Treatment of an acute motor and sensory axonal neuropathy with propionate in a 33-year-old male

Background: The aim of this work was to report a case of an acute motor and sensory axonal neuropathy (AMSAN) treated with propionate to evaluate its therapeutic potential in AMSAN. Materials and methods: The patient was investigated by clinical examination, electroneurography, high-resolution nerve ultrasound and confocal corneal microscopy at baseline and the 2 month follow up. We compared the outcome with those of five other patients with acute motor axonal neuropathy (AMAN) and AMSAN of who were referred to our neurology department in the past 5 years. Results: Considering the poor prognosis of patients with acute axonal neuropathies and in comparison with the previously treated patients with AMAN or AMSAN in our clinic, the regression of our patient’s symptoms and the improvement in the additional examinations under propionate seemed exceptionally good. Conclusion: Propionate may have an additional therapeutic effect in autoimmune inflammatory neuropathies

Serum neurofilament light chain as outcome marker for intensive care unit patients

Objective!#!Neurofilament 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.!##!Methods!#!Thirty 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.!##!Results!#!NfL 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).!##!Conclusion!#!NfL could be used as a prognostic marker for outcome of ICU patients, especially in patients with impairment of the central nervous system

New Approaches to Critical Illness Polyneuromyopathy: High-Resolution Neuromuscular Ultrasound Characteristics and Cytokine Profiling

Background!#!Diagnosis of intensive care unit acquired weakness (ICUAW) is challenging. Pathogenesis of underlying critical illness polyneuromyopathy (CIPNM) remains incompletely understood. This exploratory study investigated whether longitudinal neuromuscular ultrasound examinations and cytokine analyses in correlation to classical clinical and electrophysiological assessment contribute to the understanding of CIPNM.!##!Methods!#!Intensive care unit patients were examined every 7 days until discharge from hospital. Clinical status, nerve conduction studies, electromyography as well as ultrasound of peripheral nerves and tibial anterior muscle were performed. Cytokine levels were analyzed by a bead-based multiplex assay system.!##!Results!#!Of 248 screened patients, 35 patients were included at median of 6 days (IQR: 8) after admission to intensive care unit. Axonal damage was the main feature of CIPNM. At the peak of CIPNM (7 days after inclusion), nerve ultrasound showed cross-sectional area increase of tibial nerve as a sign of inflammatory edema as well as hypoechoic nerves as a possible sign of inflammation. Cytokine analyses showed signs of monocyte and macrophage activation at this stage. Fourteen days after inclusion, cytokines indicated systemic immune response as well as profiles associated to neovascularization and regeneration.!##!Conclusions!#!Exploratory neuromuscular ultrasound and cytokine analyses showed signs of inflammation like macrophage and monocyte activation at the peak of CIPNM followed by a systemic immune response parallel to axonal damage. This underlines the role of both axonal damage and inflammation in pathogenesis of CIPNM

Nerve echogenicity in polyneuropathies of various etiologies

Echogenicity of peripheral nerves in high-resolution ultrasound (HRUS) provides insight into the structural damage of peripheral nerves in various polyneuropathies. The aim of this study was to compare nerve echogenicity in different primarily axonal or demyelinating polyneuropathies to examine the significance of this parameter. Performing semi-automated echogenicity analysis and applying Image J, we retrospectively used HRUS images of 19 patients with critical illness polyneuropathy (CIP), and 27 patients with chemotherapy-induced polyneuropathy (CIN) and compared them to 20 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The fraction of black representing echogenicity was measured after converting the images into black and white. The nerves of patients with progressive CIDP significantly differed from the hyperechogenic nerves of patients with other polyneuropathies at the following sites: the median nerve at the forearm ($\it p$ < 0.001), the median nerve at the upper arm ($\it p$ < 0.004), and the ulnar nerve at the upper arm ($\it p$ < 0.001). The other polyneuropathies showed no notable differences. Altogether, the comparison of echogenicity between different polyneuropathies supports the assumption that there are differences depending on the genesis of the structural nerve damage. However, these differences are slight, and cannot be used to show clear differences between each polyneuropathy form

The distribution of infarctions for patients with (AHI > 10) and without (AHI < 10) SRBD.

<p>Blue shades: less frequent infarcted regions; Red shades: more frequent infarcted regions. Brainstem infarcts appear to be more frequent in patients with SRBD (arrow, not significant).</p

Image_1_Comparative analysis of albumin quotient and total CSF protein in immune-mediated neuropathies: a multicenter study on diagnostic implications.TIF

IntroductionBlood-cerebrospinal fluid (CSF) barrier dysfunction is pivotal for diagnosing immune-mediated neuropathies, especially in spinal nerve root inflammation. Typically, either total CSF protein or the CSF to serum albumin ratio (QAlb) is measured. Total CSF protein measurements have limitations, notably its fixed reference value regardless of age, in contrast to the age-dependent reference for QAlb. Our goal was to evaluate both markers in patients with immune-mediated neuropathies.MethodsIn our multicenter research, we collected retrospective CSF data from patients suffering from immune-mediated neuropathies across four German research centers. These parameters were analyzed in relation to their clinical characteristics.ResultsOut of 419 samples, 36 (8.6%) displayed a notable variation between total CSF protein and QAlb values. A detailed analysis revealed that patients displaying elevated QAlb but normal total CSF protein levels were significantly younger at disease onset (p = 0.01), at the time of diagnosis (p = 0.005), and when undergoing lumbar puncture (p = 0.001) compared to patients with elevated CSF protein and normal QAlb levels. These effects were especially evident for the subgroup of samples derived by female patients.DiscussionOur work confirms the crucial role of QAlb in diagnosing immune-mediated neuropathies and particularly its efficacy as a marker for evaluating the blood-CSF barrier in patients with an earlier disease onset. Considering the significance of the albumin quotient, its assessment is especially advisable in younger patients of female sex to avoid missing a potential barrier dysfunction that might be falsely negative when using total protein.</p

Baseline characteristics of patients with and without sleep related breathing disorder (SRBD).

<p>Baseline characteristics of patients with and without sleep related breathing disorder (SRBD).</p

High-resolution nerve ultrasound to assess nerve echogenicity, fascicular count, and cross-sectional area using semiautomated analysis

BACKGROUND AND PURPOSE Little is known about echogenicity and fascicular structure observed in high-resolution nerve ultrasound (HRUS) in both healthy subjects and patients with peripheral nerve disease. The aim of this study was to evaluate the reliability of echogenicity, fascicle count, and fascicle size analysis, to create standard values and compare these parameters to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS Median, ulnar, radial, tibial, and fibular nerve of 79 healthy subjects and patients were scanned by one examiner using HRUS. Image analysis regarding echogenicity, fascicle count, and fascicle cross-sectional area (CSA) was performed by two independent raters semiautomatically using ImageJ. Pearson correlation coefficient $\it r$ reflected interrater reliability (IR), and intraclass correlation coefficient (ICC) determined intrarater reliability (IAR). Results of healthy subjects were compared to 20 patients with CIDP by analysis of variance. RESULTS IR was very good for echogenicity ($\it r$ = .9) and good for fascicle count and size of the largest fascicle ($\it r$ = .64/.56). IAR was very good for all three parameters (ICC = .9/.83/.74). Healthy subjects had a wide range of values. CIDP patients were in range of healthy subjects. Clinically progressive CIDP patients (defined as an increase in Overall Disability Sum Score by $\geq$1 point) had a lower fraction of black than healthy controls and stable CIDP patients ($\it P$ < .001). CONCLUSION Semiautomated evaluation of echogenicity, fascicle count, and fascicle CSA is reliable. Cutoff values to differentiate between healthy persons and CIDP do not exist. Echogenicity is useful for detecting clinically progressive CIDP patients and should be used in clinical context or intraindividual course

Table_1_Comparative analysis of albumin quotient and total CSF protein in immune-mediated neuropathies: a multicenter study on diagnostic implications.docx

IntroductionBlood-cerebrospinal fluid (CSF) barrier dysfunction is pivotal for diagnosing immune-mediated neuropathies, especially in spinal nerve root inflammation. Typically, either total CSF protein or the CSF to serum albumin ratio (QAlb) is measured. Total CSF protein measurements have limitations, notably its fixed reference value regardless of age, in contrast to the age-dependent reference for QAlb. Our goal was to evaluate both markers in patients with immune-mediated neuropathies.MethodsIn our multicenter research, we collected retrospective CSF data from patients suffering from immune-mediated neuropathies across four German research centers. These parameters were analyzed in relation to their clinical characteristics.ResultsOut of 419 samples, 36 (8.6%) displayed a notable variation between total CSF protein and QAlb values. A detailed analysis revealed that patients displaying elevated QAlb but normal total CSF protein levels were significantly younger at disease onset (p = 0.01), at the time of diagnosis (p = 0.005), and when undergoing lumbar puncture (p = 0.001) compared to patients with elevated CSF protein and normal QAlb levels. These effects were especially evident for the subgroup of samples derived by female patients.DiscussionOur work confirms the crucial role of QAlb in diagnosing immune-mediated neuropathies and particularly its efficacy as a marker for evaluating the blood-CSF barrier in patients with an earlier disease onset. Considering the significance of the albumin quotient, its assessment is especially advisable in younger patients of female sex to avoid missing a potential barrier dysfunction that might be falsely negative when using total protein.</p