Usefulness of somatosensory evoked potentials for monitoring the clinical course of patients with chronic inflammatory demyelinating polyradiculoneuropathy.

INTRODUCTION/AIMS: Somatosensory evoked potentials (SSEPs) are described as a supportive tool to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, there is a lack of studies determining the effectiveness of SSEPs in monitoring the clinical course of individuals with this condition. The aims of this study are to evaluate the utility of SSEPs in monitoring patients with CIDP and to assess their association with clinical outcomes following immunomodulatory therapy. METHODS: This was a single-center retrospective observational study that included patients who met European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP between 2018 and 2023. SSEPs were performed at diagnosis and during follow-up after the start of immunomodulatory treatment. Fisher's exact test was employed to assess the association between clinical improvement and SSEP improvement. RESULTS: Eighteen patients were included in the study. Ten patients had a typical CIDP pattern and 11 were male. In 17, SSEPs were abnormal prior to the start of immunomodulatory treatment. In patients who showed clinical improvement with immunomodulatory therapy, we observed that 15/17 had partial or complete improvement in SSEPs. Patients who showed no clinical improvement with first-line treatment exhibited worsening SSEPs. There was a significant association between clinical and SSEPs improvement (p = 0.009). DISCUSSION: We observed a positive association between improvement in SSEPs and clinical improvement in patients with CIDP. Our data suggest that SSEPs may be useful for monitoring the clinical course of patients with CIDP, but additional, larger studies are needed.

Effectiveness of handheld near infrared spectrometer for traceability of Angus steaks.

The feasibility of Near Infrared Spectroscopy was assessed for aging traceability of steaks of Angus beef (Biceps femoris) individually vacuum-packaged, as well as for the prediction of the refrigeration storage time (0, 7, and 14 days). For this purpose, a total of 288 steaks homogeneously distributed among the sampling times were used. The model developed by Partial Least Squares-Discriminant Analysis offered high discrimination ability between aged beef vs. non-aged. The accuracy after external validation exceeded 90%. Regarding the predictive capacity of the storage time, it was greater on the set of aged samples, in which the accuracy achieved values higher than 96%, while the accuracy decreased to 75% for the non-aged samples. Results obtained support the ability of NIRS technology to be considered in any digital transformation strategy for traceability across the meat supply chain.

NIRS (Near Infrared Spectroscopy) classification of sliced Duroc dry-cured ham under various packaging systems and storage temperature and time.

The potential of Near Infrared Spectroscopy (NIRS) was assessed for storage temperature discrimination (4 °C ± 2 vs. 20 °C ± 2) and for the prediction of the length of time that sliced Duroc dry-cured ham was in storage, considering the following packaging types; vacuum (n = 133) and modified atmosphere (MAP) (n = 133), without opening the package. The models, obtained by means of Partial least squares-discriminant analysis, indicated successful classification of the product according to storage temperature after validation (accuracy values of 100.00% in vacuum and between 92.00 and 100% in MAP). Furthermore, good accuracy was obtained for the assignments into storage times, with values comprised between 92.31% and 100.00% for samples under vacuum and between 91.00% and 97.00% for those under MAP, in both cases after validation. Thus, NIRS technology could help to support the preservation temperature traceability and the stocks of sliced dry-cured hams.

Limbic encephalitis secondary to neuro-Behcet disease: an uncommon presentation. / Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita.

INTRODUCTION: Limbic encephalitis (LE) can have a wide range of etiologies, most frequently infectious (especially viral) or autoimmune. Behcet's disease (BD) can present with heterogeneous neurological manifestations. However, LE is not considered a typical presentation of neuro-Behcet's disease (NBD). CASE REPORT: A 40-years-old male presented with new-onset subacute headaches, memory problems and apathy. A review of systems revealed an unrecorded past history of recurrent oral sores for years, recent malaise and fever, as well as an episode of bilateral panuveitis four months before presentation. His general and neurologic examination revealed slight fever, an isolated oral aphtha, anterograde amnesia and signs of bilateral retinal vasculitis. Brain magnetic resonance imaging displayed a pattern of limbic meningoencephalitis, and his cerebrospinal fluid showed mononuclear inflammation. The patient met BD diagnostic criteria. Considering LE is a very rare presentation of NBD, alternative etiologies were thoroughly assessed and excluded, including infectious, autoimmune and paraneoplastic encephalitis. Therefore, he was diagnosed with NBD, and he recovered well after immunosuppression. DISCUSSION: Only two cases of NBD presenting with LE have been previously reported. We report a third case of this rare presentation and compare it with the previous two. We aim to highlight this association and contribute to enlarge the rich clinical spectrum of NBD.

TITLE: Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita.Introducción. La encefalitis límbica (EL) puede tener un amplio abanico de etiologías, más frecuentemente la infecciosa (sobre todo viral) o autoinmune. La enfermedad de Behçet (EB) puede presentarse con manifestaciones neurológicas heterogéneas. Sin embargo, la EL no se considera una presentación típica del neuro-Behçet (NB). Caso clínico. Varón de 40 años que consultó por cefalea de novo subaguda, problemas de memoria y apatía. La anamnesis por sistemas reveló una historia no conocida previamente de aftas orales recurrentes durante años, fiebre y afectación general reciente, así como un episodio de panuveítis bilateral cuatro meses antes de la presentación. Su exploración general y neurológica reveló febrícula, una afta oral aislada, amnesia anterógrada y signos de vasculitis retiniana bilateral. La resonancia magnética mostró un patrón de afectación de meningoencefalitis límbica y su líquido cefalorraquídeo presentaba inflamación mononuclear. El paciente cumplía los criterios diagnósticos de la EB. Considerando que la EL es una presentación muy rara del NB, se buscaron exhaustivamente y se excluyeron otras etiologías alternativas, incluyendo las encefalitis infecciosas, autoinmunes y paraneoplásicas. En consecuencia, el paciente se diagnosticó de NB y mostró una buena recuperación con tratamiento inmunosupresor. Discusión. Sólo dos casos de NB con presentación en forma de EL se han publicado previamente. Comunicamos el tercer caso de esta rara manifestación clínica de la EB y lo comparamos con los dos anteriores, con el objetivo de destacar dicha asociación y contribuir a expandir el rico espectro clínico del NB.

[Prognostic and monitoring biomarkers in chronic inflammatory demyelinating polyneuropathy]. / Biomarcadores pronósticos y de seguimiento en la polineuropatía desmielinizante inflamatoria crónica.

INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a clinical entity with significant phenotypic variability both in its onset and in its course. Therefore, it is important to have objective biomarkers with which to monitor its evolution. In this review we present clinical, neurophysiological, neuroimaging, blood and cerebrospinal fluid (CSF) biomarkers for the monitoring and prognosis of CIDP. DEVELOPMENT: Different clinical tools have been developed and validated to monitor CIDP by assessing strength and disability. However, the best parameter for monitoring gait remains to be determined. Monitoring by neurophysiological examination is also widespread and the amplitude of the compound muscle action potential is the most commonly used. More recently, the Motor Unit Number Index sum score has been developed, which is an accurate and reproducible technique. The role of nerve ultrasonography is under development and a correlation between clinical evolution and ultrasound findings has been described. Multiple biomarkers have been described in blood and CSF, including antinodal/paranodal antibodies, neurofilament light chain, serum immunoglobulin G levels and CSF sphingomyelin levels. Genetic variants and cytokines associated with prognosis and response to treatment have also been described. CONCLUSIONS: One of the most important challenges in the management of CIDP is the monitoring of clinical changes after treatment initiation. The combination of biomarkers that allow an accurate understanding of the disease is crucial for the optimal management of CIDP.

TITLE: Biomarcadores pronósticos y de seguimiento en la polineuropatía desmielinizante inflamatoria crónica.Introducción. La polineuropatía desmielinizante inflamatoria crónica (PDIC) es una entidad clínica con una variabilidad fenotípica muy importante tanto en el inicio como en la evolución. Por lo tanto, es importante disponer de biomarcadores objetivos para monitorizar la evolución. En esta revisión presentamos los biomarcadores clínicos, neurofisiológicos, de neuroimagen, y en la sangre y el líquido cefalorraquídeo (LCR) para el seguimiento y el pronóstico de la PDIC. Desarrollo. Se han desarrollado diferentes herramientas clínicas validadas para el seguimiento de la PDIC mediante la evaluación de la fuerza y la discapacidad. No obstante, falta determinar el mejor parámetro para monitorizar la marcha. El seguimiento mediante examen neurofisiológico también está ampliamente extendido, y la amplitud del compound muscle action potential es lo más utilizado. Más recientemente, se ha desarrollado la Motor Unit Number Index sum score, que es una técnica precisa y reproducible. El papel de la ecografía de nervio se encuentra en desarrollo, y se ha descrito correlación entre la evolución clínica y los hallazgos por ecografía. Se han descrito múltiples biomarcadores en sangre y el LCR, entre los que destacan los anticuerpos antinodales/paranodales, los neurofilamentos de cadena ligera, los niveles de inmunoglobulina G en el suero y los niveles de esfingomielina en el LCR. Asimismo, se han descrito variantes genéticas y citocinas relacionadas con el pronóstico y la respuesta a los tratamientos. Conclusiones. Uno de los retos más importante en el manejo de la PDIC es la monitorización de los cambios clínicos tras el inicio del tratamiento. La combinación de biomarcadores que permitan una comprensión exacta de la enfermedad es crucial para el manejo óptimo de la PDIC.

[Guillain-Barre syndrome and thrombocytopenia after SARS-CoV-2 vaccination with Moderna. A case report]. / Síndrome de Guillain-Barré y trombocitopenia tras la vacunación contra el SARS-CoV-2 con Moderna. Descripción de un caso.

INTRODUCTION: The massive vaccination against the SARS-CoV-2 virus has demonstrated to be one of the major measures for the reduction of the morbidity and mortality that this virus causes. However, during the last months the administration of the vaccine has been also associated with some rare, but life-threatening, adverse effects. CASE REPORT: In this article we describe the case of a patient that developed a Guillain-Barre syndrome and an Idiopathic thrombocytopenic purpura nine days after the vaccination with the third dose for the SARS-CoV-2 virus (Moderna). He had received previously two doses of the AstraZeneca vaccine. Moreover, the patient was positive for auto-antibodies anti-SSA/Ro60 and auto-antibodies IgG anti-GM1 and IgG anti-GM3. DISCUSSION: Even though it is not possible to stablish a clear relation of causality between the administration of the vaccine booster for SARS-CoV-2 and the diseases developed by the patient, the association of two concomitant autoimmune processes is remarkable. As well as the positivity for the auto-antibodies anti-SSA/Ro60, which have been described in the bibliography in cases of SARS-CoV-2 infection.

TITLE: Síndrome de Guillain-Barré y trombocitopenia tras la vacunación contra el SARS-CoV-2 con Moderna. Descripción de un caso.Introducción. La vacunación masiva contra el virus SARS-CoV-2 constituye una de las principales estrategias en la reducción de la morbimortalidad que presenta dicho virus. No obstante, a lo largo de los últimos meses, su administración también se ha relacionado con diversos efectos adversos raros, pero potencialmente graves. Caso clínico. En el presente artículo describimos el caso de un paciente que desarrolló un síndrome de Guillain-Barré y una púrpura trombocitopénica idiopática nueve días después de la vacunación con la tercera dosis contra el virus SARS-CoV-2 (Moderna), con dos dosis previas de AstraZeneca. Adicionalmente, destaca la presencia de positividad para autoanticuerpos anti-SSA/Ro60 y para anticuerpos inmunoglobulina G anti-GM1 e inmunoglobulina G anti-GM3. Conclusión. Aunque no es posible establecer una relación de causalidad entre la administración del booster de la vacuna y el desarrollo de la enfermedad, es destacable la asociación de dos procesos autoinmunes concomitantes, junto con la positividad en los autoanticuerpos anti-SSA/Ro60, lo cual se ha descrito en la bibliografía en casos de infección del virus SARS-CoV-2.

Performance and carcass quality traits of Iberian × Duroc crossbred pig subject to gender and age at the beginning of the free-range finishing phase.

The traditional production of the Iberian breed pig involves a long production cycle. It might be shortened by using Iberian pigs crossed with Duroc and by reducing the growing phase, but the age-related changes on productive performance and carcass quality should be addressed. Thus, productive performance, live measurements and carcass and primal cut traits were evaluated on Iberian × Duroc 50:50 crossed pigs according to animal age at the beginning of the free-range finishing phase (Montanera): 10, 12 and 14 months old (IBxD10 (n = 15), IBxD12 (n = 17) and IBxD14 (n = 18) animal batches, respectively) and gender (immunologically castrated female -consisted of the Improvac® vaccination- and surgically castrated males). During the growing period, animals were fed with restrictions; 1.49, 1.29 and 1.20 ± 0.023 (mean ± SEM) kg/day of commercial feeds to start Montanera with similar BW; 103.9, 102.9 and 102.1 ± 0.22 kg, for IBxD10, IBxD12 and IBxD14, respectively. IBxD14 animals yielded the highest average daily gain (ADG) and BW after Montanera, as well as larger rump height and croup width. In contrast, these animals had the lowest carcass yield. Although animals from IBxD10 yielded hams of inferior size, this could be of interest to the sector, as there is a certain segment of the market that demands hams of smaller size and, generally, this is difficult to obtain with the traditional Montanera production system. The gender had no major effects on performance and carcass and primal cut traits, so both immunologically castrated female and surgically castrated males are suitable for finishing in Montanera.

Physico-chemical and sensory characterization of sliced Iberian chorizo from raw material of three commercial categories and stability during refrigerated storage packaged under vacuum and modified atmospheres.

Physico-chemical and sensory analysis of Iberian chorizo manufactured from three commercial categories raw material compiled in the current Spanish Iberian Quality standard (Black, Red; 100% Iberian and 50% Iberian × Duroc pigs, respectively, under Montanera, and White; 50% Iberian × Duroc pigs commercially fed) and packed under vacuum and modified atmosphere (MAP) were carried out, in order to address the influence of the genotype and animal production system and packaging on quality parameters, as well as the stability of these throughout long-term storage. Montanera categories showed higher values of γ-tocopherol, mono- and poly-unsaturated fatty acids, greater scores of flavour and taste and higher lipid oxidation during the whole storage. MAP preserved better colour, especially redness and lipid oxidative status. Iberian chorizo could attain a differentiated quality because of physico-chemical and sensorial characteristics derived from the Montanera that would be adequate preserved in both vacuum and MAP throughout long-term storage.

[Myotonic dystrophy type 1: a series of 107 patients]. / Distrofia miotónica de tipo 1: una serie de 107 pacientes.

INTRODUCTION: Myotonic dystrophy type 1 is the most common muscular dystrophy in adults. It is a genetic disorder of autosomal dominant inheritance and one of its most striking features is its multi-systemic involvement with a wide clinical phenotype. PATIENTS AND METHODS: Data from 107 patients with a genetically confirmed diagnosis of the disease were retrospectively analysed from the database of a national reference division for neuromuscular diseases. Demographic and clinical data were collected over a 7-year period. RESULTS: The most frequent age of symptom onset was adulthood (66.4%). 35% showed exclusive distal weakness and a majority (63.6%) had clinical myotonia. Only 10 patients lacked neuromuscular symptoms at diagnosis and up to 9.5% were restricted to a wheelchair. The implantation of a pacemaker or cardioverter-defibrillator was conducted in 16 patients but no sudden cardiac death was detected. A venous thromboembolic disease incidence rate of 5.6 cases per 1000 patient-year was identified. More than half of the patients (54%) in the series developed respiratory failure. 13 patients died during the follow-up period, with respiratory failure being the main cause of death. CONCLUSIONS: The follow-up and clinical management of patients with DM1 should be multidisciplinary. In our series, the main cause of morbidity and mortality was respiratory disorders, whereas the incidence of cardiac disorders was lower. In addition, there is a notable frequency of complications derived from falls, which can have serious consequences. Finally, a higher than expected incidence of thromboembolic events was identified, which deserves further study in other cohorts of patients.

TITLE: Distrofia miotónica de tipo 1: una serie de 107 pacientes.Introducción. La distrofia miotónica de tipo 1 (DM1) es la distrofia muscular más frecuente en adultos, aunque puede comenzar a cualquier edad. Genéticamente determinada y de transmisión dominante, se caracteriza por la afectación constante, aunque variable, de múltiples sistemas. Pacientes y métodos. Se analizaron retrospectivamente datos de 107 pacientes con diagnóstico genético de DM1 en seguimiento en una unidad de referencia nacional en enfermedades neuromusculares raras. Se recopilaron datos demográficos y clínicos de un período de seguimiento de siete años. Resultados. El 66,4% de los pacientes comenzó en la edad adulta. El 35,5% tenía debilidad distal exclusiva y la mayoría (63,6%) presentaba miotonía clínica. Sólo 10 pacientes no tenían síntomas neuromusculares en el diagnóstico. En un 8,6%, las caídas ocasionaron complicaciones graves y hasta un 9,5% perdió la deambulación autónoma. Se implantó un dispositivo cardíaco en 16 pacientes y no se registró ninguna muerte súbita de origen cardíaco. Se identificó una tasa de incidencia de enfermedad tromboembólica venosa de 5,6 casos/1.000 pacientes-año. Un 54% de los pacientes desarrolló insuficiencia respiratoria. Durante el seguimiento fallecieron 13 pacientes y la insuficiencia respiratoria fue la principal causa de muerte (38,5%). Conclusiones. El manejo clínico y el seguimiento de los pacientes con DM1 debe ser multidisciplinar. En nuestra serie, la principal causa de morbimortalidad fueron los trastornos respiratorios, mientras que la incidencia de complicaciones cardiológicas graves fue baja. Destacan, además, las complicaciones derivadas de las caídas, que pueden tener consecuencias graves. Finalmente, se identificó una incidencia mayor de la esperada de eventos tromboembólicos, que merece ser estudiada en mayor profundidad.

What functional proteomic and biochemical analysis tell us about animal stress in beef?

Animal management practices may influence the animal's susceptibility to stress, with detrimental effects on the ultimate meat quality. In this field, proteomics is a promising tool that reveals the biological pathways underpinning the effect of animal's pre-slaughter stress (PSS) on the ultimate meat quality. The objective of this work was to study the effect of a pre-slaughter procedure that may promote stress, such as mixing unfamiliar animals during the transport and lairage period, on the post-mortem muscle proteome of young bulls reared under two different farm management systems (Intensive or Extensive). Comparative proteomics and biochemical analysis reveal the effect of PSS on biochemical pathways involved in the meat colour development, muscle redox status, energy metabolism and autophagy. This work highlights the potential of some muscle proteins such as Beclin-1 (autophagy marker), CKM (biomarker of energy transduction) and proteins of the energy metabolism (ALDOA, PYGM, PGM1, PKM, GPI) as potential biomarkers to discriminate beef samples according to the incidence of PSS. SIGNIFICANCE: Meat scientists are interested in the study of individual animal-based measurements that allow the detection of stress situations which could have negative effects on meat quality. In this context, the mechanisms underlying the adverse effects of pre-slaughter stress on the post-mortem muscle metabolism need to be elucidated in order to understand how animal stress may influence the conversion of muscle into meat. In this work, the study of proteome changes in the post-mortem muscle has allowed the identification of significant biomarkers of these processes that could be used as tools for detecting inappropriate strategies that may induce increased animal stress and, in consequence, may compromise the ultimate beef quality.

Is production system a relevant attribute in consumers' food preferences? The case of Iberian dry-cured ham in Spain.

Spanish consumers have a strong preference for Iberian meat products, as they perceive them to be of extra sensorial and nutritional quality. The production of these meat products depends on multiple variables, such as genetics, livestock production systems and, above all, the feed provided. The aim of this paper is to study the preferences of Spanish consumers for the various types of Iberian dry-cured ham, analysing whether they are willing to pay the premium required by the highest-quality products. The methodological approach combined a sensory analysis and a choice-based conjoint experiment with obtained through tasting sessions in Extremadura (SW of Spain). Findings of the sensory test have shown that there are significant differences in odour, texture and taste, explained mainly by the type of feed pigs were fed. The main results of the choice experiment have also shown that the type of feed is the most preferred attribute by consumers, in line with the sensory analysis.

Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita / Limbic encephalitis secondary to neuro-Behçet disease: an uncommon presentation

Introducción: La encefalitis límbica (EL) puede tener un amplio abanico de etiologías, más frecuentemente la infecciosa (sobre todo viral) o autoinmune. La enfermedad de Behçet (EB) puede presentarse con manifestaciones neurológicas heterogéneas. Sin embargo, la EL no se considera una presentación típica del neuro-Behçet (NB). Caso clínico: Un varón de 40 años consultó por cefalea de novo subaguda, problemas de memoria y apatía. La anamnesis por sistemas reveló una historia no conocida previamente de aftas orales recurrentes durante años, fiebre y afectación general reciente, así como un episodio de panuveítis bilateral cuatro meses antes de la presentación. Su exploración general y neurológica reveló febrícula, una afta oral aislada, amnesia anterógrada y signos de vasculitis retiniana bilateral. La resonancia magnética mostró un patrón de afectación de meningoencefalitis límbica y su líquido cefalorraquídeo presentaba inflamación mononuclear. El paciente cumplía los criterios diagnósticos de la EB. Considerando que la EL es una presentación muy rara del NB, se buscaron exhaustivamente y se excluyeron otras etiologías alternativas, incluyendo las encefalitis infecciosas, autoinmunes y paraneoplásicas. En consecuencia, el paciente se diagnosticó de NB y mostró una buena recuperación con tratamiento inmunosupresor. Discusión: Sólo dos casos de NB con presentación en forma de EL se han publicado previamente. Comunicamos el tercer caso de esta rara manifestación clínica de la EB y lo comparamos con los dos anteriores, con el objetivo de destacar dicha asociación y contribuir a expandir el rico espectro clínico del NB.(AU)

Introduction: Limbic encephalitis (LE) can have a wide range of etiologies, most frequently infectious (especially viral) or autoimmune. Behçet’s disease (BD) can present with heterogeneous neurological manifestations. However, LE is not considered a typical presentation of neuro-Behçet’s disease (NBD). Case report: A 40-years-old male presented with new-onset subacute headaches, memory problems and apathy. A review of systems revealed an unrecorded past history of recurrent oral sores for years, recent malaise and fever, as well as an episode of bilateral panuveitis four months before presentation. His general and neurologic examination revealed slight fever, an isolated oral aphtha, anterograde amnesia and signs of bilateral retinal vasculitis. Brain magnetic resonance imaging displayed a pattern of limbic meningoencephalitis, and his cerebrospinal fluid showed mononuclear inflammation. The patient met BD diagnostic criteria. Considering LE is a very rare presentation of NBD, alternative etiologies were thoroughly assessed and excluded, including infectious, autoimmune and paraneoplastic encephalitis. Therefore, he was diagnosed with NBD, and he recovered well after immunosuppression. Discussion: Only two cases of NBD presenting with LE have been previously reported. We report a third case of this rare presentation and compare it with the previous two. We aim to highlight this association and contribute to enlarge the rich clinical spectrum of NBD.(AU)

Biomarcadores pronósticos y de seguimiento en la polineuropatía desmielinizante inflamatoria crónica / Prognostic and monitoring biomarkers in chronic inflammatory demyelinating polyneuropathy

Introducción: La polineuropatía desmielinizante inflamatoria crónica (PDIC) es una entidad clínica con una variabilidad fenotípica muy importante tanto en el inicio como en la evolución. Por lo tanto, es importante disponer de biomarcadores objetivos para monitorizar la evolución. En esta revisión presentamos los biomarcadores clínicos, neurofisiológicos, de neuroimagen, y en la sangre y el líquido cefalorraquídeo (LCR) para el seguimiento y el pronóstico de la PDIC. Desarrollo: Se han desarrollado diferentes herramientas clínicas validadas para el seguimiento de la PDIC mediante la evaluación de la fuerza y la discapacidad. No obstante, falta determinar el mejor parámetro para monitorizar la marcha. El seguimiento mediante examen neurofisiológico también está ampliamente extendido, y la amplitud del compound muscle action potential es lo más utilizado. Más recientemente, se ha desarrollado la Motor Unit Number Index sum score, que es una técnica precisa y reproducible. El papel de la ecografía de nervio se encuentra en desarrollo, y se ha descrito correlación entre la evolución clínica y los hallazgos por ecografía. Se han descrito múltiples biomarcadores en sangre y el LCR, entre los que destacan los anticuerpos antinodales/paranodales, los neurofilamentos de cadena ligera, los niveles de inmunoglobulina G en el suero y los niveles de esfingomielina en el LCR. Asimismo, se han descrito variantes genéticas y citocinas relacionadas con el pronóstico y la respuesta a los tratamientos. Conclusiones: Uno de los retos más importante en el manejo de la PDIC es la monitorización de los cambios clínicos tras el inicio del tratamiento. La combinación de biomarcadores que permitan una comprensión exacta de la enfermedad es crucial para el manejo óptimo de la PDIC.(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a clinical entity with significant phenotypic variability both in its onset and in its course. Therefore, it is important to have objective biomarkers with which to monitor its evolution. In this review we present clinical, neurophysiological, neuroimaging, blood and cerebrospinal fluid (CSF) biomarkers for the monitoring and prognosis of CIDP. Development: Different clinical tools have been developed and validated to monitor CIDP by assessing strength and disability. However, the best parameter for monitoring gait remains to be determined. Monitoring by neurophysiological examination is also widespread and the amplitude of the compound muscle action potential is the most commonly used. More recently, the Motor Unit Number Index sum score has been developed, which is an accurate and reproducible technique. The role of nerve ultrasonography is under development and a correlation between clinical evolution and ultrasound findings has been described. Multiple biomarkers have been described in blood and CSF, including antinodal/paranodal antibodies, neurofilament light chain, serum immunoglobulin G levels and CSF sphingomyelin levels. Genetic variants and cytokines associated with prognosis and response to treatment have also been described. Conclusions: One of the most important challenges in the management of CIDP is the monitoring of clinical changes after treatment initiation. The combination of biomarkers that allow an accurate understanding of the disease is crucial for the optimal management of CIDP.(AU)

Síndrome de Guillain-Barré y trombocitopenia tras la vacunación contra el SARS-CoV-2 con Moderna. Descripción de un caso / Guillain-Barré syndrome and thrombocytopenia after SARS-CoV-2 vaccination with Moderna. A case report

Introducción: La vacunación masiva contra el virus SARS-CoV-2 constituye una de las principales estrategias en la reducción de la morbimortalidad que presenta dicho virus. No obstante, a lo largo de los últimos meses, su administración también se ha relacionado con diversos efectos adversos raros, pero potencialmente graves. Caso clínico: En el presente artículo describimos el caso de un paciente que desarrolló un síndrome de Guillain-Barré y una púrpura trombocitopénica idiopática nueve días después de la vacunación con la tercera dosis contra el virus SARS-CoV-2 (Moderna), con dos dosis previas de AstraZeneca. Adicionalmente, destaca la presencia de positividad para autoanticuerpos anti-SSA/Ro60 y para anticuerpos inmunoglobulina G anti-GM1 e inmunoglobulina G anti-GM3. Conclusión: Aunque no es posible establecer una relación de causalidad entre la administración del booster de la vacuna y el desarrollo de la enfermedad, es destacable la asociación de dos procesos autoinmunes concomitantes, junto con la positividad en los autoanticuerpos anti-SSA/Ro60, lo cual se ha descrito en la bibliografía en casos de infección del virus SARS-CoV-2.(AU)

Introduction: The massive vaccination against the SARS-CoV-2 virus has demonstrated to be one of the major measures for the reduction of the morbidity and mortality that this virus causes. However, during the last months the administration of the vaccine has been also associated with some rare, but life-threatening, adverse effects. Case report: In this article we describe the case of a patient that developed a Guillain-Barré syndrome and an Idiopathic thrombocytopenic purpura nine days after the vaccination with the third dose for the SARS-CoV-2 virus (Moderna). He had received previously two doses of the AstraZeneca vaccine. Moreover, the patient was positive for auto-antibodies anti-SSA/Ro60 and auto-antibodies IgG anti-GM1 and IgG anti-GM3. Discussion: Even though it is not possible to stablish a clear relation of causality between the administration of the vaccine booster for SARS-CoV-2 and the diseases developed by the patient, the association of two concomitant autoimmune processes is remarkable. As well as the positivity for the auto-antibodies anti-SSA/Ro60, which have been described in the bibliography in cases of SARS-CoV-2 infection.(AU)

Distrofia miotónica de tipo 1: una serie de 107 pacientes / Myotonic dystrophy type 1: a series of 107 patients

Introducción: La distrofia miotónica de tipo 1 (DM1) es la distrofia muscular más frecuente en adultos, aunque puede comenzar a cualquier edad. Genéticamente determinada y de transmisión dominante, se caracteriza por la afectación constante, aunque variable, de múltiples sistemas. Pacientes y métodos: Se analizaron retrospectivamente datos de 107 pacientes con diagnóstico genético de DM1 en seguimiento en una unidad de referencia nacional en enfermedades neuromusculares raras. Se recopilaron datos demográficos y clínicos de un período de seguimiento de siete años. Resultados: El 66,4% de los pacientes comenzó en la edad adulta. El 35,5% tenía debilidad distal exclusiva y la mayoría (63,6%) presentaba miotonía clínica. Sólo 10 pacientes no tenían síntomas neuromusculares en el diagnóstico. En un 8,6%, las caídas ocasionaron complicaciones graves y hasta un 9,5% perdió la deambulación autónoma. Se implantó un dispositivo cardíaco en 16 pacientes y no se registró ninguna muerte súbita de origen cardíaco. Se identificó una tasa de incidencia de enfermedad tromboembólica venosa de 5,6 casos/1.000 pacientes-año. Un 54% de los pacientes desarrolló insuficiencia respiratoria. Durante el seguimiento fallecieron 13 pacientes y la insuficiencia respiratoria fue la principal causa de muerte (38,5%). Conclusiones: El manejo clínico y el seguimiento de los pacientes con DM1 debe ser multidisciplinar. En nuestra serie, la principal causa de morbimortalidad fueron los trastornos respiratorios, mientras que la incidencia de complicaciones cardiológicas graves fue baja. Destacan, además, las complicaciones derivadas de las caídas, que pueden tener consecuencias graves. Finalmente, se identificó una incidencia mayor de la esperada de eventos tromboembólicos, que merece ser estudiada en mayor profundidad.(AU)

Introduction: Myotonic dystrophy type 1 is the most common muscular dystrophy in adults. It is a genetic disorder of autosomal dominant inheritance and one of its most striking features is its multi-systemic involvement with a wide clinical phenotype. Patients and methods: Data from 107 patients with a genetically confirmed diagnosis of the disease were retrospectively analysed from the database of a national reference division for neuromuscular diseases. Demographic and clinical data were collected over a 7-year period. Results: The most frequent age of symptom onset was adulthood (66.4%). 35% showed exclusive distal weakness and a majority (63.6%) had clinical myotonia. Only 10 patients lacked neuromuscular symptoms at diagnosis and up to 9.5% were restricted to a wheelchair. The implantation of a pacemaker or cardioverter-defibrillator was conducted in 16 patients but no sudden cardiac death was detected. A venous thromboembolic disease incidence rate of 5.6 cases per 1000 patient-year was identified. More than half of the patients (54%) in the series developed respiratory failure. 13 patients died during the follow-up period, with respiratory failure being the main cause of death. Conclusions: The follow-up and clinical management of patients with DM1 should be multidisciplinary. In our series, the main cause of morbidity and mortality was respiratory disorders, whereas the incidence of cardiac disorders was lower. In addition, there is a notable frequency of complications derived from falls, which can have serious consequences. Finally, a higher than expected incidence of thromboembolic events was identified, which deserves further study in other cohorts of patients.(AU)

Changes of pH produced by immersion of endodontic sealers.

When evaluating the antimicrobial effect of endodontic sealers it is important to study the variation of pH, since this is one of the properties that may inhibit the microbial growth and influence biocompatibility. The aim of this study was to evaluate the capacity of several endodontic sealers without calcium hydroxide to modify the pH of the solution in which they were immersed. The sealers used were: Endomethasone C-Septodont (EC), Bio Seal-Ogna (BS), Diaket-Espe (D), Procosol-StarDental (P), AH26-Dentsply (AH), Ketac Endo-Espe (KE), AHPlus-Dentsply (AHP), Endion-Voco (E), Endomethasone-Septodont (EM), EZ Fill-Essential Dental Systems (EZ). Samples of each material were placed in tubes with phosphate saline solution (PBS). The pH was determined at 2, 5, 12, 20, 30, 60, and 90 days. A tube with solution alone was taken as control. Statistical analysis of the results was performed by ANOVA. Material, sample, time and material-time interaction elicited statistically significant differences (p < 0.05). The variation of pH produced by immersion of endodontic sealers depends on the composition of the material.

Study of variability in antioxidant composition and fatty acids profile of Longissimus dorsi and Serratus ventralis muscles from Iberian pigs reared in two different Montanera seasons.

This investigation was designed to evaluate the effects of variations in antioxidant and fatty acids composition of acorns and grass from two Montanera (free-range system and feeding based on acorns and grass) seasons (2006/07 and 2007/08) on the antioxidant composition and fatty acids profile of m. Longissimus dorsi (LD) and m. Serratus ventralis (SV) from Iberian pigs reared under these Montanera seasons. Acorn and grass composition was affected by Montanera season and consequently, LD and SV muscles showed different contents of α-tocopherol, total phenols, hydrophilic and lipophilic antioxidant activity and fatty acid profile, according with the composition of acorns and grass ingested. Results suggest a lack of uniformity in meat quality between different seasons. This could be due to the variable nature of extensive pig production as reflected in the variability in the composition of the diet (acorns and grass).

Effect of production system on physical-chemical, antioxidant and fatty acids composition of Longissimus dorsi and Serratus ventralis muscles from Iberian pig.

The effect of three production systems of Iberian pigs namely Montanera (free-range system and feeding based on acorns and grass), Recebo (free-range system and nutrition based in combination of acorns, grass and mixed feeds) and Intensive (confinement with mixed feeds) on some quality traits of Longissimus dorsi (LD) and Serratus ventralis (SV) muscles were studied. Muscles from pigs raised in the Montanera system showed significantly higher CIE L(∗), a(∗) and b(∗) values and higher haem pigment content than those from Intensive system. Similarly, muscles from pigs raised in the Montanera system had significantly higher contents of α and γ-tocopherol and phenolic compounds contents and higher lipophilic and hydrophilic activity antioxidant than those from pigs raised in the Intensive system. Fatty acids profiles from Montanera pigs had significantly higher monounsaturated (MUFA) and polyunsaturated (PUFA) fatty acids and lower saturated fatty acids (SFA) than those from pigs raised in the Intensive system. In relation to muscle effect, LD showed lower intramuscular fat (IMF), α-tocopherol, phenolic compounds, lipid oxidation and PUFA, but higher MUFA than SV.

Changes of pH produced by immersion of endodontic sealers

When evaluating the antimicrobial effect of endodontic sealers it is important to study the variation of pH, since this is one of the properties that may inhibit the microbial growth and influence biocompatibility. The aim of this study was to evaluate the capacity of several endodontic sealers without calcium hydroxide to modify the pH of the solution in which they were immersed. The sealers used were: Endomethasone C-Septodont (EC), Bio Seal-Ogna (BS), Diaket-Espe (D), Procosol-StarDental (P), AH26-Dentsply (AH), Ketac Endo-Espe (KE), AHPlus-Dentsply (AHP), Endion-Voco (E), Endomethasone-Septodont (EM), EZ Fill-Essential Dental Systems (EZ). Samples of each material were placed in tubes with phosphate saline solution (PBS). The pH was determined at 2, 5, 12, 20, 30, 60, and 90 days. A tube with solution alone was taken as control. Statistical analysis of the results was performed by ANOVA. Material, sample, time and material-time interaction elicited statistically significant differences (p < 0.05). The variation of pH produced by immersion of endodontic sealers depends on the composition of the material.