Anti-titin antibodies in a cohort of myasthenia gravis patients.

Background: Anti-titin antibodies have been previously associated with thymoma-associated myasthenia gravis (MG) and a more clinically severe form of MG. While currently only serving as a disease biomarker, its possible utility as an indicator of underlying thymus malignancy may be of value in clinical practice. Methods: Data was retrospectively collected and analyzed from 2013 to 2022 using an institutional record of MG patients. Anti-titin antibodies were assessed using Line Blot immunoassay. Results: From 130 MG cases, 32 (24.6%) were anti-titin positive. Anti-titin positive cases were associated with older age of disease onset [median (IQR): 63.0 (44.3-70.8) vs. 35.5 (24.8-60.8) years] (P<0.01). Thymectomy was performed in 46 (35.4%) MG patients, 12 of which anti-titin positive (26.1%). Thymectomy samples from anti-titin positive patients comprised 10 (83.3%) cases of thymoma and 2 (16.7%) cases of thymus hyperplasia. There was a tendency towards anti-titin positive patients having more thymoma while anti-titin negative displayed more hyperplasia (P<0.01). Anti-titin positivity correlated with thymoma in patients with age of onset bellow 50 years (P=0.028). Anti-titin positivity was significantly associated with generalized MG in the late-onset group (P=0.005). Conclusions: The presence of anti-titin antibodies appears to correlate with underlying thymoma in early-onset MG cases and with generalized MG in late-onset cases. Prospective studies are needed to further study this association.

Blood and CSF anti-neuronal antibodies testing in psychotic syndromes: a retrospective analysis from a tertiary psychiatric hospital.

A Consensus of Psychoimmunology Experts (Pollak et al., 2019) established a set of red flags and proposed diagnostic criteria for psychosis of autoimmune origin (AIP). Previous studies on AIP are limited by the scarcity of CSF analysis, preventing the valorization of blood anti-neuronal antibodies (Ab). The aims of this study are to determine the relative frequency and characterize AIP in a cohort of psychotic patients that underwent CSF workup. This work is a retrospective study in a tertiary psychiatric hospital. Clinical and paraclinical data were collected from medical records, and patients were classified according to Pollak et al. (2019) criteria. From 68 patients, ten (14.7%) had positive anti-neuronal antibodies (Ab): n = 5 in CSF and blood (n = 4 anti-NMDAr, n = 1 -GAD65), and n = 5 in blood only (n = 1 anti-GABAb, n = 1 -GAD65, n = 1 -SOX1, n = 1 -NMDAr, n = 1 -zic4). After 5- (2-10)-year follow-up, n = 6/68 (8.8%) had AIP diagnosis in context of autoimmune encephalitis (AE), and the remaining (n = 4/10, blood-only Ab) alternative diagnoses (n = 2 dementia, n = 1 schizophrenia, n = 1 intellectual disability). Ten of the 13 patients that fulfilled criteria for possible AIP were mimics, and only three AE had criteria for probable AIP. All AIP developed neurological manifestations (mostly cognitive dysfunction); EEG was usually abnormal (66.7%), and all had normal MRI. We found statistically significant associations between AIP/AE and systemic autoimmune disease, presentation with seizures and EEG abnormalities. All AE developed neurological symptoms alongside psychosis. Ab positivity occurred predominantly in AE but also in other neuropsychiatric disorders. Clinical suspicion based on the knowledge of the described presentations of established Ab is crucial in the psychotic patient approach.

Distinct phenotypes in a cohort of anti-CASPR2 associated neurological syndromes.

INTRODUCTION: Anti-contactin-associated protein-like 2 (CASPR2) is classically associated with limbic encephalitis (LE), Morvan syndrome and peripheral nerve hyperexcitability (PNH). Additional clinical features have been previously recognized. OBJECTIVE: To describe a cohort of patients with anti-CASPR2-associated neurological syndromes from a tertiary referral centre. METHODS: Retrospective analysis of patients with positive serum anti-CASPR2 antibodies in the period between 2014 and 2021. RESULTS: Nineteen patients were identified, 11 (57.9%) male, with a median age at symptom onset of 49.0 (31.3-63.0) years and a median time to diagnosis of 1.0 (0.0-1.8) years. The most common clinical syndromes were LE (7 cases, 36.8%), Morvan syndrome (4, 21.1%) and PNH (2, 10.5%). Six patients presented with atypical phenotypes (31.6%), comprising dysautonomia (orthostatic hypotension and Adie's Pupil), motor tics/stereotypies, obsessive-compulsive disorder, and brainstem involvement. The most common presenting symptoms were seizures (31.6%), PNH (21.1%) and cognitive dysfunction (15.8%). One LE patient had a disease duration of 2,5 years and was initially diagnosed with dementia. CSF was normal in most cases. Brain MRI showed temporal lobe hyperintensities in 4 LE cases (57.1%). All PNH cases had myokymic discharges of fasciculations in the electromyography. Two patients had associated thymoma and 1 had lung adenocarcinoma. Eight patients (42.1%) received treatment during the acute phase and 26.3% maintenance treatment. Approximately half of the treated cases improved or stabilised, with 4 (21.1%) deaths in the whole cohort. CONCLUSION: Anti-CASPR2-associated neurological disorders may present with isolated atypical phenotypes, a slowly progressive clinical course, and with normal CSF or imaging findings.

Comparison between a bulk-fill resin-based composite and three luting materials on the cementation of fiberglass-reinforced posts.

Objectives: This study verified the possibility of cementing fiberglass-reinforced posts using a flowable bulk-fill composite (BF), comparing its push-out bond strength and microhardness with these properties of 3 luting materials. Materials and Methods: Sixty endodontically treated bovine roots were used. Posts were cemented using conventional dual-cured cement (CC); self-adhesive cement (SA); dual-cured composite (RC); and BF. Push-out bond strength (n = 10) and microhardness (n = 5) tests were performed after 1 week and 4 months of storage. Two-way repeated measures analysis of variance (ANOVA), 1-way ANOVA, t-test, and Tukey post-hoc tests were applied for the push-out bond strength and microhardness results; and Pearson correlation test was applied to verify the correlation between push-out bond strength and microhardness results (α = 0.05). Results: BF presented higher push-out bond strength than CC and SA in the cervical third before aging (p < 0.01). No differences were found between push-out bond strength before and after aging for all the luting materials (p = 0.84). Regarding hardness, only SA presented higher values measured before than after aging (p < 0.01). RC and BF did not present 80% of the maximum hardness at the apical regions. A strong positive correlation was found between the luting materials' push-out bond strength and microhardness (p < 0.01, R2 = 0.7912). Conclusions: The BF presented comparable or higher push-out bond strength and microhardness than the luting materials, which indicates that it could be used for cementing resin posts in situations where adequate light curing is possible.

Systemic relations among the variables involved in occupational accidents of the nursing team in a psychiatric hospital.

BACKGROUND: The occupational activities carried out in hospital environments pose occupational risks to professionals. In psychiatric hospitals, due to the characteristics of the patients treated, professionals are also subjected to other risks, such as physical aggression. OBJECTIVE: This research aimed to identify the systemic context, highlighting the cause-and-effect relationships that culminate in occupational accidents that occurred with the nursing staff in a psychiatric hospital in Brazil. METHODS: The current study is an applied research and was divided into three stages. First, the collection of data related to the case study was made and accidents were analyzed and occupational hazards were identified. In the second stage, from the collected information, occupational safety indicators were defined. Lastly, in the third stage, the qualitative aspect of System Dynamics was applied to perform the systemic analysis and to identify how the different variables were related. RESULTS: The results showed that physical aggression was the main cause of accidents. Regarding safety indicators, while both the level of use of Personal Protective Equipment (PPE) by professionals and the high level of PPE protection were positive aspects, the level of training of professionals to use PPE was a negative aspect. The Causal Link Diagram (CLD) showed that the perception of risk influenced the level of use of PPE and those organizational measures influenced the accident rate. CONCLUSION: In conclusion, the systemic analysis of the system dynamics can optimize the diagnostic process of occupational accidents in psychiatric hospitals, and especially help to identify the cause and effect among the variables involved.

Stimuli in 3 Acts: A normative study on action-statements, action videos and object photos.

The study of action observation and imagery, separately and combined, is expanding in diverse research areas (e.g., sports psychology, neurosciences), making clear the need for action-related stimuli (i.e., action statements, videos, and pictures). Although several databases of object and action pictures are available, norms on action videos are scarce. In this study, we validated a set of 60 object-related everyday actions in three different formats: action-statements, and corresponding dynamic (action videos) and static (object photos) stimuli. In Study 1, ratings of imageability, image agreement, action familiarity, action frequency, and action valence were collected from 161 participants. In Study 2, a different sample of 115 participants rated object familiarity, object valence, and object-action prototypicality. Most actions were rated as easy to imagine, familiar, and neutral or positive in valence. However, there was variation in the frequency with which participants perform these actions on a daily basis. High agreement between participants' mental image and action videos was also found, showing that the videos depict a conventional way of performing the actions. Objects were considered familiar and positive in valence. High ratings on object-action prototypicality indicate that the actions correspond to prototypical actions for most objects. 3ActStimuli is a comprehensive set of stimuli that can be useful in several research areas, allowing the combined study of action observation and imagery.

Can we learn from errors? Retrieval facilitates the correction of false memories for pragmatic inferences.

Errorful learning suggests that, when perfect learning has not yet been attained, errors can enhance future learning if followed by corrective feedback. Research on memory updating has shown that after retrieval, memory becomes more malleable and prone to change. Thus, retrieval of a wrong answer might provide a good context for the incorporation of feedback. Here, we tested this hypothesis using sentences including pragmatic sentence implications, commonly used for the study of false memories. Across two experiments with young adults, we hypothesized that corrective feedback would be more efficient at reducing false memories if provided immediately after retrieval, when memory is more malleable than after being exposed to the material. Participants' memory was assessed as a function of the type of learning task (Experiment 1: retrieval vs. restudy; and Experiment 2: active vs. passive recognition); and whether participants received corrective feedback or not. In both experiments, we observed that retrieval not only improved correct recall (replicating the testing effect) but also promoted the correction of false memories. Notably, corrective feedback was more effective when given after errors that were committed during retrieval rather than after restudy (Experiment 1) or after passive recognition (Experiment 2). Our results suggest that the benefits of retrieval go beyond the testing effect since it also facilitates false memories correction. Retrieval seems to enhance memory malleability, thus improving the incorporation of feedback, compared to the mere presentation of the information. Our results support the use of learning strategies that engage in active and explicit retrieval because, even if the retrieved information is wrong-when immediate feedback is provided-memory updating is promoted and errors are more likely to be corrected.

Late onset neuromyelitis optica spectrum disorders (LONMOSD) from a nationwide Portuguese study: Anti-AQP4 positive, anti-MOG positive and seronegative subgroups.

INTRODUCTION: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. OBJECTIVE: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). METHODS: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. RESULTS: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). CONCLUSIONS: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.

Anti-NMDAr Encephalitis and COVID-19 in a Patient With Systemic pANCA-Vasculitis and Recurrent Varicella Zoster Infection.

Introduction: There is a complex interplay between systemic autoimmunity, immunosuppression, and infections. Any or all of these can result in neurologic manifestations, requiring diligence on the part of neurologists. Case report: We herein report a case of a patient on immunosuppressive treatment for a vasculitis that resulted in zoster meningoencephalitis. This was further complicated by the development of anti-NMDAr encephalitis, the etiology of which is undetermined and further discussed in this paper. The patient eventually developed COVID-19 during hospitalization, succumbing to the respiratory infection. Conclusion: This case emphasizes that post-infectious autoimmune disorders are becoming increasingly recognized and that they should still be considered in patients who are on immunosuppression. Practitioners should be aware of the complex relationship between autoimmunity and immunosuppression and consider both throughout the disease course.

Autoimmune encephalitis: suspicion in clinical practice and mimics.

Despite the existence of well-established diagnostic criteria for autoimmune encephalitis, there are diseases capable of mimicking it. This study sought to retrospectively evaluate the reasons for testing and the final diagnosis of patients admitted to a Neurology ward tested for anti-NMDAR antibodies and estimate sensitivity and specificity of current diagnostic criteria. The threshold for testing was lower than that of the prevailing diagnostic criteria, and the proportion of autoimmune encephalitis mimics was high. Searching for alternative diagnoses is of pivotal importance in cases of autoimmune encephalitis suspicion, and diagnostic criteria may need expanding so that no autoimmune encephalitis is missed.

Imagination Reduces False Memories for Everyday Action Sentences: Evidence From Pragmatic Inferences.

Human memory can be unreliable, and when reading a sentence with a pragmatic implication, such as "the karate champion hit the cinder block," people often falsely remember that the karate champion "broke" the cinder block. Yet, research has shown that encoding instructions affect the false memories we form. On the one hand, instructing participants to imagine themselves manipulating the to-be-recalled items increase false memories (imagination inflation effect). But on the other hand, instructions to imagine have reduced false memories in the DRM paradigm (imagination facilitation effect). Here, we explored the effect of imaginal encoding with pragmatic inferences, a way to study false memories for information about everyday actions. Across two experiments, we manipulated imaginal encoding through the instructions given to participants and the after-item filler task (none vs. math operations). In Experiment 1, participants were either assigned to the encoding condition of imagine+no filler; pay attention+math; or memorize+math. In Experiment 2, the encoding instructions (imagine vs. memorize) and the filler task (none vs. math) were compared across four separate conditions. Results from the two experiments showed that imagination instructions lead to better memory, by showing a higher proportion of correct responses and better performance in a memory benefit index. Similarly, a significant reduction of false memories was observed across both experiments, even though a complementary Bayesian analysis only supported this conclusion for Experiment 1. The findings show that imaginal encoding improves memory, suggesting the engagement of a distinctiveness heuristic and source-monitoring process.

Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study.

INTRODUCTION: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. OBJECTIVE: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. METHODS: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. RESULTS: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. CONCLUSION: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries.

Memory updating after retrieval: when new information is false or correct.

We conducted three experiments testing the malleability of memory in incorporating new information following retrieval. All experiments used associative lists typical of the DRM paradigm [Deese, J. (1959). On the prediction of occurrence of particular verbal intrusions in immediate recall. Journal of Experimental Psychology, 58(1), 17-22; Roediger, H. L., & McDermott, K. B. (1995). Creating false memories: Remembering words not presented in lists. Journal of Experimental Psychology: Learning, Memory, and Cognition, 21(4), 803-814]. This paradigm enabled the evaluation of the integration of false information and correct information with the original information. In Experiment 1, participants studied DRM lists, and in a later phase either retrieved or restudied the lists and were presented with never-presented critical lures. The results of Experiment 1 showed that compared to restudy, retrieval enhanced the integration of subsequent false information, as measured by later recall in a follow-up test. In Experiments 2 and 3, after initial study, participants retrieved or studied incorrect information and received corrective feedback. The results showed that retrieval led to more error correction than restudy, when feedback was presented immediately. In general, this research suggests retrieval facilitates incorporation of new, related information, regardless of whether it is false or correct.

Frequency of Fabry disease in a juvenile idiopathic arthritis cohort.

BACKGROUND: Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). During childhood, classic FD symptomatology is rare. The majority of children may show non-specific symptoms, including in the musculoskeletal system. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients is unknown. OBJECTIVE: This study aimed to identify the frequency of FD in a JIA cohort, characterizing early clinical symptoms, enzyme titers, and GLA genotyping. METHODS: Children with JIA followed in a tertiary Children Hospital cohort were selected. Clinical, laboratory and familiar information were recorded. Molecular genetic testing to detect GLA gene mutations was performed in girls and enzymatic analysis in boys. RESULTS: In 89 patients (56.2% female, age at disease onset: 8.93 ± 4.35 years), one male (1.12%) patient presented pathogenic mutation in GLA gene, c.1244 T > C p.L415P, one female patient had a variant of uncertain significance c.38C > T (p.Ala13Val). Three additional (3.4%) patients had the enzymatic activity of alpha-galactosidase slightly decreased. We observed the presence of intronic variants in 44.44% of patients in our cohort: c.1000-22C > T; c.370-81_-77del; c.640-16A > G; c.10C > T; c.548-125C > G and c.-12G > A. These variants and their combination were associated with clinical symptoms in our cohort. CONCLUSIONS: The incidence of FD in our cohort was 1.12%. Intronic variants were associated with symptoms previously described in the literature. Screening for FD in JIA may be a reasonable strategy for those with an atypical pattern of pain.

Interplay between different manual toothbrushes and brushing loads on erosive tooth wear.

OBJECTIVE: To investigate the effect of different types of manual toothbrushes and brushing loads on the progression of erosive tooth wear (ETW) on enamel. METHODS: Bovine enamel specimens (n = 10) were submitted to a 5-day erosive-abrasive cycling model (0.3 % citric acid for 5 min, artificial saliva for 60 min, 4x/day). Toothbrushing was carried out 2x/day for 15 s, according to the toothbrushes tested (ultra-soft (a): Curaprox 5460; ultra-soft (b): Sensodyne Repair & Protect; soft (a): Colgate Slim Soft; soft (b): Oral-B Indicator Plus; medium: Johnson's Professional; hard: Tek) and brushing loads (1.5 N, 3 N). Surface loss (SL, in µm) was assessed by optical profilometry on conclusion of the cycling. Some of the toothbrush characteristics were evaluated. Data were statistically analyzed (α = 0.05). RESULTS: For the 1.5 N load, the hard brush showed the highest SL value, with statistical significance. The other toothbrushes did not differ significantly, except that ultra-soft (a) caused significantly higher SL than ultra-soft (b). For the 3 N load, hard and soft (a) exhibited the highest SL. Soft (b) and medium had the lowest SL value, with statistical significance. Only soft (a) and ultra-soft (b) showed significant difference between loads, with lower SL for the load of 1.5 N. None of the toothbrush characteristics were significantly correlated with SL. CONCLUSIONS: Although different degrees of enamel surface loss were observed with use of the different toothbrushes, no association was found between the toothbrush characteristics and SL. Depending on the toothbrush, the force of brushing was capable of modulating the ETW of enamel. Based on the brushing loads usually applied by healthy individuals, hard brushes are not recommended for use by patients with ETW. CLINICAL SIGNIFICANCE: The use of hard bristle brushes is not recommended for use by individuals who exert healthy forces when brushing their teeth. The toothbrush characteristics are of secondary importance in terms of causing enamel loss in ETW.

Systemic inflammation as a risk factor for portal vein thrombosis in cirrhosis: a prospective longitudinal study.

BACKGROUND AND AIMS: Various risk factors for portal vein thrombosis (PVT) development in patients with cirrhosis have been identified, but the role of systemic inflammatory reaction is unknown. The study aims to assess the association between markers of systemic inflammation and PVT in cirrhosis. METHODS: Between January 2014 and October 2015, 107 outpatients with cirrhosis and no PVT were recruited, and followed till February 2017. White blood cell count, serum concentrations of high-sensitive C-reactive protein, ferritin, tumor necrosis factor-alpha and interleukin-6 (IL-6) were evaluated at baseline and every 3 or 6 months till PVT diagnosis or end of follow-up. RESULTS: Median age, model for end-stage liver disease (MELD) score and follow-up period of the studied population was 55 years (IQR 46-62 years), 9.6 points (IQR 7.5-12 points) and 19 months (12-24 months), respectively. PVT developed in 10.3% of the patients. Lymphocyte count below 1.2 ´ 109/L [hazard ratio, 6.04; 95% confidence interval (CI), 1.29-28.2; P = 0.022], IL-6 above 5.5 pg/mL (hazard ratio, 5.64; 95% CI, 1.21-26.33; P = 0.028) and neutrophil-to-lymphocyte ratio (hazard ratio, 1.46; 95% CI, 1.04-2.04; P = 0.028) were associated with a higher risk of PVT development. IL-6 and lymphopenia remained associated with subsequent PVT development after adjustment for nonselective beta-blockers, spleen size, portosystemic collaterals, oesophageal varices (grade ≥2) and ascites, but also with alcohol as the cause for cirrhosis and MELD ≥13. CONCLUSION: In patients with cirrhosis, markers of systemic inflammation IL-6 and lymphopenia are predictive of PVT independently of markers of portal hypertension. These results draw our attention on a factor so far overlooked in the pathogenesis of PVT.

Mechanical Properties of the Mandible in Postmenopausal Women Under Aledronate Treatment.

AIMS: This study evaluated the mechanical properties of mandibular bone tissue retrieved from postmenopausal women under alendronate treatment. METHODS: Twenty postmenopausal women were divided into two groups: healthy postmenopausal subjects (control group) and osteoporotic subjects treated with alendronate (alendronate group). Mandibular bone samples were retrieved with a trephine bur at the time of dental implant placement and fixed in 4% formalin. Samples were processed for hard tissue histology, and the bone surface was analyzed for nanohardness measurement. Nanohardness and elastic modulus were evaluated by using a Berkovich tip with elastic modulus of 1.016x106 MPa, Poisson coefficient of 0.3, and a load of 100 mN. Each cycle was configured with a load time of 18 seconds (speed of 1 mN/second), the discharge time of 18 seconds, and a rest time of 5 seconds during indentation at a depth of 10 µm. RESULTS: The control group presented the highest values for nanohardness and elastic modulus (p less than 0.05) in relation to the osteoporotic subjects. CONCLUSIONS: Within the limitations of the study, it can be concluded that treatment with alendronate negatively influenced the mechanical properties of mandibular bone in postmenopausal women by reducing bone nanohardness and elastic modulus.

Paraneoplastic neurological syndromes with onconeural antibodies: A single center retrospective study.

INTRODUCTION: Paraneoplastic neurological syndromes (PNS) are immune-mediated diseases that occur in patients with tumors and can be associated with onconeural antibodies. Our aim was to describe our cohort of patients with PNS. METHODS: Retrospective analysis of a cohort of patients followed in a Portuguese tertiary center, with autoantibodies against intracellular antigens from our PNS panel, between 2012 and 2017. RESULTS: Among the 882 patients with suspected PNS (1029 samples), 37 (4.2%) had positive and 27 (3.1%) weak positive antibodies. A total of 17 (29.3%) PNS were diagnosed, 5 were classic syndromes. Autoantibodies found were anti-Yo, anti-Hu, anti-titin, anti-Ma2, anti-SOX1, anti-Ri and anti-CV2/CRMP5. They were associated with thymoma, breast, colon, parotid gland and lung (small-cell, neuroendocrine or carcinoid) cancer. Among the 17 PNS patients, no tumor was found in 4 (mean follow-up of 46 months); no patients improved with tumor treatment, while 8 improved with immunotherapy; ten patients (59%) died during follow-up. Twenty (60.6%) patients with positive antibodies and 21 (84.0%) with weak positive were not diagnosed with a PNS. CONCLUSIONS: PNS had highly heterogenous clinical presentations. Response to tumor treatment was overall poor, with an unfavorable prognosis. In our cohort, only 29.3% of the patients with positive antibodies were diagnosed with a PNS.