Role of multisegmental nerve ultrasound in the diagnosis of leprosy neuropathy.

INTRODUCTION/AIMS: Leprosy is the most common treatable peripheral neuropathy worldwide. The detection of peripheral nerve impairment is essential for its diagnosis and treatment, in order to prevent stigmatizing deformities and disabilities. This study was performed to identify neural thickening through multisegmental ultrasound (US). METHODS: We assessed US measurements of cross-sectional areas (CSAs) of ulnar, median and tibial nerves at two points (in the osteofibrous tunnel and proximal to the tunnel), and also of the common fibular nerve at the fibular head level in 53 leprosy patients (LP), and compared with those of 53 healthy volunteers (HV), as well as among different clinical forms of leprosy. RESULTS: US evaluation detected neural thickening in 71.1% (38/53) of LP and a mean number of 3.6 enlarged nerves per patient. The ulnar and tibial were the most frequently affected nerves. All nerves showed significantly higher measurements in LP compared with HV, and also greater asymmetry, with significantly higher values for ulnar and tibial nerves. We found significant CSAs differences between tunnel and pre-tunnel points for ulnar and tibial nerves, with maximum values proximal to the tunnel. All clinical forms of leprosy evaluated showed neural enlargement through US. DISCUSSION: Our findings support the role of multisegmental US as a useful method for diagnosing leprosy neuropathy, revealing that asymmetry, regional and non-uniform thickening are characteristics of the disease. Furthermore, we observed that neural involvement is common in different clinical forms of leprosy, reinforcing the importance of including US evaluation of peripheral nerves in the investigation of all leprosy patients.

Monoclonal gammopathy-associated peripheral neuropathies: Uncovering pearls and challenges.

Monoclonal gammopathy-related peripheral neuropathies encompass a spectrum of clinical presentations in which the monoclonal protein directly damages the tissues, including the peripheral nervous system. Given the prevalence of both peripheral neuropathy and monoclonal gammopathy in the general population, these conditions may overlap in clinical practice, posing a challenge for clinicians in determining causality. Therefore, a comprehensive understanding of primary clinical syndromes and their neurophysiological patterns is of great importance for accurate differential diagnoses and effective treatment strategies. In this article, we examine the main forms of monoclonal gammopathies that affect the peripheral nerve. We explore the clinical and electrophysiological aspects and their correlation with each syndrome's corresponding monoclonal protein type. This knowledge is essential for healthcare professionals to diagnose better and manage patients presenting with monoclonal gammopathy-related peripheral nervous system involvement.

Corneal confocal microscopy detects early nerve regeneration after pharmacological and surgical interventions: Systematic review and meta-analysis.

Corneal confocal microscopy (CCM) is an ophthalmic imaging technique that enables the identification of corneal nerve fibre degeneration and regeneration. To undertake a systematic review and meta-analysis of studies utilizing CCM to assess for corneal nerve regeneration after pharmacological and surgical interventions in patients with peripheral neuropathy. Databases (EMBASE [Ovid], PubMed, CENTRAL and Web of Science) were searched to summarize the evidence from randomized and non-randomized studies using CCM to detect corneal nerve regeneration after pharmacological and surgical interventions. Data synthesis was undertaken using RevMan web. Eighteen studies including 958 patients were included. CCM identified an early (1-8 months) and longer term (1-5 years) increase in corneal nerve measures in patients with peripheral neuropathy after pharmacological and surgical interventions. This meta-analysis confirms the utility of CCM to identify nerve regeneration following pharmacological and surgical interventions. It could be utilized to show a benefit in clinical trials of disease modifying therapies for peripheral neuropathy.

[Electrophysiological diagnostics in choosing treatment strategy for neuropathies in children with transcondylar fractures]. / Rol' elektrofiziologicheskoi diagnostiki v vybore taktiki lecheniya neiropatii u detei s chrezmyshchelkovymi perelomami.

BACKGROUND: A common complication of transcondylar fractures (TCF) in children is neuropathy requiring not only therapeutic but also surgical treatment. Despite numerous reports, clear criteria for selecting patients for surgical treatment have not been defined. OBJECTIVE: To clarify the role of clinical and electrophysiological diagnostics in choosing treatment tactics for neuropathies in children with TCF. MATERIAL AND METHODS: There were 20 patients with neuropathies after TCF between 2020 and 2022. Of these, 10 ones were selected for surgical treatment according to electrophysiological diagnostic data. Inclusion criteria: age 6-12 years, closed TCF within previous 3-12 months, symptoms of neuropathy confirmed by electroneuromyography (ENMG), no nerve disruption according to ultrasound data. Exclusion criteria: elbow joint contracture and post-traumatic ulnar nerve dislocation. All patients underwent needle myography with functional assessment of motor and sensory fibers, spontaneous activity in muscles, recruitment pattern and motor unit potentials. Intraoperative electrophysiological diagnostics included stimulation of motor fascicles with registration of M-responses from the target muscles. The follow-up period was 3-6 months. RESULTS: The study included 20 patients aged 6-12 years without peripheral nerve disruption. A group of 10 patients who required surgical treatment was identified. The control group consisted of 10 patients who did not require surgical treatment. To choose treatment tactics, we considered ENMG data. Surgical procedure was determined according to intraoperative neuromonitoring (IONM) data. CONCLUSION: When choosing treatment strategy, surgeons should consider objective ENMG and IONM criteria, as well as fascicular anatomy.

The role of tuning fork in the evaluation of musculoskeletal disorders and pallesthesia: A scoping review.

BACKGROUND: Musculoskeletal and neurological conditions disorders are important conditions that need to be assessed in clinical practice. The tuning fork (TF) has been proposed as a practical tool to investigate suspected fractures and for the evaluation of pallesthesia in subjects with peripheral neuropathy. OBJECTIVE: the aim of this study is to define whether the tuning fork can be useful in the clinical evaluation of patients with musculoskeletal disorders and deep somatosensory dysfunctions. METHODS: This scoping review was performed in accordance with Joanna Briggs Institute. MEDLINE, Cochrane Library, PEDro, CINAHL, Web of Science, UpToDate, Scopus Database were consulted. RESULTS: 14 studies were included in the final analysis. Nine studies regard the use of tuning fork to detect fractures. If the tuning fork was used with a stethoscope, the test reached a high sensitivity ranging between 83% and 94%. Five studies investigated the tool to evaluate pallesthesia dysfunctions among which possible differences between biceps femoris strain and simple clinical rules for detecting peripheral neuropathy. CONCLUSION: The 128 Hz tuning fork could be potentially useful to detect some type of traumatic fractures. The Rydel-Seiffer tuning fork appears to be a useful tool for assessing potential nerve conduction deficits in the evaluation of pallesthesia.

Femoral and obturator neuropathies.

The femoral and obturator nerves both arise from the L2, L3, and L4 spinal nerve roots and descend into the pelvis before emerging in the lower limbs. The femoral nerve's primary function is knee extension and hip flexion, along with some sensory innervation to the leg. The obturator nerve's primary function is thigh adduction and sensory innervation to a small area of the medial thigh. Each may be injured by a variety of potential causes, many of them iatrogenic. Here, we review the anatomy of the femoral and obturator nerves and the clinical features and potential etiologies of femoral and obturator neuropathies. Their necessary investigations, including electrodiagnostic studies and imaging, their prognosis, and potential treatments, are discussed in this chapter.

Focal inflammatory neuropathies.

This chapter focuses on neuropathies that present with focal involvement of nerve roots, plexus, and/or peripheral nerves associated with autoimmune and inflammatory mechanisms that present with focal involvement of nerve roots, plexus and/or peripheral nerves. The clinical presentation, diagnosis, and treatment of focal autoimmune demyelinating neuropathies, focal nonsystemic vasculitic disorders (diabetic and nondiabetic radiculoplexus neuropathies, postsurgical inflammatory neuropathy, and neuralgic amyotrophy), and focal neuropathies associated with sarcoidosis and bacterial and viral infections are reviewed.

Longitudinal follow-up and prognostic factors in nitrous oxide-induced neuropathy.

BACKGROUND AND AIM: Recreational use of nitrous oxide (N2O) has been associated with the development of severe nitrous oxide-induced neuropathy (N2On). Follow-up of these patients poses challenges, and their clinical progression remains largely unknown. The identification of prognostic factors is made difficult by the lack of standardized longitudinal assessments in most studies. The objective was to document the course of neuropathy through systematic follow-up assessments in N2On patients to identify prognostic factors for persistent disability after 6 months. METHODS: We gathered demographic, clinical, biological, and electrophysiological data from N2On patients hospitalized in the Referral center in Marseille, both at baseline and during a standardized follow-up assessment at 6 months. RESULTS: We retrospectively included 26 N2On patients (mean age 22.6 ± 4.4). Significant improvements were observed in all main clinical scores including Rankin, ONLS, and MRC testing (p < .01). Electrophysiological studies (EDX) revealed a predominantly motor neuropathy with marked reduction in CMAP in the lower limbs at baseline, and no significant improvement in motor parameters (p = .543). Rankin score at 6 months correlated with the initial weekly N2O consumption (r = .43, p = .03) and the CMAP sum score in the lower limbs at the first EDX (r = -.47, p = .02). Patients with and without myelitis showed similar Rankin and ONLS score after 6 months. INTERPRETATION: The clinical course generally improved favorably at 6 months with notable amelioration in the primary disability scores, sensory deficits, and ataxia. However, distal motor impairment associated with peripheral neuropathy persisted, with distal axonal loss emerging as the main prognostic factor for long-term disability in these young patients.

The role of electrodiagnosis in focal neuropathies.

Electrodiagnostic (EDX) testing plays an important role in confirming a mononeuropathy, localizing the site of nerve injury, defining the pathophysiology, and assessing the severity and prognosis. The combination of nerve conduction studies (NCS) and needle electromyography findings provides the necessary information to fully assess a nerve. The pattern of NCS abnormalities reflects the underlying pathophysiology, with focal slowing or conduction block in neuropraxic injuries and reduced amplitudes in axonotmetic injuries. Needle electromyography findings, including spontaneous activity and voluntary motor unit potential changes, complement the NCS findings and further characterize chronicity and degree of axon loss and reinnervation. EDX is used as an objective marker to follow the progression of a mononeuropathy over time.

Neurological features of Hansen disease: a retrospective, multicenter cohort study.

To elucidate the neurological features of Hansen disease. The medical records of patients with confirmed Hansen disease transferred from the neurology department were reviewed, and all medical and neurological manifestations of Hansen disease were assessed. Eleven patients with confirmed Hansen disease, 10 with newly detected Hansen disease and 1 with relapsed Hansen disease, who visited neurology departments were enrolled. The newly detected patients with Hansen disease were classified as having lepromatous leprosy (LL, n = 1), borderline lepromatous leprosy (BL, n = 2), borderline leprosy (BB, n = 2), borderline tuberculoid leprosy (BT, n = 1), tuberculoid leprosy (TT, n = 2), or pure neural leprosy (PNL, n = 2). All of the patients with confirmed Hansen were diagnosed with peripheral neuropathy (100.00%, 11/11). The symptoms and signs presented were mainly limb numbness (100.00%, 11/11), sensory and motor dysfunction (100.00%, 11/11), decreased muscle strength (90.90%, 10/11), and skin lesions (81.81%, 9/11). Nerve morphological features in nerve ultrasonography (US) included peripheral nerve asymmetry and segmental thickening (100.00%, 9/9). For neuro-electrophysiology feature, the frequency of no response of sensory nerves was significantly higher than those of motor nerves [(51.21% 42/82) vs (24.70%, 21/85)(P = 0.0183*)] by electrodiagnostic (EDX) studies. Nerve histological features in nerve biopsy analysis included demyelination (100.00%, 5/5) and axonal damage (60.00%, 3/5). In addition to confirmed diagnoses by acid-fast bacteria (AFB) staining (54.54%, 6/11) and skin pathology analysis (100.00%, 8/8), serology and molecular technology were positive in 36.36% (4/11) and 100.00% (11/11) of confirmed patients of Hansen disease, respectively. It is not uncommon for patients of Hansen disease to visit neurology departments due to peripheral neuropathy. The main pathological features of affected nerves are demyelination and axonal damage. The combination of nerve US, EDX studies, nerve biopsy, and serological and molecular tests can improve the diagnosis of Hansen disease.

Paraproteinemic neuropathies.

The diagnostic evaluation of a peripheral neuropathy includes testing for the presence of monoclonal gammopathy, which can be found in about 10% of patients with peripheral neuropathy. Our role, as physicians, is to determine whether the neuropathy is directly related to the gammopathy or whether the co-occurrence of these two disorders is purely coincidental. The evaluating physician needs to be familiar with the different types of neuropathies associated with monoclonal gammopathies, their clinical and electrodiagnostic characteristics, and their appropriate diagnostic evaluation and management. Testing for monoclonal protein disorders includes serum protein electrophoresis (SPEP) and immunofixation of blood, and in some cases of urine, as well as measurement of free light chains and quantitative immunoglobulins. Specific antibody testing is directed by paraprotein type and neuropathy phenotype. Patients with abnormal free light chains in association with sensory and autonomic neuropathy should be evaluated for AL amyloidosis. When a lambda monoclonal protein is identified together with a clinical phenotype of chronic inflammatory demyelinating neuropathy (CIDP), a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome should be considered. Patients with IgM paraprotein associated neuropathy should be assessed for distal acquired demyelinating sensorimotor (DADS) neuropathy, with or without anti myelin associated glycoprotein (MAG) antibody or CANOMAD syndrome. In many cases, a monoclonal gammopathy of uncertain significance (MGUS) is incidental and unrelated to the neuropathy. Collaboration with oncology is critical in evaluating patients with monoclonal proteins to assess for underlying plasma cell neoplasms or B cell lymphomas.

Development and validation of a risk nomogram model for predicting peripheral neuropathy in patients with type 2 diabetes mellitus.

Objective: Diabetic peripheral neuropathy frequently occurs and presents severely in individuals suffering from type 2 diabetes mellitus, representing a significant complication. The objective of this research was to develop a risk nomogram for DPN, ensuring its internal validity and evaluating its capacity to predict the condition. Methods: In this retrospective analysis, Suqian First Hospital's cohort from January 2021 to June 2022 encompassed 397 individuals diagnosed with T2DM. A random number table method was utilized to allocate these patients into two groups for training and validation, following a 7:3 ratio. By applying univariate and multivariable logistic regression, predictive factors were refined to construct the nomogram. The model's prediction accuracy was assessed through metrics like the ROC area, HL test, and an analysis of the calibration curve. DCA further appraised the clinical applicability of the model. Emphasis was also placed on internal validation to confirm the model's dependability and consistency. Results: Out of 36 evaluated clinicopathological characteristics, a set of four, duration, TBIL, TG, and DPVD, were identified as key variables for constructing the predictive nomogram. The model exhibited robust discriminatory power, evidenced by an AUC of 0.771 (95% CI: 0.714-0.828) in the training cohort and an AUC of 0.754 (95% CI: 0.663-0.845) in the validation group. The congruence of the model's predictions with actual findings was corroborated by the calibration curve. Furthermore, DCA affirmed the clinical value of the model in predicting DPN. Conclusion: This research introduces an innovative risk nomogram designed for the prediction of diabetic peripheral neuropathy in individuals suffering from type 2 diabetes mellitus. It offers a valuable resource for healthcare professionals to pinpoint those at elevated risk of developing this complication. As a functional instrument, it stands as a viable option for the prognostication of DPN in clinical settings.

Axillary and musculocutaneous neuropathies.

This chapter covers axillary and musculocutaneous neuropathies, with a focus on clinically relevant anatomy, electrodiagnostic approaches, etiologic considerations, and management principles. Disorders of the lateral antebrachial cutaneous nerve, a derivative of the musculocutaneous nerve, are also reviewed. We emphasize the importance of objective findings, including the physical examination and electrodiagnostic evaluation in confirming the isolated involvement of each nerve which, along with the clinical history, informs etiologic considerations. Axillary and musculocutaneous neuropathies are both rare in isolation and most frequently occur in the setting of trauma. Less commonly encountered etiologies include external compression or entrapment, neoplastic involvement, or immune-mediated disorders including neuralgic amyotrophy, postsurgical inflammatory neuropathy, multifocal motor neuropathy, vasculitic neuropathy, and multifocal chronic inflammatory demyelinating polyradiculoneuropathy.

Neurological affection and serum neurofilament light chain in wild type transthyretin amyloidosis.

In contrast to inherited transthyretin amyloidosis (A-ATTRv), neuropathy is not a classic leading symptom of wild type transthyretin amyloidosis (A-ATTRwt). However, neurological symptoms are increasingly relevant in A-ATTRwt as well. To better understand the role of neurological symptoms in A-ATTRwt, A-ATTRwt patients were prospectively characterized at Amyloidosis Center Charité Berlin (ACCB) between 2018 and 2023 using detailed neurological examination, quality of life questionnaires, and analysis of age- and BMI-adapted serum neurofilament light chain (NFL) levels. 16 out of 73 (21.9%) patients presented with a severe neuropathy which we defined by a Neuropathy Impairment Score (NIS) of 20 or more. In this group, quality of life was reduced, peripheral neuropathy was more severe, and spinal stenosis and joint replacements were frequent. Age- and BMI matched serum NFL levels were markedly elevated in patients with a NIS ≥ 20. We therefore conclude that highly abnormal values in neuropathy scores such as the NIS occur in A-ATTRwt, and have an important impact on quality of life. Both peripheral neuropathy and spinal canal stenosis are likely contributors. Serum NFL may serve as a biomarker for neurological affection in patients with A-ATTRwt. It will be important to consider neurological aspects of A-ATTRwt for diagnosis, clinical follow-up, and future treatment development.

Peripheral Neuropathy.

Peripheral neuropathy is a commonly encountered diagnosis in both neurology and primary care office settings. It is important for primary care providers to identify, characterize, and diagnose patients with neuropathy. This study aims to describe the clinical presentation, diagnostic work up, and treatment options for this entity, as well as the identification of atypical features that should prompt specialized laboratory testing, electrodiagnostic testing, and neurologic consultation.

[How to Perform Autoantibody Testing for Autoimmune and Inflammatory Diseases of Peripheral Nerves and Muscles].

Measurement of autoantibodies is essential for the management of several peripheral nerve and muscle diseases. The clinical significance of autoantibody testing differs for each antibody. In addition, clinicians must understand several issues including the accuracy of the test, isotype and subclass distribution, and its relationship to disease activity. Moreover, many autoantibody tests are not covered by health insurance. With limited medical resources, clinicians are required to be up-to-date with the latest information to utilize test results in daily practice without misunderstanding.

[Utilization of Peripheral Neuropathology Tests].

Neuropathological findings rarely lead to a definitive diagnosis of autoimmune and inflammatory peripheral nerve diseases, and indications for invasive nerve biopsy with subsequent disability should be carefully determined. In addition to disease-specific pathological findings, identifying findings that facilitate differential diagnosis in clinical practice is necessary. This article reviews the neuropathological findings that are valuable in the differential diagnosis of autoimmune and inflammatory peripheral nerve diseases.

[Anti-Myelin-associated Glycoprotein Neuropathy].

Anti-myelin-associated glycoprotein (MAG) neuropathy, which occurs secondary to immunoglobulin (Ig)M paraproteinemia such as monoclonal gammopathy of undetermined significance, is characterized by slow progression, sensory or sensorimotor disturbances, and ataxia. The estimated prevalence of this neuropathy in Japan is 0.28 per 100,000 population with male preponderance. This neuropathy is diagnosed based on the detection of M protein and anti-MAG antibodies in patients' serum. Nerve conduction studies show prolonged distal latency, and histopathological evaluation of sural nerve biopsies shows widely spaced myelin on electron microscopy. Usually, immunotherapy, including administration of intravenous Ig and corticosteroids, is ineffective, and rituximab is beneficial in approximately 50% of patients. Novel therapies, such as administration of Bruton's tyrosine kinase inhibitors are expected to benefit patients with the MYD88L265P mutation.