Diffusion tensor imaging in anisotropic tissues: application of reduced gradient vector schemes in peripheral nerves.

BACKGROUND: In contrast to the brain, fibers within peripheral nerves have distinct monodirectional structure questioning the necessity of complex multidirectional gradient vector schemes for DTI. This proof-of-concept study investigated the diagnostic utility of reduced gradient vector schemes in peripheral nerve DTI. METHODS: Three-Tesla magnetic resonance neurography of the tibial nerve using 20-vector DTI (DTI20) was performed in 10 healthy volunteers, 12 patients with type 2 diabetes, and 12 age-matched healthy controls. From the full DTI20 dataset, three reduced datasets including only two or three vectors along the x- and/or y- and z-axes were built to calculate major parameters. The influence of nerve angulation and intraneural connective tissue was assessed. The area under the receiver operating characteristics curve (ROC-AUC) was used for analysis. RESULTS: Simplified datasets achieved excellent diagnostic accuracy equal to DTI20 (ROC-AUC 0.847-0.868, p ≤ 0.005), but compared to DTI20, the reduced models yielded mostly lower absolute values of DTI scalars: median fractional anisotropy (FA) ≤ 0.12; apparent diffusion coefficient (ADC) ≤ 0.25; axial diffusivity ≤ 0.96, radial diffusivity ≤ 0.07). The precision of FA and ADC with the three-vector model was closest to DTI20. Intraneural connective tissue was negatively correlated with FA and ADC (r ≥ -0.49, p < 0.001). Small deviations of nerve angulation had little effect on FA accuracy. CONCLUSIONS: In peripheral nerves, bulk tissue DTI metrics can be approximated with only three predefined gradient vectors along the scanner's main axes, yielding similar diagnostic accuracy as a 20-vector DTI, resulting in substantial scan time reduction. RELEVANCE STATEMENT: DTI bulk tissue parameters of peripheral nerves can be calculated with only three predefined gradient vectors at similar diagnostic performance as a standard DTI but providing a substantial scan time reduction. KEY POINTS: • In peripheral nerves, DTI parameters can be approximated using only three gradient vectors. • The simplified model achieves a similar diagnostic performance as a standard DTI. • The simplified model allows for a significant acceleration of image acquisition. • This can help to introduce multi-b-value DTI techniques into clinical practice.

Ultrahigh-frequency ultrasound of fascicles in the common fibular, superficial fibular, and sural nerves.

INTRODUCTION/AIMS: While ultrasound assessment of cross-sectional area and echogenicity has gained popularity as a biomarker for various neuropathies, there is a scarcity of data regarding fascicle count and density in neuropathies or even healthy controls. The aim of this study was to determine whether fascicles within select lower limb nerves (common fibular, superficial fibular, and sural nerves) can be counted in healthy individuals using ultrahigh-frequency ultrasound (UHFUS). METHODS: Twenty healthy volunteers underwent sonographic examination of the common fibular, superficial fibular, and sural nerves on each lower limb using UHFUS with a 48 MHz linear transducer. Fascicle counts and density in each examined nerve were determined by a single rater. RESULTS: The mean fascicle number for each of the measured nerves included the following: common fibular nerve 9.85 (SD 2.29), superficial fibular nerve 5.35 (SD 1.59), and sural nerve 6.73 (SD 1.91). Multivariate linear regression analysis revealed a significant association between cross-sectional area and fascicle count for all three nerves. In addition, there was a significant association seen in the common fibular nerve between fascicle density and height, weight, and body mass index. Age and sex did not predict fascicle count or density (all p > .13). DISCUSSION: UHFUS enabled the identification and counting of fascicles and fascicle density in the common fibular, superficial fibular, and sural nerves. Knowledge about normal values and normal peripheral nerve architecture is needed in order to further understand and identify pathological changes that may occur within each nerve in different disease states.

Expanding the Clinical Spectrum of DRP2-Associated Charcot-Marie-Tooth Disease.

BACKGROUND AND OBJECTIVES: Germline truncating variants in the DRP2 gene (encoding dystrophin-related protein 2) cause the disruption of the periaxin-DRP2-dystroglycan complex and have been linked to Charcot-Marie-Tooth disease. However, the causality and the underlying phenotype of the genetic alterations are not clearly defined. METHODS: This cross-sectional retrospective observational study includes 9 patients with Charcot-Marie-Tooth disease (CMT) with DRP2 germline variants evaluated at 6 centers throughout Spain. RESULTS: We identified 7 Spanish families with 4 different DRP2 likely pathogenic germline variants. In agreement with an X-linked inheritance, men harboring hemizygous DRP2 variants presented with an intermediate form of CMT, whereas heterozygous women were asymptomatic. Symptom onset was variable (36.6 ± 16 years), with lower limb weakness and multimodal sensory loss producing a mild-to-moderate functional impairment. Nerve echography revealed an increase in the cross-sectional area of nerve roots and proximal nerves. Lower limb muscle magnetic resonance imaging confirmed the presence of a length-dependent fatty infiltration. Immunostaining in intradermal nerve fibers demonstrated the absence of DRP2 and electron microscopy revealed abnormal myelin thickness that was also detectable in the sural nerve sections. DISCUSSION: Our findings support the causality of DRP2 pathogenic germline variants in CMT and further define the phenotype as a late-onset sensory and motor length-dependent neuropathy, with intermediate velocities and thickening of proximal nerve segments.

Imaging Biomarkers of Peripheral Nerves: Focus on Magnetic Resonance Neurography and Ultrasonography.

Peripheral neuropathy is a prevalent and debilitating condition affecting millions of individuals globally. Magnetic resonance neurography (MRN) and ultrasonography (US) are noninvasive methods offering comprehensive visualization of peripheral nerves, using anatomical and functional imaging biomarkers to ensure accurate evaluation. For optimized MRN, superior and high-resolution two-dimensional and three-dimensional imaging protocols are essential. The anatomical MRN and US imaging markers include quantitative measures of nerve and fascicular size and signal, and qualitative markers of course and morphology. Among them, quantitative markers of T2-signal intensity ratio are sensitive to nerve edema-like signal changes, and the T1-mapping technique reveals nerve and muscle tissue fatty and fibrous compositional alterations.The functional markers are derived from physiologic properties of nerves, such as diffusion characteristics or blood flow. They include apparent diffusion coefficient from diffusion-weighted imaging and fractional anisotropy and tractography from diffusion tensor imaging to delve into peripheral nerve microstructure and integrity. Peripheral nerve perfusion using dynamic contrast-enhanced magnetic resonance imaging estimates perfusion parameters, offering insights into nerve health and neuropathies involving edema, inflammation, demyelination, and microvascular alterations in conditions like type 2 diabetes, linking nerve conduction pathophysiology to vascular permeability alterations.Imaging biomarkers thus play a pivotal role in the diagnosis, prognosis, and monitoring of nerve pathologies, thereby ensuring comprehensive assessment and elevating patient care. These biomarkers provide valuable insights into nerve structure, function, and pathophysiology, contributing to the accurate diagnosis and management planning for peripheral neuropathy.

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.

Nerve block, nerve damage, and fluid injection pressure: overturning the myth.

Histological and micro-ultrasound evidence rebuffs deep-rooted views on the nature of nerve block, nerve damage, and injection pressure monitoring. We propose that the ideal position of the needle tip for nerve block is between the innermost circumneural fascial layer and outer epineurium, with local anaesthetic passing circumferentially through adipose tissue. Thin, circumferential, subepineural expansion that is invisible to the naked eye was identified using micro-ultrasound, and could account for variability of outcomes in clinical practice. Pressure monitoring cannot differentiate between intrafascicular and extrafascicular injection. High injection pressure only indicates intraneural extrafascicular spread, not intrafascicular spread, because it is not possible to inject into the stiff endoneurium in most human nerves.

The impact of biological sex in peripheral nerve blockade: A prospective pharmacodynamic, pharmacokinetic and morphometric study in volunteers.

STUDY OBJECTIVE: The impact of biological sex in peripheral regional anaesthesia is largely unknown. We therefore designed a prospective study in volunteers to investigate the impact of biological sex on pharmacodynamic, pharmacokinetic and morphometric characteristics for peripheral nerve blockade. METHODS: The initial study plan was powered to include 90 volunteers to find a difference of 35 min in duration of sensory block (primary outcome variable) with 80% power and alpha error at 5%. After discussions in ethical review, a pilot study of 2 x 12 volunteers from each sex were studied. Female and male volunteers received ultrasound guided nerve blockade with 3.0 mL ropivacaine 7.5 mg mL-1. Sensory duration of blockade, as the primary outcome, was evaluated by pinprick testing. Secondary outcomes were sensory onset time of blockade, pharmacokinetic characteristics and the visibility of ulnar nerves using ultrasound. Analyses included Mann-Whitney U-statistics with P<0.05 (two-sided) as significant. RESULTS: After 24 participants, the median (IQR) duration of sensory blockade was 450 (420; 503) min in women and 480 (450; 510) min in men (P = 0.49). Sensory onset time of blockade, and ultrasound visibility of nerves were also similar between the study groups. The total drug exposure across time (AUC0-infinity) was significantly higher in women (P = 0.017). After a the planned power re-analysis after these 24 study paticipants, which suggested that > 400 subjects would be required with 80% power and alpha error of 5% to find significance for the primary outcome parameter for marginal differences, we terminated the study at this point. CONCLUSIONS: We did not detect significant differences between female and male study participants in terms of pharmacodynamic and morphometric characteristics after ultrasound guided ulnar nerve blocks. Women did show significantly greater pharmacokinetic ropivacaine exposures. The results of this study indicate that peripheral regional block pharmacodynamic characteristics are independent of the biological sex, whereas pharmacokinetic parameters are sex-dependent.

The diagnostic value of ultrasonography in evaluation of the intraneural vascular anomalies of peripheral nerves.

BACKGROUND: The imaging diagnosis of intraneural vascular anomalies of peripheral nerves mostly depended on magnetic resonance imaging (MRI), whereas high-frequency ultrasonography evaluation of intraneural vascular anomalies has been seldom done. PURPOSE: To evaluate the diagnostic value of ultrasonography in the diagnosis of intraneural vascular anomalies of peripheral nerves. MATERIAL AND METHODS: A total of 69 consecutive patients seen at Shandong Provincial Hospital Affiliated to Shandong First Medical University between February 2013 and June 2022, each with a clinical suspicion of intraneural vascular anomaly, were included. The ultrasonographic images of intraneural vascular anomalies of peripheral nerves were analyzed and the ultrasonographic features were summarized. These data were compared with MRI, which served as the gold standard for the diagnosis of intraneural vascular anomalies. The kappa statistic was adopted to determine the level of agreement. The sensitivity, specificity, positive and negative predictive values, accuracy, and Youden index of high-frequency ultrasonography as a diagnostic tool were assessed. RESULTS: Ultrasonography findings were positive in 20 of 69 patients with a clinical suspicion of intraneural vascular anomaly. The diagnosis was confirmed by MRI in 21 patients. There was one false-positive result and two false-negative results by ultrasonography. The κ value was 0.896. The sensitivity, specificity, positive and negative predictive values, accuracy, and Youden index of ultrasonography were 90.5%, 97.9%, 95%, 95.9%, 95.7%, and 0.884, respectively. CONCLUSION: Ultrasonography could be an accurate, reliable, and convenient imaging tool for the diagnosis of intraneural vascular anomalies of peripheral nerves.

Multiparametric Quantitative MRI of Peripheral Nerves in the Leg: A Reliability Study.

BACKGROUND: Patients with polyneuropathies typically have demyelination and/or axonal degeneration in peripheral nerves. Currently, there is a lack of imaging biomarkers to track the changes in these pathologies. PURPOSE: To develop and evaluate the reliability of a multiparametric quantitative magnetic resonance imaging (qMRI) method of peripheral nerves in the leg. STUDY TYPE: Prospective. SUBJECTS: Seventeen healthy volunteers (36.2 ± 13.8 years old, 9 males) with 10 of them scanned twice for test-retest. FIELD STRENGTH/SEQUENCE: 3 T, three-dimensional gradient echo and diffusion tensor imaging. ASSESSMENT: A qMRI protocol and processing pipeline was established for quantifying the following nerve parameters that are sensitive to myelin and axonal pathologies: magnetization transfer (MT) ratio (MTR), MT saturation index (MTsat), T2 *, T1 , proton density (PD), fractional anisotropy (FA), and mean/axial/radial diffusivities (MD, AD, and RD). The qMRI protocol also measures the volume of nerve fascicles (fVOL) and the fat fraction (FF) of muscles. STATISTICAL TESTS: The intersession reproducibility and inter-rater reliability of each qMRI parameter were assessed by Bland-Altman analysis and intraclass correlation coefficient (ICC). Pairwise Pearson correlation analyses were performed to investigate the intrinsic association between qMRI parameters. Distal-to-proximal variations were evaluated by paired t-tests with Bonferroni-Holm multiple comparison corrections. P < 0.05 was considered statistically significant. RESULTS: The MTR, MTsat, T2 *, T1 , PD, FA, AD, and fVOL of the sciatic and tibial nerves, and the FF of leg muscles, had an overall good-to-excellent test-retest agreement (ICC varying from 0.78 to 0.99). All the qMRI parameters had good-to-excellent inter-rater reliability (ICC > 0.80). The data demonstrated a pattern of distal-to-proximal changes of an increased nerve MTsat and FA, and a decreased nerve T1 , PD, MD, and RD, as well as a significantly increased muscle FF. DATA CONCLUSION: The proposed multiparametric qMRI method of the peripheral nerves is highly reproducible and provided healthy control data which will be used in developing monitoring biomarkers in patients with polyneuropathies. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 2.

Neurological injury following peripheral nerve blocks: a narrative review of estimates of risks and the influence of ultrasound guidance.

BACKGROUND: Peripheral nerve injury or post-block neurological dysfunction (PBND) are uncommon but a recognized complications of peripheral nerve blocks (PNB). A broad range of its incidence is noted in the literature and hence a critical appraisal of its occurrence is needed. OBJECTIVE: In this review, we wanted to know the pooled estimates of PBND and further, determine its pooled estimates following various PNB over time. Additionally, we also sought to estimate the incidence of PBND with or without US guidance. EVIDENCE REVIEW: A literature search was conducted in six databases. For the purposes of the review, we defined PBND as any new-onset sensorimotor disturbances in the distribution of the performed PNB either attributable to the PNB (when reported) or reported in the context of the PNB (when association with a PNB was not mentioned). Both prospective and retrospective studies which provided incidence of PBND at timepoints of interest (>48 hours to <2 weeks; >2 weeks to 6 weeks, 7 weeks to 5 months, 6 months to 1 year and >1 year durations) were included for review. Incidence data were used to provide pooled estimates (with 95% CI) of PBND at these time periods. Similar estimates were obtained to know the incidence of PBND with or without the use of US guidance. Additionally, PBND associated with individual PNB were obtained in a similar fashion with upper and lower limb PNB classified based on the anatomical location of needle insertion. FINDINGS: The overall incidence of PBND decreased with time, with the incidence being approximately 1% at <2 weeks' time (Incidence per thousand (95% CI)= 9 (8; to 11)) to approximately 3/10 000 at 1 year (Incidence per thousand (95% CI)= 0. 3 (0.1; to 0.5)). Incidence of PBND differed for individual PNB with the highest incidence noted for interscalene block. CONCLUSIONS: Our review adds information to existing literature that the neurological complications are rarer but seem to display a higher incidence for some blocks more than others. Use of US guidance may be associated with a lower incidence of PBND especially in those PNBs reporting a higher pooled estimates. Future studies need to standardize the reporting of PBND at various timepoints and its association to PNB.

The role of preoperative ultrasound in the management of peripheral nerve injuries.

BACKGROUND: Peripheral nerve injury refers to any damage or trauma to the nerves located outside the central nervous system. Ultrasonography is a reliable, cheap, and minimally invasive method in clinical practice to give physicians useful information about nerve injury. OBJECTIVES: to assess the power of ultrasound in determining the presence, localization, and extent of neural damage in patients with clinical evidence of peripheral nerve lesions before surgery. METHODS: This cross-sectional study was conducted on 78 patients (56 females and 22 males, aged from 9 to 52 years) who had different pathologies including entrapment, tumoral, post-traumatic, and post-surgical nerve injuries at the Neurosurgery and Physical Medicine, Rheumatology, and Rehabilitation Departments, Tanta University Hospitals. All studied patients had preoperative evaluation; neurological examination, electrodiagnostic studies, and sonographic examinations with linear array transducers (frequencies ranging from 7.5 to 16 MHz). RESULTS: The most common pathological condition was entrapment neuropathy (39 patients) (50%). Ultrasound complemented the electrodiagnostic studies by determining the site of entrapment manifested by increased mean maximum cross-sectional area of the nerve proximal to the site of entrapment and nerve hypoechogenicity. In post-traumatic and iatrogenic neuropathies (35 patients) (44.9%), the ultrasound finding revealed neuroma in continuity in nine cases (11.5%), complete neurotmesis with stump neuroma in eighteen patients (23.1%), and eight cases (10.3%) showed perineural adhesion. In all cases, the nerve was hypoechoic at the site of injury. The presence of hyperechoic fibrous tissue could indicate perineural adhesion and the necessity for neurolysis. This study also included three (3.8%) cases had schwannoma, and one case (1.3%) had neurofibroma. Ultrasound was used to confirm the diagnosis by determining the tumor's size and vascular supply. CONCLUSIONS: Ultrasonography is a diagnostic and surgical planning tool that is becoming more and more useful for the management of peripheral nerve injuries. Its high resolution and real-time capability provide safe and cost-effective scans that aid in determining the extent of injuries. For patients with peripheral nerve injuries, ultrasound is advised to be added to the routine clinical and neurophysiological evaluation. It is also advised to use ultrasound as a first-line imaging modality for tumors thought to be of nerve origin.

Evaluation of Serum Neurofilament Light Chain and Nerve Ultrasound in Diabetic Neuropathy.

OBJECTIVE: To assess the role of serum neurofilament light chain (NfL) levels in individuals with diabetic polyneuropathy (DPN) compared with controls, as well as to highlight the different sonographic changes in DPN and determine if NfL correlates with sonographic, clinical, and functional parameters. METHODS: Diabetic individuals with signs or symptoms consistent with peripheral nerve involvement were recruited. They were evaluated by examination, functional neuropathy severity scores, laboratory assessments (including NfL), nerve conduction studies (NCS), and ultrasound. Ultrasound was performed of the bilateral median, ulnar, tibial, fibular, sural, and vagus nerves, and cervical roots 5 and 6. Results were compared with age, sex, and body mass index matched healthy controls. RESULTS: A total of 320 nerves from 20 patients and 480 nerves from 30 controls were evaluated. NfL was significantly elevated in those with diabetes with a mean and standard deviation of 6.95 ± 2.95 pg/mL in the diabetic group and 2.83 ± 0.77 pg/mL in controls (P < .001). Nerve cross-sectional area and serum NfL levels correlated significantly with clinical and functional parameters and with each other (P < .05). CONCLUSION: Individuals with DPN have significantly higher NfL levels than healthy controls, and NfL levels correlate with ultrasonographic parameters. These findings may be useful for the diagnosis, prognosis, and disease monitoring of those with DPN, though further exploration is needed.

Diffusion Tensor Imaging of Peripheral Nerves: Current Status and New Developments.

Diffusion tensor imaging (DTI) is an emerging technique for peripheral nerve imaging that can provide information about the microstructural organization and connectivity of these nerves and complement the information gained from anatomical magnetic resonance imaging (MRI) sequences. With DTI it is possible to reconstruct nerve pathways and visualize the three-dimensional trajectory of nerve fibers, as in nerve tractography. More importantly, DTI allows for quantitative evaluation of peripheral nerves by the calculation of several important parameters that offer insight into the functional status of a nerve. Thus DTI has a high potential to add value to the work-up of peripheral nerve pathologies, although it is more technically demanding. Peripheral nerves pose specific challenges to DTI due to their small diameter and DTI's spatial resolution, contrast, location, and inherent field inhomogeneities when imaging certain anatomical locations. Numerous efforts are underway to resolve these technical challenges and thus enable wider acceptance of DTI in peripheral nerve MRI.

Fluorescence imaging of peripheral nerve function and structure.

Peripheral nerve injuries are common and can cause catastrophic consequences. Although peripheral nerves have notable regenerative capacity, full functional recovery is often challenging due to a number of factors, including age, the type of injury, and delayed healing, resulting in chronic disorders that cause lifelong miseries and significant financial burdens. Fluorescence imaging, among the various techniques, may be the key to overcome these restrictions and improve the prognosis because of its feasibility and dynamic real-time imaging. Intraoperative dynamic fluorescence imaging allows the visualization of the morphological structure of the nerve so that surgeons can reduce the incidence of medically induced injury. Axoplasmic transport-based neuroimaging allows the visualization of the internal transport function of the nerve, facilitating early, objective, and accurate assessment of the degree of regenerative repair, allowing early intervention in patients with poor recovery, thereby improving prognosis. This review briefly discusses peripheral nerve fluorescent dyes that have been reported or could potentially be employed, with a focus on their role in visualizing the nerve's function and anatomy.

Exploration of Neurofilament Light Chain and Nerve Ultrasound in Leprotic Neuropathy.

OBJECTIVES: To explore neurofilament light chain (NfL) levels in leprotic neuropathy compared to controls, and to determine if the changes correlate with ultrasonographic nerve findings. METHODS: Individuals with leprosy with signs or symptoms suggestive of peripheral nerve involvement were recruited. They were evaluated by clinical examination, functional scores, laboratory assessments (including NfL), nerve conduction studies (NCS), and ultrasound. Ultrasound was conducted in bilateral median, ulnar, tibial, fibular, sural, and vagus nerves as well as cervical roots 5 and 6. Results were compared to age, sex, and body mass index matched healthy controls. RESULTS: A total of 320 nerves from 20 patients and 480 nerves from 30 controls were evaluated. NfL was significantly elevated in those with leprosy with a mean and standard deviation of 7.50 + 2.83 compared with 3.42 + 1.18 in controls (P < .001). Ultrasound showed focal enlargement of the nerves, particularly at entrapment sites. Additionally, there were noticeable changes in neural Doppler signal, echogenicity, and epineural thickness among the measured nerve sites. NfL levels in those with leprosy correlated closely with nerve cross-sectional area at all sites (P < .05). Functional and clinical assessment scores correlated with NfL and sonographic cross-sectional area as well (P ≤ .05). CONCLUSIONS: NfL is elevated in leprotic neuropathy. Ultrasound showed specific morphological changes in individuals with leprosy, and nerve enlargement correlated with NfL levels. Thus, both modalities may be useful for the diagnosis, prognosis, and disease monitoring in those with leprotic neuropathy, and further investigations are warranted.

Enlarged cross-sectional area in peripheral nerves in Swedish patients with hereditary V30M transthyretin amyloidosis.

INTRODUCTION: In hereditary transthyretin amyloidosis (ATTRv), two different fibrillar forms causing the amyloid deposition, have been identified, displaying substantially cardiac or neuropathic symptoms. Neuropathic symptoms are more frequent in early-onset patients, whereas late-onset patients, besides cardiac symptoms, seem to develop carpal tunnel syndrome, more often. With ultrasonography (US) of peripheral nerves, it is possible to distinguish structural changes, and enlarged cross-sectional area (CSA). The main purpose of this study was, for the first time, to elucidate US of peripheral nerves in Swedish ATTRv patients at an early stage of the disease, and to evaluate possible early enlarged CSA. MATERIAL AND METHODS: This prospective study included first visit data of 13 patients, aged 30-88 years, of which 11 with late-onset age. All had a positive V30M mutation. Eight men and six women (aged 28-74 years) served as controls. RESULTS: Significantly enlarged CSA was seen in ATTRv patients for the tibial nerve at the ankle (p = .001), the sural nerve (p < .001), the peroneal nerve at the popliteal fossa (p = .003), and the ulnar nerve at the middle upper arm (p = .007). CONCLUSION: US of peripheral nerves could be a valuable tool in disease evaluation and could facilitate monitoring of disease progression.

Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies.

Background and Objectives: Ultrasound (US) is a non-invasive tool for the in vivo detection of peripheral nerve alterations. Materials and Methods: In this study, we applied nerve US to assist the discrimination between the spectrum of amyotrophic lateral sclerosis (ALS, n = 11), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n = 5), and genetically confirmed Charcot-Marie-Tooth disease (CMT, n = 5). All participants and n = 15 controls without neurological diseases underwent high-resolution US of the bilateral tibial nerve. The nerve cross-sectional area (CSA) and nerve microvascular blood flow were compared between the groups and related to cerebrospinal fluid (CSF) measures, clinical symptoms, and nerve conduction studies. The analyses are part of a larger multimodal study on the comparison between US and 7 Tesla (7T) magnetic resonance neurography (MRN). Results: The patients and controls were matched with respect to their demographical data. CMT had the longest disease duration, followed by CIDP and ALS. CSA was related to age, weight, and disease duration. CSA was larger in CMT and CIDP compared to ALS and controls. The blood flow was greatest in CIDP, and higher than in CMT, ALS, and controls. In ALS, greater CSA was correlated with greater CSF total protein and higher albumin quotient. The US measures did not correlate with clinical scores or nerve conduction studies in any of the subgroups. Conclusion: Our results point towards the feasibility of CSA and blood flow to discriminate between ALS, CIDP, and CMT, even in groups of small sample size. In ALS, larger CSA could indicate an inflammatory disease subtype characterized by reduced blood-nerve barrier integrity. Our upcoming analysis will focus on the additive value of 7T MRN in combination with US to disentangle the spectrum between more inflammatory or more degenerative disease variants among the disease groups.

Peripheral Neuropathy in Diabetes: What Can MRI Do?

Diabetes peripheral neuropathy (DPN) is commonly asymptomatic in the early stage. However, once symptoms and obvious defects appear, recovery is not possible. Diagnosis of neuropathy is based on physical examinations, questionnaires, nerve conduction studies, skin biopsies, and so on. However, the diagnosis of DPN is still challenging, and early diagnosis and immediate intervention are very important for prevention of the development and progression of diabetic neuropathy. The advantages of MRI in the diagnosis of DPN are obvious: the peripheral nerve imaging is clear, the lesions can be found intuitively, and the quantitative evaluation of the lesions is the basis for the diagnosis, classification, and follow-up of DPN. With the development of magnetic resonance technology, more and more studies have been conducted on detection of DPN. This article reviews the research field of MRI in DPN.