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Background and Objectives: The 2023 criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) perform well in adults but have not been assessed in children., Methods: This prospective observational nationwide study includes children and adults with demyelinating syndromes or encephalitis, whose serum or CSF was found MOG-immunoglobulin G (IgG) positive at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic of Barcelona (Spain). Exclusion criteria were lack of clinical information and follow-up <1 year, and serum unavailable for antibody testing. The primary outcome was to assess the accuracy of the 2023 MOGAD criteria, using as gold standard the most plausible diagnosis after a follow-up >1 year. MOGAD criteria were retrospectively applied assessing core syndromes, supportive clinical-radiological features, and MOG-IgG titers. Patients tested ≤3 months of a disease attack (acute phase) or afterward (remission) were considered separately. The positive predictive value (PPV) of the criteria (true-positive [patients classified as MOGAD and MOGAD diagnosis last follow-up] divided by total positive [all patients classified as MOGAD]), and its 95% CI, was calculated with the Wilson procedure., Results: A total of 257 patients (133 children) were included in the study (median age 15 years [interquartile range 6-38], 54% female). Among 202 patients assessed during a disease attack, 158 (78%) had high MOG-IgG serum titers, 36 (18%) low titers, and 8 (4%) antibodies only in CSF. No differences were identified between patients with high and low titers, but those with low titers were more likely to have an alternative diagnosis at last follow-up (2/36 [6%] vs 0/158, p = 0.012). Supportive features were present in 230 of 257 (89%) patients, regardless of age, MOG-IgG titers, and core syndromes except for optic neuritis in adults whose assessment with orbital MRI was not systematic. Overall, 240 of 257 (94%) patients were well classified by the MOGAD criteria (e.g., 236 eventually having MOGAD and 4 alternative diagnoses), and 17 were wrongly classified (e.g., 11 eventually having MOGAD and 6 alternative diagnoses). Although the criteria classified better during disease attacks than during remissions (187 [96%] vs 49 [89%] serum MOG-IgG-positive patients were well-classified, p = 0.038), the PPV was high in both settings (99% [95% CI 97-100] vs 98% [95% CI 89-100])., Discussion: The 2023 MOGAD criteria correctly identified most children and adults with MOGAD. The highest accuracy occurred when they were applied during disease attacks., Classification of Evidence: This study provides Class IV evidence that the 2023 MOGAD criteria accurately identify adults and children with MOGAD.

Purpose: Fifty percent of cranial CT scans performed achieve no benefit and entail risks. Our aim is to determine the yield of non-traumatic urgent cranial-CT and develop a pretest clinical probability scale approach., Methods: Adult patients seen in our emergency department between 2017-2021 and referred for urgent cranial-CT for non-traumatic reasons were retrospectively recruited and randomly selected. Presenting complaint (PC), demographic variables, Relevant radiological findings (RRF) on the urgent cranial-CT and Relevant clinical-radiological findings (RCRF: admission need or RRF detection on the urgent cranial-CT or cranial CT/MRI in the following three months) were recruited., Results: We recruited 702 patients, with median age 62 [47-76] years, 363 (51.7%) females. RCRF were observed in 404 (57.55%); of these, 352 (50.1%) required admission. RRF were detected in 190 (27.06%): 36 acute ischemic and 27 acute hemorrhagic lesions, 115 masses, 9 edema, and 27 hydrocephalus. Predictive PC for urgent cranial-CT were motor, speech, sensory deficits, sudden alteration of mental status, epileptic seizure, cognitive impairment, neurological symptoms in cancer patients, acute headache without a prior history and with meningeal signs; nausea, vomiting, or hypertensive crisis; visual deficits, and dizziness. This algorithm provided sensitivity, specificity, positive predictive value, and negative predictive value (NPV, 95%CI in brackets) of 92.1% (89-94.5%), 27.5% (22.5-33.0%), 63.3% (59.2-67.2%), and 71.9% (62.7-80.0%), to diagnose RCRF, and 97.4% (93.4-99.1%), 21.3% (17.8-25.1%), 31.5% (27.7-35.4%), and 95.6% (90.1-98.6%), to diagnose RRF. In patients not requiring admission (n = 350), the NPV for RRF was 98.8% (93.6-100%); the negative likelihood ratio 0.08 (0.01-0.57), and sensitivity remained at 97.8% (82.2-99.9%). Applying it would have avoided performing 85/350 urgent cranial-CT (24.29%). To find one RRF, we would have gone from performing 7.8 (350/45) to 5.9 (265/45) CTs, failing to diagnose 1/45 (2.2%) RRF., Conclusions: This proposed clinical scale could potentially decrease 24% of urgent cranial-CT., (© 2024. The Author(s), under exclusive licence to American Society of Emergency Radiology (ASER).)

Introduction: Depression is one of the most prevalent psychiatric conditions in adulthood, reaching figures of around 20%. The methodologies used to study depression are varied, and range from a self-administered test to structured psychiatric assessment. Several studies of patients with multiple sclerosis (MS) have been conducted in the last 20 years, and figures of around 35% have been found for depressive symptoms, while depressive disorders are less frequent, at approximately 21%., Aims: The aim of this study is to evaluate the usefulness of patient self-reported scales such as the Beck depression inventory (BDI) for identifying depressive symptoms in patients with MS, and to analyse their correlation with the diagnosis of clinical depression or depressive disorder using the psychiatric clinical interview based on the criteria of the Diagnostic and statistical manual of mental disorders, fifth edition., Patients and Methods: This is a multicentre descriptive cross-sectional study of patients with MS and depressive symptoms. The BDI and the Hamilton depression rating scale (HDRS) were used, and the patients with the highest scores underwent psychiatric assessment., Results: A total of 191 patients were included; 81 of these (40.5%) had depressive symptomatology in the pathological range according to the BDI (cut-off point of 14), and 20 had a severe score (above 28). Nineteen patients with severe depressive symptoms according to both scales were selected and finally evaluated by a psychiatrist, who also assessed five patients who according to the neurologist had severe depressive symptoms despite a BDI score of almost 28, but did not reach that level. The suspected major depressive disorder was confirmed in only four (21%) cases with BDI scores indicative of severe symptoms. There is no correlation between the severity score as evidenced by the BDI and the psychiatric assessment. A major depressive disorder was diagnosed in 16 (66.6%) of the 24 patients with BDI > 26 evaluated by psychiatry. A score above 26 on the BDI enables identification of 75% of cases of depressive disorder without subtyping. The correlation between the HDRS and the BDI was statistically significant (r = 0.8; p < 0)., Conclusions: The BDI is a useful screening test for identifying patients with depressive symptoms; in specific terms, a score above 26 is probably indicative of a depressive disorder that may benefit from psychiatric assessment.

Introduction: Teriflunomide is a once-daily oral immunomodulator approved for relapsing forms of multiple sclerosis (MS) or relapsing-remitting multiple sclerosis (RRMS; depending on the local label), based on extensive evidence from clinical trials and a real-world setting on efficacy, tolerability and patient-reported benefits. The TERICARE study assessed the impact of teriflunomide treatment over 2 years on health-related quality of life (HRQoL) and some of the most common and disabling symptoms of MS, such as fatigue and depression., Methods: This prospective observational study in Spain included RRMS patients treated with teriflunomide for ≤ 4 weeks. The following patient-reported outcomes (PROs) were collected at baseline and every 6 months for 2 years: the 29-item Multiple Sclerosis Impact Scale version 2 (MSIS-29), the 21-item Modified Fatigue Impact Scale (MFIS-21), the Beck Depression Inventory (BDI-II), the Short Form (SF)-Qualiveen and the Treatment Satisfaction Questionnaire for Medication v1.4 (TSQM). Annualised relapse rate (ARR), disability progression according to the Expanded Disability Status Scale (EDSS), and no evidence of disease activity (NEDA-3) were also assessed., Results: A total of 325 patients were analysed. Patients had a mean (SD) age of 43.2 years (10.4), a mean baseline EDSS score of 1.75 (1.5), a mean number of relapses in the past 2 years of 1.5 (0.7), and 64% had received prior disease-modifying therapy (DMT). Patients showed significant improvements in the psychological domain of MSIS-29 from 35.9 (26.6) at baseline to 29.4 (25.5) at 18 months (p = 0.004) and 29.0 (24.6) at 24 months (p = 0.002). Levels of fatigue and depression were also reduced. After 2 years of treatment with teriflunomide, ARR was reduced to 0.17 (95% CI 0.14-0.21) from the baseline of 0.42 (95% CI 0.38-0.48), representing a 60.1% reduction. Mean EDSS scores remained stable during the study, and 79.9% of patients showed no disability progression. 54.7% of patients achieved NEDA-3 in the first 12 months, which increased to 61.4% during months 12-24. Patients reported increased satisfaction with treatment over the course of the study, regardless of whether they were DMT naive or not., Conclusion: Teriflunomide improves psychological aspects of HRQoL and maintains low levels of fatigue and depression. Treatment with teriflunomide over 2 years is effective in reducing ARR and disability progression., (© 2023. The Author(s).)

Multiple sclerosis (MS) is a chronic immune-mediated, inflammatory, and degenerative disease that is up to three times more frequent in young women. MS does not alter fertility and has no impact on fetal development, the course of pregnancy, or childbirth. The Pregnancy in Multiple Sclerosis Study in 1998 showed that pregnancy, mostly in untreated women, did not adversely affect MS, as disease activity decreased during pregnancy (although it significantly increased in the first trimester postpartum). These findings, together with the limited information available on the potential risks of fetal exposure to disease modifying treatments (DMTs), meant that women were advised to delay the onset of DMTs, stop them prior to conception, or, in case of unplanned pregnancy, discontinue them when pregnancy was confirmed. Now, many women with MS receive DMTs before pregnancy and, despite being considered a period of MS stability, up to 30% of patients could relapse in the first trimester postpartum. Factors associated with an increased risk of relapse and disability during pregnancy and postpartum include relapses before and during pregnancy, a greater disability at the time of conception, the occurrence of relapses after DMT cessation before conception, and the use of high-efficacy DMTs before conception, especially natalizumab or fingolimod. Strategies to prevent postpartum activity are needed in some patients, but consensus is lacking regarding the therapeutic strategies for women with MS of a fertile age. This, along with the increasing number of DMTs, means that the decision-making processes in aspects related to family planning and therapeutic strategies before, during, and after pregnancy are increasingly more complex. The purpose of this review is to provide an update on pregnancy-related issues in women with MS, including recommendations for counseling, general management, use of DMTs in pre-pregnancy, pregnancy, and postpartum periods, and breastfeeding-related aspects of DMTs., Competing Interests: RV has received lecture fees or travel grants from Biogen, Merck, Novartis, Roche, Sanofi, and Teva, personal fees from Sanofi-Genzyme, and reports no other potential conflicts of interest for this work., (© 2022 Villaverde-González.)

Objective: To determine the effect of disease-modifying drugs (DMDs) on disease activity rebound in patients discontinuing natalizumab (NTZ)., Methods: Twenty-one patients with relapsing-remitting multiple sclerosis (RRMS) treated with NTZ for ≥1 year and who switched to DMDs (glatiramer acetate [GA] or interferon) were followed up for 12 months in clinical practice. Clinical outcomes after NTZ cessation were assessed every 3 months for 1 year and MRI was performed at 12 months., Results: Twelve months after switching from NTZ to DMDs, there were no significant differences in the annualized relapse rate (ARR) compared to the days that NTZ was used (0.3 vs. 0.1; p = 0.083); and the ARR never reached similar values to those prior to NTZ use (1.61; p < 0.001). The percentage of relapse-free patients after switching from NTZ was 71.4%. These patients did not have lower disease activity before NTZ compared with those with clinical relapses (1.3 vs. 1.7; p = 0.302), but they had lower Expanded Disability Status Scale scores (3.4 vs. 5.7; p = 0.001). DMDs had beneficial effects on MRI parameters, as 10 of 16 patients (62.5%) presented no evidence of radiological activity 12 months after NTZ discontinuation., Conclusions: Patients with RRMS and moderate disability who discontinued NTZ for safety reasons may benefit from the DMDs GA and interferon with no known risk for progressive multifocal leukoencephalopathy., (© 2017 S. Karger AG, Basel.)

Background: The prevalence of multiple sclerosis (MS) varies throughout the world, and available epidemiological data suggest a progressively increasing prevalence of MS in Spain. The objective of this study was to calculate MS prevalence in Health District III of the autonomous community of Murcia in Spain., Methods: This is an observational, cross-sectional, descriptive study. The prevalence of MS in Health District III in the Region of Murcia, which includes the municipalities of Lorca, Totana, Águilas, Puerto Lumbreras and Aledo, was calculated from the total population (171,040 inhabitants), and among native Spanish citizens only (137,659 persons). Healthcare and demographic data were obtained from three sources: 1) OMI-AP: the local primary care computer system containing the medical records of all subscribers; 2) the medical record database of the Hospital Rafael Mendez (the single hospital in the district); and 3) the records of the AEMA III Multiple Sclerosis Association to which patients from this healthcare district belong. Data from these three sources were combined to check the accuracy and completeness of the patient records., Results: The prevalence of MS among the general population of this district, including non-Spanish individuals, was 71.9 per 100,000 inhabitants (95% CI=60-85). Prevalence among the native Spanish population was 82.0 per 100,000 (95% CI=68-98). Considering prevalence by sex, it was 118.1 per 100,000 (95% CI: 95-146) in the female native Spanish population, and 45.4 per 100,000 (95% CI: 31-64) in the male native Spanish population. The prevalence in the native Spanish population in this district was calculated by sex and age (grouped by decades). A peak was observed among women aged between 20 and 29 years: 234.2 per 100,000 inhabitants (95% CI: 151-361)., Conclusion: Our results suggest that the population in this healthcare district presents a risk of MS similar to that recently reported in other regions of Spain, which is higher than in previous decades., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Aim: To examine the use of extra-hospital emergency systems in the urgent care of stroke patients in our region and their influence on the time required to reach hospital, the time needed to perform an urgent computerised axial tomography (CAT) scan and the delay in receiving attention from the specialist., Patients and Methods: Samples were collected from 232 stroke patients out of the total number admitted to our hospitals. Data about the stroke were collected prospectively, and included the arrival time, the time required to perform the CAT scan and the time the specialist devoted to attending the patient. Data were also gathered about the different extra-hospital transport and emergency systems. A statistical analysis was performed to determine the effect of using the extra-hospital emergency procedures on the different variables., Results: A total of 53.6% of patients arrived within the first three hours. 38.7% went straight to hospital, 25% visited extra-hospital Emergency Services first, and 18.5% made a prior visit to Primary Care. 51.5% found their own way to the hospital and 46.7% arrived by ambulance. Mean time taken to perform an urgent CAT scan: 190.4 minutes; mean time required for specialist attention: 25.65 hours. The only statistically significant relation was the use of extra-hospital emergency systems and health care transport according to the type of stroke: both were more likely to be used in cases of haemorrhagic stroke., Conclusions: In hospitals in the Murcia region, the use of the extra-hospital emergency system and the means of transport utilised do not affect the time stroke patients take to reach hospital or the time needed to perform an urgent CAT scan or the delay in receiving attention from a specialist; the aetiology of the stroke does, however, influence the use of such services.

Introduction: Foreign accent syndrome (FAS) is a little known disorder affecting language which has been described in a few cases after acute strokes or traumatic brain injuries, but until now has not been reported in multiple sclerosis (MS). It is characterised by the appearance of what is perceived to be a foreign accent in the language of the patient. Although it could be included within the dysprosodias that accompany motor aphasias, it should be considered as an entity in its own right, since it may appear without the accompanying aphasia. Aphasia is an infrequent manifestation of MS and even less so when it appears as an initial symptom of the disease. When it does occur it usually accompanies large demyelinating lesions in the dominant hemisphere, and it is usually of a motor type., Case Report: Patient, aged 38 years, who presented FAS that accompanied mild non fluent aphasia as the first manifestation of MS with pseudotumoral lesions. Initially the clinical features were interpreted as a somatoform disorder, which delayed diagnosis., Conclusions: Like aphasia, FAS can occur in MS as a manifestation of a cortical language disorder. It is important to recognise this in order to prevent mistaken diagnoses.

Introduction: Oat (small) cell carcinoma is the type of tumour most frequently associated to neurologic paraneoplastic syndromes. It is usually located in the lungs although it has been described in some other locations. Cerebellar symptoms may appear alone, associated to anti Yo antibodies ( Breast and gynaecologic carcinomas), or as manifestation of a more generalized paraneoplastic encephalopathy, associated to signs and symptoms of some other neurologic systems affected., Case Report: A 52 year old patient consulted due to a pancerebellar clinical picture, which started about two months before, and later associated to polineuropathy. Abdominal CT showed a 4 cm mass in the head of the pancreas. Pathologic evaluation demonstrated a poorly differentiated small cell pancreatic tumour. Anti Hu antibodies in high titres were found both in serum and cerebrospinal fluid., Discussion: The association of anti Hu immunity and paraneoplastic encephalomyelitis has been observed in patients with neuroblastoma, seminomas, colorectal, breast and prostate carcinomas and some types of sarcoma. Only about 1% of pancreatic malignancies correspond to small cell type. We have not found any previous report about the association between a paraneoplastic syndrome and pancreatic poorly differentiated small cell carcinoma.