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Background and purpose: Monitoring of the disease course of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) remains challenging because nerve conduction studies do not adequately correlate with functional disability. The prognostic value of pathological spontaneous activity (PSA) in needle electromyography (EMG) in different CIDP subgroups in a longitudinal context has, to date, not been analysed. We aimed to determine whether PSA was a prognostic marker or a marker of disease activity in a cohort of patients with CIDP. Methods: A total of 127 patients with CIDP spectrum disorder were retrospectively analysed over 57 ± 47 months regarding the occurrence of PSA (fibrillations and positive sharp waves). The presence of PSA at diagnosis, newly occurring PSA, and continuously present PSA were longitudinally correlated with clinical disability using the Inflammatory Neuropathy Cause and Treatment Overall Disability Sum Score (INCAT‐ODSS) and CIDP subtype. Results: Pathological spontaneous activity occurred in 49.6% of all CIDP patients at first diagnosis. More frequent evidence of PSA was significantly associated with a higher INCAT‐ODSS at the last follow‐up. Continuous and new occurrence of PSA were associated with higher degree of disability at the last follow‐up. The majority of patients with sustained evidence of PSA were characterized by an atypical phenotype, higher degree of disability, and the need for escalation of treatment. Conclusions: Pathological spontaneous activity was associated with a higher degree of disability and occurred more frequently in atypical CIDP variants according to the longitudinal data of a large cohort of patients with CIDP. Our results showed that EMG examination was an adequate marker for disease progression and should be evaluated during the disease course. [ABSTRACT FROM AUTHOR]

Background. Nerve conduction studies represent the gold standard in the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and play a central role in the EFNS/PNS criteria. However, the highly extensive examination protocol of those criteria and the question how to further monitor are relevant problems in daily medical practice. Hence, the aim of this cross-sectional study is to establish a practical and applicable strategy a) conducting the EFNS/PNS criteria and b) monitoring disease related disability in a large cohort of CIDP patients. Methods. As part of a prospective cohort study, we conducted a bilateral neurographic examination of the median nerve (mot.+sens.), ulnar nerve (mot.+sens.), radial nerve (sens.), tibial nerve (mot.), peroneal nerve (mot.+sens.), and sural nerve (sens.) in 95 CIDP patients. The EFNS/PNS criteria were applied on the collected neurographic data and compared with the INCAT overall disability sum score (INCAT-ODSS) and INCAT sensory score (ISS) to quantify disease disabilities. Results. In total, 95 CIDP patients (f:m 1:3.32; mean age 59±12y; 52% atypical CIDP; mean disease duration 74±60 months) were recruited, thereof 25 at initial diagnosis. All patients fulfilled the electrophysiological EFNS/PNS diagnosis criteria for CIDP. At initial diagnosis, the median nerve met the single parameters of the EFNS/PNS criteria most frequently with 72%, followed by the tibial nerve with 66%, ulnar nerve with 46%, and peroneal nerve with 22%. In total, the criterion of prolonged distal cMAP duration was met most frequently (32%), followed by an abnormal temporal dispersion (16%) and absence of F-waves (13%). However, we identified axonal damage as the parameter related with clinical disability (p < 0.001), not demyelination. Thus, distal cMAP and sNAP amplitudes could be identified as predictive markers of disability, especially in the upper limbs (related to INCAT-ODSS: ulnar nerve: R2 = 0.219; median nerve: R2 = 0.099; peroneal nerve: R2 = 0.044; tibial nerve: R2 = 0.034; related to ISS: ulnar nerve: R2 = 0.118; radial nerve: R2 = 0.112; sural nerve: R2 = 0.096; peroneal nerve: R2 = 0.084; median nerve: R2 = 0.082). The neurographic parameters of the upper limbs might relate to a greater extent to the total disability than those of the lower limbs, since nerves of the lower limbs have already shown extensive axonal damage (71% of distal cMAP amplitudes of the peroneal nerve and 45% of the tibial nerve <1 mV). Conclusions. Although the majority of CIDP patients reveal symptoms predominant of the legs, the examination of the upper limbs has proven to be essential for both initial diagnosis and disease monitoring, underlining CIDP as a systemic disease. Especially the determination of the distal cMAP duration was a sensitive parameter in the detection of CIDP. The total disability is largely determined by the degree of axonal damage. Therefore, neuroprotection should have an important additional role in the CIDP therapy in the future. [ABSTRACT FROM AUTHOR]

Background: Antibodies to human full-length myelin oligodendrocyte glycoprotein (MOG-IgG) as detected by new-generation cell-based assays have recently been described in patients presenting with acute demyelinating disease of the central nervous system, including patients previously diagnosed with multiple sclerosis (MS). However, only limited data are available on the relevance of MOG-IgG testing in patients with chronic progressive demyelinating disease. It is unclear if patients with primary progressive MS (PPMS) or secondary progressive MS (SPMS) should routinely be tested for MOG-IgG.Objective: To evaluate the frequency of MOG-IgG among patients classified as having PPMS or SPMS based on current diagnostic criteria.Methods: For this purpose, we retrospectively tested serum samples of 200 patients with PPMS or SPMS for MOG-IgG using cell-based assays. In addition, we performed a review of the entire English language literature on MOG-IgG published between 2011 and 2017.Results: None of 139 PPMS and 61 SPMS patients tested was positive for MOG-IgG. Based on a review of the literature, we identified 35 further MOG-IgG tests in patients with PPMS and 55 in patients with SPMS; the only reportedly positive sample was positive just at threshold level and was tested in a non-IgG-specific assay. In total, a single borderline positive result was observed among 290 tests.Conclusion: Our data suggest that MOG-IgG is absent or extremely rare among patients with PPMS or SPMS. Routine screening of patients with typical PPMS/SPMS for MOG-IgG seems not to be justified. [ABSTRACT FROM AUTHOR]

Introduction. In this study we evaluated the autonomic neural function in Parkinson's disease (PD) by combining the use of clinical scoring, functional evaluation with head-up tilt test (HUTT) and morphological studies of the vagus nerve (VN) with nerve ultrasound (NU). This allowed us to correlate morphological changes of the VN with markers of parasympathetic dysfunction from HUTT. Patients/Methods. In our study we used NU to compare the mean values of the cross-sectional area (CSA) of the VNs of 80 PD patients with those of controls (n=40) and patients with chronic inflammatory demyelinating polyneuropathy (CIDP) (n=76). In 69 cases HUTT data was analyzed concerning the presence of orthostatic hypotension (OH), postural orthostatic tachycardia syndrome (POTS) and chronotropic incompetence (CI). Functional haemodynamic parameters in HUTT were used to evaluate parasympathetic functions and correlate them with VN CSA (n=65). Results. In PD, the CSA of right / left VN was significantly smaller compared to the right/left VN of the control and CIDP patients (Table 1). Based on HUTT, 60.5 % of the patients were diagnosed with autonomic dysfunction (OH n=11, CI n=31, POTS n=1). Patients with OH showed significantly higher UPDRS-III values than those with CI (p=0.025). The CSA of the VN correlated inversely with heart rate and positively with parameters representing parasympathetic modulation through vagal activity (Fig. 1). Conclusion. By using NU significant morphological differences of the CSA of the VN were detected in CIDP, PD and a healthy control group. Furthermore, we demonstrated for the first time that anatomical characteristics of the VN correlate with parameters of parasympathetic function from the spectral analysis of cardiovascular parameters in HUTT for PD. This finding enables a new interpretation of the anatomical characteristics of the VN for PD patients and diagnosis of autonomic dysfunction. ▪ Fig. 1. Schematic presentation of the inverse correlation of the cross-sectional area of the right vagus nerve with the heart rate. Positive correlation with markers of parasympathetic activity HFnu-RRI. (Spearman Correlation). Abbreviations: HR=heart rate, HFnuRRI = high frequency power of the RR-Interval, bpm= beats per minute, CSA= cross-sectional area Table 1. Demographical and epidemiological characteristics as well as the cross-sectional area (CSA) of the vagus nerve. All values are provided as mean ± standard deviation. ▪ Abbreviations: INCAT/ODSS = inflammatory neuropathy cause and treatment/overall disability sum score, MDS-UPDRS III= MDS-Unified Parkinson's Disease Rating Scale, SCOPA-AUT= Scales for Outcomes in Parkinson's Disease- Autonomic Dysfunction, PDQ39 = Parkinson's Disease Questionnaire, CSA = cross sectional area. [ABSTRACT FROM AUTHOR]

Face recognition is a primary social skill which depends on a distributed neural network. A pronounced face recognition deficit in the absence of any lesion is seen in congenital prosopagnosia. This study investigating 24 congenital prosopagnosic subjects and 25 control subjects aims at elucidating its neural basis with fMRI and voxel-based morphometry. We found a comprehensive behavioral pattern, an impairment in visual recognition for faces and buildings that spared long-term memory for faces with negative valence. Anatomical analysis revealed diminished gray matter density in the bilateral lingual gyrus, the right middle temporal gyrus, and the dorsolateral prefrontal cortex. In most of these areas, gray matter density correlated with memory success. Decreased functional activation was found in the left fusiform gyrus, a crucial area for face processing, and in the dorsolateral prefrontal cortex, whereas activation of the medial prefrontal cortex was enhanced. Hence, our data lend strength to the hypothesis that congenital prosopagnosia is explained by network dysfunction and suggest that anatomic curtailing of visual processing in the lingual gyrus plays a substantial role. The dysfunctional circuitry further encompasses the fusiform gyrus and the dorsolateral prefrontal cortex, which may contribute to their difficulties in long-term memory for complex visual information. Despite their deficits in face identity recognition, processing of emotion related information is preserved and possibly mediated by the medial prefrontal cortex. Congenital prosopagnosia may, therefore, be a blueprint of differential curtailing in networks of visual cognition. [ABSTRACT FROM AUTHOR]

Objective We studied the immunmodulatory effects of triamcinolone applied intrathecally in the animal model of experimental autoimmune neuritis (EAN) in Lewis rats. Background Therapeutic efficacy of intravenous corticosteroids in chronic autoimmune neuritis has been shown in several randomized, placebo-controlled studies, although the exact mechanisms of action remain unclear. Intrathecal drug administration is an alternate route of delivery in order to bypass the blood-nerve barrier and achieve a direct effect on the nerve roots. Methods Active EAN was induced by immunization with the P2 aa 53–78 myelin peptide in Lewis rats followed by intrathecal application of 0.6 mg/kg or 1.2 mg/kg traimcinolone once daily on day 11 p.i (post-immunisation). The clinical severity score was assessed daily until day 23 p.i.. Nerve conduction studies and histological analyses of the sciatic nerves for demyelination and inflammatory infiltrates as well as flow cytometric analyses of the peripheral lymph nodes and spleen were performed at the disease maximum (day 18 p.i.). Results Therapeutic treatment with 0.6 and 1.2 mg/kg Triamcinolone applied intrathecally on day 11 p.i. significantly ameliorated clinical signs of neuritis by reducing proximal as well as distal demyelination in the nerve conduction studies. Further, histological analyses revealed a significantly lower degree of T cells and macrophages infiltrates accompanied with a decrease of proinflammatory cytokines (IFN-g and TNF-a) and an increase of anti-inflammatory cytokines (IL-10, IL-4) in the sciatic nerves at the disease maximum. Furthermore, Schwann cells death markers (caspase-3) showed a reduced Schwann cells apoptosis on triamcinolone treatment. Conclusions Intrathecal administration of triamcinolone in the rat model of autoimmune neuritis is a safe and effective immunomodulatory option with a anti-degenerative potential. Further studies are required in order to investigate the safety and efficacy of intrathecal triamcinolone application in patients with autoimmune neuritis. [ABSTRACT FROM AUTHOR]