Survey on the usage of therapeutic erythrocytapheresis in transfusion services in Italy for the treatment of polycythemia vera, secondary erythrocytosis and hemochromatosis.

INTRODUCTION: Erythrocytapheresis, an apheresis treatment which selectively removes red blood cells, is an alternative to therapeutic phlebotomy, over which it has several advantages. Actually there is a high degree of variability in the use of this treatment. This prompted SIdEM (Italian Society of Hemapheresis and Cell Manipulation) to conduct a survey on the use of erythrocytapheresis in the Italian Transfusion Services. The purpose is to monitor this activity in the treatment of Polycythemia Vera (pv), secondary erythrocytosis and hemochromatosis. MATERIALS AND METHODS: A data collection file was sent to the SIdEM regional delegates who, in turn, involved the Transfusion Centers in the areas they cover. The data collected were processed on a Microsoft Excel spreadsheet. RESULTS: 75 centers from 14 Italian regions responded to the Survey: 36 centers (48 %) use erythrocytapheresis (35 centers perform therapeutic apheresis and 1 center only donor apheresis), 39 centers (52 %) do not (15 centers perform therapeutic apheresis, 18 centers only donor apheresis and 6 centers do not perform either therapeutic apheresis or donor apheresis). Although most centers have a substantially uniform attitude concerning the indications for which erythrocytapheresis is used, the survey shows that there are still differences more evident in the treatment of secondary erythrocytosis than in the treatment of pv or hemochromatosis. CONCLUSIONS: This survey has been useful to document the current Italian reality and to raise awareness about the need for improvement in optimizing and standardizing the use of a therapy with a great potential to exploit properly.

Clinical presentation, genotype-phenotype correlations, and outcome of pancreatic neuroendocrine tumors in Von Hippel-Lindau syndrome.

PURPOSE: Data regarding the clinical management and follow-up of pancreatic neuroendocrine tumors (PanNETs) associated with Von Hippel-Lindau (VHL) syndrome are limited. This study aimed to assess clinical presentation, genotype-phenotype correlations, treatment and prognosis of PanNETs in a series of VHL syndrome patients. METHODS: Retrospective analysis of data of patients observed between 2005 and 2020. RESULTS: Seventeen patients, including 12 probands and 5 relatives (mean age 30.8 ± 18.4; 7 males), were recruited. PanNETs were found in 13/17 patients (77.5%) at a median age of 37 years: 4/13 (30.7%) at the time of VHL diagnosis and 9 (69.3%) during follow up. Six (46.1%) PanNET patients underwent surgery, whereas seven were conservatively treated (mean tumor diameter: 40 ± 10.9 vs. 15 ± 5.3 mm respectively). Four patients (30.7%) had lymph node metastases and a mean tumor diameter significantly larger than the nonmetastatic PanNETs (44.2 ± 9.3 vs. 17.4 ± 7 mm, p = 0.00049, respectively). Five (83.3%) operated patients had stable disease after a median follow up of 3 years whereas one patient showed liver metastases. Six (85.7%) non-resected PanNETs were stable after a median follow-up of 2 years, whereas one patient developed a new small PanNET and a slight increase in diameter of a pre-existing PanNET. No correlation was found between the type of germline mutation and malignant behavior of PanNETs. CONCLUSIONS: PanNETs are a common disease of the VHL syndrome and can be the presenting feature. Tumor size rather than genetic mutation is a prognostic factor of malignancy.

Importance of main pancreatic duct dilatation in IPMN undergoing surveillance.

BACKGROUND: The association between risk of pancreatic cancer and a dilated main pancreatic duct (MPD) in intraductal papillary mucinous neoplasm (IPMN) is debated. The aim of this study was to assess the role of MPD size in predicting pancreatic cancer in resected IPMNs and those kept under surveillance. METHODS: All patients with IPMN referred to the Pancreas Institute, University of Verona Hospital Trust, from 2006 to 2016 were included. The primary endpoint was the occurrence of malignancy detected at surgery or during follow-up. RESULTS: The final cohort consisted of 1688 patients with a median follow-up of 60 months. Main pancreatic duct dilatation was associated with other features of malignancy in both the resected and surveillance groups. In patients who underwent resection, only a MPD of at least 10 mm was an independent predictor of malignancy. In patients kept under surveillance, MPD dilatation was not associated with malignancy. Fifteen of 71 patients (21 per cent) with malignancy in the resection cohort had a dilated MPD alone, whereas only one of 30 (3 per cent) under surveillance with MPD dilatation alone developed malignancy. Patients with a dilated MPD and other worrisome features had an increased 5-year cumulative incidence of malignancy compared with those with a non-dilated duct (11 versus 1·2 per cent; P < 0·001); however, the risk of malignancy was not significantly increased in patients with a dilated MPD alone (4 versus 1·2 per cent; P = 0·448). CONCLUSION: In patients under surveillance, a dilated MPD alone was not associated with an increased incidence of malignancy in IPMN.

Nerve growth factor derivative NGF61/100 promotes outgrowth of primary sensory neurons with reduced signs of nociceptive sensitization.

Nerve Growth Factor (NGF) is being considered as a therapeutic candidate for Alzheimer's disease. However, the development of an NGF-based therapy is limited by its potent pain activity. We have developed a "painless" derivative form of human NGF (NGF61/100), characterized by identical neurotrophic properties but a reduced nociceptive sensitization activity in vivo. Here we characterized the response of rat dorsal root ganglia neurons (DRG) to the NGF derivative NGF61/100, in comparison to that of control NGF (NGF61), analyzing the expression of noxious pro-nociceptive mediators. NGF61/100 displays a neurotrophic activity on DRG neurons comparable to that of control NGF61, despite a reduced activation of PLCγ, Akt and Erk1/2. NGF61/100 does not differ from NGF61 in its ability to up-regulate Substance P (SP) and Calcitonin Gene Related Peptide (CGRP) expression. However, upon Bradykinin (BK) stimulation, NGF61/100-treated DRG neurons release a much lower amount of SP and CGRP, compared to control NGF61 pre-treated neurons. This effect of painless NGF is explained by the reduced up-regulation of BK receptor 2 (B2R), respect to control NGF61. As a consequence, BK treatment reduced phosphorylation of the transient receptor channel subfamily V member 1 (TRPV1) in NGF61/100-treated cultures and induced a significantly lower intracellular Ca2+ mobilization, responsible for the lower release of noxious mediators. Transcriptomic analysis of DRG neurons treated with NGF61/100 or control NGF allowed identifying a small number of nociceptive-related genes that constitute an "NGF pain fingerprint", whose differential regulation by NGF61/100 provides a strong mechanistic basis for its selective reduced pain sensitizing actions.

What is the most accurate lymph node staging method for perihilar cholangiocarcinoma? Comparison of UICC/AJCC pN stage, number of metastatic lymph nodes, lymph node ratio, and log odds of metastatic lymph nodes.

BACKGROUND: We compared the prognostic performance of the International Union Against Cancer/American Joint Committee on Cancer (UICC/AJCC) 7th edition pN stage, number of metastatic LNs (MLNs), LN ratio (LNR), and log odds of MLNs (LODDS) in patients with perihilar cholangiocarcinoma (PCC) undergoing curative surgery in order to identify the best LN staging method. METHODS: Ninety-nine patients who underwent surgery with curative intent for PCC in a single tertiary hepatobiliary referral center were included in the study. Two approaches were used to evaluate and compare the predictive power of the different LN staging methods: one based on the estimation of variable importance with prediction error rate and the other based on the calculation of the receiver operating characteristic (ROC) curve. RESULTS: LN dissection was performed in 92 (92.9%) patients; 49 were UICC/AJCC pN0 (49.5%), 33 pN1 (33.3%), and 10 pN2 (10.1%). The median number of LNs retrieved was 8. The prediction error rate ranged from 42.7% for LODDS to 47.1% for UICC/AJCC pN stage. Moreover, LODDS was the variable with the highest area under the ROC curve (AUC) for prediction of 3-year survival (AUC = 0.71), followed by LNR (AUC = 0.60), number of MLNs (AUC = 0.59), and UICC/AJCC pN stage (AUC = 0.54). CONCLUSIONS: The number of MLNs, LNR, and LODDS appear to better predict survival than the UICC/AJCC pN stage in patients undergoing curative surgery for PCC. Moreover, LODDS seems to be the most accurate and predictive LN staging method.

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part V - EUS-Guided Therapeutic Interventions (short version).

The fifth section of the Guidelines on Interventional Ultrasound (INVUS) of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) assesses the evidence for all the categories of endoscopic ultrasound-guided treatment reported to date. Celiac plexus neurolysis and block, vascular intervention, drainage of fluid collections, drainage of biliary and pancreatic ducts, and experimental tumor ablation techniques are discussed. For each topic, all current evidence has been extensively analyzed and summarized into major recommendations for reader consultation (short version; the long version is published online).

On the instability and constraints of the interaction between number representation and spatial attention in healthy humans: A concise review of the literature and new experimental evidence.

The relationship between number and space representation is still one of the most debated topics in studies of mathematical cognition. Here we offer a concise review of two important behavioral effects that have pointed out the use of a spatially left-to-right oriented mental number line (MNL) in healthy participants: the SNARC effect and the attentional SNARC effect (Att-SNARC). Following a brief summary of seminal investigations on the introspective properties of the MNL, we review recent empirical evidence and theories on the functional origin of the SNARC effect, where upon left/right response choices faster reaction times are found for small numbers with left-side responses and for large numbers with right-side responses. Then we offer a summary of the studies that have investigated whether the mere perception of visual Arabic numbers presented at central fixation can engender spatially congruent lateral shifts of attention, ie, leftward for small numbers and rightward for large ones, ie, the Att-SNARC effect. Finally, we summarize four experiments that tested whether the Att-SNARC depends on an active rather than passive processing of centrally presented digit cues. In line with other recent studies, these experiment do not replicate the original Att-SNARC and show that the mere perception of Arabic numerals does not trigger automatic shifts of attention. These shifts are instead found when the task requires the explicit left/right spatial coding of digit cues, ie, Spatial Att-SNARC (Fattorini et al., 2015b). Nonetheless, the reliability of the Spatial Att-SNARC effect seems not as strong as that of conventional SNARC effects where left/right codes are mapped onto responses rather than directly mapped on digit cues. Comparing the magnitude of digits to a numerical reference, ie, "5," also produced a Magnitude Comparison Att-SNARC that was weaker than the spatial one. However, the reliability of this Magnitude Comparison Att-SNARC should be considered with caution because, like in a study by Zanolie and Pecher (2014), we recently failed to replicate this effect in a separate behavioral-event-related potentials study in preparation (Fattorini et al., 2015a). All together the results from the present series of experiments support the hypothesis that spatial coding is not an intrinsic part of number representation and that number-space interaction is determined by the use of stimulus- or response-related spatial codes in the task at hand.

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part IV - EUS-guided interventions: General Aspects and EUS-guided Sampling (Short Version).

The fourth part of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) Guidelines on Interventional Ultrasound describes general aspects of endoscopic ultrasound-guided diagnostic and therapeutic interventions and assesses the evidence for endoscopic ultrasound-guided sampling. Endoscopic ultrasound combines the most advanced high-resolution ultrasound imaging of lesions within the wall and in the vicinity of the gastrointestinal tract and safe and effective fine needle-based tissue acquisition from these lesions. The guideline addresses the indications, contraindications, techniques, adverse events, training and clinical impact of EUS-guided sampling. Advantages and drawbacks are weighed in comparison with image-guided percutaneous biopsy. Based on the most current evidence, clinical practice recommendations are given for crucial preconditions and steps of EUS-guided sampling as well as for safe performance. Additionally, the guideline deals with the principles and reliability of cytopathological reporting in endoscopic ultrasound-guided sampling (short version; the long version is published online).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part III - Abdominal Treatment Procedures (Short Version).

The third part of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) Guidelines on Interventional Ultrasound assesses the evidence for ultrasound-guided and assisted interventions in abdominal treatment procedures. Recommendations for clinical practice are presented covering indications, contraindications, safety and efficacy of the broad variety of these techniques. In particular, drainage of abscesses and fluid collections, interventional tumor ablation techniques, interventional treatment of symptomatic cysts and echinococcosis, percutaneous transhepatic cholangiography and drainage, percutaneous gastrostomy, urinary bladder drainage, and nephrostomy are addressed (short version; a long version is published online).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part IV - EUS-guided Interventions: General aspects and EUS-guided sampling (Long Version).

The fourth part of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) Guidelines on Interventional Ultrasound describes general aspects of endoscopic ultrasound-guided diagnostic and therapeutic interventions and assesses the evidence for endoscopic ultrasound-guided sampling. Endoscopic ultrasound combines the most advanced high-resolution ultrasound imaging of lesions within the wall and in the vicinity of the gastrointestinal tract and safe and effective fine needle based tissue acquisition from these lesions. The guideline addresses the indications, contraindications, techniques, adverse events, training and clinical impact of EUS-guided sampling. Advantages and drawbacks are weighed in comparison with image-guided percutaneous biopsy. Based on the most current evidence, clinical practice recommendations are given for crucial preconditions and steps of EUS-guided sampling as well as for safe performance. Additionally, the guideline deals with the principles and reliability of cytopathological reporting in endoscopic ultrasound-guided sampling (long version).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part V.

The fifth section of the Guidelines on Interventional Ultrasound (INVUS) of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) assesses the evidence for all the categories of endoscopic ultrasound-guided treatment reported to date. Celiac plexus neurolysis and block, vascular intervention, drainage of fluid collections, drainage of biliary and pancreatic ducts, and experimental tumor ablation techniques are discussed. For each topic, all current evidence has been extensively analyzed and summarized into major recommendations for reader consultation in clinical practice (long version).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part III - Abdominal Treatment Procedures (Long Version).

The third part of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) Guidelines on Interventional Ultrasound (INVUS) assesses the evidence for ultrasound-guided and assisted interventions in abdominal treatment procedures. Recommendations for clinical practice are presented covering indications, contraindications, and safe and effective performance of the broad variety of these techniques. In particular, drainage of abscesses and fluid collections, interventional tumor ablation techniques, interventional treatment of symptomatic cysts and echinococcosis, percutaneous transhepatic cholangiography and drainage, percutaneous gastrostomy, urinary bladder drainage, and nephrostomy are addressed (long version).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part II. Diagnostic Ultrasound-Guided Interventional Procedures (Short Version).

This is the second part of the series on interventional ultrasound guidelines of the Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB). It deals with the diagnostic interventional procedure. General points are discussed which are pertinent to all patients, followed by organ-specific imaging that will allow the correct pathway and planning for the interventional procedure. This will allow for the appropriate imaging workup for each individual interventional procedure (Long version/ short version; the long version is published online).

EFSUMB Guidelines on Interventional Ultrasound (INVUS), Part II. Diagnostic Ultrasound-Guided Interventional Procedures (Long Version).

This is the second part of the series on interventional ultrasound guidelines of the Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB). It deals with the diagnostic interventional procedure. General points are discussed which are pertinent to all patients, followed by organ-specific imaging that will allow the correct pathway and planning for the interventional procedure. This will allow for the appropriate imaging workup for each individual interventional procedure (Long version).

Diagnostic performance of contrast-enhanced ultrasound (CEUS) and contrast-enhanced endoscopic ultrasound (ECEUS) for the differentiation of pancreatic lesions: a systematic review and meta-analysis.

PURPOSE: To evaluate CEUS for the diagnosis of pancreatic diseases and its application in the clinical routine with a focus on the value of CEUS in ductal pancreatic carcinoma and its use for the differentiation of neoplastic and non-neoplastic lesions. MATERIALS AND METHODS: All prospective and retrospective studies published in any language by March 6, 2014 were included based on the following criteria: use of contrast-enhanced ultrasound (CEUS) and contrast-enhanced endoscopic ultrasound (ECEUS) as the imaging methods, use of histology as the reference method and availability of a complete translation. Two authors analyzed the titles and abstracts of the search results to identify all relevant publications. Two independent readers then analyzed the full articles to identify those meeting the inclusion criteria. Details regarding study design, patient characteristics, interventions, and results were then independently extracted by two radiologists and one reviewer with methodological expertise. Sensitivity, specificity and diagnostic odds ratio (DOR) were used to obtain overall estimates. RESULTS: 1293 articles were initially identified. 27 studies met the inclusion criteria. CEUS was the index test in 23 studies while ECEUS was the index test in 4 studies. The primary study objective was met by 20 studies with respect to ductal adenocarcinoma. CEUS sensitivity was evaluated in all studies. The pooled estimate of CEUS sensitivity for the diagnosis of ductal adenocarcinoma was 0.89 (95 % CI, 0.85 - 0.92). 15 out of 20 studies examined CEUS specificity. The average specificity was 0.84 (95 % CI, 0.77 - 0.89). The pooled estimate for DOR was 61.12 (95 % CI, 34.81 - 107.32). With regard to the secondary study objective, the pooled sensitivity and specificity were 0.95 (95 % CI, 0.93 - 0.96) from 14 studies and 0.72 (95 % CI, 0.58 - 0.83) from 13 studies, respectively. The pooled DOR was 57.63 (95 % CI, 33.62 - 98.78). CONCLUSION: The sensitivity, specificity, and DOR results show the high value of CEUS for the characterization and differentiation of ductal adenocarinomas from other pancreatic diseases and for cystic pancreatic lesions. For this reason and due to their noninvasive nature, CEUS and ECEUS should be used as the first methods for characterizing neoplastic pancreatic lesions, especially since these are often incidental findings. The methods improve the quality of ultrasound diagnostics and result in faster diagnosis and better disease management.

Behavioural, psychological, and temperamental predictors of risk suicide trend after brief psychodynamic psychotherapy.

BACKGROUND: Evidence has shown that psychotherapy is effective for depression, whereas the outcome for suicide risk is unclear. AIM: It was to investigate whether possible pre-treatment predictors of suicide risk (SR) decrease after a brief psychodynamic psychotherapy treatment and at follow-up. PATIENTS AND METHODS: Forty-one patients were assessed at: baseline (T0) for clinical history, clinical family history, physical diseases, type of suffered abuse; after the treatment (T1); and, at six-month follow-up (T2) for mood ratings, temperamental features, and SR levels. RESULTS: The levels of depression and cyclothymia decreased at T1 and T2 compared to T0; however, the distribution of the patients with high SR level was similar between T0 and T1, and at T2 it increased. T1-T0 SR (Δ1SR) was correlated with suicidality in the last month and with depression levels at T0; T2-T0 SR (Δ2SR) was correlated with many historical, clinical, and temperamental variables; T2-T1 SR (Δ3SR) was correlated with the presence of previous psychotherapy, abuse, and anxiety. Linear regression models revealed that Δ1SR was predicted by the suicidality in the last month; Δ2SR was not significantly predicted by any variable; and, Δ3SR was predicted by anxiety. CONCLUSIONS: The treatment was able to decrease the depression but not the SR. Findings confirm the difficulty of affecting SR and the importance of carefully considering the anxiety and the previous experiences of abuse in order to manage the interruption of the psychotherapy.

A preliminary evaluation of a reusable digital sterilization indicator prototype.

BACKGROUND: Sterilization of critical and semicritical instruments used in patient care must undergo a terminal process of sterilization. Use of chemical and physical indicators are important in providing information on the sterilizer's performance during each cycle. Regular and periodic monitoring of sterilizers using biological indicators is necessary in periodically validating performance of sterilizers. Data loggers or independent digital parametric indicators are innovative devices that provide more information than various classes chemical indicators. In this study we evaluated a prototype of an independent digital parametric indicator's use in autoclaves. AIM: The purpose of this study was to evaluate the performance of an independent digital indicator/data logger prototype (DS1922F) that could be used for multiple cycles within an autoclave.MG Materials and methods: Three batches of the DS1922F (150 samples) were used in this study that was conducted in a series. The first batch was challenged with 300 sterilization cycles within an autoclave and the data loggers evaluated to study failures and the reason for failure, make corrections and improve the prototype design. After changes made based on studying the first batch, the second batch of the prototype (150 samples) were challenged once again with 300 sterilization cycles within an autoclave and failure studied again in further improvement of the prototype. The final batch (3rd batch) of the prototype (150 samples) was challenged again but with 600 cycles to see how long they would last. Kaplan-Meier survival analysis analyses of all three batches was conducted (α = 0.05) and failed samples qualitatively studied in understanding the variables involved in the failure of the prototype, and in improving quality. RESULTS: Each tested batch provided crucial information on device failure and helped in improvement of the prototype. Mean lifetime survival of the final batch (Batch 3) of prototype was 498 (480, 516) sterilization cycles in an autoclave. CONCLUSION: In this study, the final batch of the DS1922F prototype data logger was found to be robust in withstanding the challenge of 600 autoclave cycles, with a mean lifetime of more than 450 cycles, multiple times more than prescribed number of cycles. CLINICAL SIGNIFICANCE: Instrument reprocessing is among the important aspects of infection control. While stringent procedures are followed in instrument reprocessing within the clinic in assuring patient safety, regular use of sterilization process indicators and periodic biological validation of the sterilizer's performance is necessary. Chemical indicators for use in Autoclaves provide information on whether the particular cycle's parameters were achieved but do not provide at what specific point in time or temperature the failure occurred. Data loggers and associated reader software as the tested prototype in this evaluation (DS1922F), are designed to provide continuous information on time and temperature of the prescribed cycle. Data loggers provide immediate information on the process as opposed to Biological Indicators that take from days to a week in obtaining a confirmatory result. Further, many countries do not have the sterilization monitoring service infrastructure to meet the demands of the end users. In the absence of sterilization monitoring services, use of digital data loggers for each sterilization cycle is more pragmatic.

Malignant focal liver lesions at contrast-enhanced ultrasonography and magnetic resonance with hepatospecific contrast agent.

The aim of this study was to compare the diagnostic accuracy of the late phase of CEUS and the hepatobiliary phase of CE-MR with Gd-BOPTA in the characterization of focal liver lesions in terms of benignity and malignancy. A total of 147 solid focal liver lesions (38 focal nodular hyperplasias, 1 area of focal steatosis, 3 regenerative nodules, 8 adenomas, 11 cholangiocarcinomas, 36 hepatocellular carcinomas and 49 metastases) were retrospectively evaluated in a multicentre study, both with CEUS, using sulphur hexafluoride microbubbles (SonoVue, Bracco, Milan, Italy) and CE-MR, performed with Gd-BOPTA (Multihance, Bracco, Milan, Italy). All lesions thought to be malignant were cytohistologically proven, while all lesions thought to be benign were followed up. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values and accuracy were calculated for the late phase of CEUS and the hepatobiliary phase of CE-MRI, respectively, and in combination. Analysis of data revealed 42 benign and 105 malignant focal liver lesions. We postulated that all hypoechoic/hypointense lesions on the two phases were malignant. The diagnostic errors were 13/147 (8.8%) by CEUS and 12/147 (8.2%) by CE-MR. Sensitivity, specificity, PPV, NPV and accuracy of the late phase of CEUS were 90%, 93%, 97%, 80% and 91%, 93%, 97%, 81% and 92% for the hepatobiliary phase of CE-MRI, respectively. If we considered both techniques, the misdiagnosis diminished to 3/147 (2%) and sensitivity, specificity, PPV, NPV and accuracy were 98%, 98%, 99%, 95% and 98%. The combination of the late phase of CEUS and the hepatobiliary phase of CE-MR in the characterization of solid focal liver lesions in terms of benignity and malignancy is more accurate than the two techniques used separately.

Dysregulated microRNAs in amyotrophic lateral sclerosis microglia modulate genes linked to neuroinflammation.

MicroRNAs (miRNAs) regulate gene expression at post-transcriptional level and are key modulators of immune system, whose dysfunction contributes to the progression of neuroinflammatory diseaseas such as amyotrophic lateral sclerosis (ALS), the most widespread motor neuron disorder. ALS is a non-cell-autonomous disease targeting motor neurons and neighboring glia, with microgliosis directly contributing to neurodegeneration. As limited information exists on miRNAs dysregulations in ALS, we examined this topic in primary microglia from superoxide dismutase 1-G93A mouse model. We compared miRNAs transcriptional profiling of non-transgenic and ALS microglia in resting conditions and after inflammatory activation by P2X7 receptor agonist. We identified upregulation of selected immune-enriched miRNAs, recognizing miR-22, miR-155, miR-125b and miR-146b among the most highly modulated. We proved that miR-365 and miR-125b interfere, respectively, with the interleukin-6 and STAT3 pathway determining increased tumor necrosis factor alpha (TNFα) transcription. As TNFα directly upregulated miR-125b, and inhibitors of miR-365/miR-125b reduced TNFα transcription, we recognized the induction of miR-365 and miR-125b as a vicious gateway culminating in abnormal TNFα release. These results strengthen the impact of miRNAs in modulating inflammatory genes linked to ALS and identify specific miRNAs as pathogenetic mechanisms in the disease.