Retinal sensitivity and gaze fixation evaluated by microperimetry in subjects with type 2 diabetes: two independent parameters that explore different neuronal circuits.

BACKGROUND AND AIMS: Retinal sensitivity (RS) and gaze fixation (GF) assessed by retinal microperimetry are useful and complementary tools for identifying mild cognitive impairment (MCI) in patients with type 2 diabetes (T2D). The hypothesis is that RS and GF examine different neural circuits: RS depends only on the visual pathway while GF reflects white matter complex connectivity networks. The aim of the study is to shed light to this issue by examining the relationship of these two parameters with visual evoked potentials (VEP), the current gold standard to examine the visual pathway. MATERIALS AND METHODS: Consecutive T2D patients > 65 years were recruited from the outpatient clinic. Retinal microperimetry (MAIA 3rd generation) and visual evoked potentials (VEP) (Nicolet Viking ED). RS (dB), GF (BCEA63%, BCEA95%) (MAIA) and VEP (Latency P100ms, Amplitude75-100 uV) were analyzed. RESULTS: Thirty three patients (45% women, 72.1 ± 4.6 years) were included. VEP parameters significantly correlated with RS but not with GF. CONCLUSIONS: These results confirm that RS but not GF depends on the visual pathway, reinforcing the concept that they are complementary diagnostic tools. Used together can further increase the value of microperimetry as screening test for identifying T2D population with cognitive impairment.

[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.

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)

Debilidad como complicación del paciente crítico por COVID-19: características clínicas y factores pronósticos en una serie de casos / Weakness as a complication of COVID-19 in critically ill patients: clinical features and prognostic factors in a case series

Introducción: La debilidad es una complicación frecuente en el enfermo crítico por COVID-19. Se describen sus características, y los factores que pueden condicionarla y predecirla. Pacientes y métodos: Estudio observacional descriptivo prospectivo con pacientes ingresados en la unidad de cuidados intensivos (UCI) por COVID-19 entre abril y mayo de 2020 con debilidad muscular. Se consideró una afectación clínica grave un equilibrio motor igual o inferior a 3/5 según la escala de fuerza muscular modificada del Medical Research Council. Se han realizado 25 estudios analíticos, 16 estudios neurofisiológicos y una biopsia muscular; seguimiento telefónico al mes; análisis comparativo entre los grupos con y sin afectación grave, y determinación de puntos de corte de parámetros analíticos para predecir afectación grave mediante curvas ROC. Resultados: Se incluyó a 25 pacientes con 58 años (desviación estándar ± 9) de edad media. La mediana de estancia en la UCI fue de 27,5 días. Todos los electromiogramas mostraban un patrón miógeno y el 75%, también una neuropatía. El grupo con afectación clínica grave tenía mayores niveles de dímero-D (p = 0,08), lactato deshidrogenasa (p = 0,03) e interleucina 6 (p = 0,10), y la combinación de la alteración de dos cualquiera de estos tres parámetros pronosticaba la afectación grave con una sensibilidad del 100% y una especificidad del 76,9%. Al mes de seguimiento, el 36% no podía deambular autónomamente y el 92% seguía con debilidad muscular. Conclusiones: La debilidad en el enfermo por COVID-19 grave tiene una repercusión clínica importante. Su detección y estudio precoces mediante predictores de su desarrollo pueden permitir un mejor manejo. La ausencia en algunos casos de los factores de riesgo clásicos para la debilidad adquirida en la UCI sugiere una fisiopatología diferente.(AU)

Introduction: Weakness is a frequent complication in those critically ill due to COVID-19. This study describes its characteristics and the factors that can condition and predict it. Patients and methods: We conducted a prospective, descriptive, observational study of patients admitted to the intensive care unit (ICU) due to COVID-19 between April and May 2020 with muscle weakness. A motor balance equal to or lower than 3/5 according to the modified Medical Research Council muscle strength scale was considered to be severe clinical impairment. Altogether 25 analytical studies, 16 neurophysiological studies and one muscle biopsy were performed, with a telephone follow-up at one month, a comparative analysis between the groups with and without severe compromise, and determination of cut-off points for analytical parameters to predict severe involvement using ROC curves. Results: The sample consisted of 25 patients with a mean age of 58 years (standard deviation ± 9). The median length of stay in the ICU was 27.5 days. All the electromyograms exhibited a myogenic pattern and 75% also showed neuropathy. The group with severe clinical involvement had higher levels of D-dimer (p = 0.08), lactate dehydrogenase (p = 0.03) and interleukin-6 (p = 0.10), and the combination of the alteration of any two of these three parameters predicted severe involvement with a sensitivity of 100% and a specificity of 76.9%. At one month of follow-up, 36% were unable to walk autonomously and 92% continued with muscle weakness. Conclusions: Weakness in severe COVID-19 patients has a major clinical impact. Its early detection and study by means of predictors of its development may allow for better management. The absence in some cases of classical risk factors for ICU-acquired weakness suggests a different pathophysiology.(AU)

[Weakness as a complication of COVID-19 in critically ill patients: clinical features and prognostic factors in a case series]. / Debilidad como complicación del paciente crítico por COVID-19: características clínicas y factores pronósticos en una serie de casos.

INTRODUCTION: Weakness is a frequent complication in those critically ill due to COVID-19. This study describes its characteristics and the factors that can condition and predict it. PATIENTS AND METHODS: We conducted a prospective, descriptive, observational study of patients admitted to the intensive care unit (ICU) due to COVID-19 between April and May 2020 with muscle weakness. A motor balance equal to or lower than 3/5 according to the modified Medical Research Council muscle strength scale was considered to be severe clinical impairment. Altogether 25 analytical studies, 16 neurophysiological studies and one muscle biopsy were performed, with a telephone follow-up at one month, a comparative analysis between the groups with and without severe compromise, and determination of cut-off points for analytical parameters to predict severe involvement using ROC curves. RESULTS: The sample consisted of 25 patients with a mean age of 58 years (standard deviation ± 9). The median length of stay in the ICU was 27.5 days. All the electromyograms exhibited a myogenic pattern and 75% also showed neuropathy. The group with severe clinical involvement had higher levels of D-dimer (p = 0.08), lactate dehydrogenase (p = 0.03) and interleukin-6 (p = 0.10), and the combination of the alteration of any two of these three parameters predicted severe involvement with a sensitivity of 100% and a specificity of 76.9%. At one month of follow-up, 36% were unable to walk autonomously and 92% continued with muscle weakness. CONCLUSIONS: Weakness in severe COVID-19 patients has a major clinical impact. Its early detection and study by means of predictors of its development may allow for better management. The absence in some cases of classical risk factors for ICU-acquired weakness suggests a different pathophysiology.

TITLE: Debilidad como complicación del paciente crítico por COVID-19: características clínicas y factores pronósticos en una serie de casos.Introducción. La debilidad es una complicación frecuente en el enfermo crítico por COVID-19. Se describen sus características, y los factores que pueden condicionarla y predecirla. Pacientes y métodos. Estudio observacional descriptivo prospectivo con pacientes ingresados en la unidad de cuidados intensivos (UCI) por COVID-19 entre abril y mayo de 2020 con debilidad muscular. Se consideró una afectación clínica grave un equilibrio motor igual o inferior a 3/5 según la escala de fuerza muscular modificada del Medical Research Council. Se han realizado 25 estudios analíticos, 16 estudios neurofisiológicos y una biopsia muscular; seguimiento telefónico al mes; análisis comparativo entre los grupos con y sin afectación grave, y determinación de puntos de corte de parámetros analíticos para predecir afectación grave mediante curvas ROC. Resultados. Se incluyó a 25 pacientes con 58 años (desviación estándar ± 9) de edad media. La mediana de estancia en la UCI fue de 27,5 días. Todos los electromiogramas mostraban un patrón miógeno y el 75%, también una neuropatía. El grupo con afectación clínica grave tenía mayores niveles de dímero-D (p = 0,08), lactato deshidrogenasa (p = 0,03) e interleucina 6 (p = 0,10), y la combinación de la alteración de dos cualquiera de estos tres parámetros pronosticaba la afectación grave con una sensibilidad del 100% y una especificidad del 76,9%. Al mes de seguimiento, el 36% no podía deambular autónomamente y el 92% seguía con debilidad muscular. Conclusiones. La debilidad en el enfermo por COVID-19 grave tiene una repercusión clínica importante. Su detección y estudio precoces mediante predictores de su desarrollo pueden permitir un mejor manejo. La ausencia en algunos casos de los factores de riesgo clásicos para la debilidad adquirida en la UCI sugiere una fisiopatología diferente.

Recomendaciones para la utilización clínica del estudio de potenciales evocados motores en la esclerosis múltiple / Recommendations for the clinical use of motor evoked potentials in multiple sclerosis

Objetivo: Establecer una guía clínica para la utilización clínica del estudio de potenciales evocados motores (PEM) en el diagnóstico y el seguimiento de la esclerosis múltiple (EM). Disponer de unas recomendaciones para la utilización clínica de los PEM contribuye a racionalizar y optimizar los recursos en el proceso diagnóstico y de seguimiento en los pacientes con EM. Método: Hemos llevado a cabo una extensa revisión de la literatura médica y puesto en común nuestros propios datos para consensuar recomendaciones para el uso clínico de los PEM en el estudio de la EM. Resultados: Los PEM contribuyen, junto con la resonancia magnética medular o cerebral, al diagnóstico y evaluación de los pacientes cuyo inicio clínico es un síndrome medular, que presentan hallazgos de neuroimagen poco específicos o que presentan criterios clínicos de EM con neuroimagen cerebral normal. Conclusiones: Es aconsejable realizar un estudio de potenciales evocados multimodales en pacientes con sospecha de EM para documentar la afectación de la vía motora como apoyo al diagnóstico de diseminación en espacio (AU)

Objective: To establish clinical guidelines for the clinical use and interpretation of motor evoked potentials (MEP) in diagnosing and monitoring patients with multiple sclerosis (MS). Recommendations for MEP use and interpretation will help us rationalise and optimise resources used in MS patient diagnosis and follow up. Method: We completed an extensive literature review and pooled our own data to produce a consensus statement with recommendations for the clinical use of MEPs in the study of MS. Results: MEPs, in addition to spinal and cranial magnetic resonance imaging (MRI), help us diagnose and assess MS patients whose disease initially presents as spinal cord syndrome and those with non-specific brain MRI findings, or a normal brain MRI and clinical signs of MS. Conclusions: Whenever possible, a multimodal evoked potential study should be performed on patients with suspected MS in order to demonstrate involvement of the motor pathway which supports a diagnosis of dissemination in space (AU)

[Recommendations for the clinical use of motor evoked potentials in multiple sclerosis]. / Recomendaciones para la utilización clínica del estudio de potenciales evocados motores en la esclerosis múltiple.

OBJECTIVE: To establish clinical guidelines for the clinical use and interpretation of motor evoked potentials (MEP) in diagnosing and monitoring patients with multiple sclerosis (MS). Recommendations for MEP use and interpretation will help us rationalise and optimise resources used in MS patient diagnosis and follow up. METHOD: We completed an extensive literature review and pooled our own data to produce a consensus statement with recommendations for the clinical use of MEPs in the study of MS. RESULTS: MEPs, in addition to spinal and cranial magnetic resonance imaging (MRI), help us diagnose and assess MS patients whose disease initially presents as spinal cord syndrome and those with non-specific brain MRI findings, or a normal brain MRI and clinical signs of MS. CONCLUSIONS: Whenever possible, a multimodal evoked potential study should be performed on patients with suspected MS in order to demonstrate involvement of the motor pathway which supports a diagnosis of dissemination in space.

Phenotypic variability in a Spanish family with MNGIE.

Clinical, biochemical, and genetic features of a Spanish family with mitochondrial neurogastrointestinal encephalomyopathy are reported. The proband presented with severe gastrointestinal dysmotility and the affected sister had extraocular muscle weakness. In both affected individuals, biochemical defects of thymidine phosphorylase and a pathogenic G-to-A transition mutation at nucleotide 435 in the thymidine phosphorylase gene were identified. The first thymidine phosphorylase mutation identified in Spain showed phenotypic variability at onset.

Parálisis facial de bell recurrente: nuestra experiencia / Recurrent bell's palsy: our experience

La parálisis facial recurrente se describe raramente en la literatura. Presentamos un estudio comparativo de la parálisis facial idiopática en sus formas de presentación única (PFIU) y recurrente (PFIR). Asimismo comparamos entre las formas recurrentes las que son recurrentes ipsilaterales (PFRA) con las recurrentes contralaterales (PFRR), observando que las parálisis de presentación única se comportan de forma similar a las formas de parálisis recurrentes contralaterales (AU)

Recurrent facial palsy is noted infrequently in the literature. We present a comparative study of recurrent Bell's palsy (RBP) with regard to non-recurrent Bell's palsy (NRBP). Afterwards we compare among RBP cases those of ipsilateral recurrence (RRBP) to those of contralateral one (ARBP). We constate that non-recurrent Bell'palsy formes behave similarly to the contralateral recurrent forms (AU)

Late-onset metachromatic leukodystrophy clinically presenting as isolated peripheral neuropathy: compound heterozygosity for the IVS2+1G-->A mutation and a newly identified missense mutation (Thr408Ile) in a Spanish family.

We report the case of a 50-year-old woman and her 32-year-old daughter, both of whom are affected with adult-onset metachromatic leukodystrophy (MLD) clinically presenting as peripheral neuropathy. Arylsulfatase A (ARSA) activities were markedly reduced, and electrophysiology showed a severe demyelinating neuropathy with features of chronic acquired demyelinating polyneuropathy. Molecular genetic studies of the family revealed that the proband and her affected daughter are compound heterozygotes for the common IVS2+ 1G-->A mutation and a newly identified missense mutation, Thr408Ile. This case indicates that adult metachromatic leukodystrophy should be considered in adult patients with demyelinating peripheral neuropathy of unknown etiology.

[Spastic paraparesias by HTLV-1: early identification of a new case. Review of the Spanish casuistics]. / Paraparesia espástica por HTLV-I: identificación temprana de un nuevo caso. Revisión de la casuística española.

This is a study of the early identification of a new case of tropical spastic paraparesis/HLTV-1-associated myelopathy in a Spanish patient not previously reported on. The patient is 48 years of age, and he has been living in Sierra Leone for the past seventeen years, where he has worked as a surgeon. On first examination, he had been suffering from symptoms of a spastic paraparesis for the previous five months. This diagnosis was confirmed using serological tests (EIA and Western-blot) on the blood and cerebrospinal fluid (CSF). We have studied the cases outlined in medical literature, paying special attention to the time elapsed between the onset of symptoms and the date on which the diagnosis was made.

Paraparesia espástica por HTLV-I: identificación temprana de un nuevo caso. Revisión de la casuística española / Spastic paraparesias by HTLV-1: early identification of a new case. Review of the Spanish casuistics

Presentamos la identificación temprana de un nuevo caso de paraparesia espástica asociada al virus HTLV-I. El paciente, un español de 48 años, había desarrollado su actividad quirúrgica durante los últimos 17 años en Sierra Leona. Presentaba un síndrome de paraparesia espástica sin alteración esfinteriana de 5 meses de evolución. El diagnóstico se confirmó mediante la determinación de anticuerpos frente al HTLV-I en sangre y líquido cefalorraquídeo (EIA y Western blot). Hemos revisado en la bibliografía los casos descritos, prestando especial atención al tiempo transcurrido entre el inicio de los síntomas y la fecha en que se estableció el diagnóstico (AU)

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[Recurrent Bell's facial palsy: our experience]. / Parálisis facial de Bell recurrente: nuestra experiencia.

Recurrent facial palsy is noted infrequently in the literature. We present a comparative study of recurrent Bell's palsy (RBP) with regard to non-recurrent Bell's palsy (NRBP). Afterwards we compare among RBP cases those of ipsilateral recurrence (RRBP) to those of contralateral one (ARBP). We constate that non-recurrent Bell'palsy formes behave similarly to the contralateral recurrent forms.

MRI findings in Möbius syndrome: correlation with clinical features.

The authors studied the MRI findings of three patients with Möbius syndrome. Möbius syndrome is a rare congenital disorder characterized by complete or partial facial diplegia accompanied by other cranial nerve palsies. MRI demonstrated brainstem hypoplasia with straightening of the fourth ventricle floor, indicating an absence of the facial colliculus. These MRI features suggest the diagnosis of Möbius syndrome and correlate with the clinical and neurophysiologic findings.

[Recurrent peripheral facial paralysis. Our case load from 1995]. / Parálisis facial periférica recidivante. Nuestra casuística del año 1995.

Of 117 cases of peripheral facial palsy seen in our emergency room in 1995, 10 (8.5%) were relapses. Seven were males and 3, females. Mean age at the first appearance was 23.6 years. The mean interval between the first facial palsy and the first recurrence was 10.1 years, and between the first and second recurrence, 4.6 years. Five of the 10 patients (50%) had a second recurrence. Two of the 10 patients (20%) had a family history of facial palsy. Only 1 (10%) was diabetic. Seven of 10 patients achieved a complete functional recovery and 3 had sequelae. Computed tomography was normal in every patient. In our series, a family history of facial palsy and low ENoG results were related with a poorer prognosis. ENoG scan was used as a prognostic and follow-up factor.

Effects of rHuEPO on Q-EEG and event-related potentials in chronic renal failure.

Quantitative electroencephalography is a powerful tool to evaluate brain function, and preliminary data have shown its usefulness in the evaluation of patients with chronic renal failure (CRF). In this study, baseline values of different quantitative EEG variables, as well as data from the P300 component of the visual event-related potential, in 43 patients with chronic renal failure, were compared with those of a group of healthy subjects and with the results obtained after 3, 6, 9 and 12 months of treatment of these patients with rHuEPO. Baseline total power was much lower in patients with CRF than in healthy subjects, and the distribution of power among the frequency bands was also abnormal. rHuEPO promptly normalized total power and progressively improved power distribution, although full normality was not achieved. Mean dominant frequencies in brain areas were abnormal in patients with CRF, and progressive improvement was seen along the study. The latency of P300, which was increased before treatment, decreased in all subjects, but normal values were not reached. The same applies to the hypomanic and psychopathic scores of psychological tests. Altogether, brain dysfunction of CRF seems to substantially improve by treatment of the anemia with rHuEPO.