Evaluation of DiaSorin Liaison® calprotectin fecal assay adapted for pleural effusion.

Objectives: Calprotectin (CP) is a calcium and zinc binding protein that is widely measured on faecal samples but its determination in other biological fluids might be of interest. The aim of this work was to validate the measurement of CP in pleural fluid by chemiluminescence. Methods: LIAISON®XL, a fully automated chemiluminescence analyzer, was used for CP quantification on pleural fluid. A validation protocol was designed using both quality control materials provided by the manufacturer and pools of pleural fluid samples. Stability, imprecision, bias, linearity, detection capability and carry over effect were evaluated. Results: CP was stable on pleural fluid at least one week, under refrigerated conditions, and four weeks at -80 °C. The observed intra- and inter-day imprecision was between 2.2 and 6.49 %, with a negative bias under 5.51 %. The linearity of the method was verified up to 2,000 ng/mL. The LoQ for the assay was 48.52 ng/mL. A statistically significant carry-over effect was observed after measuring CP concentrations above the upper limit of linearity, but given the observed magnitude, a clinically relevant impact should not be expected. Conclusions: DiaSorin Liaison® calprotectin assay allows reliable measurement of CP in pleural fluid.

Real-world safety and effectiveness of cenobamate in patients with focal onset seizures: Outcomes from an Expanded Access Program.

OBJECTIVE: This study investigated early, real-world outcomes with cenobamate (CNB) in a large series of patients with highly drug-resistant epilepsy within a Spanish Expanded Access Program (EAP). METHOD: This was a multicenter, retrospective, observational study in 14 hospitals. Inclusion criteria were age ≥18 years, focal seizures, and EAP authorization. Data were sourced from patient clinical records. Primary effectiveness endpoints included reductions (100%, ≥90%, ≥75%, and ≥50%) or worsening in seizure frequency at 3-, 6-, and 12-month visits and at the last visit. Safety endpoints included rates of adverse events (AEs) and AEs leading to discontinuation. RESULTS: The study included 170 patients. At baseline, median epilepsy duration was 26 years and median number of seizures/month was 11.3. The median number of prior antiseizure medications (ASMs) and concomitant ASMs were 12 and 3, respectively. Mean CNB dosages/day were 176 mg, 200 mg, and 250 mg at 3, 6, and 12 months. Retention rates were 98.2%, 94.5%, and 87% at 3, 6, and 12 months. At last available visit, the rate of seizure freedom was 13.3%; ≥90%, ≥75%, and ≥50% responder rates were 27.9%, 45.5%, and 63%, respectively. There was a significant reduction in the number of seizures per month (mean: 44.6%; median: 66.7%) between baseline and the last visit (P < 0.001). Responses were maintained regardless of the number of prior or concomitant ASMs. The number of concomitant ASMs was reduced in 44.7% of patients. The cumulative percentage of patients with AEs and AEs leading to discontinuation were 68.2% and 3.5% at 3 months, 74.1% and 4.1% at 6 months, and 74.1% and 4.1% at 12 months. The most frequent AEs were somnolence and dizziness. SIGNIFICANCE: In this highly refractory population, CNB showed a high response regardless of prior and concomitant ASMs. AEs were frequent but mostly mild-to-moderate, and few led to discontinuation.

Donepezil alone and combined with intensive language-action therapy on depression and apathy in chronic post-stroke aphasia: A feasibility study.

This study explored the feasibility and effectiveness of a short-term (10-week) intervention trial using Donepezil administered alone and combined with intensive language action therapy (ILAT) for the treatment of apathy and depression in ten people with chronic post-stroke aphasia. Outcome measures were the Western Aphasia Battery and the Stroke Aphasia Depression Questionnaire-21. Structural magnetic resonance imaging and 18fluorodeoxyglucose positron emission tomography were acquired at baseline and after two endpoints (Donepezil alone and Donepezil-ILAT). The intervention was found to be feasible to implement. Large treatment effects were found. Donepezil alone and combined with ILAT reduced aphasia severity, while apathy and depression only improved with Donepezil-ILAT. Structural and functional neuroimaging data did not show conclusive results but provide hints for future research. Given these overall positive findings on feasibility, language and behavioral benefits, further studies in larger sample sizes and including a placebo-control group are indicated.

Impact of spill-in counts from off-target regions on [18F]Flortaucipir PET quantification.

BACKGROUND: [18F]Flortaucipir (FTP) PET quantification is usually hindered by spill-in counts from off-target binding (OFF) regions. The present work aims to provide an in-depth analysis of the impact of OFF in FTP PET quantification, as well as to identify optimal partial volume correction (PVC) strategies to minimize this problem. METHODS: 309 amyloid-beta (Aß) negative cognitively normal subjects were included in the study. Additionally, 510 realistic FTP images with different levels of OFF were generated using Monte Carlo simulation (MC). Images were corrected for PVC using both a simple two-compartment and a multi-region method including OFF regions. FTP standardized uptake value ratio (SUVR) was quantified in Braak Areas (BA), the hippocampus (which was not included in Braak I/II) and different OFF regions (caudate, putamen, pallidum, thalamus, choroid plexus (ChPlex), cerebellar white matter (cerebWM), hemispheric white matter (hemisWM) and cerebrospinal fluid (CSF)) using the lower portion of the cerebellum as a reference region. The correlations between OFF and cortical SUVRs were studied both in real and in simulated PET images, with and without PVC. RESULTS: In-vivo, we found correlations between all OFF and target regions, especially strong for the hemisWM (slope>0.63, R2>0.4). All the correlations were attenuated but remained significant after applying PVC, except for the ChPlex. In MC simulations, the hemisWM and CSF were the main contributors to PVE in all BA (slopes 0.15-0.26 and 0.13-0.21 respectively). The hemisWM (slope=0.2), as well as the ChPlex (slope=0.02), influenced SUVRs in the hippocampus. The CerebWM was negatively correlated with all target regions (slope<-0.02, R2>0.8). While no other correlations between OFF and target regions were found, hemisWM was correlated with all OFF regions but the cerebWM (slopes 0.06-0.33). HemisWM correlations attenuated (slopes<0.06) when applying two-compartment PVC, but the hippocampus-ChPlex and the cerebWM correlations required more complex PVC with dedicated compartments for these regions. In-vivo, PVC removed a notably higher fraction of the correlation between OFF regions found to be affected by PVE in the simulation studies and BA (≈50%) than for OFF regions not affected by PVE (16%). CONCLUSION: HemisWM is the main driver of spill-in effects in FTP PET, affecting both target regions and the rest of OFF regions. PVC successfully reduces PVE, even when using a simple two-compartment method. Despite PVC, non-zero correlations were still observed between target and OFF regions in vivo, which suggests the existence of biological or tracer-related contributions to these correlations.

Intensity normalization methods in brain FDG-PET quantification.

BACKGROUND: The lack of standardization of intensity normalization methods and its unknown effect on the quantification output is recognized as a major drawback for the harmonization of brain FDG-PET quantification protocols. The aim of this work is the ground truth-based evaluation of different intensity normalization methods on brain FDG-PET quantification output. METHODS: Realistic FDG-PET images were generated using Monte Carlo simulation from activity and attenuation maps directly derived from 25 healthy subjects (adding theoretical relative hypometabolisms on 6 regions of interest and for 5 hypometabolism levels). Single-subject statistical parametric mapping (SPM) was applied to compare each simulated FDG-PET image with a healthy database after intensity normalization based on reference regions methods such as the brain stem (RRBS), cerebellum (RRC) and the temporal lobe contralateral to the lesion (RRTL), and data-driven methods, such as proportional scaling (PS), histogram-based method (HN) and iterative versions of both methods (iPS and iHN). The performance of these methods was evaluated in terms of the recovery of the introduced theoretical hypometabolic pattern and the appearance of unspecific hypometabolic and hypermetabolic findings. RESULTS: Detected hypometabolic patterns had significantly lower volumes than the introduced hypometabolisms for all intensity normalization methods particularly for slighter reductions in metabolism . Among the intensity normalization methods, RRC and HN provided the largest recovered hypometabolic volumes, while the RRBS showed the smallest recovery. In general, data-driven methods overcame reference regions and among them, the iterative methods overcame the non-iterative ones. Unspecific hypermetabolic volumes were similar for all methods, with the exception of PS, where it became a major limitation (up to 250 cm3) for extended and intense hypometabolism. On the other hand, unspecific hypometabolism was similar far all methods, and usually solved with appropriate clustering. CONCLUSIONS: Our findings showed that the inappropriate use of intensity normalization methods can provide remarkable bias in the detected hypometabolism and it represents a serious concern in terms of false positives. Based on our findings, we recommend the use of histogram-based intensity normalization methods. Reference region methods performance was equivalent to data-driven methods only when the selected reference region is large and stable.

"Need to Know" or the Strong Urge to Find Names of Unique Entities in Acquired Obsessive-Compulsive Disorder.

The two forms of obsessive-compulsive disorder (OCD), idiopathic and acquired, have been linked to abnormalities in the fronto-striato-thalamo-cortical circuitry, involving the orbitofrontal cortex, anterior cingulate cortex, thalamus, and striatum. Accumulating evidence indicates that damage to other brain regions (ie, temporal lobes) is also implicated in the pathogenesis of both types of OCD. In addition, some discrete OCD symptoms have received less attention because of their presumed low occurrence and difficultly of categorization. Among these, one intriguing and potentially severe type of obsessive thinking is the so-called "need to know" (NtK), which is a strong urge to access certain information, particularly proper names. In some patients, this monosymptomatic presentation may constitute the major feature of OCD. Here we report the cases of two patients who developed NtK obsessions with tenacious time-consuming, answer-seeking compulsions as the only or more disabling symptomatology in association with malignant tumors involving the right temporal lobe and connected fronto-subcortical circuits.

QModeling: a Multiplatform, Easy-to-Use and Open-Source Toolbox for PET Kinetic Analysis.

Kinetic modeling is at the basis of most quantification methods for dynamic PET data. Specific software is required for it, and a free and easy-to-use kinetic analysis toolbox can facilitate routine work for clinical research. The relevance of kinetic modeling for neuroimaging encourages its incorporation into image processing pipelines like those of SPM, also providing preprocessing flexibility to match the needs of users. The aim of this work was to develop such a toolbox: QModeling. It implements four widely-used reference-region models: Simplified Reference Tissue Model (SRTM), Simplified Reference Tissue Model 2 (SRTM2), Patlak Reference and Logan Reference. A preliminary validation was also performed: The obtained parameters were compared with the gold standard provided by PMOD, the most commonly-used software in this field. Execution speed was also compared, for time-activity curve (TAC) estimation, model fitting and image generation. QModeling has a simple interface, which guides the user through the analysis: Loading data, obtaining TACs, preprocessing the model for pre-evaluation, generating parametric images and visualizing them. Relative differences between QModeling and PMOD in the parameter values are almost always below 10-8. The SRTM2 algorithm yields relative differences from 10-3 to 10-5 when [Formula: see text] is not fixed, since different, validated methods are used to fit this parameter. The new toolbox works efficiently, with execution times of the same order as those of PMOD. Therefore, QModeling allows applying reference-region models with reliable results in efficient computation times. It is free, flexible, multiplatform, easy-to-use and open-source, and it can be easily expanded with new models.

Perampanel in routine clinical use in idiopathic generalized epilepsy: The 12-month GENERAL study.

OBJECTIVE: To analyze the effectiveness and tolerability of perampanel across different seizure types in routine clinical care of patients with idiopathic generalized epilepsy (IGE). METHODS: This multicenter, retrospective, 1-year observational study collected data from patient records at 21 specialist epilepsy units in Spain. All patients who were aged ≥12 years, prescribed perampanel before December 2016, and had a confirmed diagnosis of IGE were included. RESULTS: The population comprised 149 patients with IGE (60 with juvenile myoclonic epilepsy, 51 generalized tonic-clonic seizures [GTCS] only, 21 juvenile absence epilepsy, 10 childhood absence epilepsy, 6 adulthood absence epilepsy, and one Jeavons syndrome). Mean age was 36 years. The retention rate at 12 months was 83% (124/149), and 4 mg was the most common dose. At 12 months, the seizure-free rate was 59% for all seizures (88/149); 63% for GTCS (72/115), 65% for myoclonic seizures (31/48), and 51% for absence seizures (24/47). Seizure frequency was reduced significantly at 12 months relative to baseline for GTCS (78%), myoclonic (65%), and absence seizures (48%). Increase from baseline seizure frequency was seen in 5.2% of patients with GTCS seizures, 6.3% with myoclonic, and 4.3% with absence seizures. Perampanel was effective regardless of epilepsy syndrome, concomitant antiepileptic drugs (AEDs), and prior AEDs, but retention and seizure freedom were significantly higher when used as early add-on (after ≤2 prior AEDs) than late (≥3 prior AEDs). Adverse events were reported in 50% of patients over 12 months, mostly mild or moderate, and irritability (23%), somnolence (15%), and dizziness (14%) were most frequent. SIGNIFICANCE: In routine clinical care of patients with IGE, perampanel improved seizure outcomes for GTCS, myoclonic seizures, and absence seizures, with few discontinuations due to adverse events. This is the first real-world evidence with perampanel across different seizure types in IGE.

Hemisferectomía funcional: seguimiento a largo plazo en una serie de cinco casos / Functional hemispherectomy: long-term follow-up in a series of five patients

Introducción. La hemisferectomía funcional es una de las técnicas quirúrgicas con intención paliativa que se pueden realizar en pacientes con epilepsia farmacorresistente y síndromes hemisféricos. Se basa en la desconexión neuronal del hemisferio afectado preservando el árbol vascular. Objetivo. Analizar el pronóstico y la seguridad a largo plazo de las hemisferectomías realizadas en nuestro centro. Pacientes y métodos. Revisión retrospectiva de los casos intervenidos, recogiendo las siguientes variables clínicas: edad, sexo, edad de inicio de la epilepsia, tipo de crisis, etiología de la epilepsia, edad de intervención, pronóstico posquirúrgico y posibles complicaciones. El seguimiento mínimo fue de cinco años. Resultados. Cinco pacientes (60% mujeres) fueron intervenidos entre 1999 y 2010. La edad de inicio de la epilepsia fue de 36 meses, y el tiempo de evolución hasta la cirugía, de 7 años. El tipo de crisis más habitual fueron las crisis parciales simples motoras con generalización secundaria (n = 5). Tres pacientes permanecieron libres de crisis tras la cirugía, y otro paciente mejoró más de un 90%. El tiempo medio de seguimiento fue de 13 años. Como complicaciones, una paciente sufrió una meningitis bacteriana sin secuelas posteriores. A los seis años de la cirugía, un paciente presentó una hidrocefalia que requirió la implantación de una válvula de derivación ventriculoperitoneal. Conclusiones. La hemisferectomía funcional constituye un procedimiento quirúrgico eficaz para el tratamiento de pacientes con epilepsia farmacorresistente, patología hemisférica extensa y crisis limitadas a ese hemisferio. Hay complicaciones que pueden aparecer tardíamente, por lo que se aconseja un seguimiento a largo plazo de estos pacientes (AU)

Introduction. Functional hemispherectomy consists in palliative epilepsy surgical procedure usually performed in patients with pharmaco-resistant epilepsy and hemispheric syndromes. It is based on the neural disconnection of the affected hemisphere with preservation of the vascular supply. Aim. To analyze long-term prognosis and safety of the hemispherectomies performed in our institution. Patients and methods. Retrospective analysis collecting the following variables: age, gender, age of epilepsy onset, type of seizures, etiology, age of epilepsy surgery, prognosis and potential surgical complications. All patients had a minimum of five years of follow up. Results. Five patients (60% females) underwent hemispherotomy between 1999 and 2010. Age of epilepsy onset was 36 months and time of evolution until surgery was 7 years. The most frequent type of seizures were simple motor seizures with secondary generalization (n = 5). Three patients remained seizure free persistently after surgery and another patient had a more than 90% improvement. Time of follow up was 13 years. One patient suffered a bacterial meningitis without sequelae. Six years after surgery a patient suffered hydrocephalous requiring ventriculoperitoneal shunt. Conclusions. Functional hemispherectomy constitutes an effective method to treat patients with pharmaco-resistant epilepsy, extensive unihemispheric pathology and seizures limited to that hemisphere. Late complications may occur thus long-term follow-up is needed (AU)

Dolor abdominal: una manifestación infrecuente de epilepsia temporal / Abdominal pain: an infrequent manifestation of temporal epilepsy

No disponible

[Clinical experience with lacosamide in Galicia: the GALACO study]. / Experiencia clinica con lacosamida en Galicia: estudio GALACO.

INTRODUCTION: Lacosamide is a sodium channel blocker antiepileptic drug authorized as an adjunctive therapy for focal seizures in adolescents and adults. AIM: To analyze the efficacy and safety of lacosamide in Galicia according to its use in daily clinical practice. PATIENTS AND METHODS: Retrospective observational study in patients who started treatment with lacosamide between January 2014 and June 2013 in 10 hospitals in Galicia, Spain. Its efficacy and safety at 3, 6 and 12 months after starting lacosamide was assessed. RESULTS: We included 184 patients with a mean age of 44.2 ± 17.4 years old; 56.5% (n = 104) were male; 173 patients constituted the efficacy population. Mean duration of epilepsy was 18.8 ± 15.5 years. Seizure frequency was 2.5 ± 1.6 episodes/month. After 12 months, 68.2% of patients (n = 118) had >= 50% improvement (responders) and among them, 54 (45.8% of responder patients) were seizure free. Twenty-three percent (n = 43) suffered from adverse events after 12 months, being dizziness (10.3%) and instability (3.3%) the most frequently reported. After the 12 month visit, 87.5% of patients (n = 161) continued treatment with lacosamide. CONCLUSIONS: Lacosamide provides a very good efficacy and safety profile for patients with focal refractory epilepsy. High percentage of responders may be related to a less refractory population compared to other daily clinical practice studies. It constitutes an attractive therapeutic option for the treatment of focal epilepsies.

TITLE: Experiencia clinica con lacosamida en Galicia: estudio GALACO.Introducción. La lacosamida es un fármaco antiepiléptico bloqueante de los canales de sodio, autorizado en adolescentes y adultos como tratamiento coadyuvante en crisis de inicio focal. Objetivo. Analizar los resultados de eficacia y seguridad de la lacosamida en Galicia en su uso de acuerdo con la práctica clínica habitual. Pacientes y métodos. Estudio retrospectivo observacional en pacientes que iniciaron tratamiento con lacosamida entre enero de 2013 y junio de 2014 en 10 hospitales de Galicia. Se evaluó su eficacia y seguridad a los 3, 6 y 12 meses del inicio del tratamiento. Resultados. Se incluyeron 184 pacientes con edad media de 44,2 ± 17,4 años; el 56,5% (n = 104) eran varones. Conforman la población de eficacia 173 pacientes. El tiempo medio de evolución de la epilepsia fue de 18,8 ± 15,5 años. La frecuencia de crisis era de 2,5 ± 1,6 episodios/mes. A los 12 meses, el 68,2% de los pacientes (n = 118) presentaba una mejoría igual o superior al 50% (pacientes respondedores) y, de ellos, 54 (el 45,8% de los respondedores) estaban libres de crisis. El 23,4% (n = 43) refirió efectos adversos a los 12 meses, principalmente mareos (10,3%) e inestabilidad (3,3%). Después de la visita de los 12 meses, continuaba con lacosamida el 87,5% de los pacientes (n = 161). Conclusiones. La lacosamida ofrece un perfil de eficacia y seguridad muy favorable para pacientes con epilepsia focal refractaria. El elevado porcentaje de respondedores podría atribuirse a una población de epilépticos menos refractarios que en otros estudios de práctica clínica. Constituye una opción terapéutica atractiva para el tratamiento de epilepsias de inicio focal.

Experiencia clínica con lacosamida en Galicia: estudio GALACO / Clinical experience with lacosamide in Galicia: the GALACO study

Introducción. La lacosamida es un fármaco antiepiléptico bloqueante de los canales de sodio, autorizado en adolescentes y adultos como tratamiento coadyuvante en crisis de inicio focal. Objetivo. Analizar los resultados de eficacia y seguridad de la lacosamida en Galicia en su uso de acuerdo con la práctica clínica habitual. Pacientes y métodos. Estudio retrospectivo observacional en pacientes que iniciaron tratamiento con lacosamida entre enero de 2013 y junio de 2014 en 10 hospitales de Galicia. Se evaluó su eficacia y seguridad a los 3, 6 y 12 meses del inicio del tratamiento. Resultados. Se incluyeron 184 pacientes con edad media de 44,2 ± 17,4 años; el 56,5% (n = 104) eran varones. Conforman la población de eficacia 173 pacientes. El tiempo medio de evolución de la epilepsia fue de 18,8 ± 15,5 años. La frecuencia de crisis era de 2,5 ± 1,6 episodios/mes. A los 12 meses, el 68,2% de los pacientes (n = 118) presentaba una mejoría igual o superior al 50% (pacientes respondedores) y, de ellos, 54 (el 45,8% de los respondedores) estaban libres de crisis. El 23,4% (n = 43) refirió efectos adversos a los 12 meses, principalmente mareos (10,3%) e inestabilidad (3,3%). Después de la visita de los 12 meses, continuaba con lacosamida el 87,5% de los pacientes (n = 161). Conclusiones. La lacosamida ofrece un perfil de eficacia y seguridad muy favorable para pacientes con epilepsia focal refractaria. El elevado porcentaje de respondedores podría atribuirse a una población de epilépticos menos refractarios que en otros estudios de práctica clínica. Constituye una opción terapéutica atractiva para el tratamiento de epilepsias de inicio focal (AU)

Introduction. Lacosamide is a sodium channel blocker antiepileptic drug authorized as an adjunctive therapy for focal seizures in adolescents and adults. Aim. To analyze the efficacy and safety of lacosamide in Galicia according to its use in daily clinical practice. Patients and methods. Retrospective observational study in patients who started treatment with lacosamide between January 2014 and June 2013 in 10 hospitals in Galicia, Spain. Its efficacy and safety at 3, 6 and 12 months after starting lacosamide was assessed. Results. We included 184 patients with a mean age of 44.2 ± 17.4 years old; 56.5% (n = 104) were male; 173 patients constituted the efficacy population. Mean duration of epilepsy was 18.8 ± 15.5 years. Seizure frequency was 2.5 ± 1.6 episodes/month. After 12 months, 68.2% of patients (n = 118) had ≥ 50% improvement (responders) and among them, 54 (45.8% of responder patients) were seizure free. Twenty-three percent (n = 43) suffered from adverse events after 12 months, being dizziness (10.3%) and instability (3.3%) the most frequently reported. After the 12 month visit, 87.5% of patients (n = 161) continued treatment with lacosamide. Conclusions. Lacosamide provides a very good efficacy and safety profile for patients with focal refractory epilepsy. High percentage of responders may be related to a less refractory population compared to other daily clinical practice studies. It constitutes an attractive therapeutic option for the treatment of focal epilepsies (AU)

Paquimeningitis hipertrófica secundaria a enfermedad relacionada con IgG4: descripción de un caso y revisión de la bibliografía / Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature

Introducción. La paquimeningitis hipertrófica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopática o secundaria a procesos infecciosos, autoinmunes o neoplásicos. La recientemente descrita ‘enfermedad relacionada con IgG4’ podría ser la causa de bastantes cuadros considerados criptogénicos. Caso clínico. Mujer de 54 años, con historia de asma bronquial, que consultó por cefalea, vértigo y pérdida de audición por su oído izquierdo. En la resonancia magnética cerebral con gadolinio se objetivó engrosamiento y realce dural, que se extendía desde la pared lateral del seno cavernoso izquierdo y la parte medial del lóbulo temporal al ángulo pontocerebeloso y parte del tentorio homolaterales. El líquido cefalorraquídeo presentaba 10 leucocitos/μL (90% mononucleares), con 1 g/L de proteínas y sin consumo de glucosa. El estudio anatomopatológico mostró fibrosis y un infiltrado linfoplasmocitario, con 16 células plasmáticas IgG4+ por campo de gran aumento. El resto de estudios analíticos y microbiológicos resultaron normales o negativos. La tasa plasmática de IgG4 estaba dentro de los límites normales. Tratada con esteroides, se produjo mejoría clínica acompañada de la práctica desaparición de las alteraciones detectadas en la neuroimagen. Conclusiones. La paquimeningitis hipertrófica como manifestación de la enfermedad relacionada con IgG4 puede diagnosticarse basándose en los hallazgos de la resonancia magnética si la IgG4 plasmática está elevada. En casos dudosos, habrá que recurrir a la biopsia meníngea. La corticoterapia suele ser eficaz y representa la primera línea terapéutica (AU)

Introduction. Hypertrophic pachymeningitis is an infrequent disorder that produces focal or diffuse thickening of the dura mater. It can be idiopathic or secondary to infectious, autoimmune or neoplastic processes. The recently described ‘IgG4- related disease’ could be the cause of many cases considered cryptogenic. Case report. A 54-year-old woman, with a history of bronchial asthma, presented with headache, dizziness and hearing loss on her left ear. The brain MRI study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left cavernous sinus and medial temporal lobe to cerebellopontine angle and ipsilateral tentorium. CSF had 10 leukocytes/μL (90% mononuclear), with 1 g/L protein and without glucose consumption. Pathology showed fibrosis and lymphoplasmacytic infiltrate, with 16 IgG4+ plasma cells per high power field. The rest of analytical and microbiological studies were normal or negative. The plasma IgG4 rate was within normal limits. After treatment with steroids there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. Conclusions. Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be diagnosed based on MRI findings if plasma IgG4 is elevated. In doubtful cases we must resort to meningeal biopsy. Corticosteroid therapy is usually effective and it is the first line treatment (AU)

[Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature]. / Paquimeningitis hipertrofica secundaria a enfermedad relacionada con IgG4: descripcion de un caso y revision de la bibliografia.

INTRODUCTION: Hypertrophic pachymeningitis is an infrequent disorder that produces focal or diffuse thickening of the dura mater. It can be idiopathic or secondary to infectious, autoimmune or neoplastic processes. The recently described 'IgG4-related disease' could be the cause of many cases considered cryptogenic. CASE REPORT: A 54-year-old woman, with a history of bronchial asthma, presented with headache, dizziness and hearing loss on her left ear. The brain MRI study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left cavernous sinus and medial temporal lobe to cerebellopontine angle and ipsilateral tentorium. CSF had 10 leukocytes/µL (90% mononuclear), with 1 g/L protein and without glucose consumption. Pathology showed fibrosis and lymphoplasmacytic infiltrate, with 16 IgG4+ plasma cells per high power field. The rest of analytical and microbiological studies were normal or negative. The plasma IgG4 rate was within normal limits. After treatment with steroids there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. CONCLUSIONS: Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be diagnosed based on MRI findings if plasma IgG4 is elevated. In doubtful cases we must resort to meningeal biopsy. Corticosteroid therapy is usually effective and it is the first line treatment.

TITLE: Paquimeningitis hipertrofica secundaria a enfermedad relacionada con IgG4: descripcion de un caso y revision de la bibliografia.Introduccion. La paquimeningitis hipertrofica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopatica o secundaria a procesos infecciosos, autoinmunes o neoplasicos. La recientemente descrita 'enfermedad relacionada con IgG4' podria ser la causa de bastantes cuadros considerados criptogenicos. Caso clinico. Mujer de 54 años, con historia de asma bronquial, que consulto por cefalea, vertigo y perdida de audicion por su oido izquierdo. En la resonancia magnetica cerebral con gadolinio se objetivo engrosamiento y realce dural, que se extendia desde la pared lateral del seno cavernoso izquierdo y la parte medial del lobulo temporal al angulo pontocerebeloso y parte del tentorio homolaterales. El liquido cefalorraquideo presentaba 10 leucocitos/µL (90% mononucleares), con 1 g/L de proteinas y sin consumo de glucosa. El estudio anatomopatologico mostro fibrosis y un infiltrado linfoplasmocitario, con 16 celulas plasmaticas IgG4+ por campo de gran aumento. El resto de estudios analiticos y microbiologicos resultaron normales o negativos. La tasa plasmatica de IgG4 estaba dentro de los limites normales. Tratada con esteroides, se produjo mejoria clinica acompañada de la practica desaparicion de las alteraciones detectadas en la neuroimagen. Conclusiones. La paquimeningitis hipertrofica como manifestacion de la enfermedad relacionada con IgG4 puede diagnosticarse basandose en los hallazgos de la resonancia magnetica si la IgG4 plasmatica esta elevada. En casos dudosos, habra que recurrir a la biopsia meningea. La corticoterapia suele ser eficaz y representa la primera linea terapeutica.

General closed-form bit-error rate expressions for coded M-distributed atmospheric optical communications.

In this Letter, general closed-form expressions for the average bit error rate in atmospheric optical links employing rate-adaptive channel coding are derived. To characterize the irradiance fluctuations caused by atmospheric turbulence, the Málaga or M distribution is employed. The proposed expressions allow us to evaluate the performance of atmospheric optical links employing channel coding schemes such as OOK-GSc, OOK-GScc, HHH(1,13), or vw-MPPM with different coding rates and under all regimes of turbulence strength. A hyper-exponential fitting technique applied to the conditional bit error rate is used in all cases. The proposed closed-form expressions are validated by Monte-Carlo simulations.

Recidiva después de 25 años de encefalitis por anticuerpos antirreceptor de NMDA / Recurrence of encephalitis after 25 years due to anti-NMDA receptor antibodies

No disponible