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1.
Muscle Nerve ; 70(1): 101-110, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38698725

ABSTRACT

INTRODUCTION/AIMS: Whole-body magnetic resonance neurography (MRN) is an imaging modality that shows peripheral nerve signal change in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We aimed to explore the diagnostic potential of whole-body MRN and its potential as a monitoring tool after immunotherapy in treatment-naïve CIDP patients. METHODS: Whole-body MRN using coronal 3-dimensional short tau inversion recovery (STIR) sampling perfection with application-optimized contrasts by using different flip angle evolution (SPACE) techniques was performed in patients being investigated for CIDP and in healthy controls. Baseline clinical neuropathy scales and electrophysiologic parameters were collected, and MRN findings were compared before and after CIDP treatment. RESULTS: We found highly concordant symmetrical thickening and increased T2 signal intensities in the brachial/lumbosacral plexus, femoral, or sciatic nerves in five of the eight patients with a final diagnosis of CIDP and none of the healthy controls. There were no treatment-related imaging changes in five patients with CIDP who completed a follow-up study. Diffuse, symmetrical thickening, and increased T2 signal in root, plexus, and peripheral nerves were found in two patients ultimately excluded due to a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome in addition to signal changes in the muscles, bony lesions, organomegaly, and lymphadenopathy. DISCUSSION: Whole-body MRN imaging shows promise in detecting abnormalities in proximal nerve segments in patients with CIDP. Future studies evaluating the role of MRN in assessing treatment response should consider follow-up scans after treatment durations of more than 4 months.


Subject(s)
Magnetic Resonance Imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Whole Body Imaging , Humans , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnostic imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/physiopathology , Male , Female , Middle Aged , Magnetic Resonance Imaging/methods , Aged , Whole Body Imaging/methods , Adult , Peripheral Nerves/diagnostic imaging , Neural Conduction/physiology
2.
World J Gastroenterol ; 30(16): 2233-2248, 2024 Apr 28.
Article in English | MEDLINE | ID: mdl-38690027

ABSTRACT

BACKGROUND: Perineural invasion (PNI) has been used as an important pathological indicator and independent prognostic factor for patients with rectal cancer (RC). Preoperative prediction of PNI status is helpful for individualized treatment of RC. Recently, several radiomics studies have been used to predict the PNI status in RC, demonstrating a good predictive effect, but the results lacked generalizability. The preoperative prediction of PNI status is still challenging and needs further study. AIM: To establish and validate an optimal radiomics model for predicting PNI status preoperatively in RC patients. METHODS: This retrospective study enrolled 244 postoperative patients with pathologically confirmed RC from two independent centers. The patients underwent pre-operative high-resolution magnetic resonance imaging (MRI) between May 2019 and August 2022. Quantitative radiomics features were extracted and selected from oblique axial T2-weighted imaging (T2WI) and contrast-enhanced T1WI (T1CE) sequences. The radiomics signatures were constructed using logistic regression analysis and the predictive potential of various sequences was compared (T2WI, T1CE and T2WI + T1CE fusion sequences). A clinical-radiomics (CR) model was established by combining the radiomics features and clinical risk factors. The internal and external validation groups were used to validate the proposed models. The area under the receiver operating characteristic curve (AUC), DeLong test, net reclassification improvement (NRI), integrated discrimination improvement (IDI), calibration curve, and decision curve analysis (DCA) were used to evaluate the model performance. RESULTS: Among the radiomics models, the T2WI + T1CE fusion sequences model showed the best predictive performance, in the training and internal validation groups, the AUCs of the fusion sequence model were 0.839 [95% confidence interval (CI): 0.757-0.921] and 0.787 (95%CI: 0.650-0.923), which were higher than those of the T2WI and T1CE sequence models. The CR model constructed by combining clinical risk factors had the best predictive performance. In the training and internal and external validation groups, the AUCs of the CR model were 0.889 (95%CI: 0.824-0.954), 0.889 (95%CI: 0.803-0.976) and 0.894 (95%CI: 0.814-0.974). Delong test, NRI, and IDI showed that the CR model had significant differences from other models (P < 0.05). Calibration curves demonstrated good agreement, and DCA revealed significant benefits of the CR model. CONCLUSION: The CR model based on preoperative MRI radiomics features and clinical risk factors can preoperatively predict the PNI status of RC noninvasively, which facilitates individualized treatment of RC patients.


Subject(s)
Magnetic Resonance Imaging , Neoplasm Invasiveness , Rectal Neoplasms , Humans , Rectal Neoplasms/diagnostic imaging , Rectal Neoplasms/pathology , Rectal Neoplasms/surgery , Magnetic Resonance Imaging/methods , Male , Retrospective Studies , Female , Middle Aged , Aged , Predictive Value of Tests , Prognosis , Preoperative Period , Peripheral Nerves/diagnostic imaging , Peripheral Nerves/pathology , Adult , Risk Factors , Rectum/diagnostic imaging , Rectum/pathology , Rectum/surgery , ROC Curve , Radiomics
3.
J Neurol Sci ; 460: 122995, 2024 May 15.
Article in English | MEDLINE | ID: mdl-38583391

ABSTRACT

BACKGROUND AND PURPOSE: We previously reported that nerve enlargement assessment by nerve ultrasonography of the intermediate upper limb is applicable for distinguishing demyelinating Charcot-Marie-Tooth disease (CMT) from chronic inflammatory demyelinating polyneuropathy (CIDP). However, differences in the severity and distribution patterns of lower extremity nerve enlargement have not been established for either disease. Therefore, we examined the utility of lower extremity nerve ultrasonography for differentiating between CMT and CIDP. METHODS: Twelve patients with demyelinating CMT and 17 patients with CIDP were evaluated. The median, ulnar, tibial, and fibular nerves were evaluated in three regions: the distal upper extremity, intermediate upper extremity, and lower extremity. Of the 14 selected screening sites, the number of sites that exhibited nerve enlargement (enlargement site number, ESN) in each region was determined. RESULTS: The screening ESNs in the intermediate region and lower extremities were greater in patients with demyelinating CMT than in patients with CIDP and greater than the ESN in the distal region (p = 0.010, p = 0.001, and p = 0.101, respectively). The ESNs in the intermediate region and lower extremities significantly differed among patients with typical CIDP, CIDP variants, and demyelinating CMT (p = 0.084 and p < 0.001). Among the 14 selected screening sites, the combined upper and lower extremity ESNs exhibited the highest AUC (0.92; p < 0.001). CONCLUSIONS: Combining the upper and lower extremities for ultrasonographic nerve measurement more accurately distinguishes CIDP from demyelinating CMT.


Subject(s)
Charcot-Marie-Tooth Disease , Lower Extremity , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Ultrasonography , Humans , Charcot-Marie-Tooth Disease/diagnostic imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnostic imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Male , Female , Middle Aged , Ultrasonography/methods , Adult , Aged , Lower Extremity/diagnostic imaging , Lower Extremity/innervation , Diagnosis, Differential , Peripheral Nerves/diagnostic imaging , Peripheral Nerves/pathology , Young Adult
4.
Clin Nucl Med ; 49(6): e292-e294, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38557810

ABSTRACT

ABSTRACT: We present a case of sarcoidosis with a rare presentation of involvement of peripheral nerves of the lower limbs and subcutaneous nodules detected on 18 F-FDG PET/CT. The patient also had involvement of the spinal nerves and dura, histologically proven to be sarcoidosis. There were other manifestations of systemic sarcoidosis like metabolically active cervical and mediastinal lymphadenopathy. This case highlights the role of 18 F-FDG PET/CT in evaluating the uncommon sites of sarcoid involvement. Although many cases of sarcoid involvement of central nervous system have been reported, peripheral nerves involvement in the extremities was not found on a literature search.


Subject(s)
Central Nervous System Diseases , Fluorodeoxyglucose F18 , Peripheral Nerves , Positron Emission Tomography Computed Tomography , Sarcoidosis , Humans , Sarcoidosis/diagnostic imaging , Central Nervous System Diseases/diagnostic imaging , Peripheral Nerves/diagnostic imaging , Peripheral Nerves/pathology , Extremities/diagnostic imaging , Tomography, X-Ray Computed , Middle Aged , Female , Male , Positron-Emission Tomography , Multimodal Imaging
5.
Eur Radiol Exp ; 8(1): 37, 2024 Apr 02.
Article in English | MEDLINE | ID: mdl-38561526

ABSTRACT

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.


Subject(s)
Diabetes Mellitus, Type 2 , Diffusion Tensor Imaging , Humans , Diffusion Tensor Imaging/methods , Anisotropy , Peripheral Nerves/diagnostic imaging , Diffusion Magnetic Resonance Imaging
6.
Neurology ; 102(7): e209174, 2024 Apr 09.
Article in English | MEDLINE | ID: mdl-38513194

ABSTRACT

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.


Subject(s)
Charcot-Marie-Tooth Disease , Germ-Line Mutation , Female , Humans , Male , Charcot-Marie-Tooth Disease/genetics , Charcot-Marie-Tooth Disease/pathology , Myelin Sheath/pathology , Peripheral Nerves/diagnostic imaging , Phenotype , Cross-Sectional Studies , Retrospective Studies , Pedigree , Young Adult , Middle Aged , Aged
7.
Muscle Nerve ; 69(5): 631-636, 2024 May.
Article in English | MEDLINE | ID: mdl-38456323

ABSTRACT

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.


Subject(s)
Peripheral Nerves , Sural Nerve , Humans , Sural Nerve/diagnostic imaging , Sural Nerve/pathology , Ultrasonography , Peripheral Nerves/diagnostic imaging , Peroneal Nerve/diagnostic imaging , Peroneal Nerve/pathology , Lower Extremity
8.
Sci Rep ; 14(1): 4643, 2024 02 26.
Article in English | MEDLINE | ID: mdl-38409319

ABSTRACT

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.


Subject(s)
Polyneuropathies , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Humans , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnostic imaging , Prospective Studies , Ultrasonography , Ulnar Nerve/diagnostic imaging , Myelin-Associated Glycoprotein , Autoantibodies , Peripheral Nerves/diagnostic imaging , Neural Conduction
10.
Semin Musculoskelet Radiol ; 28(1): 92-102, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38330973

ABSTRACT

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.


Subject(s)
Diabetes Mellitus, Type 2 , Peripheral Nervous System Diseases , Humans , Diffusion Tensor Imaging/methods , Diabetes Mellitus, Type 2/pathology , Peripheral Nerves/diagnostic imaging , Peripheral Nervous System Diseases/diagnostic imaging , Magnetic Resonance Imaging/methods , Ultrasonography/methods , Biomarkers , Magnetic Resonance Spectroscopy , Edema
11.
PLoS One ; 19(1): e0297095, 2024.
Article in English | MEDLINE | ID: mdl-38277353

ABSTRACT

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.


Subject(s)
Anesthetics, Local , Nerve Block , Humans , Male , Female , Ropivacaine/pharmacology , Prospective Studies , Anesthetics, Local/pharmacology , Pilot Projects , Amides , Peripheral Nerves/diagnostic imaging , Nerve Block/methods , Volunteers
12.
Acta Radiol ; 65(2): 241-246, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38224998

ABSTRACT

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.


Subject(s)
Peripheral Nerves , Vascular Malformations , Humans , Peripheral Nerves/diagnostic imaging , Vascular Malformations/diagnostic imaging , Ultrasonography , Magnetic Resonance Imaging , Predictive Value of Tests
13.
Br J Anaesth ; 132(5): 1022-1026, 2024 May.
Article in English | MEDLINE | ID: mdl-38182528

ABSTRACT

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.


Subject(s)
Nerve Block , Peripheral Nervous System Diseases , Humans , Ultrasonography, Interventional , Nerve Block/adverse effects , Peripheral Nerves/diagnostic imaging , Anesthetics, Local , Sciatic Nerve/diagnostic imaging
14.
J Neurol ; 271(5): 2494-2502, 2024 May.
Article in English | MEDLINE | ID: mdl-38261029

ABSTRACT

BACKGROUND: To specify peripheral nerve affection in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) by correlating high-resolution nerve ultrasound and nerve conduction studies. METHODS: We assessed a cohort of 11 ARSACS patients with standardized nerve conduction studies and high-resolution ultrasound of peripheral nerves and compared nerve ultrasound findings to a healthy control group matched for age, sex, size and weight. RESULTS: Mean age of patients was 39.0 (± 14.1) years and disease duration at assessment 30.6 (± 12.5) years. All patients presented with a spasticity, ataxia and peripheral neuropathy. Neuropathy appeared to be primarily demyelinating in 9/11 cases and was not classifiable in 2/11 cases due to not evocable potentials. Nerve ultrasound revealed a normal ultrasound pattern sum score (UPSS) in each ARSACS patient and no significant nerve enlargement compared to the control group. CONCLUSIONS: Peripheral neuropathy in ARSACS showed primarily demyelinating rather than axonal characteristics and presented without nerve enlargement. As demyelinating neuropathies do commonly present enlarged nerves we recommend further genetic testing of the SACS gene in patients who present with this combination of demyelinating neuropathy without nerve enlargement. ARSACS cases that initially presented only with neuropathy without spasticity or ataxia and therefore were misdiagnosed as Charcot-Marie-Tooth disease are supporting this suggestion.


Subject(s)
Demyelinating Diseases , Muscle Spasticity , Neural Conduction , Spinocerebellar Ataxias , Spinocerebellar Ataxias/congenital , Ultrasonography , Humans , Male , Female , Adult , Middle Aged , Neural Conduction/physiology , Demyelinating Diseases/diagnostic imaging , Muscle Spasticity/diagnostic imaging , Muscle Spasticity/etiology , Muscle Spasticity/physiopathology , Spinocerebellar Ataxias/diagnostic imaging , Spinocerebellar Ataxias/complications , Young Adult , Peripheral Nervous System Diseases/diagnostic imaging , Peripheral Nervous System Diseases/diagnosis , Peripheral Nervous System Diseases/pathology , Peripheral Nervous System Diseases/physiopathology , Peripheral Nerves/diagnostic imaging , Peripheral Nerves/pathology , Cohort Studies
15.
J Magn Reson Imaging ; 59(2): 563-574, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37191075

ABSTRACT

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.


Subject(s)
Diffusion Tensor Imaging , Polyneuropathies , Male , Humans , Young Adult , Adult , Middle Aged , Diffusion Tensor Imaging/methods , Reproducibility of Results , Prospective Studies , Leg/diagnostic imaging , Peripheral Nerves/diagnostic imaging , Magnetic Resonance Imaging/methods , Biomarkers
17.
Reg Anesth Pain Med ; 49(2): 122-132, 2024 Feb 05.
Article in English | MEDLINE | ID: mdl-37940348

ABSTRACT

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.


Subject(s)
Anesthesia, Conduction , Nerve Block , Humans , Retrospective Studies , Prospective Studies , Nerve Block/adverse effects , Peripheral Nerves/diagnostic imaging
18.
J Ultrasound Med ; 43(4): 683-696, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38124499

ABSTRACT

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.


Subject(s)
Diabetes Mellitus , Diabetic Neuropathies , Humans , Diabetic Neuropathies/diagnostic imaging , Intermediate Filaments , Ultrasonography/methods , Peripheral Nerves/diagnostic imaging , Body Mass Index , Neural Conduction/physiology
19.
Clin Neurol Neurosurg ; 236: 108083, 2024 01.
Article in English | MEDLINE | ID: mdl-38104445

ABSTRACT

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.


Subject(s)
Neuroma , Peripheral Nerve Injuries , Male , Female , Humans , Peripheral Nerve Injuries/diagnostic imaging , Peripheral Nerve Injuries/surgery , Cross-Sectional Studies , Peripheral Nerves/diagnostic imaging , Peripheral Nerves/surgery , Ultrasonography/methods
20.
Semin Musculoskelet Radiol ; 27(6): 641-648, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37935210

ABSTRACT

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.


Subject(s)
Diffusion Tensor Imaging , Peripheral Nerves , Humans , Diffusion Tensor Imaging/methods , Peripheral Nerves/diagnostic imaging , Magnetic Resonance Imaging
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