COVID-19-associated Guillain-Barré syndrome in the early pandemic experience in Lombardia (Italy).

OBJECTIVE: To estimate the incidence and describe clinical characteristics and outcome of GBS in COVID-19 patients (COVID19-GBS) in one of the most hit regions during the first pandemic wave, Lombardia. METHODS: Adult patients admitted to 20 Neurological Units between 1/3-30/4/2020 with COVID19-GBS were included as part of a multi-center study organized by the Italian society of Hospital Neuroscience (SNO). RESULTS: Thirty-eight COVID19-GBS patients had a mean age of 60.7 years and male frequency of 86.8%. CSF albuminocytological dissociation was detected in 71.4%, and PCR for SARS-CoV-2 was negative in 19 tested patients. Based on neurophysiology, 81.8% of patients had a diagnosis of AIDP, 12.1% of AMSAN, and 6.1% of AMAN. The course was favorable in 76.3% of patients, stable in 10.5%, while 13.2% worsened, of which 3 died. The estimated occurrence rate in Lombardia ranges from 0.5 to 0.05 GBS cases per 1000 COVID-19 infections depending on whether you consider positive cases or estimated seropositive cases. When we compared GBS cases with the pre-pandemic period, we found a reduction of cases from 165 to 135 cases in the 2-month study period in Lombardia. CONCLUSIONS: We detected an increased incidence of GBS in COVID-19 patients which can reflect a higher risk of GBS in COVID-19 patients and a reduction of GBS events during the pandemic period possibly due to a lower spread of more common respiratory infectious diseases determined by an increased use of preventive measures.

Dynamic Diatom-Bacteria Consortia in Synthetic Plankton Communities.

Microalgae that form phytoplankton live and die in a complex microbial consortium in which they co-exist with bacteria and other microorganisms. The dynamics of species succession in the plankton depends on the interplay of these partners. Bacteria utilize substrates produced by the phototrophic algae, while algal growth can be supported by bacterial exudates. Bacteria might also use chemical mediators with algicidal properties to attack algae. To elucidate whether specific bacteria play universal or context-specific roles in the interaction with phytoplankton, we investigated the effect of cocultured bacteria on the growth of 8 microalgae. An interaction matrix revealed that the function of a given bacterium is highly dependent on the cocultured partner. We observed no universally algicidal or universally growth-promoting bacteria. The activity of bacteria can even change during the aging of an algal culture from inhibitory to stimulatory or vice versa. We further established a synthetic phytoplankton/bacteria community with the centric diatom, Coscinodiscus radiatus, and 4 phylogenetically distinctive bacterial isolates, Mameliella sp., Roseovarius sp., Croceibacter sp., and Marinobacter sp. Supported by a Lotka-Volterra model, we show that interactions within the consortium are specific and that the sum of the pairwise interactions can explain algal and bacterial growth in the community. No synergistic effects between bacteria in the presence of the diatom was observed. Our survey documents highly species-specific interactions that are dependent on algal fitness, bacterial metabolism, and community composition. This species specificity may underly the high complexity of the multi-species plankton communities observed in nature. IMPORTANCE The marine food web is fueled by phototrophic phytoplankton. These algae are central primary producers responsible for the fixation of ca. 40% of the global CO2. Phytoplankton always co-occur with a diverse bacterial community in nature. This diversity suggests the existence of ecological niches for the associated bacteria. We show that the interaction between algae and bacteria is highly species-specific. Furthermore, both, the fitness stage of the algae and the community composition are relevant in determining the effect of bacteria on algal growth. We conclude that bacteria should not be sorted into algicidal or growth supporting categories; instead, a context-specific function of the bacteria in the plankton must be considered. This functional diversity of single players within a consortium may underly the observed diversity in the plankton.

Imaging features and ultraearly hematoma growth in intracerebral hemorrhage associated with COVID-19.

PURPOSE: Intracerebral hemorrhage (ICH) is an uncommon but deadly event in patients with COVID-19 and its imaging features remain poorly characterized. We aimed to describe the clinical and imaging features of COVID-19-associated ICH. METHODS: Multicenter, retrospective, case-control analysis comparing ICH in COVID-19 patients (COV19 +) versus controls without COVID-19 (COV19 -). Clinical presentation, laboratory markers, and severity of COVID-19 disease were recorded. Non-contrast computed tomography (NCCT) markers (intrahematoma hypodensity, heterogeneous density, blend sign, irregular shape fluid level), ICH location, and hematoma volume (ABC/2 method) were analyzed. The outcome of interest was ultraearly hematoma growth (uHG) (defined as NCCT baseline ICH volume/onset-to-imaging time), whose predictors were explored with multivariable linear regression. RESULTS: A total of 33 COV19 + patients and 321 COV19 - controls with ICH were included. Demographic characteristics and vascular risk factors were similar in the two groups. Multifocal ICH and NCCT markers were significantly more common in the COV19 + population. uHG was significantly higher among COV19 + patients (median 6.2 mL/h vs 3.1 mL/h, p = 0.027), and this finding remained significant after adjustment for confounding factors (systolic blood pressure, antiplatelet and anticoagulant therapy), in linear regression (B(SE) = 0.31 (0.11), p = 0.005). This association remained consistent also after the exclusion of patients under anticoagulant treatment (B(SE) = 0.29 (0.13), p = 0.026). CONCLUSIONS: ICH in COV19 + patients has distinct NCCT imaging features and a higher speed of bleeding. This association is not mediated by antithrombotic therapy and deserves further research to characterize the underlying biological mechanisms.

New-onset myasthenia gravis after mRNA SARS-CoV-2 vaccination: a case series.

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease that targets acetylcholine receptor (AChR) of the neuromuscular junction. New-onset MG after SARS-CoV-2 vaccination has rarely been reported. CASE PRESENTATION: We report about three patients who presented new-onset myasthenia gravis after receiving mRNA SARS-CoV-2 vaccination. The patients were all males and older than 55 years. All the patients presented with ocular and bulbar symptoms. The interval between vaccine administration and MG onset ranged from 3 days after the first dose to 10 days after the second dose. All the patients had elevated serum AChR antibodies and responded to pyridostigmine. Two out of three patients were successfully treated with IVIG or plasma exchange and with long-term immunosuppression. CONCLUSIONS: MG is a rare disease; clinicians should be aware of possible new-onset MG after SARS-CoV-2 vaccination, especially with the current recommendation of booster doses. The hyperstimulation of the innate immune system or the exacerbation of a subclinical pre-existing MG could be possible explanations.

Accuracy of the clinical diagnosis of dementia with Lewy bodies (DLB) among the Italian Dementia Centers: a study by the Italian DLB study group (DLB-SINdem).

INTRODUCTION: Dementia with Lewy bodies (DLB) may represent a diagnostic challenge, since its clinical picture overlaps with other dementia. Two toolkits have been developed to aid the clinician to diagnose DLB: the Lewy Body Composite Risk Score (LBCRS) and the Assessment Toolkit for DLB (AT-DLB). We aim to evaluate the reliability of these two questionnaires, and their ability to enhance the interpretation of the international consensus diagnostic criteria. METHODS: LBCRS and AT-DLB were distributed to 135 Italian Neurological Centers for Cognitive Decline and Dementia (CDCDs), with the indication to administer them to all patients with dementia referred within the subsequent 3 months. We asked to subsequently apply consensus criteria for DLB diagnosis, to validate the diagnostic accuracy of the two toolkits. RESULTS: A total of 23 Centers joined the study; 1854 patients were enrolled. We found a prevalence of possible or probable DLB of 13% each (26% total), according to the consensus criteria. LBCRS toolkit showed good reliability, with a Cronbach alpha of 0.77, stable even after removing variables from the construct. AT-DLB toolkit Cronbach alpha was 0.52 and, after the subtraction of the "cognitive fluctuation" criterion, was only 0.31. Accuracy, sensitivity, and specificity were higher for LBCRS vs. AT-DLB. However, when simultaneously considered in the logistic models, AT-DLB showed a better performance (p < 0.001). Overall, the concordance between LBCRS positive and AT-DLB possible/probable was of 78.02% CONCLUSIONS: In a clinical setting, the LBCRS and AT-DLB questionnaires have good accuracy for DLB diagnosis.

Energy Autonomous Wireless Sensor Nodes for Freight Train Braking Systems Monitoring.

Nowadays, railway freight transportation is becoming more and more crucial since it represents the best alternative to road transport in terms of sustainability, pollution, and impact on the environment and on public health. Upgrading the potentiality of this kind of transportation, it would be possible to avoid delays in goods deliveries due to road accidents, traffic jams, and other situation occurring on roads. A key factor in this framework is therefore represented by monitoring and maintenance of the train components. Implementing a real time monitoring of the main components and a predictive maintenance approach, it would be possible to avoid unexpected breakdowns and consequently unavailability of wagons for unscheduled repair activities. As highlighted in recent statistical analysis, one of the elements more critical in case of failure is represented by the brake system. In this view, a real time monitoring of pressure values in some specific points of the system would provide significant information on its health status. In addition, since the braking actions are related to the load present on the convoy, thanks to this kind of monitoring, it would be possible to appreciate the different behavior of the system in case of loaded and unloaded trains. This paper presented an innovative wireless monitoring system to perform brake system diagnostics. A low-power system architecture, in terms of energy harvesting and wireless communication, was developed due to the difficulty in applying a wired monitoring system to a freight convoy. The developed system allows acquiring brake pressure data in critical points in order to verify the correct behavior of the brake system. Experimental results collected during a five-month field test were provided to validate the approach.

Analysis of Wind-Induced Vibrations on HVTL Conductors Using Wireless Sensors.

In a world accelerating the energy transition towards renewable sources, high voltage transmission lines represent strategic infrastructure for power delivery. Being slender and low-damped structures, HVTL conductors are affected by wind-induced vibrations that can lead to severe fatigue issues in conductors and other components. Vibration monitoring could represent a key activity to assess the safety level of the line and perform condition-based maintenance activities. This work proposes an innovative approach based on the knowledge of the physical phenomena and smart technological devices. A wireless monitoring system based on MEMS accelerometers and energy harvesting techniques has been designed to measure the fymax parameter in the field, which represents a fatigue indicator useful to identify the different wind-induced phenomena and assess the conductors' strain level. A field test on a Canadian transmission line was used in the check of the efficiency of the system and collection of significant data. Vibrations due to vortex shedding were identified with a maximum value of fymax = 50 m/s, while subspan oscillation and galloping were not observed. We show the novel method can detect the different wind-induced phenomena and pave the way to the development of suitable software able to compute a conductor's residual fatigue life.

Alzheimer's Disease: From Immune Homeostasis to Neuroinflammatory Condition.

Alzheimer's Disease is the most common cause in the world of progressive cognitive decline. Although many modifiable and non-modifiable risk factors have been proposed, in recent years, neuroinflammation has been hypothesized to be an important contributing factor of Alzheimer's Disease pathogenesis. Neuroinflammation can occur through the combined action of the Central Nervous System resident immune cells and adaptive peripheral immune system. In the past years, immunotherapies for neurodegenerative diseases have focused wrongly on targeting protein aggregates Aß plaques and NFT treatment. The role of both innate and adaptive immune cells has not been fully clarified, but several data suggest that immune system dysregulation plays a key role in neuroinflammation. Recent studies have focused especially on the role of the adaptive immune system and have shown that inflammatory markers are characterized by increased CD4+ Teff cells' activities and reduced circulating CD4+ Treg cells. In this review, we discuss the key role of both innate and adaptive immune systems in the degeneration and regeneration mechanisms in the pathogenesis of Alzheimer's Disease, with a focus on how the crosstalk between these two systems is able to sustain brain homeostasis or shift it to a neurodegenerative condition.

CD4+ T-cell Transcription Factors in Idiopathic REM Sleep Behavior Disorder and Parkinson's Disease.

BACKGROUND: CD4+ T-cell dysregulation occurs in Parkinson's disease (PD); however, it is unknown whether it contributes to PD development. The objective of this study was to investigate transcription factor gene expression in CD4+ T cells in idiopathic rapid eye movement sleep behavior disorder, the strongest risk factor for prodromal PD. METHODS: Expression of transcription factors (TBX21, STAT1, STAT3, STAT4, STAT6, RORC, GATA3, FOXP3, and NR4A2) was measured in CD4+ T cells from 33 polysomnographically confirmed idiopathic rapid eye movement sleep behavior disorder subjects and compared with expression in cells from matched healthy subjects and antiparkinson drugs-naive PD patients. RESULTS: Compared with healthy subjects, idiopathic rapid eye movement sleep behavior disorder subjects and PD patients had lower TBX21, STAT3, and STAT4, and higher FOXP3 expression. TBX21 expression discriminated healthy subjects from idiopathic rapid eye movement sleep behavior disorder subjects and PD patients, but not idiopathic rapid eye movement sleep behavior disorder subjects with PD. CONCLUSIONS: In idiopathic rapid eye movement sleep behavior disorder subjects CD4+ T cells exhibit a peculiar molecular signature strongly resembling cells from PD patients, suggesting early involvement of peripheral immunity in PD. © 2020 International Parkinson and Movement Disorder Society.

Enhanced production of heterologous proteins by a synthetic microbial community: Conditions and trade-offs.

Synthetic microbial consortia have been increasingly utilized in biotechnology and experimental evidence shows that suitably engineered consortia can outperform individual species in the synthesis of valuable products. Despite significant achievements, though, a quantitative understanding of the conditions that make this possible, and of the trade-offs due to the concurrent growth of multiple species, is still limited. In this work, we contribute to filling this gap by the investigation of a known prototypical synthetic consortium. A first E. coli strain, producing a heterologous protein, is sided by a second E. coli strain engineered to scavenge toxic byproducts, thus favoring the growth of the producer at the expense of diverting part of the resources to the growth of the cleaner. The simplicity of the consortium is ideal to perform an in depth-analysis and draw conclusions of more general interest. We develop a coarse-grained mathematical model that quantitatively accounts for literature data from different key growth phenotypes. Based on this, assuming growth in chemostat, we first investigate the conditions enabling stable coexistence of both strains and the effect of the metabolic load due to heterologous protein production. In these conditions, we establish when and to what extent the consortium outperforms the producer alone in terms of productivity. Finally, we show in chemostat as well as in a fed-batch scenario that gain in productivity comes at the price of a reduced yield, reflecting at the level of the consortium resource allocation trade-offs that are well-known for individual species.

Design and Field Validation of a Low Power Wireless Sensor Node for Structural Health Monitoring.

Smart monitoring systems are currently gaining more attention and are being employed in several technological areas. These devices are particularly appreciated in the structural field, where the collected data are used with purposes of real time alarm generation and remaining fatigue life estimation. Furthermore, monitoring systems allow one to take advantage of predictive maintenance logics that are nowadays essential tools for mechanical and civil structures. In this context, a smart wireless node has been designed and developed. The sensor node main tasks are to carry out accelerometric measurements, to process data on-board, and to send wirelessly synthetic information. A deep analysis of the design stage is carried out, both in terms of hardware and software development. A key role is played by energy harvesting integrated in the device, which represents a peculiar feature and it is thanks to this solution and to the adoption of low power components that the node is essentially autonomous from an energy point of view. Some prototypes have been assembled and tested in a laboratory in order to check the design features. Finally, a field test on a real structure under extreme weather conditions has been performed in order to assess the accuracy and reliability of the sensors.

Engineering orthogonal synthetic timer circuits based on extracytoplasmic function σ factors.

The rational design of synthetic regulatory circuits critically hinges on the availability of orthogonal and well-characterized building blocks. Here, we focus on extracytoplasmic function (ECF) σ factors, which are the largest group of alternative σ factors and hold extensive potential as synthetic orthogonal regulators. By assembling multiple ECF σ factors into regulatory cascades of varying length, we benchmark the scalability of the approach, showing that these 'autonomous timer circuits' feature a tuneable time delay between inducer addition and target gene activation. The implementation of similar timers in Escherichia coli and Bacillus subtilis shows strikingly convergent circuit behavior, which can be rationalized by a computational model. These findings not only reveal ECF σ factors as powerful building blocks for a rational, multi-layered circuit design, but also suggest that ECF σ factors are universally applicable as orthogonal regulators in a variety of bacterial species.

Correction: Transient acute-onset tetraparesis in a COVID-19 patient.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Correction to: Efficacy of safinamide on non-motor symptoms in a cohort of patients affected by idiopathic Parkinson's disease.

The original version of this article contains an error in Table 2. The Authors realized that they submitted the previous version of Table 2. The correct version of Table 2 is shown here.

Efficacy of safinamide on non-motor symptoms in a cohort of patients affected by idiopathic Parkinson's disease.

The primary endpoint of this work was to evaluate the effect of safinamide on non-motor symptoms (NMS) in patients affected by idiopathic Parkinson's disease (PD) complicated by motor fluctuations. We retrospectively collected data from 20 subjects affected by idiopathic PD in treatment with L-dopa alone or in combination with dopamine agonists, who began to be treated with safinamide due to the occurrence of motor fluctuations. Secondary endpoints included SCales for Outcomes in Parkinson's disease (SCOPA) Motor Scale, cognitive assessment, the Hoehn and Yahr stage, Clinical Impression of Severity Index for Parkinson's Disease, Hospital Anxiety And Depression Scale, Physical and Mental Fatigue, Parkinson's disease Sleep Scale, Parkinson's Disease Questionnaire-8 (PDQ-8) and EQ-5D. Each one of these scales/questionnaires was performed at baseline and T1. For efficacy analyses, continuous variables were treated with descriptive statistics, using mean and standard deviations. A non-parametric test (the Friedman test) was carried out to evaluate the statistical significance of the results observed. We found a statistically significant reduction of the total score of NMS, of 6 domains out of 9, and 13 items out of 30. A statistically significant reduction of SCOPA Motor Scale, PDQ-8, and CISI was also detected. In conclusion, our data showed a positive effect of safinamide on NMS and confirm its positive effect on motor symptomatology.

Microdomain formation is a general property of bacterial membrane proteins and induces heterogeneity of diffusion patterns.

BACKGROUND: Proteins within the cytoplasmic membrane display distinct localization patterns and arrangements. While multiple models exist describing the dynamics of membrane proteins, to date, there have been few systematic studies, particularly in bacteria, to evaluate how protein size, number of transmembrane domains, and temperature affect their diffusion, and if conserved localization patterns exist. RESULTS: We have used fluorescence microscopy, single-molecule tracking (SMT), and computer-aided visualization methods to obtain a better understanding of the three-dimensional organization of bacterial membrane proteins, using the model bacterium Bacillus subtilis. First, we carried out a systematic study of the localization of over 200 B. subtilis membrane proteins, tagged with monomeric mVenus-YFP at their original gene locus. Their subcellular localization could be discriminated in polar, septal, patchy, and punctate patterns. Almost 20% of membrane proteins specifically localized to the cell poles, and a vast majority of all proteins localized in distinct structures, which we term microdomains. Dynamics were analyzed for selected membrane proteins, using SMT. Diffusion coefficients of the analyzed transmembrane proteins did not correlate with protein molecular weight, but correlated inversely with the number of transmembrane helices, i.e., transmembrane radius. We observed that temperature can strongly influence diffusion on the membrane, in that upon growth temperature upshift, diffusion coefficients of membrane proteins increased and still correlated inversely to the number of transmembrane domains, following the Saffman-Delbrück relation. CONCLUSIONS: The vast majority of membrane proteins localized to distinct multimeric assemblies. Diffusion of membrane proteins can be suitably described by discriminating diffusion coefficients into two protein populations, one mobile and one immobile, the latter likely constituting microdomains. Our results show there is high heterogeneity and yet structural order in the cell membrane, and provide a roadmap for our understanding of membrane organization in prokaryotes.

Parkinson's disease patients have a complex phenotypic and functional Th1 bias: cross-sectional studies of CD4+ Th1/Th2/T17 and Treg in drug-naïve and drug-treated patients.

BACKGROUND: Parkinson's disease (PD) affects an estimated 7 to 10 million people worldwide, and only symptomatic treatments are presently available to relieve the consequences of brain dopaminergic neurons loss. Neuronal degeneration in PD is the consequence of neuroinflammation in turn influenced by peripheral adaptive immunity, with CD4+ T lymphocytes playing a key role. CD4+ T cells may however acquire proinflammatory phenotypes, such as T helper (Th) 1 and Th17, as well as anti-inflammatory phenotypes, such as Th2 and the T regulatory (Treg) one, and to what extent the different CD4+ T cell subsets are imbalanced and their functions dysregulated in PD remains largely an unresolved issue. METHODS: We performed two cross-sectional studies in antiparkinson drug-treated and drug-naïve PD patients, and in age- and sex-matched healthy subjects. In the first one, we examined circulating Th1, Th2, Th17, and in the second one circulating Treg. Number and frequency of CD4+ T cell subsets in peripheral blood were assessed by flow cytometry and their functions were studied in ex vivo assays. In both studies, complete clinical assessment, blood count and lineage-specific transcription factors mRNA levels in CD4+ T cells were independently assessed and thereafter compared for their consistency. RESULTS: PD patients have reduced circulating CD4+ T lymphocytes, due to reduced Th2, Th17, and Treg. Naïve CD4+ T cells from peripheral blood of PD patients preferentially differentiate towards the Th1 lineage. Production of interferon-γ and tumor necrosis factor-α by CD4+ T cells from PD patients is increased and maintained in the presence of homologous Treg. This Th1-biased immune signature occurs in both drug-naïve patients and in patients on dopaminergic drugs, suggesting that current antiparkinson drugs do not affect peripheral adaptive immunity. CONCLUSIONS: The complex phenotypic and functional profile of CD4+ T cell subsets in PD patients strengthen the evidence that peripheral adaptive immunity is involved in PD, and represents a target for the preclinical and clinical assessment of novel immunomodulating therapeutics.

Predictors of outcome in a large retrospective cohort of patients with transverse myelitis.

BACKGROUND: Transverse myelitis (TM) is an inflammatory disorder that can be idiopathic or associated with central nervous system autoimmune/dysimmune inflammatory diseases, connective tissue autoimmune diseases, or post-infectious neurological syndromes. Prognosis of initial TM presentations is uncertain. OBJECTIVE: To identify outcome predictors in TM. METHODS: Retrospective study on isolated TM at onset. Scores ⩾3 on the modified Rankin scale (mRS) marked high disability. RESULTS: A total of 159 patients were identified. TM was classified as follows: idiopathic (I-TM, n = 53), post-infectious (PI-TM, n = 48), associated with multiple sclerosis (MS-TM, n = 51), or neuromyelitis optica spectrum disorders/connective tissue autoimmune diseases/neurosarcoidosis ( n = 7). At follow-up (median, 55 months; interquartile range, 32-80), 42 patients were severely disabled, and patients with I-TM or PI-TM showed the worst outcomes. Predictors of disability were infectious antecedents, sphincter and pyramidal symptoms, high mRS scores, blood-cerebrospinal fluid barrier damage, lumbar magnetic resonance imaging (MRI) lesions on univariate analysis, and older age (odds ratio (OR), 1.1; 95% confidence interval (CI), 1.0-1.1), overt/subclinical involvement of the peripheral nervous system (PNS) (OR, 9.4; 95% CI, 2.2-41.0), complete TM (OR, 10.8; 95% CI, 3.4-34.5) on multivariate analysis. CONCLUSION: Our findings help define prognosis and therapies in TM at onset. Infectious antecedents and PNS involvement associate with severe prognosis. Nerve conduction studies and lumbar MRI could improve the prognostic assessment of this condition.

Utility of somatosensory evoked potentials in the assessment of response to IVIG in a long-lasting case of chronic immune sensory polyradiculopathy.

BACKGROUND: Chronic immune sensory polyradiculopathy (CISP) identifies a progressive acquired peripheral dysimmune neuropathy recognized as a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant. We describe a young woman with a thirteen-year history of CISP with a belated variable response to intravenous immunoglobulin (IVIG) and an almost erratic anticipation of symptoms between IVIG cycles. The association of IVIG and corticosteroids, immunosuppressants, plasmapheresis, did not lead to clinical improvement and was characterized by significant side effects. We evaluated a combined clinical and somatosensory evoked potentials (SSEPs) approach aimed to identify possible predictive parameters concerning the effect and duration of each IVIG administration. Neurologic disability was evaluated using INCAT - Overall Disability Sum Score (INCAT-ODSS). CASE PRESENTATION: A 30-year-old woman presented on 2004 for the subacute onset of asymmetric paresthesias in the lower limbs over the previous six months. The symptoms had been relapsing-remitting during the first four months, followed by a slow progression, resulting in limbs ataxia and a progressive gait disturbance requiring Canadian crutches. Motor and sensory nerve conduction studies and electromyographic evaluation were into normal limits. Median SSEPs were normal, while tibial SSEPs were characterised by the bilateral absence of both lumbar and cortical responses. Cerebrospinal fluid detected an increased protein concentration, while spinal MRI showed a pronounced thickening of the sacral nerve roots, together with a tube-shaped enlargement. These findings led to the diagnosis of CISP and the patient was treated with IVIG reaching a stable remission over the following 9 years. In early 2014, the patient began to show a variable response to treatment with erratic anticipation of sensory disturbances, and a more pronounced walking disability: corticosteroids, plasmapheresis, mycophenolate mofetil and cyclophosphamide were uneffective and burdened by relevant side effects. To better assess the response to IVIG in terms of time-effect, consistency and duration, we have combined a scheduled clinical and SSEPs evaluation during and after each IVIG cycle. CONCLUSIONS: The correlation between the neurophysiological data and the INCAT-ODSS scores has allowed the modulation of IVIG cycles with a significant reduction of the clinical fluctuations and disability. SSEPs may therefore represent an useful and recommended additional aid for the treatment schedule of this rare clinical form.