Escorial Research Articles

Assessment of the Historical Gardens and Buildings Lighting Interaction through Virtual Reality: The Case of Casita de Arriba de El Escorial

Green areas and parks are places where people’s quality of life improves, places of recreation and relaxation, in which to carry out various social activities. Among these, the historic gardens represent the union between green areas and historic architecture. Light is considered one of the main factors influencing the perception of such spaces at night; therefore, lighting solutions should be properly balanced. Immersive virtual reality is a tool that allows for lighting design management from different viewpoints. This study investigates how the lighting of a monumental garden affects people’s perception of its architecture at night. With this aim, a detailed 3D model of the western part of Escorial’s monumental complex in Spain was built in DIALux evo 11 and Unreal Engine 4.27 and used to propose six different lighting scenarios. Participants viewed the scenarios through a head-mounted display and provided subjective feedback on the perceived light strength of the environment and architecture. Results highlight that illuminating surrounding areas affects the environment and building perception, as well as the order in which elements capture observer attention. In particular, lit elements between the observer and the façade can reduce façade importance.

Diagnostic utility of Gold Coast criteria for amyotrophic lateral sclerosis in Asia

Objective: This study aimed to reveal the diagnostic utility of Gold Coast (GC) criteria in Japanese patients with amyotrophic lateral sclerosis (ALS) by comparing the sensitivity/specificity with revised El Escorial (R-EE) and Awaji criteria, because its utility has not been studied in Asian ALS. Methods: Consecutive 639 patients (529 with ALS and 110 with ALS mimics), who were suspected of ALS and referred to three Japanese ALS centers, were enrolled. Diagnostic accuracy and characteristics of false positive and negative in GC criteria were compared with those of the Awaji and R-EE criteria. Patients were categorized as definite, probable or possible ALS according to each criterion. Results: The sensitivity of GC criteria (96.8%, 95% confidence interval [CI]: 95.3–98.3%) was higher than that of Awaji (89.6%, 95% CI: 87.0–92.2%) and R-EEC (89.2, 95% CI: 86.6–91.8%) criteria (both, p < 0.001). The specificity was also higher with GC criteria (77.3%, 95% CI: 69.5–85.1%) than Awaji (65.5%, 95% CI: 56.6–74.4%) and R-EEC (66.4, 95% CI: 57.6–75.2%) criteria (both, p < 0.01). Using GC criteria, patients with cervical spondylosis and Parkinson’s syndrome tended to be diagnosed with ALS (i.e. “false positive”). Additionally, ALS patients diagnosed only by GC criteria less frequently had upper motor neuron (UMN) signs, compared with the other two criteria. Conclusion: Gold Coast criteria improve diagnostic accuracy for ALS in an Asian population, especially in patients with subtle UMN signs.

Epidemiology of amyotrophic lateral sclerosis in the Krasnoyarsk region

Background. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that causes paralysis of muscles due to degeneration of motor neurons. According to available data, the incidence of ALS in different regions of the world is from 0.6 to 3.8 per 100,000 population per year. Data obtained during the study of the epidemiology of ALS at the regional level can be used in planning medical resources, in particular, calculating the necessary respiratory equipment and funds for nutritional support.Aim. Clinical and epidemiological analysis of ALS cases registered in the Krasnoyarsk region for the period 2018–2022.Materials and methods. A clinical and epidemiological analysis of ALS cases registered in the Krasnoyarsk region from 2018 to 2022 was conducted. To do this, we used data from the specialized office of neuromuscular pathology of the Regional Clinical Hospital in Krasnoyarsk and the Krasnoyarsk Regional Medical Information and Analytical Center. Diagnosis verification was carried out in accordance with the generally accepted El Escorial and Gold Coast criteria. The functional state and degree of neurological deficit were assessed using the ALS-FRS-R scale.Results. Over the period from 2018 to 2022, 78 cases of ALS were registered. The incidence rate was 0.54 cases per 100,000 population per year. In 2022, the prevalence rate was 1.16 cases per 100 thousand population. The time from symptom onset to diagnosis of the disease ranged from 4 to 57 months, with the average time to diagnosis being 17 months. Most patients were diagnosed with ALS when their ALS-FRS-R score decreased by 7–11 points. The division of patients into groups of rapid, moderate, and slow diagnostic correlates with life expectancy. Patients who are diagnosed quickly have a higher rate of disease progression, which results in a low life expectancy.Conclusion. The creation of specialized centers will improve the diagnosis and management of patients with ALS and plan the costs of palliative care. The increase in the incidence and detection of ALS emphasizes the need to develop new approaches to the treatment and rehabilitation of patients with ALS.

Evolution of the Amyotrophic Lateral Sclerosis Diagnostic Criteria Towards the Gold Coast Criteria

Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disorder characterized by progressive degeneration of upper and lower motor neurons (LMNs). Careful history taking, neurological examination, and electromyography are used to confirm its clinical diagnosis. The El Escorial, revised El Escorial and Awaji criteria have proposed varying degrees of diagnostic probability for ALS. However, such categories may cause uncertainty among patients regarding the certainty of diagnosis. Cases labeled as “possible ALS” also risk exclusion from clinical trials. To clarify and simplify the diagnostic criteria of ALS, the previous diagnostic categories of possible, probable, and definite were abandoned in the newly suggested criteria, Gold Coast criteria. The simplified criteria offer practical utility for precise diagnosis of LMN-predominant ALS and clinical trial recruitment going forward.

Genealogies of Spanish Architectural Knowledge in Roman Print

This article analyzes an album of prints published in Rome in the sixteenth and seventeenth centuries, organized in the tradition of the Speculum romanae magnificentiae. The album shows the various uses of architecture and sculpture, both antique and modern, in early modern Rome, and contains an engraving of El Escorial, published in 1606, on its last page. The plate reproduces the famous image realized in Madrid in 1589 for Juan de Herrera’s Estampas and is based on a plate published by Abraham Ortelius in Antwerp in 1591. The article analyzes the album from the point of view of the Roman print of El Escorial and argues that the album works, through its structure, to introduce the building to the print-buying public in Spanish Rome.

Traduir a l’edat mitjana: el repte d’editar la Legenda aurea de Jacobus de Voragine en català

The Catalan translation of the Legenda Aurea by Jacobus de Voragine has been considered by Meyer and by Corominesas a version very close to its Latin model. However, a review of the preserved manuscripts allows us to note remarkabledifferences from the Latin model as well as among the Catalan manuscripts. In this work we take as a basis the manuscriptE (ms. N-III-5, Madrid, Real Biblioteca del Monasterio de San Lorenzo de El Escorial) to analyse structural, editorialand textual changes in the Catalan compilation: structural changes, such as those related to the arrangement of chapters;editorial changes, such as the deletion of extensive fragments in some chapters or the replacement of the version of achapter with another one other than that by Voragine; and textual changes, such as the variants that characterize the twobranches into which the translation evolves. These changes allow us to characterize the formation and the evolution of thiscompilation. We take as a point of reference manuscript E, representative of one of the two branches of the translation,in contrast with P (ms. Espagnol 44, Paris, Bibliothèque nationale de France), representative of the other. In E thereare important internal differences in the wording of each chapter and differences with respect to the other preservedmanuscripts. In some chapters, the wording of E is very different from that of P, with a less literal version of the Latin text,a wording that will serve as a model for the later print tradition. Editing this compilation based on manuscript E involvestaking into account the rest of the preserved manuscript evidence, as well as the Latin text, in addition to considering thechapters added in this manuscript.

Incidence of Motor Neuron Disease (MND) in Patients Diagnosed with Cervical Spondylotic Myelopathy (CSM)

Objective: Motor neuron disease (MND) can occur in patients with cervical spondylotic myelopathy. The purpose of this study was to determine the prevalence of motor neuron disease in individuals suffering from cervical spondylotic myelopathy (CSM). Materials &amp; Methods: A prospective study was conducted at the Lady Reading Hospital. The patients(n = 70) included were between ages 55 to 75 with established clinical and radiological diagnoses of CSM. Motor neuron disease was diagnosed clinically by Awaji criteria. The patients were evaluated with NCS/EMG (Nerve Conduction Studies/Electromyography) and followed for 6 months. The motor neuron disease was confirmed by modified El Escorial criteria. Results: 40 patients had bilateral upper limb weakness (57%), however, the rest of the 30 patients had unilateral upper extremity weakness (42.8%). Hoffman’s sign was positive in 60% of patients. Lhermitte's sign was also positive in 31% of patients. The majority of patients (55.7%) were found with grade 4 for deltoid followed by 51.4% patients for biceps brachii. MRI showed anterior cord compression at the level of C5 and C6 in 27.14% of patients and C6 C7 in 32.8% of patients. 15.7% of patients were suspected of having both CSM and MND. Conclusion: CSM and MND can occur in the same patients. The incidence of MND is 5.7% in patients with cervical spondylotic myelopathy, hence in CSM patients, workup must be done for MND as it affects the outcome of surgery in these patients.

Disease survival and progression in TARDBP ALS patients from Sardinia, Italy.

Common genes implicated in amyotrophic lateral sclerosis (ALS) development may also influence its progression rate. The C9orf72 mutations featured a faster progression rate while the European SOD1 mutations were associated with a slower progression. In this study, we assessed the relationship between TARDBP and ALS progression/survival. ALS incident patients (2010-2019) were diagnosed by El Escorial revised criteria and staged over the disease course by the King's staging system. Disease progression was analysed by Kaplan-Meier survival curves and Cox regression models, with survival measured from symptom onset to death/tracheostomy or censor date. The study population included 76 patients carrying TARDBP mutations (A382T/G295S), 28 patients carrying the C9orf72 GGGGCC expansion, and 158 patients who had no evidence of causative genetic mutations (nmALS group). TARDBP patients reached death/tracheostomy later than C9orf72 and nmALS patients, independently of possible prognostic indicators (sex, age at ALS onset, diagnostic delay, phenotype at onset, and family history of ALS). On King's staging, the time elapsed between disease onset (King's stage 1) and involvement of the second body region (King's stage 2B) was similar in TARDBP and nmALS patients but longer in TARDBP than in C9orf72 patients. TARDBP patients reached King's stages 3 and 4 later than C9orf72 and nmALS patients. TARDBP patients have a better survival/prognosis than C9orf72-positive and nmALS patients. King's staging also suggested that the higher survival rate and the slower progression associated with the TARDBP mutation could mainly be attributed to the longer time elapsed between King's stages 2B to 3.

Management of amyotrophic lateral sclerosis in clinical practice: Results of the expert consensus using the Delphi methodology

Amyotrophic lateral sclerosis (ALS) is a rare disease characterized by a progressive and irreversible degeneration of upper and lower motor neurons leading to death. In France, limited data exist describing the criteria used in clinical practice for diagnosis and follow-up, and how novel therapies may fit in. The objective of this Delphi panel was to obtain an overview of current French practices in ALS diagnosis, management, and follow-up by determining the scales and criteria used in clinical practice outside of clinical trials, as well as the place of a future treatment like AMX0035, acting on endoplasmic reticulum (ER) stress and mitochondrial dysfunction, in the current therapeutic strategies. A questionnaire was administered to 24 ALS healthcare providers practicing in ALS centers in France. Two rounds of remote voting were organized, before proposition of final consensus statements. Consensus was considered reached when at least 66% of the voters agreed. Consensus were obtained to define the new Gold Coast criteria as the ones used in clinical practice to establish the diagnosis of ALS, thus replacing the revised El Escorial criteria, considered too complex and now mainly used to characterize the patient populations to be included in clinical trials. The clinical factors considered to establish ALS diagnosis are mainly the demonstration of progression of the motor deficit and elimination of differential diagnoses. The ALSFRS-R scale is used in daily clinical practice to assess patient's functional impairment in terms of number of points lost, with the bulbar, respiratory, and fine motor subscores being the most important to evaluate independently. A critical medical need was identified regarding the provision of new therapeutic alternatives in ALS. The panel members would support the earliest management of patients. In this landscape, based on data from a very encouraging phase II (Centaur trial), AMX0035 represents a new tool of choice in current treatment strategies for all patients for whom experts are confident in the diagnosis of ALS, in combination with riluzole. These results will need to be confirmed by the ongoing phase III trial (Phoenix trial).

Clinical Profile of Various Mnd Cases that Presented to a Tertiary Centre over the Past 3 Years

INTRODUCTION: Motor neurone disease (MND) is a disabling and ultimately fatal disease of the motor system, with few effective treatments. Considerable heterogeneity is observed in the clinical motor features of MND, with extra-motor manifestations now also recognized as part of the condition. Progressive motor weakness and bulbar dysfunction lead to premature death, usually from respiratory failure. Diagnosis remains clinical. There are no specific laboratory markers which make the diagnosis independent of the clinical pattern but there are a few appropriate investigations to exclude mimics. The clinical syndrome once observed is recognized easily because of a distinctive appearance and course. OBJECTIVES: To demonstrate and compare the etiology, demography &amp; clinical profile of various forms of Motor Neuron Diseases DESIGN: Cross sectional (Observational) study SETTING: Sapthagiri Institute of Medical Sciences &amp; Research Centre METHODS: 30 MND cases diagnosed clinically (as per Awaji Shima modification of the El Escorial Criteria) with few supportive investigations that presented to our centre over the past 3 years were compiled and compared CONCLUSIONS: Mortality rates in MND depends on type of MND and subtypes like Madras Motor Neuron disease, SMA, PLS has the slowest progression and best prognosis of all the subtypes. Secondary MND due to exposure to heavy metals has faster progression and worst prognosis

Clinical factors and vascular endothelial growth factor as determinants of disease progression in Indian patients with amyotrophic lateral sclerosis

Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. Prognostication remains sub-optimally defined. We aimed to assess clinical determinants of disease progression rates in Indian patients with ALS and to assess the role of vascular endothelial growth factor (VEGF) in disease progression. Methods In this cross-sectional study, consecutive patients with clinically definite/probable ALS according to the revised El Escorial criteria and controls were included. Patients were classified into fast or slow progressors based on disease progression rate (DPR). Serum and CSF VEGF level was assessed for patients and controls. Results Of 142 patients recruited, 93 (65.5%) were male. Mean age at enrollment was 49.37 ± 12.65 years. Mean duration of symptoms was 20.53 ± 20.88 months. Mean DPR was 1.14 ± 0.94. Based on DPR, 81 (57%) patients were slow progressors and 61 (43%) were fast progressors. Univariate analysis demonstrated a statistically significant association of DPR with age at onset, symptom duration, time to spread, wasting of small muscles of the hand, frontal release signs, and neurophysiologic bulbar abnormalities. On multivariate analysis, age at onset and symptom duration had a significant association with disease progression. The CSF VEGF levels of ALS patients (46.18 ± 27.8) were significantly elevated compared to controls (25.95 ± 25.64 pg/ml) (p = 0.001), but not serum VEGF. Conclusion Age at symptom onset and duration of disease had a significant impact on disease progression in Indian patients with ALS. CSF VEGF levels were significantly elevated in ALS compared to controls, indicating the role of CSF VEGF as a potential biomarker.

Materiality and Language at El Escorial

Abstract David Bestué’s recent take on Philip II’s reification of centrality (El Escorial: Imperio y estómago, 2021) is an open contestation of the royal site’s conception—and later reception—as an immutable, transcendental edifice meant to “stop the entropy of physical processes.” Distancing himself from castizo evocations of El Escorial as “piedra lírica” (Ortega y Gasset) and “líneas puras” (Unamuno), Bestué guides us through the building’s secluded sewers, the granite quarries that nourished its construction, and its many scenes of fire and dereliction to dismantle the monastery’s value as purely symbolic permanence. As Bestué explains, his book functions “no como un acto más de significación, otra capa, sino como un corte.” This mechanistic notion of the written word as a device with which to shape abstraction back into physicality, however, is not a novel addition to the literature on El Escorial. This article revisits José de Sigüenza’s Fundación del monasterio de El Escorial (1605)—the most exhaustive and authoritative chronicle of its construction—as a reaction to the official representational apparatus of the building. Whereas the language of Philip II’s instructions allied with Juan de Herrera’s designs to project an immutability beyond space and time, Sigüenza, this article shows, enacts a ventriloquist mimicking of artisanal practice that vindicates process and local experience in conflict with the notion of a petrified center of empire. It also examines, as reverberations of these disparities, late nineteenth- to early twentieth-century appraisals of Sigüenza’s text that have projected the unblemished geometrical lines of the Escorial onto the friar’s writing. Its overall aim is to shed light on the lingering tensions around language, architecture, and place that characterize the most instrumentalized—and yet paradoxically dematerialized—of Iberian materialities.

Lack of Association between CD33 rs3865444 and Amyotrophic Lateral Sclerosis: A Case-Control Study.

Microglial activation is considered to assume a role in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). To date, the relationship between ALS and the rs3865444 polymorphism of the cluster of differentiation 33 (CD33) has not been explored. The current report aimed to investigate the potential connection between CD33 rs3865444 and ALS. Patients diagnosed with sporadic ALS according to the revised El Escorial criteria, as well as age and sex matched community controls, were enrolled. Two evenly numbered, age and sex matched groups of 155 participants each were genotyped. No association was found between rs3865444 and ALS [log-additive odds ratio (OR) = 0.83 (0.57, 1.22), over-dominant OR = 0.86 (0.55, 1.36), recessive OR = 0.73 (0.25, 2.17), dominant OR = 0.82 (0.52, 1.29), co-dominant OR1 = 0.68 (0.23, 2.05) and co-dominant OR2 = 0.84 (0.53, 1.33)]. Moreover, no relationship was established between rs3865444 and the age of ALS onset based on both unadjusted and sex adjusted Cox-proportional hazards models. Finally, no association between rs3865444 and ALS was found in subgroup analyses based on the site of ALS onset (bulbar or spinal) and sex. The current analysis is the first to report that rs3865444 is not linked to ALS. Larger multi-racial studies are required to confirm these findings.

Lorca’s New York Drawings: Fabulous Beasts, Leashed Wild Animals and the So-called Escena de domador

Ten drawings that Lorca produced in New York or shortly thereafter feature animales fabulosos. This article proposes that he found inspiration not only in the illuminations of the Beato de Liébana manuscript held at El Escorial but also in two others recently acquired by the Morgan Library. Furthermore, the exploration of other medieval images depicting leashed wild animals, alongside the elucidation of certain elements of circus culture observable at Coney Island—the sideshow strongman—lead to a significant reinterpretation of the relationship between figure and beast in one of those ten works, namely the Escena de domador con animal fabuloso.

Gender-specific association of uric acid and survival in sporadic amyotrophic lateral sclerosis patients

ObjectiveTo investigate the relationship between serum uric acid (UA) and survival in sporadic amyotrophic lateral sclerosis (sALS) patients. MethodA total of 801 sporadic amyotrophic lateral sclerosis (sALS) patients fulfilled the revised El Escorial criteria were enrolled and followed up in the study. Baseline clinical data and laboratory variables including gender, age, age of onset, site of onset, disease duration, body mass index (BMI), uric acid (UA), creatinine (Cr), and creatine kinase (CK) were collected during enrollment. Multivariate Cox regression models were used to evaluate the survival-related factors after adjustment for confounders. ResultsThe serum UA level was significantly lower in female patients than that in male patients (243.5 vs 314.9 μmol/L, p < 0.001). Gender, BMI, Cr, CK were significantly associated with the level of uric acid according to the linear regression analysis. In the multivariate Cox regression analysis, higher serum UA level (>268.0 μmol/L) was an independent protective factor for prolonged survival among female patients (HR = 0.69, P = 0.042) after adjustment for confounders. ConclusionThe present study provided further support that higher UA was a protective factor for survival in sALS patients, especially in female.

An Irish Rebel in New Spain: The Tumultuous Life and Tragic Death of William Lamport

An Irish Rebel in New Spain: The Tumultuous Life and Tragic Death of William Lamport

Parasympathetic cardiac dysfunction in Amyotrophic Lateral Sclerosis (P10-8.015)

<h3>Objective:</h3> Study the presence of cardiac dysautonomia in ALS patients. <h3>Background:</h3> Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the upper and lower motor neurons. Diffuse neuronal affection has been observed in more frequent neurological diseases of degenerative etiology. There are isolated and sometimes contradictory studies addressing cardiac dysautonomia in ALS. Both orthostatic hypotension (sympathetic nervous system) and reduced RR interval variability (parasympathetic nervous system) are associated with a higher morbimortality. <h3>Design/Methods:</h3> Demographic variables, ALS-FRS and MRC scores and forced vital capacity (FVC) were registered in 20 ALS patients (with definite or probable diagnosis according to El Escorial Revisited criteria) attending a regular neurological control. We performed dynamometry, orthostatic testing and manually evaluated the RR interval variability in resting, deep breathing and post Valsalva maneuver electrocardiograms. All patients signed the informed consent approved by the hospital’s ethics committee. Logistic regression was used to address the relationship between cardiac parasympathetic dysautonomia and the independent relevant variables. <h3>Results:</h3> The mean age was 52 years (SD 13.79), with 75% of male patients and 85% of spinal onset. The mean ALS-FRS and FVC scores were 25.65 (SD 10.55) and 67% (SD 21). Only one patient had orthostatic hypotension while 12 (58.8%) showed reduced RR interval variability, with a strong tendency of association with age and not any other variable. <h3>Conclusions:</h3> Parasympathetic cardiac dysautonomia was very frequent in ALS patients. Larger studies are needed to confirm this finding since its diagnosis could help to tailor treatment and follow up in order to reduce other possible causes of morbimortality in ALS patients. <b>Disclosure:</b> Cecilia Quarracino has nothing to disclose. Natalia Bohorquez Morera has nothing to disclose. Dr. Capani has nothing to disclose. The institution of Santiago Perez-Lloret has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ELEA LAboratories. Gabriel Rodriguez has nothing to disclose.

MicroRNA Expression in ALS: A Systematic Review and Bioinformatic Analysis (P8-8.013)

<h3>Objective:</h3> To identify dysregulated microRNAs in patients with ALS compared to controls. <h3>Background:</h3> ALS is a neurodegenerative disease characterized by degeneration of upper and lower motor neurons. The diagnosis is challenging and is delayed due to a lack of adequate tools. Currently, circulating microRNAs have evidence in their favor as diagnostic biomarkers in ALS. <h3>Design/Methods:</h3> We performed a systematic review and conducted a database search in PubMed, Embase, Scopus, and Web of Science until July 2021. We included articles about patients with ALS diagnosed by Revised El Escorial Criteria, who measure the expression of microRNA and compare it with controls. On the other hand, we excluded articles based on animal models, reviews, letters, and articles with no complete text. We used two independent reviewers for article selection, quality assessment, and data extraction. We used the BIOCROSS (biomarker-based cross-sectional studies) tool to assess the risk of bias. We conducted the bioinformatic analysis using the DIANA tools mirPath v.3.0 software. <h3>Results:</h3> We included 24 original articles and identified 23 microRNAs repeated twice or more times in the articles, showing consistency and significant expression in ALS patients compared to controls. From these 23 miRNAs, 10 were up-regulated (hsa-miR-338-3p, hsa-miR-100, hsa-miR-206, hsa-miR-133a/b, hsa-miR-31, hsa-miR-34c, hsa-miR-424, hsa-miR-36073p, and hsa-miR-542-3p) and 13 were down-regulated (hsa-miR-451, hsa-miR-638, hsa-miR-149, hsa-miR183, hsa-miR-3613-5p, hsa-miR-425-5p, hsa-miR-30c-5p, hsa-let-7a, hsa-miR-26b-5p, hsa-let-7f-5p, hsa-miR-409-3p, hsa-miR-148b-3p, and hsa-miR584-5p). Bioinformatic analysis suggests these microRNAs are involved with three genetic pathways: prion disease, fatty acid synthesis, and fatty acid metabolism. <h3>Conclusions:</h3> The 23 microRNA we identified might be the beginning of a panel to diagnose ALS in its early stages. The genetic pathways shown in the bioinformatic analysis can provide clues to the pathophysiology of ALS. In addition, there is a need to conduct more studies regarding the potential of miRNAs as biomarkers; these studies should be standardized to avoid variability among them. <b>Disclosure:</b> Dr. Aguilera Peña has nothing to disclose. Dr. Cardenas Cruz has nothing to disclose. Dr. Correa has nothing to disclose.

Low intensity exercise training in patients with amyotrophic lateral sclerosis – factors influencing eligibility and compliance to the clinical trial

ABSTRACT Background To date, there are no evidence-based recommendations for physical therapy in amyotrophic lateral sclerosis (ALS). The reason is a low number of related clinical trials (CTs), restricted sample sizes and a high dropout rate. It may influence the profile of the participants, while the final results might not translate to the general ALS population. Objective To analyze factors affecting the ALS patients’ enrollment and retention to the study, and to describe a profile of participants as compared to the eligible group. Methods A total of 104 ALS patients were offered participation in a CT of low-intensity exercises at home. Forty-six patients were recruited. Demographic and clinical data (El Escorial criteria, site of onset, diagnosis delay, disease duration, amyotrophic lateral sclerosis functional rating scale – revised [ALSFRS-R], Medical Research Council [MRC], hand-held dynamometry) were analyzed every 3 months. Results Male gender, younger age and a higher ALSFRS predicted enrollment, while male gender, higher ALSFRS-R and MRC predicted retention in the study. A long commute to the study site and a fast disease progression were the main reasons influencing both enrollment and retention. Despite a high dropout rate, study participants were representative for the general ALS population. Conclusion The above demographic, clinical and logistic factors need to be considered when designing studies in ALS population.

Increasing prevalence 2015–2019 of amyotrophic lateral sclerosis in Sardinia, Italy

BackgroundWhile amyotrophiclateral sclerosis (ALS) incidence has increased during the last decades, structured evidence on increased prevalence is lacking. After reporting a significant yearly increase of ALS incidence over a 10-year period, we checked for increased prevalence in Southern Sardinia over a quinquennium.MethodsALS patients (El Escorial Criteria) recruited from the study area and followed at ALS Centre, University of Cagliari, were included. Prevalence was computed for January 1, 2015 and January 1, 2019 and was calculated for the overall ALS population as well as for tracheostomized and non-tracheostomized patients.ResultsWe observed a non-significant trend for greater ALS prevalence in 2019 than in 2015 (18.31 per 100,000 vs. 15.26 per 100,000; rate ratio: 1.83, p = 0.01). By contrast, a significantly raising 2015 to 2019 ALS prevalence was observed in tracheostomized patients. No significant difference could be detected in non-tracheostomized.ConclusionsWe provided the highest prevalence rate to date reported in the worldwide literature, and also showed a non-significant raising ALS prevalence in the Sardinian population over a quinquennium. The trend in raising ALS prevalence was likely due to extended survival due to invasive interventions.