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2.
Life (Basel) ; 12(10)2022 Sep 24.
Article in English | MEDLINE | ID: mdl-36294919

ABSTRACT

Understanding how plants respond and adapt to extraterrestrial conditions is essential for space exploration initiatives. Deleterious effects of the space environment on plant development have been reported, such as the unbalance of cell growth and proliferation in the root meristem, or gene expression reprogramming. However, plants are capable of surviving and completing the seed-to-seed life cycle under microgravity. A key research challenge is to identify environmental cues, such as light, which could compensate the negative effects of microgravity. Understanding the crosstalk between light and gravity sensing in space was the major objective of the NASA-ESA Seedling Growth series of spaceflight experiments (2013-2018). Different g-levels were used, with special attention to micro-g, Mars-g, and Earth-g. In spaceflight seedlings illuminated for 4 days with a white light photoperiod and then photostimulated with red light for 2 days, transcriptomic studies showed, first, that red light partially reverted the gene reprogramming induced by microgravity, and that the combination of microgravity and photoactivation was not recognized by seedlings as stressful. Two mutant lines of the nucleolar protein nucleolin exhibited differential requirements in response to red light photoactivation. This observation opens the way to directed-mutagenesis strategies in crop design to be used in space colonization. Further transcriptomic studies at different g-levels showed elevated plastid and mitochondrial genome expression in microgravity, associated with disturbed nucleus-organelle communication, and the upregulation of genes encoding auxin and cytokinin hormonal pathways. At the Mars g-level, genes of hormone pathways related to stress response were activated, together with some transcription factors specifically related to acclimation, suggesting that seedlings grown in partial-g are able to acclimate by modulating genome expression in routes related to space-environment-associated stress.

3.
Br J Ophthalmol ; 106(10): 1463-1468, 2022 10.
Article in English | MEDLINE | ID: mdl-33926863

ABSTRACT

PURPOSE: To ascertain the anatomic factors that help achieve non-surgical sealing in full thickness macular hole (FTMH). METHODS: Retrospective collaborative study of FTMH that closed without surgical intervention. RESULTS: A total of 78 patients (mean age 57.9 years) included 18 patients with blunt ocular trauma, 18 patients that received topical or intravitreal therapies and 42 patients with idiopathic FTMH. Mean±SD of the initial corrected visual acuity (VA) in logMAR improved from 0.65±0.54 to 0.34±0.45 (p<0.001) at a mean follow-up of 33.8±37.1 months. FTMH reopened in seven eyes (9.0%) after a mean of 8.6 months. Vitreomacular traction was noted in 12 eyes (15.8%), perifoveal posterior vitreous detachment in 42 (53.8%), foveal epiretinal membrane in 10 (12.8%), cystoid macular oedema (CME) in 49 (62.8%) and subretinal fluid (SRF) in 20 (25.6%). By multivariate analysis, initial VA correlated to the height (p<0.001) and narrowest diameter of the hole (p<0.001) while final VA correlated to the basal diameter (p<0.001). Time for closure of FTMH (median 2.8 months) correlated to the narrowest diameter (p<0.001) and the presence of SRF (p=0.001). Mean time for closure (in months) was 1.6 for eyes with trauma, 4.3 for eyes without trauma but with therapy for CME, 4.4 for eyes without trauma and without therapy in less than 200 µm in size and 24.7 for more than 200 µm. CONCLUSION: Our data suggest an observation period in new onset FTMH for non-surgical closure, in the setting of trauma, treatment of CME and size <200 µm.


Subject(s)
Retinal Perforations , Wounds, Nonpenetrating , Fovea Centralis , Humans , Middle Aged , Retinal Perforations/diagnosis , Retinal Perforations/surgery , Retrospective Studies , Tomography, Optical Coherence , Visual Acuity , Vitrectomy , Wounds, Nonpenetrating/diagnosis , Wounds, Nonpenetrating/surgery
4.
Plants (Basel) ; 10(4)2021 Apr 09.
Article in English | MEDLINE | ID: mdl-33918741

ABSTRACT

Clinorotation was the first method designed to simulate microgravity on ground and it remains the most common and accessible simulation procedure. However, different experimental settings, namely angular velocity, sample orientation, and distance to the rotation center produce different responses in seedlings. Here, we compare A. thaliana root responses to the two most commonly used velocities, as examples of slow and fast clinorotation, and to vertical and horizontal clinorotation. We investigate their impact on the three stages of gravitropism: statolith sedimentation, asymmetrical auxin distribution, and differential elongation. We also investigate the statocyte ultrastructure by electron microscopy. Horizontal slow clinorotation induces changes in the statocyte ultrastructure related to a stress response and internalization of the PIN-FORMED 2 (PIN2) auxin transporter in the lower endodermis, probably due to enhanced mechano-stimulation. Additionally, fast clinorotation, as predicted, is only suitable within a very limited radius from the clinorotation center and triggers directional root growth according to the direction of the centrifugal force. Our study provides a full morphological picture of the stages of graviresponse in the root tip, and it is a valuable contribution to the field of microgravity simulation by clarifying the limitations of 2D-clinostats and proposing a proper use.

5.
Reumatol. clín. (Barc.) ; 16(5,pt.2): 413-415, sept.-oct. 2020. tab
Article in Spanish | IBECS | ID: ibc-199736

ABSTRACT

La aparición en el campo de la oncología de moléculas terapéuticas en forma de anticuerpos monoclonales, cuyo objetivo consiste en estimular el propio sistema inmune del paciente para que sea este el encargado de destruir las células cancerígenas, ha revolucionado el tratamiento de diversos cánceres en los últimos años. Este tipo de terapia, denominada inmunoterapia, se caracteriza además por presentar efectos secundarios en forma de enfermedades autoinmunes que todavía estamos empezando a conocer. Desde el punto de vista de los efectos secundarios inmunomediados reumatológicos, podemos encontrar manifestaciones musculoesqueléticas mecánicas, inflamatorias o enfermedad autoinmune sistémica. El manejo terapéutico de estos efectos secundarios se mantiene variable debido a la ausencia de ensayos clínicos y de recomendaciones validadas, siendo el manejo multidisciplinar fundamental para tratar con éxito dichos casos. En este artículo presentamos nuestra serie de casos clínicos de pacientes en tratamiento con inmunoterapia y efectos secundarios inmunomediados reumatológicos en un hospital universitario


The appearance in the field of oncology of therapeutic molecules in the form of monoclonal antibodies, whose objective is to stimulate the patient's own immune system to be responsible for destroying cancer cells, has revolutionized the treatment of many cancers in recent years. This type of therapy, called immunotherapy, is also characterized by presenting side effects in the form of autoimmune diseases that we are still beginning to understand. From the point of view of the immune-mediated rheumatological side effects, we can find musculoskeletal manifestations, mechanical, inflammatory or systemic autoimmune diseases. The therapeutic approach to these side effects remains uncertain due to the absence of clinical trials and validated recommendations. The multidisciplinary management is crucial to successfully treat such cases. In the following manuscript, we will describe our case reports of rheumatologic immune-related adverse events in a university hospital


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Immunotherapy/adverse effects , Neoplasms/drug therapy , Rheumatic Diseases/drug therapy , Immune System Diseases/chemically induced , Immunologic Factors/adverse effects , Drug-Related Side Effects and Adverse Reactions/epidemiology
6.
Reumatol Clin (Engl Ed) ; 16(5 Pt 2): 413-415, 2020.
Article in English, Spanish | MEDLINE | ID: mdl-30471992

ABSTRACT

The appearance in the field of oncology of therapeutic molecules in the form of monoclonal antibodies, whose objective is to stimulate the patient's own immune system to be responsible for destroying cancer cells, has revolutionized the treatment of many cancers in recent years. This type of therapy, called immunotherapy, is also characterized by presenting side effects in the form of autoimmune diseases that we are still beginning to understand. From the point of view of the immune-mediated rheumatological side effects, we can find musculoskeletal manifestations, mechanical, inflammatory or systemic autoimmune diseases. The therapeutic approach to these side effects remains uncertain due to the absence of clinical trials and validated recommendations. The multidisciplinary management is crucial to successfully treat such cases. In the following manuscript, we will describe our case reports of rheumatologic immune-related adverse events in a university hospital.


Subject(s)
Immunotherapy/adverse effects , Neoplasms/therapy , Rheumatic Diseases/etiology , Aged , Aged, 80 and over , Female , Hospitals, University , Humans , Male , Middle Aged , Retrospective Studies
7.
Front Plant Sci ; 10: 1529, 2019.
Article in English | MEDLINE | ID: mdl-31850027

ABSTRACT

Introduction: Traveling to nearby extraterrestrial objects having a reduced gravity level (partial gravity) compared to Earth's gravity is becoming a realistic objective for space agencies. The use of plants as part of life support systems will require a better understanding of the interactions among plant growth responses including tropisms, under partial gravity conditions. Materials and Methods: Here, we present results from our latest space experiments on the ISS, in which seeds of Arabidopsis thaliana were germinated, and seedlings grew for six days under different gravity levels, namely micro-g, several intermediate partial-g levels, and 1g, and were subjected to irradiation with blue light for the last 48 h. RNA was extracted from 20 samples for subsequent RNAseq analysis. Transcriptomic analysis was performed using the HISAT2-Stringtie-DESeq pipeline. Differentially expressed genes were further characterized for global responses using the GEDI tool, gene networks and for Gene Ontology (GO) enrichment. Results: Differential gene expression analysis revealed only one differentially expressed gene (AT4G21560, VPS28-1 a vacuolar protein) across all gravity conditions using FDR correction (q < 0.05). However, the same 14 genes appeared differentially expressed when comparing either micro-g, low-g level (< 0.1g) or the Moon g-level with 1g control conditions. Apart from these 14-shared genes, the number of differentially expressed genes was similar in microgravity and the Moon g-level and increased in the intermediate g-level (< 0.1g), but it was then progressively reduced as the difference with the Earth gravity became smaller. The GO groups were differentially affected at each g-level: light and photosynthesis GO under microgravity, genes belonged to general stress, chemical and hormone responses under low-g, and a response related to cell wall and membrane structure and function under the Moon g-level. Discussion: Transcriptional analyses of plants under blue light stimulation suggests that root blue-light phototropism may be enough to reduce the gravitational stress response caused by the lack of gravitropism in microgravity. Competition among tropisms induces an intense perturbation at the micro-g level, which shows an extensive stress response that is progressively attenuated. Our results show a major effect on cell wall/membrane remodeling (detected at the interval from the Moon to Mars gravity), which can be potentially related to graviresistance mechanisms.

9.
Reumatol. clín. (Barc.) ; 15(5): 252-257, sept.-oct. 2019. tab
Article in Spanish | IBECS | ID: ibc-189400

ABSTRACT

OBJETIVOS: Describir la metodología del Registro Español de Artritis Psoriásica de reciente comienzo de la Sociedad Española de Reumatología (REAPSER), así como sus fortalezas y limitaciones. El objetivo principal del proyecto es identificar factores pronósticos de la evolución clínica y radiográfica en una cohorte de pacientes que padecen artritis psoriásica (APs) diagnosticada con menos de 2 años de evolución. MATERIAL Y MÉTODO: Estudio observacional, prospectivo (2 años de seguimiento; periodicidad anual de las visitas), multicéntrico. La intención en la visita basal fue reflejar la situación del paciente antes de que la evolución de la enfermedad se viese modificada por los tratamientos pautados en los servicios de reumatología. Los pacientes fueron invitados a participar consecutivamente en una de sus visitas habituales al reumatólogo. El tamaño muestral finalmente alcanzado fue de 211 pacientes. Se recogen datos sociodemográficos; de situación laboral; historia familiar; antecedentes personales y comorbilidad; antropométricos; estilo de vida; uso de los servicios de salud; situación clínica al diagnóstico de APs; afectación articular y dolor espinal; dolor y valoración global de la enfermedad; entesitis, dactilitis y uveítis; afectación cutánea y ungueal; situación funcional y calidad de vida; evaluación radiográfica; determinaciones analíticas; tratamiento; brotes en esqueleto axial y periférico. CONCLUSIONES: El estudio REAPSER incluye una cohorte de pacientes con APs de inicio reciente reclutados antes de que la evolución de la enfermedad se viese modificada por la prescripción de FAME en los servicios de reumatología. Se espera que la información exhaustiva recogida en las visitas suponga una amplia fuente de datos para futuros análisis


AIMS: To describe the methodology of REAPSER (Spanish Registry of Recent-onset Psoriatic Arthritis), its strengths and limitations. The aim of this study is to identify prognostic factors for the clinical and radiographic course in a cohort of patients with psoriatic arthritis (PsA) diagnosed within 2 years of symptom evolution. METHODS: Multicenter, observational and prospective study (with 2-year follow-up including annual visits). Baseline visit intended to reflect patient situation before the disease course was modified by treatments prescribed in rheumatology departments. Patients were invited to participate consecutively in one of their routine visits to the rheumatologist. 211 patients were included. Following data were collected: sociodemographic variables; employment situation; family history; personal history and comorbidities; anthropometric data; lifestyle; use of healthcare services; clinical situation at the time of PsA diagnosis; joint involvement and spinal pain; pain and overall assessment; enthesitis, dactylitis and uveitis; skin and nail involvement; functional situation and quality of life; radiographic evaluation; analytical determinations; treatment; axial and peripheral flare-ups. CONCLUSIONS: The REAPSER study includes a cohort of patients with recent-onset PsA, before the disease course was modified by disease-modifying antirheumatic drugs prescribed in rheumatology departments. Exhaustive information collected in each visit is expected to be an important data source for future analysis


Subject(s)
Humans , Male , Female , Adult , Arthritis, Psoriatic/diagnostic imaging , Disease Progression , Records , Cohort Studies , Follow-Up Studies , Medical History Taking , Patient Selection , Prognosis , Prospective Studies , Radiography , Spain , Time Factors
11.
Reumatol Clin (Engl Ed) ; 15(5): 252-257, 2019.
Article in English, Spanish | MEDLINE | ID: mdl-30522944

ABSTRACT

AIMS: To describe the methodology of REAPSER (Spanish Registry of Recent-onset Psoriatic Arthritis), its strengths and limitations. The aim of this study is to identify prognostic factors for the clinical and radiographic course in a cohort of patients with psoriatic arthritis (PsA) diagnosed within 2years of symptom evolution. METHODS: Multicenter, observational and prospective study (with 2-year follow-up including annual visits). Baseline visit intended to reflect patient situation before the disease course was modified by treatments prescribed in rheumatology departments. Patients were invited to participate consecutively in one of their routine visits to the rheumatologist. 211 patients were included. Following data were collected: sociodemographic variables; employment situation; family history; personal history and comorbidities; anthropometric data; lifestyle; use of healthcare services; clinical situation at the time of PsA diagnosis; joint involvement and spinal pain; pain and overall assessment; enthesitis, dactylitis and uveitis; skin and nail involvement; functional situation and quality of life; radiographic evaluation; analytical determinations; treatment; axial and peripheral flare-ups. CONCLUSIONS: The REAPSER study includes a cohort of patients with recent-onset PsA, before the disease course was modified by disease-modifying antirheumatic drugs prescribed in rheumatology departments. Exhaustive information collected in each visit is expected to be an important data source for future analysis.


Subject(s)
Arthritis, Psoriatic/diagnostic imaging , Disease Progression , Registries , Adult , Cohort Studies , Female , Follow-Up Studies , Humans , Male , Medical History Taking , Patient Selection , Prognosis , Prospective Studies , Radiography , Spain , Time Factors
12.
Reumatol. clín. (Barc.) ; 14(6): 379-381, nov.-dic. 2018. ilus
Article in Spanish | IBECS | ID: ibc-176032

ABSTRACT

Las miopatías necrosantes autoinmunes (NAM) son unas entidades raras y emergentes de las miopatías inflamatorias idiopáticas (MII). Su presencia se ha asociado a conectivopatías, infecciones virales, neoplasias, anticuerpos frente a la partícula de reconocimiento de la señal (anti-SRP) y a anticuerpos frente a la proteína 3-hidroxi-3-metilglutaril-coenzima A reductasa (HMG-CR) (asociada al empleo o no de estatinas). Las NAM asociadas al anti-SRP presentan unas características clínicas y anatomopatológicas que las diferencian del resto de las MII, resultando en un peor pronóstico. Hasta el momento han sido muy pocos los casos publicados tratados con rituximab y con diferente respuesta clínica. Presentamos el caso de una mujer de 30 años con NAM asociada al anti-SRP refractaria a tratamiento convencional inmunosupresor y que ha sido controlada a largo plazo con la combinación de rituximab, metotrexato y corticoides


Necrotizing autoimmune myopathy (NAM) is a rare and emerging entity of idiopathic inflammatory myopathy (IIM). They have been associated with connective tissue disorders, viral infections, malignancy, anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase protein (with or without the use of statins). Anti-SRP associated NAM has different clinical and histological characteristics that differentiate them from other IIM, resulting in a poor prognosis. Very few cases treated with rituximab have been published, with varying clinical response. Here we describe a case of anti-SRP associated NAM refractory to conventional immunosuppressants and its successful long-term management with the combination of rituximab, corticosteroids and methotrexate


Subject(s)
Humans , Female , Adult , Rituximab/therapeutic use , Muscular Diseases/drug therapy , Autoimmune Diseases/drug therapy , Myositis/drug therapy , Signal Recognition Particle/analysis , Immunosuppressive Agents/therapeutic use , Deglutition Disorders/etiology , Dysphonia/etiology , Plasmapheresis
13.
Reumatol Clin (Engl Ed) ; 14(6): 379-381, 2018.
Article in English, Spanish | MEDLINE | ID: mdl-28389200

ABSTRACT

Necrotizing autoimmune myopathy (NAM) is a rare and emerging entity of idiopathic inflammatory myopathy (IIM). They have been associated with connective tissue disorders, viral infections, malignancy, anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase protein (with or without the use of statins). Anti-SRP associated NAM has different clinical and histological characteristics that differentiate them from other IIM, resulting in a poor prognosis. Very few cases treated with rituximab have been published, with varying clinical response. Here we describe a case of anti-SRP associated NAM refractory to conventional immunosuppressants and its successful long-term management with the combination of rituximab, corticosteroids and methotrexate.


Subject(s)
Autoantibodies/blood , Autoimmune Diseases/drug therapy , Immunosuppressive Agents/therapeutic use , Myositis/drug therapy , Rituximab/therapeutic use , Signal Recognition Particle/immunology , Adult , Autoimmune Diseases/blood , Autoimmune Diseases/immunology , Female , Humans , Myositis/blood , Myositis/immunology
14.
Plant Signal Behav ; 9(4): e28289, 2014.
Article in English | MEDLINE | ID: mdl-24614101

ABSTRACT

Experiments performed in actively proliferating plant cells both in space and simulated microgravity have evidenced a common effect: cell proliferation appears enhanced whereas cell growth is depleted. Coordination of cell growth and proliferation, called meristematic competence, is a major feature of meristematic cells and its disruption may lead to important alterations in the developmental pattern of the plant. Auxin is known to be a mediator of the transduction of the gravitropic signal and a regulator of the rates of growth and proliferation in meristematic cells, as well as of their further differentiation. Therefore, gravity sensing, gravitropism, auxin levels, and meristematic competence are mutually interrelated. However, our experiments in simulated microgravity, using both mechanical and magnetic levitation technologies, have revealed that this interdependence is neither strict nor univocal and may include additional factors and mechanisms. Available data indicate that altered gravity may affect cell growth and proliferation by mechanisms alternative to the transduction of the gravitropic signal perceived by columella cells in the root tip. These mechanisms would include gravity sensing independent from statolith displacement and transduction mediators other than polar auxin transport.


Subject(s)
Arabidopsis/growth & development , Gravitropism , Meristem/physiology , Weightlessness , Arabidopsis/metabolism , Cell Enlargement , Cell Proliferation , Indoleacetic Acids/metabolism , Mechanotransduction, Cellular
15.
PLoS One ; 9(3): e91814, 2014.
Article in English | MEDLINE | ID: mdl-24618597

ABSTRACT

The "GENARA A" experiment was designed to monitor global changes in the proteome of membranes of Arabidopsis thaliana seedlings subjected to microgravity on board the International Space Station (ISS). For this purpose, 12-day-old seedlings were grown either in space, in the European Modular Cultivation System (EMCS) under microgravity or on a 1 g centrifuge, or on the ground. Proteins associated to membranes were selectively extracted from microsomes and identified and quantified through LC-MS-MS using a label-free method. Among the 1484 proteins identified and quantified in the 3 conditions mentioned above, 80 membrane-associated proteins were significantly more abundant in seedlings grown under microgravity in space than under 1 g (space and ground) and 69 were less abundant. Clustering of these proteins according to their predicted function indicates that proteins associated to auxin metabolism and trafficking were depleted in the microsomal fraction in µg space conditions, whereas proteins associated to stress responses, defence and metabolism were more abundant in µg than in 1 g indicating that microgravity is perceived by plants as a stressful environment. These results clearly indicate that a global membrane proteomics approach gives a snapshot of the cell status and its signaling activity in response to microgravity and highlight the major processes affected.


Subject(s)
Arabidopsis Proteins/metabolism , Arabidopsis/growth & development , Arabidopsis/metabolism , Microsomes/metabolism , Space Flight , Weightlessness , Membrane Proteins/metabolism , Phenotype , Protein Transport , Proteomics , Seedlings/growth & development
17.
Plant Signal Behav ; 9(9): e29637, 2014.
Article in English | MEDLINE | ID: mdl-25763699

ABSTRACT

Growing plants in space for using them in bioregenerative life support systems during long-term human spaceflights needs improvement of our knowledge in how plants can adapt to space growth conditions. In a previous study performed on board the International Space Station (GENARA A experiment STS-132) we evaluate the global changes that microgravity can exert on the membrane proteome of Arabidopsis seedlings. Here we report additional data from this space experiment, taking advantage of the availability in the EMCS of a centrifuge to evaluate the effects of cues other than microgravity on the relative distribution of membrane proteins. Among the 1484 membrane proteins quantified, 227 proteins displayed no abundance differences between µ g and 1 g in space, while their abundances significantly differed between 1 g in space and 1 g on ground. A majority of these proteins (176) were over-represented in space samples and mainly belong to families corresponding to protein synthesis, degradation, transport, lipid metabolism, or ribosomal proteins. In the remaining set of 51 proteins that were under-represented in membranes, aquaporins and chloroplastic proteins are majority. These sets of proteins clearly appear as indicators of plant physiological processes affected in space by stressful factors others than microgravity.


Subject(s)
Arabidopsis/growth & development , Arabidopsis/metabolism , Extraterrestrial Environment , Proteome/metabolism , Weightlessness/adverse effects , Arabidopsis Proteins/metabolism , Microsomes/metabolism , Seedlings/growth & development , Seedlings/metabolism , Space Flight , Stress, Physiological
18.
Rev Neurosci ; 24(5): 507-25, 2013.
Article in English | MEDLINE | ID: mdl-24077617

ABSTRACT

It has been almost 40 years since Barker, Jalinous, and Freeston designed and used the first device of transcranial magnetic stimulation (TMS). From then until now, this technique has evolved vertiginously, appearing a lot of new protocols and device modifications, which associated with new technologies complement and enhance the versatility of this technique. TMS has demonstrated to be a safe technology and become a key tool in the study of the complex brain processes. Despite this, it is as a therapeutic tool where this technique has caused a revolution. In this regard, this type of non-invasive brain stimulation has been proven useful in a variety of neurodegenerative and psychiatric disorders due to its biochemical, molecular, and cellular effects, with depression being the paradigm of the therapeutic effectiveness of this technique. This review focuses on a detailed vision of how this type of radiation modifies different biochemical and cellular processes that induce the mechanisms and pathways underlying the therapeutic effects of TMS.


Subject(s)
Brain/physiology , Mental Disorders/therapy , Neurodegenerative Diseases/therapy , Transcranial Magnetic Stimulation/methods , Brain/ultrastructure , Humans , Mitochondria/physiology , Neurons/ultrastructure , Neurotransmitter Agents/metabolism
19.
Rev. neurol. (Ed. impr.) ; 56(11): 567-572, 1 jun., 2013. ilus
Article in Spanish | IBECS | ID: ibc-112890

ABSTRACT

Introducción. La epiteliopatía pigmentaria placoide multifocal posterior aguda (EPPMPA) es una enfermedad inflamatoria rara, generalmente de etiología indeterminada, de la coriocapilar, el epitelio pigmentario y la retina externa. Afecta predominantemente a pacientes jóvenes y en algunos casos puede involucrar al sistema nervioso central en forma de ictus o de meningoencefalitis. Presentamos el caso clínico de una mujer joven con EPPMPA complicada con ictus e hipertensión intracraneal. Caso clínico. Mujer de 16 años que comienza con cefalea intensa sugestiva de hipertensión intracraneal, así como con un déficit agudo hemisférico izquierdo. La resonancia magnética craneal ponía de manifiesto lesiones embólicas o vasculíticas en diferentes territorios. No se evidenciaron datos de meningoencefalitis en el estudio del líquido cefalorraquídeo, pero sí de hipertensión intracraneal asociada. La presencia de lesiones muy específicas en el polo ocular posterior permitió el diagnóstico de EPPMPA complicada con ictus isquémico, probablemente por mecanismo vasculítico. Un amplio estudio etiológico fue negativo para identificar un factor desencadenante claro del proceso. Se inició tratamiento corticoideo con buena evolución clínica y radiológica. Conclusiones. La EPPMPA es una entidad rara que generalmente entraña buen pronóstico; sin embargo, en algunos casos puede complicarse con afectación del sistema nervioso central, y el ictus isquémico secundario a vasculitis es la complicación más grave. Ante un paciente joven con ictus que presente sintomatología visual y lesiones coriorretinianas, debe considerarse la EPPMPA en su diagnóstico etiológico (AU)


Introduction. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory disease, generally of unknown aetiology, affecting the choriocapillaris, the pigment epithelium and the outer retina. It predominantly affects young patients and in some cases may involve the central nervous system in the form of strokes or meningoencephalitis. We report the clinical case of a young female with APMPPE that was complicated by stroke and intracranial hypertension. Case report. Our patient was a 16-year-old female who began with intense headaches suggesting intracranial hypertension, as well as with an acute deficit in the left hemisphere. A magnetic resonance scan of the head revealed embolic or vasculitic lesions in different territories. No evidence of meningoencephalitis was found in the cerebrospinal fluid analysis, but signs of associated intracranial hypertension were observed. The presence of very specific lesions in the posterior pole of the eye led to a diagnosis of APMPPE complicated by ischaemic stroke, probably caused by a vasculitic mechanism. An extensive aetiological study failed to identify a clear precipitating factor underlying the process. Treatment with corticoids was established, with good clinical and radiological progression. Conclusions. APMPPE is an infrequent condition that generally has a good prognosis. In some cases, however, complications may arise owing to involvement of the central nervous system, and ischaemic stroke secondary to vasculitis is the most severe complication. In young patients with stroke who present visual symptoms and chorioretinital lesions, APMPPE must be considered in the aetiological diagnosis (AU)


Subject(s)
Humans , Stroke/etiology , Brain Ischemia/etiology , Vasculitis, Central Nervous System/complications , Hemangioendothelioma, Epithelioid/pathology , Retinal Pigment Epithelium/pathology
20.
Rev Neurol ; 56(11): 567-72, 2013 Jun 01.
Article in Spanish | MEDLINE | ID: mdl-23703059

ABSTRACT

INTRODUCTION: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory disease, generally of unknown aetiology, affecting the choriocapillaris, the pigment epithelium and the outer retina. It predominantly affects young patients and in some cases may involve the central nervous system in the form of strokes or meningoencephalitis. We report the clinical case of a young female with APMPPE that was complicated by stroke and intracranial hypertension. CASE REPORT: Our patient was a 16-year-old female who began with intense headaches suggesting intracranial hyper-tension, as well as with an acute deficit in the left hemisphere. A magnetic resonance scan of the head revealed embolic or vasculitic lesions in different territories. No evidence of meningoencephalitis was found in the cerebrospinal fluid analysis, but signs of associated intracranial hypertension were observed. The presence of very specific lesions in the posterior pole of the eye led to a diagnosis of APMPPE complicated by ischaemic stroke, probably caused by a vasculitic mechanism. An extensive aetiological study failed to identify a clear precipitating factor underlying the process. Treatment with corticoids was established, with good clinical and radiological progression. CONCLUSIONS: APMPPE is an infrequent condition that generally has a good prognosis. In some cases, however, complications may arise owing to involvement of the central nervous system, and ischaemic stroke secondary to vasculitis is the most severe complication. In young patients with stroke who present visual symptoms and chorioretinital lesions, APMPPE must be considered in the aetiological diagnosis.


TITLE: Epiteliopatia pigmentaria placoide multifocal posterior aguda. Una rara causa de ictus isquemico.Introduccion. La epiteliopatia pigmentaria placoide multifocal posterior aguda (EPPMPA) es una enfermedad inflamatoria rara, generalmente de etiologia indeterminada, de la coriocapilar, el epitelio pigmentario y la retina externa. Afecta predominantemente a pacientes jovenes y en algunos casos puede involucrar al sistema nervioso central en forma de ictus o de meningoencefalitis. Presentamos el caso clinico de una mujer joven con EPPMPA complicada con ictus e hipertension intracraneal. Caso clinico. Mujer de 16 anos que comienza con cefalea intensa sugestiva de hipertension intracraneal, asi como con un deficit agudo hemisferico izquierdo. La resonancia magnetica craneal ponia de manifiesto lesiones embolicas o vasculiticas en diferentes territorios. No se evidenciaron datos de meningoencefalitis en el estudio del liquido cefalorraquideo, pero si de hipertension intracraneal asociada. La presencia de lesiones muy especificas en el polo ocular posterior permitio el diagnostico de EPPMPA complicada con ictus isquemico, probablemente por mecanismo vasculitico. Un amplio estudio etiologico fue negativo para identificar un factor desencadenante claro del proceso. Se inicio tratamiento corticoideo con buena evolucion clinica y radiologica. Conclusiones. La EPPMPA es una entidad rara que generalmente entrana buen pronostico; sin embargo, en algunos casos puede complicarse con afectacion del sistema nervioso central, y el ictus isquemico secundario a vasculitis es la complicacion mas grave. Ante un paciente joven con ictus que presente sintomatologia visual y lesiones coriorretinianas, debe considerarse la EPPMPA en su diagnostico etiologico.


Subject(s)
Brain Ischemia/etiology , Choroid Diseases/complications , Retinal Diseases/complications , Vasculitis/complications , Adolescent , Choroid Diseases/diagnosis , Diagnosis, Differential , Female , Fluorescein Angiography , Headache/etiology , Hemianopsia/etiology , Hemiplegia/etiology , Humans , Intracranial Hypertension/etiology , Magnetic Resonance Imaging , Meningoencephalitis/diagnosis , Neuroimaging , Papilledema/etiology , Pharyngitis/complications , Retinal Diseases/diagnosis , Retinal Pigment Epithelium/pathology , Speech Disorders/etiology
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