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Introduction and objective Fabry disease (FD) is an X-linked lysosomal storage disorder due to a deficiency of the α-galactosidase A enzyme. Although women were historically considered only carriers, many studies have contradicted this fact. The main aim of this work was to set the first Spanish study out of the on-going registries on health status and management of women diagnosed with FD who were not receiving enzyme replacement therapy (ERT). Material and methods An epidemiological, cross-sectional, descriptive and multicentre study was assessed in women diagnosed for FD who were not receiving ERT. Assessments on symptomatology and severity were collected using several clinical questionnaires. Additionally, clinical information and lab tests were obtained from clinical records. Results Thirty-three women with a mean age of 45.6 ± 16.2 years were studied. Symptom onset was at a median age of 35.5 years old (range: 30.0–51.5), being diagnosed a median of 2 years later (range: 1.0–1.5). Missense mutations were the most prevalent mutation (n = 22, 68.8%). Although 69% considered themselves as asymptomatic, 22 (66.7%) showed at least one FD-related clinical symptom. Using Mainz Severity Score index and Fabry International Prognosis Index neurological symptomatology obtained higher scores both for severity and prognostic. The EQ-5D questionnaire showed 42.2% patients referring to some anxiety or depression, and 30.3% thought that their life was somehow altered by the pain. 62.5% were not receiving any treatment and ERT was offered only to one patient (3.6%) who refused it. Conclusions Although most of the heterozygous women for FD had not received ERT or either symptomatic treatment, they present symptoms of disease. Careful follow-up of female patients or some adjuvant treatment may be considered to delay progressive organ damage and improve patient quality of life.

We are grateful to Angela Tate for revising the English text. We are also grateful to the reviewers for their insightful suggestions., This paper analyzes, through computational simulations, which spectral filters increase the number of discernible colors (NODC) of subjects with normal color vision, as well as red–green anomalous trichromats and dichromats. The filters are selected from a set of filters in which we have modeled spectral transmittances. With the selected filters we have carried out simulations performed using the spectral reflectances captured either by a hyperspectral camera or by a spectrometer. We have also studied the effects of these filters on color coordinates. Finally, we have simulated the results of two widely used color blindness tests: Ishihara and Farnsworth–Munsell 100 Hue (FM100). In these analyses the selected filters are compared with the commercial filters from EnChroma and VINO companies. The results show that the increase in NODC with the selected filters is not relevant. The simulation results show that none of these chosen filters help color vision deficiency (CVD) subjects to pass the set of color blindness tests studied. These results obtained using standard colorimetry support the hypothesis that the use of color filters does not cause CVDs to have a perception similar to that of a normal observer., This research was supported by the Spanish State Agency for Research (AEI) and the Ministry for Economy, Industry and Competitiveness (MIMECO) by means of grant number FIS2017-89258-P with European Union FEDER (European Regional Development Funds) support, and by the Spanish Ministry of Science, Innovation, and Universities, with support from the European Regional Development Funds under grant number RTI2018-094738-B-I00.

Spectral images were captured of paper samples varnished with two water-soluble materials: gum arabic and egg white. The samples were submitted to degradation processes that partially or totally eliminated the varnish from the substrate (water immersion and ageing). The spectral information was used to obtain average color data and to characterize the spatial and color inhomogeneity across pixels, showing that the pixel spectral data are critical for an accurate characterization of the degradation process of the varnishes. Since the varnishes typically become yellower with ageing, this study introduces two novel and simple-to-compute yellowness indices based on the spectral information, which are validated against a standard colorimetric index (ASTM-E313 2015). The potential uses of spectral information are demonstrated with several pieces of a real antique map sample by comparing the spectral information measured before and after cleaning the sample. To sum up, the main contributions of this study are the characterization of the spatial homogeneity through pixel-based spectral and color information and the proposal of spectral-based yellowing indices for two critical applications (ageing process follow-up and effect of cleaning), as demonstrated with synthetic and historical samples of varnished paper respectively., Spanish Ministry of Economy and Competitiveness, under research Grant DPI2015-64571-R. Spanish State Agency of Research (AEI) and the Ministry for Economy, Industry and Competitiveness (MIMECO) by means of the Grant Number FIS2017-89258-P with European Union FEDER (European Regional Development Funds) support.

Objective: Sonographic assessment of the optic nerve sheath diameter (ONSD) is a useful technique in detecting raised intracranial pressure (ICP) in neurocritical care patients. Its utility in idiopathic intracranial hypertension (IIH) is less known. The aim of this study was to evaluate the diagnostic accuracy of ONSD for detecting IIH. Material and methods: Ultrasound measurement of ONSD was performed in 19 patients with IIH and in 11 patients with different neurological diseases without raised ICP that required undergoing a lumbar puncture. The validity of this technique for diagnosing IIH was established with cerebrospinal fluid manometry values. Results: Patients with IIH showed significantly enlarged ONSD than those without IIH. The best cut-off point for detecting raised ICP was 6.3 mms, with a sensitivity, specificity and positive likelihood ratio of 94.7%, 90.9% and 10.4, respectively. After a therapeutic lumbar puncture an 87% of cases had a partial reduction of ONSD values. Conclusion: Sonographic assessment of ONSD seems to be a useful and reliable technique for detecting raised ICP. While the spinal manometry is not replaced in usual clinical settings, transorbital sonography alternatively allows a suitable and harmless screening of patients with suspected IIH. It would be desirable to perform an internal validation of the technique in each hospital in order to get the optimal cut-off point.

Resumen Introduccion El tratamiento trombolitico del ictus isquemico agudo es una medida de alta eficacia, pero que acarrea riesgos importantes. A diferencia del tratamiento del infarto agudo de miocardio, el hematoma subdural como consecuencia del tratamiento trombolitico intravenoso del ictus isquemico no ha sido descrito previamente. Caso clinico Presentamos una paciente con un ictus isquemico que recibe tratamiento trombolitico intravenoso, y sufre un hematoma subdural de gran tamano en la convexidad derecha como consecuencia del mismo. Conclusiones El hematoma subdural tras tratamiento trombolitico intravenoso es excepcional. Este caso puede ser la primera publicacion de un hematoma subdural tras tratamiento trombolitico intravenoso del ictus isquemico.

This paper discusses the contribution of Spanish neurologist Manuel Peraita (1908-1950) to the study of deficiency neuropathy in the setting of the Spanish Civil War (1936-1939). The clinical characteristics of "paraesthetic-causalgic syndrome" or "Madrid syndrome" as described by Peraita are discussed, and the syndrome is presented in relation to other similar conditions, including Strachan's syndrome and burning feet syndrome.

Background and Purpose— We sought to evaluate the effects of administration of microbubbles (MBs) on the beginning, speed, and degree of middle cerebral artery (MCA) recanalization during systemic thrombolysis and continuous 2-MHz pulsed-wave transcranial Doppler (TCD) monitoring. Methods— We evaluated 111 patients with acute stroke attributable to MCA occlusion treated with intravenous tissue plasminogen activator (tPA). Thirty-eight patients were treated with tPA plus continuous 2-hour TCD monitoring plus 3 doses of 2.5 g (400 mg/mL) of galactose-based MBs given at 2, 20, and 40 minutes after tPA bolus (MB group). These patients were compared with 73 patients who were allocated to receive tPA plus continuous 2-hour TCD ultrasound (US) monitoring (tPA/US group) or tPA plus placebo monitoring (tPA group), most of whom were enrolled in a previous study of US-enhanced thrombolysis. The beginning, degree, and time to maximum completeness of recanalization during the first 2 hours of tPA bolus were recorded. Results— Median prebolus National Institutes of Health Stroke Scale (NIHSS) score was 18. Eighty patients (72%) had a proximal and 31 (28%) a distal MCA occlusion on TCD. Thirty-seven patients (33%) received tPA/US, 38 (34%) received tPA/US/MB, and 36 (32%) were treated with tPA alone. Stroke severity, time to treatment, location of MCA occlusion, and presence of carotid artery disease were similar among groups. Two-hour recanalization was seen in 14 (39%), 25 (68%), and 27 patients (71%) in the tPA, tPA/US, and tPA/US/MB groups, respectively ( P =0.004). Two-hour complete recanalization rate was significantly ( P =0.038) higher in the tPA/US/MB group (54.5%) compared with tPA/US (40.8%) and tPA (23.9%) groups. The time to beginning of recanalization after tPA bolus was 26±18 minutes in the tPA/US group and 19±12 minutes in the tPA/US/MB group ( P =0.12). Four patients (3.6%) experienced symptomatic intracranial hemorrhage: 2 (5.5%), 1 (2.7%), and 1 patient (2.6%) who received tPA only, tPA/US, and tPA/US/MB, respectively, experienced symptomatic intracranial hemorrhage. At 24 hours, 31%, 41%, and 55% of tPA, tPA/US, and tPA/US/MB improved >4 points in the NIHSS score. Conclusions— Administration of MBs induces further acceleration of US-enhanced thrombolysis in acute stroke, leading to a more complete recanalization and to a trend toward better short- and long-term outcome.

Background and Purpose— We evaluated the impact of admission hyperglycemia (HG) on stroke outcome in relation to the timing of reperfusion in patients treated with tissue plasminogen activator (tPA). Methods— We studied 138 consecutive stroke patients with a documented middle cerebral artery (MCA) occlusion treated with intravenous tPA 140 mg/dL. National Institutes of Health Stroke Scale (NIHSS) scores were obtained at baseline and 24 hour. Transcranial Doppler monitoring of recanalization was conducted during the first 12 hour of stroke onset. mRS was used to assess outcome at 3 months. Results— Median baseline NIHSS score was 17 points. At baseline, 42 (37.3%) patients were hyperglycemic and 96 (62.7%) normoglycemic. Reperfusion was achieved 16 points (odds ratio [OR], 3.32; 95% CI, 2.18 to 24.7; P =0.032) and admission glucose level >140 mg/dL (OR, 5.65; 95% CI, 1.97 to 16.18; P =0.002) independently predicted poor outcome (modified Rankin scale, 3 to 6) at 3 months. After adjusting by age, stroke severity, site of MCA occlusion, and degree of recanalization, the contribution of HG for poor outcome was higher as shorter the time to reperfusion. The highest odds for poor outcome related to HG corresponded to patients who recanalized P =0.002), as compared with those who recanalized between 3 and 6 hours (OR, 2.1; 95% CI, 1.1 to 16; P =0.034) and between 6 to 12 hours (OR, 1.1; 95% CI, 0.7 to 21; P =0.43). Moreover, baseline glucose level was negatively correlated ( r =−0.45; P =0.001) with the degree of improvement in the NIHSS score at 24 hours after early (3 hours) or no recanalization. Conclusion— The impact of admission HG on stroke outcome varies depending on the time to tPA-induced reperfusion. The detrimental effect of acute HG is higher after early than after delayed or no reperfusion. Ultra-early glycemic control before reperfusion may improve the efficacy of thrombolytic therapy.

The aim of this article is to contribute to the analysis of the origins of psychiatric semiology, which by emphasizing subjectivity in clinical practice, gave birth to psychopathology as the scientific and intellectual enterprise of alienism. In other words, beyond simple anatomical and clinical observation, there was an effort to ‘listen to’ and ‘read’ the patient’s delirium. In essence, the basic thesis which this short paper seeks to defend is that, despite a growing anatomical and clinical mind-set and a clear interest in physically locating mental illness within the body, during the Romantic period, psychiatry was able to construct a semiology largely based on the experience of the ego, on the inner world of the individual. This makes it possible to establish, from a clinical perspective, that the birth of alienism – of psychiatry – must be situated within the framework of a modernity in which the culture of subjectivity was one of its most characteristic features.

This article examines the influence of the French alienist Alexandre Brierre de Boismont in the first development of the Spanish psychiatric profession during the third quarter of the 19th century. As an outstanding figure of French psychological medicine, Brierre enjoyed great scientific prestige among Spanish doctors, but he also took an active part in promoting and legitimizing the cause of alienism in Spain. For instance, he was involved in projects for the reform or creation of new mental hospitals, supported the admission of some Spanish colleagues into the Société Médico-Psychologique and made a decisive contribution to the social recognition of the professional and medico-legal expertise of alienists in Spain. His case is thus an excellent example of the important role played by international relations and the scientific and professional networks of European alienism in spreading the discourses and practices of the emerging psychological medicine.

We describe a nutritional intervention by the Rockefeller Foundation's International Health Division in Spain after the Spanish Civil War, delineating the relationships between the technicians sent by the Rockefeller Foundation and the Spanish health authorities. We analyze reports of the nutritional situation in Spain in the early 1940s and the design and outcomes of a nutrition survey conducted in a district of Madrid by American and Spanish nutritionists. This nutritional survey, which was based on food intake interviews and was complemented with anthropometric measurements, clinical examinations, and blood tests, found several symptoms and signs of malnutrition. The Rockefeller Foundation's nutritional research was an important historical precedent for later studies made in emergency situations or armed conflicts. Similar surveys have been carried out in the last several decades by distinguished academic departments of public health and epidemiology and by humanitarian aid agencies.

This paper makes a fresh analysis of the work of the French alienist J. E. D. Esquirol. In addition to a critical study of his classifi cation of mental diseases, it analyses his main contributions to semiology: the difference between hallucinations and illusions, etc., and it also places the psychiatric clinic into the context of the so-called anatomoclinical mentality.

Psychiatric care and mental hygiene in Spain during the 1930s was influenced by the presence of ideological, social, and political polarization. This peculiarity of Spain's history makes it impossible to find more or less uniformed discourses and attitudes in the country. This paper analyzes the eugenic discourse developed by Spanish pro-Fascist psychiatrists, concerning mental and racial hygiene. Some special cases are highlighted that illustrate the situation of the mentally ill during the Spanish Civil War: the evacuation of asylums near the front and the attempt to apply the psychiatric reform promoted by the Second Republic regime.Despite ideological and scientific differences, both mass sterilization of mentally disabled patients and physical extermination of the mentally ill cannot be identified as typical features of Spanish psychiatry, before or after the Civil War.

Background and Purpose— After acute stroke, an increased level of C-reactive protein (CRP) measured at discharge predicts unfavorable outcome. We sought to investigate whether CRP measured before tissue plasminogen activator (tPA) treatments may add prognostic information to guide stroke thrombolysis. Methods— Our target was 151 consecutive patients with an ischemic stroke involving the middle cerebral artery territory who received tPA within 3 hours of symptom onset. High-sensitivity CRP was measured before tPA administration, and CRP gene polymorphisms were determined (G1059C and C1444T). Functional outcome was evaluated by 3-month modified Rankin Scale (mRS). Results— A total of 143 tPA-treated patients were valid for analyses after exclusion of those with inflammatory diseases and those probably infected (CRP >6 mg/dL). Patients with history of previous stroke, hypertension, or atrial fibrillation had higher levels of CRP ( P P =0.002). Among the 94 patients with proximal middle cerebral artery occlusions, CRP level was 0.53 for 81 survivors versus 0.81 mg/dL for 13 who died ( P =0.001). CRP-survival association was found even among patients who recanalized by the end of tPA infusion ( P =0.007). A correlation between CRP and mRS was found ( r =0.36, P =0.02), although CRP polymorphisms were not related to neurological outcome. In a logistic regression model, CRP (odds ratio=8.51; 95% CI, 2.16 to 33.5; P =0.002) and age (odds ratio=6.25; 95% CI, 1.44 to 27.19; P =0.015) were the only baseline mortality predictors. Conclusions— Admission CRP predicts mortality among tPA-treated stroke patients. Very early recanalization does not ameliorate the negative prognostic impact of elevated CRP.

Background and Purpose— We aimed to determine clinical and hemodynamic predictors of early reocclusion (RO) in stroke patients treated with intravenous tissue plasminogen activator (tPA). Methods— We studied 142 consecutive stroke patients with a documented middle cerebral artery (MCA) occlusion treated with intravenous tPA. All patients underwent carotid ultrasound and transcranial Doppler (TCD) examination before tPA bolus. National Institutes of Health Stroke Scale (NIHSS) scores were performed at baseline and serially for Results— After 1 hour of tPA administration, RE occurred in 84 (61%) patients (53 partial, 31 complete). Of these, 21 (25%) patients worsened after an initial improvement and 17 (12%) of them showed RO on TCD. RO was identified at a mean time of 65±55 minutes after documented RE. RO was associated ( P =0.034) with a lower degree of 24-hour NIHSS score improvement than sustained RE, and a higher modified Rankin scale score at 3 months ( P =0.002). Age older than 75 years ( P =0.012), previous antiplatelet treatment ( P =0.048), baseline NIHSS score >16 points ( P =0.009), higher leukocytes count ( P =0.042), beginning of RE P =0.039), and ipsilateral severe carotid stenosis/occlusion ( P =0.001) were significantly associated with RO. In a logistic regression model, NIHSS score >16 at baseline (odds ratio [OR], 7.1; 95% CI, 1.3 to 32) and severe ipsilateral carotid disease (OR, 13.3; 95% CI, 3.2 to 54) remained as independent predictors of RO. Conclusions— Stroke severity and ipsilateral severe carotid artery disease independently predict RO after tPA-induced MCA RE.

Background and Purpose—We sought to investigate the impact of hyperglycemia before reperfusion on long-term outcome in patients treated with intravenous tissue plasminogen activator (tPA).Methods—Of 268 consecutive patients with a nonlacunar middle cerebral artery (MCA) stroke evaluated at 140 mg/dL. National Institutes of Health Stroke Scale (NIHSS) scores were obtained at baseline and 24 hours. Transcranial Doppler monitoring of recanalization and reocclusion was conducted during the first 24 hours. Total infarct volume was measured on CT at day 5 to 7. Modified Rankin Scale was used to assess outcome at 3 months.Results—Median NIHSS score was 17. At baseline, 31 patients (42.5%) were hyperglycemic and 42 (57.5%) normoglycemic. Early reperfusion (r=−0.43;P=0.019) but not in nonreperfused (r=−0.20;P=0.21) tPA-treated patients. Increased age (P=0.014), history of diabetes mellitus (P=0.043), admission glucose >140 mg/dL (P=0.002), and early reocclusion (P=0.004) were factors associated with poor outcome among reperfused patients. A logistic regression modeling revealed that only admission glucose value >140 mg/dL (odds ratio, 8.4; 95% CI, 1.76 to 40.02;P=0.005) emerged as an independent predictor of poor outcome despite tPA-induced recanalization. In patients with 6-hour persistent MCA occlusion, baseline NIHSS score >15 points (P=0.011) and proximal MCA occlusion (P=0.039) were variables associated with poor outcome on univariate analysis. In a logistic regression model, only NIHSS score >15 points (odds ratio, 11.9; 95% CI, 1.48 to 97.1;P=0.032) remained as an independent predictor of poor outcome and functional dependence at 3 months in nonreperfused tPA-treated patients.Conclusions—Hyperglycemia before reperfusion may in part counterbalance the beneficial effect of early restoration of blood flow, which translates into a worse outcome in hyperglycemic patients despite tPA-induced recanalization.

Este artículo está sujeto a una licencia CC BY-NC-ND 4.0, The aim of this article is to highlight the importance of the history of public health for public health research and practice itself. After summarily reviewing the current great vitality of the history of collective health oriented initiatives, we explain three particular features of the historical vantage point in public health, namely the importance of the context, the relevance of a diachronic attitude and the critical perspective. In order to illustrate those three topics, we bring up examples taken from three centuries of fight against malaria, the so called “re-emerging diseases” and the 1918 influenza epidemic. The historical approach enriches our critical perception of the social eVects of initiatives undertaken in the name of public health, shows the shortcomings of public health interventions based on single factors and asks for a wider time scope in the assessment of current problems. The use of a historical perspective to examine the plurality of determinants in any particular health condition will help to solve the longlasting debate on the primacy of individual versus population factors, which has been particularly intense in recent times., Enrique Perdiguero and Esteban Rodríguez-Ocaña are funded by the Spanish Ministry of Education and Culture (Research Projects PB97–0782-C03–02 and PB97–0782-C03–01) .

[EN] This article aims to summarise, in the first instance, some of the historiographical trends which have built a “critical” history of psychiatry from the 1960s onwards. Thereafter, it will demonstrate, with suitably significant examples, how methods and discourses were being refined and updated, before reaching a proposal for a new cultural history of psychiatry and subjectivity. In our analysis, special emphasis is placed on the “patient's view”. This renders necessary the task of identifying little consulted sources, such as the writings of the mad, and the incorporation of interdisciplinary hermeneutic tools, including, most notably, those taken from cultural studies and, in particular, written culture. Finally, it will offer a reflection on the epistemic role that this historiographical approach could play in the construction of new ways of understanding mental health, such as that represented by so-called post-psychiatry., [ES] Otra historia para otra psiquiatría. La perspectiva del paciente.- Este artículo pretende revisar, en primer lugar, algunas de las tendencias historiográficas que han configurado una historia “crítica” de la psiquiatría a partir de los años sesenta del siglo XX. Se muestra a continuación, con algunos ejemplos suficientemente significativos, de qué manera los métodos y los discursos se fueron afinando y actualizando hasta llegar a la propuesta de una novedosa historia cultural de la psiquiatría y de la subjetividad. Especial importancia se concede, en nuestro análisis, al “punto de vista del paciente”. Esto obliga a una labor de identificación de fuentes escasamente trabajadas -como los escritos de los locos-, y a la incorporación de herramientas hermenéuticas de características interdisciplinares, entre las que destacan las procedentes de los estudios culturales y, de manera particular, de la cultura escrita. Finalmente, se ofrece una reflexión sobre el papel epistemológico que este enfoque historiográfico puede tener en la construcción de nuevas formas de entender la salud mental, como la representada por la llamada post-psiquiatría.