characterized upper and lower limb tremor in chronic immune sensory polyradiculopathy (CISP) and distal acquired demyelinating neuropathy with anti-MAG antibodies (DADS-MAG), contrasted to CIDP. METHODS: This was a cross-sectional study of 38 patients (CIDP [n = 25], CISP [n = 7], DADS-MAG [n = 6]). Clinical assessment, tremor study recordings, nerve conduction studies, and somatosensory evoked potentials were performed. Balance was measured by force platform. RESULTS: Upper limb tremor was prevalent (CIDP 66%, CISP 70%, DADS-MAG 100%). Peak frequencies followed a gradient along the upper limb, unchanged by weight-loading. Lower limb tremor was also present (CIDP 32%, CISP 29%, DADS-MAG 66%) and associated with imbalance. Nerve conduction parameters correlated with upper limb tremor in DADS-MAG and CISP, and imbalance in CISP. CONCLUSIONS: Upper limb tremor is mediated by peripheral and central mechanisms regardless of distal or proximal pathology. Lower limb tremor correlates with peripheral nerve function and contributes to imbalance. SIGNIFICANCE: This study contributes to the understanding of neuropathic tremor. Addressing lower limb tremor may be of therapeutic importance for neuropathy-associated imbalance.

Natural evolution of idiopathic carpal tunnel syndrome with respect to wrist and hand anthropometry: A prospective cohort study.

OBJECTIVE: A greater wrist depth/width ratio and wrist depth/palm length ratio are known risk factors for carpal tunnel syndrome. We hypothesized that these parameters might also predict progression in patients who were not surgically treated. METHODS: Seventy-eight patients with moderately severe idiopathic carpal tunnel syndrome of at least 10 months duration at recruitment, who declined surgical treatment and steroid injection, underwent repeated neurophysiological assessments after 3 years. A > 10% increase in median SNAP latency was taken as evidence of significant deterioration. RESULTS: Patients with a wrist ratio ≥ 0.72 showed a statistically significant deterioration in SNAP latency from 5.46 (SD 2.09) to 7.16 (SD 1.56) ms and in SNAP amplitude from 30.19 (SD 13.8) to 16.62 (SD 14.42) µv. For those with a wrist-to-palm ratio ≥ 0.42, SNAP latency deteriorated from 5.27 (SD 1.21) to 7.1 (SD 1.52) ms, and amplitude from 32.78 (SD 13.76) to 19.45 (SD 16.62) µv. Patients with lower ratios did not show significant changes in any neurophysiological parameter. The relative risk of significant deterioration in SNAP latency in patients with a wrist ratio ≥ 0.72 was 2.04 (95% CI 1.27-3.27). CONCLUSION: In untreated idiopathic carpal tunnel syndrome, patients with larger wrist and wrist-to-palm ratios are more likely to show neurophysiological progression.

Electrodiagnostic methods to verify Guillain-Barré syndrome subtypes in Istanbul: A prospective multicenter study.

BACKGROUND AND AIMS: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain-Barré syndrome (GBS) in Istanbul. METHODS: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti-ganglioside antibodies were analyzed. RESULTS: One hundred seventy-seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019-February 2020) and 108 during the pandemic (March 2020-March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti-ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies. INTERPRETATION: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis.

Anti-cancer therapeutic agents and carpal tunnel syndrome: Clinical, electrodiagnostic, and ultrasound findings in seven patients.

INTRODUCTION: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy and rarely develops after drug therapy. This study describes the clinical, electrodiagnostic (EDX), and ultrasound (US) findings in seven patients who experienced CTS due to anti-cancer therapeutic agents. METHODS: All patients underwent EDX testing, and four patients had an US study. RESULTS: CTS occurred in four patients with aromatase inhibitors, two with immune checkpoint inhibitors, and one with a selective estrogen receptor modulator. The mean duration between initiation of the anti-cancer therapeutic agents and symptom onset was 6 weeks (range: 2-12 weeks). Decreased digit sensation was noted in all patients; wasting and weakness of the abductor pollicis brevis (APB) was observed in three (42.8%) patients. The compound muscle action potentials (CMAP) of the APB and sensory nerve action potentials of the second or third digit could not be recorded in two (28.5%) and four (57.1%) patients, respectively. The needle EMG detected fibrillations and positive sharp waves in the APB in two patients. The motor unit potentials of the APB were decreased with large polyphasics in three (42.8%) patients. Of the four patients who underwent US testing, all had increased cross-sectional area of the median nerve at the carpal tunnel inlet, three (75%) had thenar muscle atrophy, and two (50%) had a loss of fascicular pattern. Three (42.8%) patients underwent a CTR. CONCLUSIONS: Physicians should be cognizant of the relationship between anti-cancer therapeutic agents and CTS. EDX studies and US play important roles in the diagnostic assessment of such patients.

Subjective and Objective Differences in Patients with Unilateral and Bilateral Carpal Tunnel Syndrome and the Role of Obesity in Syndrome Severity.

BACKGROUND: It remains unclear whether physiologic differences exist in musculoskeletal ultrasound nerve measurements when comparing bilateral and unilateral carpal tunnel syndrome (CTS) patients. Similarly, the influence of body mass index on CTS severity is not well characterized. METHODS: Unilateral and bilateral CTS patients were seen from October of 2014 to February of 2021. Obese and nonobese CTS patients were compared. Median nerve cross-sectional area (CSA), Boston Carpal Tunnel Syndrome Questionnaire (BCTSQ), and six-item Carpal Tunnel Symptom Score (CTS-6) measures were obtained. Nerve conduction studies recorded distal motor latency (DML) and distal sensory latency (DSL). Statistical analysis used Wilcoxon signed rank testing for paired continuous variables, Mann-Whitney U testing for nonpaired continuous variables, and chi-square testing for continuous variables, with a significance level of P < 0.05. RESULTS: A total of 109 (218 nerves) bilateral and 112 (112 nerves) unilateral CTS patients were reviewed. Bilateral patients had larger median nerve CSAs on their more symptomatic side, when defined by BCTSQ score ( P < 0.0001), CTS-6 score ( P < 0.0001), DML ( P < 0.0001), and DSL ( P < 0.01). Bilateral patients also had higher symptom severity scale ( P < 0.01) and DSL ( P < 0.001) outcomes compared with unilateral patients. Obese patients had higher median nerve CSA ( P < 0.01), prolonged DML, and prolonged DSL ( P < 0.0001) values despite similar CTS severity (BCTSQ and CTS-6). CONCLUSIONS: Ultrasound identifies the more symptomatic side in bilateral patients, which correlates with increasing severity (NCS and BCTSQ). Obesity increases median nerve CSA and prolongs nerve conduction studies without influencing CTS severity. This information can be used when considering which diagnostic test to order for CTS.

Provocative changes in nerve conductions: Fact or fiction?

At times electrodiagnostic medical consultants (EMCs) are asked to perform studies in both a neutral position, and then again after the patient is in a provocative position that exacerbates symptoms, to assess for measurable electrophysiologic changes. While this approach might seem initially appealing, particularly when standard studies are not effective at diagnosis, empiric studies in several conditions have been unimpressive. Studies in median neuropathy at the wrist, thoracic outlet syndrome, piriformis syndrome, and radial tunnel syndrome have failed to demonstrate reproducible changes in nerve conduction studies in positions that exacerbate symptoms. Furthermore, there is lack of a plausible pathophysiologic mechanism for producing both measurable and rapidly reversible electrophysiologic changes after just a few minutes, or less, of compression. Axon loss and demyelination would not be rapidly reversible, and positional changes of 2 min or less (the durations generally studied) would be insufficient to produce measurable nerve ischemia. Last, we have gained a greater appreciation for how much nerves move within limbs with changes in joint position; this movement can lead to misleading changes in nerve conduction studies. It is thus appropriate to conclude that testing nerve conduction in provocative or symptomatic positions adds no value to electrodiagnostic testing.

Sympathetic skin response for early detection of type 2 diabetic peripheral neuropathy and nephropathy.

BACKGROUND: Diabetic peripheral neuropathy (DPN) and diabetic nephropathy (DN) are common complications of type 2 diabetes mellitus (T2DM). Although nerve conduction studies (NCS) and sympathetic skin response (SSR) can detect DPN, the more sensitive method for early diagnosis remains unclear. Furthermore, whether DPN can be used as a predictor for diabetic nephropathy needs clarification. METHODS: We evaluated nerve conduction studies, sympathetic skin response, and the diabetic nephropathy indicator microalbuminuria (MAU) in 192 patients with type 2 diabetes mellitus and 50 healthy controls. RESULTS: Patients with type 2 diabetes mellitus showed a lower sensory nerve conduction velocity (SCV), sensory active nerve potential (SNAP), motor nerve conduction velocity (MCV), and compound motor action potential (CMAP) than the controls on NCS. Abnormal rates for nerve conduction studies and sympathetic skin response were 75.0% and 83.3%, respectively, in patients with type 2 diabetes mellitus. Interestingly, 54.2% of patients with normal nerve conduction studies had an abnormal sympathetic skin response. Moreover, we found a positive correlation between sympathetic skin response and microalbuminuria for the first time. The abnormal rate of microalbuminuria was 53.8%, lower than that of abnormal nerve conduction studies or sympathetic skin response patients. CONCLUSION: Sympathetic skin response is a more sensitive method than nerve conduction studies for the early diagnosis of diabetic peripheral neuropathy. Abnormal sympathetic skin response might serve as an indicator for early diabetic nephropathy. Additionally, diabetic peripheral neuropathy may occur earlier than diabetic nephropathy in the development of type 2 diabetes mellitus.