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ObjectiveHRUS is increasingly being used in the diagnosis and evaluation of autoimmune neuropathies such as CIDP. Recently, studies focused not only on changes of nerves size, but also the fascicular structure and the echogenicity changes in CIDP. However, little is known about the alterations of echogenicity in the long-term course in CIDP. The aim of this study was to evaluate echogenicity in CIDP patients in a long-term follow-up period and to analyze the benefit of the evaluation of echogenicity compared to nerve size.Methods20 patients fulfilling the definite diagnostic criteria of CIDP received clinical examination, nerve conduction studies and HRUS every 6 months over a median follow-up time of 34 months. Patients were divided into clinically stable/regressive disease course or progressive disease course according to the development of the inflammatory neuropathy cause and treatment overall disability sum score. Echogenicity of peripheral nerves was measured semi-automated and quantitative. Echogenicity was divided into three classes by fraction of black: hypoechogenic, mixed hypo-/hyperechogenic, hyperechogenic.ResultsPatients with hyperechogenic arm nerves more frequently show clinical worsening, whereas patients with hypoechogenic arm nerves remain stable or even improved over time. In the long-term course of the disease, echogenicity mostly did not change, and if changes occured echogenicity did not correspond to ODSS changes.ConclusionEchogenicity of the arm nerves in CIDP may be used as a prognostic marker, but not as a follow-up tool for evaluating clinical changes. Further studies in a larger cohort are needed to confirm these results.

Trigeminal neuralgia (TN) is supposedly caused by an ectatic blood vessel affecting the trigeminal nerve at the root entry zone of the brain stem. Recent evidence suggests an additional central component within trigeminal pain-processing in the pathophysiology of TN. Therefore, we aimed to identify specific brain regions possibly associated with the development or maintenance of TN using magnetic resonance imaging (MRI) voxel-based morphometry (VBM). Sixty patients with classical TN were compared to 49 healthy controls. Eighteen patients had TN with concomitant constant facial pain, a condition previously described as a predictor of worse treatment outcome. We found gray matter (GM) volume reduction in TN patients compared to healthy controls in the primary somatosensory and orbitofrontal cortices, as well as the in the secondary somatosensory cortex, thalamus, insula, anterior cingulate cortex (ACC), cerebellum, and dorsolateral prefrontal cortex. GM volume decrease within the ACC, parahippocampus, and temporal lobe correlated with increasing disease duration in TN. There were no differences comparing patients with and without concomitant constant facial pain. No GM increase was found comparing patient subgroups with each other and with healthy controls. The observed changes probably reflect the impact of multiple, daily attacks of trigeminal pain in these patients similar to what was previously described in other chronic pain conditions and may be interpreted as adaptation mechanism to chronic pain in regard to neuronal plasticity. The ACC, parahippocampus and temporal lobe volume reduction in parallel with disease duration may point to a pivotal role of these structures in chronic pain. •Evidence for a central component in the pathophysiology of trigeminal neuralgia.•Nerve-vessel conflict should be regarded as risk factor.•Structures identified resemble typical of chronic pain disorders.•Frequent pain attacks lead to changes related to central adaptation mechanisms.•Patients with and without constant facial pain share these morphological changes.

ObjectiveNeurofilament light chain (NfL) in serum indicates neuro-axonal damage in diseases of the central and peripheral nervous system. Reliable markers to enable early estimation of clinical outcome of intensive care unit (ICU) patients are lacking. The aim of this study was to investigate, whether serum NfL levels are a possible biomarker for prediction of outcome of ICU patients.MethodsThirty five patients were prospectively examined from admission to ICU until discharge from the hospital or death. NfL levels were measured longitudinally by a Simoa assay.ResultsNfL was elevated in all ICU patients and reached its maximum at day 35 of ICU treatment. Outcome determined by modified Rankin Scale at the end of the follow-up period correlated with NfL level at admission, especially in the group of patients with impairment of the central nervous system (n = 25, r = 0.56, p = 0.02).ConclusionNfL could be used as a prognostic marker for outcome of ICU patients, especially in patients with impairment of the central nervous system.

The assessment of disease activity is fundamental in the management of chronic inflammatory demyelinating polyneuropathy (CIDP). Previous studies with small patient numbers found an increase of corneal immune cell infiltrates as a potential marker of inflammation in patients with CIDP. However, its clinical relevance remained unclear. The present study aimed to determine whether the amount of corneal inflammatory cells (CIC) measured by corneal confocal microscopy (CCM) detects disease activity in CIDP. CIC were measured in 142 CCM-investigations of 97 CIDP-patients. Data on clinical disease activity, disability (INCAT-ODSS) and need for therapy escalation at the timepoint of CCM, 3 and 6 months later were analyzed depending CIC-count. Pathological spontaneous activity during electromyography was examined as another possible biomarker for disease activity in comparison to CIC-count. An increased CIC-count at baseline was found in patients with clinical disease activity and disability progression in the following 3–6 months. An increase to more than 25 CIC/mm 2 had a sensitivity of 0.73 and a specificity of 0.71 to detect clinical disease activity and a sensitivity of 0.77 and a specificity of 0.64 to detect disability progression (increasing INCAT-ODSS) in the following 6 months. An increase to more than 50 CIC/mm 2 had a sensitivity of about 0.51 and a specificity of 0.91 to detect clinical disease activity and a sensitivity of 0.53 and a specificity of 0.80 to detect disability progression. CIC count is a non-invasive biomarker for the detection of disease activity in the following 6 months in CIDP.

Background Corticosteroids dominate in the treatment of chronic autoimmune neuropathies although long-term use is characterized by devastating side effects. Methods We introduce the intrathecal application of the synthetic steroid triamcinolone (TRIAM) as a novel therapeutic option in experimental autoimmune neuritis in Lewis rats Results After immunization with neuritogenic P2 peptide, we show a dose-dependent therapeutic effect of one intrathecal injection of 0.3 or 0.6 mg/kg TRIAM on clinical and electrophysiological parameters of neuritis with a lower degree of inflammatory infiltrates (T cells and macrophages) and demyelination in the sciatic nerve. In vitro studies in Schwann cell cultures showed an increased expression of IL-1 receptor antagonist and reduced expression of Toll-like receptor 4 after incubation with TRIAM as well as a protective effect of TRIAM against oxidative stress after H 2 O 2 exposure. Conclusion Intrathecal TRIAM application could be a novel immunomodulatory and potentially neuroprotective option for autoimmune neuropathies with a direct effect on Schwann cells.

The novel criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) have established imaging with nerve ultrasound (NUS) and magnetic resonance neurography (MRN) as complementary methods for CIDP diagnosis. Our goal was to investigate the role of MRN and NUS for CIDP monitoring. We longitudinally examined 12 CIDP patients from 2016 to 2022 using NUS, MRN, nerve conduction studies (NCS), and clinical parameters (inflammatory neuropathy cause and treatment (INCAT)/overall disability sum score (ODSS)). NUS evaluated the cross-sectional area (CSA) of the median, ulnar, radial, tibial, fibular, and sural nerve as well as the intranerve CSA variability (INV ) of the tibial, fibular, ulnar, and median nerve, whereas MRN evaluated T2-weighted sequences of the fibular and tibial nerve at the popliteal fossa. Five patients showed clinical improvement/stability with corresponding improved or stable NCS/NUS parameters (number of nerves with increased CSA and INV ). Seven deteriorating patients showed deteriorating NCS and either increasing or decreasing NUS markers possibly indicating inflammatory activity or degenerative CSA reduction. The difference ΔINCAT/ODSS correlated positively with NUS ΔINV (  = 0.007,  = 0.731,  = 12) and with NUS ΔCSA of the tibial nerve (  = 0.0005,  = 0.865,  = 12). Further, NUS-CSA of the tibial nerve in the popliteal fossa in 2016 correlated inversely with the difference of the INCAT/ODSS score (ΔINCAT/ODSS ;  = -0.653;  = 0.033;  = 11). Finally, the Bland-Altman analyses for the tibial and fibular nerve showed a bias of -1.903 and 2.195 mm (bias = NUS-CSA - MRN-CSA) accordingly revealing a difference between MRN and NUS measurements for deeper nerves. CSA and INV of the tibial and fibular nerve can be used for monitoring in CIDP, and increased CSA of the tibial nerve is a good prognostic marker. MRN is more reliable for evaluating inflammation in proximal leg nerve segments.

Background: One of the main goals of novel, noninvasive imaging techniques like high-resolution nerve ultrasound (HRUS) and corneal confocal microscopy (CCM) is the prediction of treatment response for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: A total of 17 patients with CIDP were examined prospectively at baseline and every 9 months over a period of 18 months using CCM to quantify corneal nerve degeneration markers and immune cell infiltration as well as HRUS to detect changes of the cross-sectional area (CSA) of the peripheral nerves. Additionally, skin biopsy of the distal and proximal leg as well as quantitative sensory testing were performed at the first follow-up visit. Results: A value of more than 30 total corneal cells/mm2 in CCM at baseline identified patients with clinical progression with a sensitivity/specificity of 100% in our cohort. Corneal nerve fiber density and length remained low and stable over the study period and intra-epidermal fiber density was markedly reduced in the majority of the patients. Furthermore, an increase in Bochum ultrasound score (BUS), which summarizes the CSA of the ulnar nerve in Guyons’ canal, the ulnar nerve in the upper arm, the radial nerve in the spiral groove and the sural nerve between the gastrocnemius muscle, and a maximum BUS of 4 at study initiation identified patients with disease progression (sensitivity 80%, specificity 88%). Conclusions: BUS and corneal total cell infiltration seem to represent early markers for clinical progression in CIDP, thus having the potential to identify at-risk patients and impact treatment decisions.

Background: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce. Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to therapy with cyclophosphamide (CYP), rituximab (RTX), and bortezomib (BTZ). Treatment response was defined as improvement or stabilization of inflammatory neuropathy cause and treatment overall disability score (INCAT-ODSS). Results: A total of 48 of 181 patients (26.5%) received therapy with CYP, RTX, or BTZ. The most frequently and first used therapy was CYP (69%). More than 40% of patients needed a second or third treatment. Overall, 71 treatments were applied in 48 patients. The combination of up to all three treatments enhanced the response-rate to 90%. Treatment within 24 months after initial diagnosis resulted in significantly higher response rate than late treatment (79% versus 50 %, p = 0.04, χ2-test, n = 46) and in lower disability in long-term follow up (INCAT-ODSS 3.8 versus 5.8, p = 0.02, t-test, n = 48). Patients with Lewis-Sumner syndrome (n = 9) and autoantibody mediated neuropathies (n = 13) had excellent response rates after treatment with RTX (90–100%). In contrast, typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) showed a response rate of 64% in CYP, 64% in RTX, and 75% in BTZ. Conclusion: Treatment with CYP, RTX, or BTZ was effective in this cohort of CIN refractory to first-line treatment. Our data increase evidence for an early use of these therapies. High efficacy of RTX in Lewis-Sumner syndrome in contrast to typical CIDP suggests a distinct pathophysiology.

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a neuroinflammatory disorder of the central nervous system, distinct from multiple sclerosis by affecting predominantly the optic nerve and the spinal cord, and mediated by antibodies directed against aquaporin 4 (AQP4-ab) as a possible pathomechanistic hallmark of NMOSD. Therapeutic options include immunosuppression with steroids or B-cell-depleting agents as baseline therapies, as well as plasma exchange (PLEX) and/or immunoadsorption (IA) during relapses. Until now, data concerning the efficacy of IA alone are scarce. Methods: Visual evoked potentials (VEPs), visual acuity and changes of symptoms at relapse leading to admission in NMOSD patients (n = 10) treated with IA in a single-centre setting were evaluated retrospectively. Results: All patients profited from the procedure and showed an amelioration of admission symptoms. Three patients improved in visual acuity, another three patients remained stable, whereas five patients showed an improvement in VEPs. Discussion: In this small cohort, IA constitutes a valid therapeutic option for patients with NMOSD as an equivalent to PLEX. Analysis in larger cohorts is warranted.

This paper presents a multi-functional strand capable of introducing prestressing force in prestressed concrete (PSC) girders and sensing their static and dynamic behavior as well. This innovative strand is developed by replacing the core steel wire of the strand used in PSC structures with a carbon fiber-reinforced polymer (CFRP) wire with a built-in optical Fiber Bragg Grating (FBG) sensor. A full-scale girder specimen was fabricated by applying this multi-function strand to check the possibility of tracking the change of prestressing force at each construction stage. Moreover, dynamic data could be secured during dynamic loading tests without installing accelerometers and made it possible to obtain the natural frequencies of the structure. The results verified the capability to effectively manage the prestressing force in the PSC bridge structure by applying the PC strand with a built-in optical sensor known for its outstanding practicability and durability.