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- Motor Excitability Measurements in Early Stage Familial Amyloid Polyneuropathy: the Influence of Tafamidis Treatment.Publication . Casanova, Isabel; Caetano, André; Díaz, Andrés; Conceição, Isabel; Brum, Marisa; de Carvalho, MamedeObjective: To test motor fiber excitability in early affected patients with transthyretin (TTR)-type familial amyloid polyneuropathy (TTR-FAP) before and during tafamidis treatment. Methods: We examined the left median nerve of 21 healthy-matched controls and 10 early affected TTR-FAP patients using the automated threshold-tracking program, QTRAC. TTR-FAP patients were tested one day before the initiation of tafamidis treatment, 3 and 6 months later. Results: The drug was well-tolerated in all patients; there was no drop-out. No statistical difference was found between healthy controls and TTR-FAP patients at study entry. On treatment, both stimulus intensity for 50% of the maximal motor response and rheobase increased significantly from entry to the last evaluation at 6 months (P<0.05). Strength duration time constant decreased significantly from the 3rd to the 6th month of evaluation (P<0.05). There was also a "fanning-out" effect on the late depolarization phase (TEd 90-100ms) as well as a shortened relative refractory period from study entry to the 6th month of evaluation. Conclusions: Threshold-tracking of median nerve motor fibers is not a helpful technique for the early diagnosis of TTR-FAP patients. Tafamidis was well-tolerated. We observed possible membrane hyperpolarization during treatment. Threshold tracking can contribute to documenting the action of new drugs to treat neuropathies. Tafamidis may change nerve electrical properties by reducing the burden of amyloid fibrils.
- HBV and VZV Seroprotection Loss in MS Patients Under DMT.Publication . Ladeira, Filipa; Oliveira, Tiago; Soares, Mafalda; Araujo, Cristina; Sousa, Ana; Brum, Marisa; Sequeira, João; Capela, CarlosBackground: Strategies recommended to decrease the risk of infection associated with the use of multiple sclerosis disease-modifying treatments include screening and immunization against common viral infections such as varicella-zoster (VZV) and hepatitis B (HBV). However, the data concerning the durability of those vaccine responses and the need for re-test is scarce. Objectives: We aimed to evaluate HBV and VZV seroprotection loss in MS patients under DMT. Methods: We conducted a cohort study including patients with basal seroprotective titers against HBV/VZV viruses and a subsequent serology performed at least 3 months apart. We evaluated predictors of seroprotection loss through a binary regression. Results: HBV seroprotection loss occurred in one-fifth of patients in a median interval of 21.3 months. Anti-CD20 treatment (OR 8.559 95%CI 3.467- 21.130, p < 000.1), age at last serology higher or equal to 55 years (OR 7.506, 95% CI 2.473-22.786, p < 0.001) and basal HBsAb titer (OR 0.992, 95%CI 0.987 -0.996, p=0.001) increase the risk of seroprotection loss. VZV seroprotection loss occurred rarely in a median interval of 21.3 months. We could not identify any factor associated with an increased risk of VZV seroprotection loss. Conclusions: Anti-CD20 drugs are associated with a loss of seroprotection against HBV in a short-interval follow-up.
- Cerebral Edema in Patients with severe Hemispheric Syndrome: Incidence, Risk Factors, and Outcomes-Data from SITS-ISTR.Publication . Escudero-Martínez, Irene; Thorén, Magnus; Ringleb, Peter; Paiva Nunes, Ana; Cappellari, Manuel; Rand, Viiu-Marika; Sobolewski, Piotr; Egido, Jose; Toni, Danilo; Chen, Shih-Yin; Tsao, Nicole; Ahmed, NiazBackground and purpose: Cerebral edema (CED) in ischemic stroke can worsen prognosis and about 70% of patients who develop severe CED die if treated conservatively. We aimed to describe incidence, risk factors and outcomes of CED in patients with extensive ischemia. Methods: Oservational study based on Safe Implementation of Treatments in Stroke-International Stroke Treatment Registry (2003-2019). Severe hemispheric syndrome (SHS) at baseline and persistent SHS (pSHS) at 24 hours were defined as National Institutes of Health Stroke Score (NIHSS) >15. Outcomes were moderate/severe CED detected by neuroimaging, functional independence (modified Rankin Scale 0-2) and death at 90 days. Results: Patients (n=8,560) presented with SHS and developed pSHS at 24 hours; 82.2% received intravenous thrombolysis (IVT), 10.5% IVT+thrombectomy, and 7.3% thrombectomy alone. Median age was 77 and NIHSS 21. Of 7,949 patients with CED data, 3,780 (47.6%) had any CED and 2,297 (28.9%) moderate/severe CED. In the multivariable analysis, age <50 years (relative risk [RR], 1.56), signs of acute infarct (RR, 1.29), hyperdense artery sign (RR, 1.39), blood glucose >128.5 mg/dL (RR, 1.21), and decreased level of consciousness (RR, 1.14) were associated with moderate/severe CED (for all P<0.05). Patients with moderate/severe CED had lower odds to achieve functional Independence (adjusted odds ratio [aOR], 0.35; 95% confidence interval [CI], 0.23 to 0.55) and higher odds of death at 90 days (aOR, 2.54; 95% CI, 2.14 to 3.02). Conclusions: In patients with extensive ischemia, the most important predictors for moderate/ severe CED were age <50, high blood glucose, signs of acute infarct, hyperdense artery on baseline scans, and decreased level of consciousness. CED was associated with worse functional outcome and a higher risk of death at 3 months.
- Editorial: Cerebral Venous Thrombosis.Publication . Heldner, Mirjam R; Aguiar de Sousa, Diana; Field, Thalia S; Coutinho, Jonathan M; Zuurbier, Susanna M
- Prognostic Value of Kappa Free Light Chains Determination in First-Ever Multiple Sclerosis Relapse.Publication . Salavisa, Manuel; Paixão, Pedro; Ladeira, Ana Filipa; Mendes, Alexandra; Correia, Ana Sofia; Viana, João Faro; Viana-Baptista, MiguelGiven its highly variable clinical course, an unmet need for objective prognostic assessment in Multiple Sclerosis (MS) persists. In this work, we suggest that CSF kappa free light chains (KFLC) determination at first relapse may provide insight into future disease activity and disability worsening. We quantified KFLC by nephelometry in paired CSF/serum samples of 28 patients, collected within one month of first-ever MS relapse, and explored correlations with clinical data on disease activity, retrospectively registered across a median follow-up time of 79 months. We documented KFLC ratio (CSF-FKLC/Serum-KFLC) as an independent predictor of second relapse occurrence and disability worsening at follow-up, in this cohort.
- Previous Disability and Benefit of Acute Phase Therapy in Functional Prognosis of Selected Patients with Ischemic Stroke.Publication . Rosa, José Lourenço; Alves, Marta; Ferreira, Patrícia; Papoila, Ana Luísa; Nunes, Ana PaivaObjectives: Patients' previous disability (PD) is a key factor when considering acute stroke therapy. PD's exact impact on functional prognosis of patients with acute ischemic stroke remains not entirely clarified. We aimed to analyze PD's influence on functional outcome three months after ischemic stroke. Materials and methods: Retrospective analysis of prospectively collected data concerning patients with acute ischemic stroke admitted to Stroke Unit of a tertiary center who underwent acute phase therapy between 2017 and 2019. Modified Rankin Scale (mRS) was used to define PD (with previous mRS≥3). Patients with PD were selected for treatment based on similar baseline characteristics to patients without PD. Patients were classified into two groups according to previous mRS: mRS<3 and mRS≥3. We defined bad outcome at three months after stroke as mRS≥3 for patients with previous mRS<3, and as a higher score than baseline mRS for patients with previous mRS≥3. Results: We identified 1169 eligible patients - 1016 patients with previous mRS<3 and 153 patients with previous mRS≥3. Most baseline characteristics did not differ significantly between them. For patients ≤75 years old, PD was associated with worse outcome (odds ratio estimate [OR] 4.50, p < 0.001). For patients >75 years old, PD was protective against worse outcome (OR 0.42, p < 0.001). In patients with previous mRS≥3 and >75 years old, there was a higher proportion of women (p = 0.005). Conclusions: PD might not be a relevant factor when considering acute stroke therapy in selected patients >75 years old, especially women. Further studies are needed to clarify these findings.
- Sporadic Creutzfeldt-Jakob Disease As a Mimic of Progressive Supranuclear Palsy.Publication . Lourenço Rosa, José; Dias, Sara P; Dias, Margarida
- Predictors of Cognitive Stability or Decline During Aging: a Longitudinal Study in Primary Care.Publication . Pavão Martins, Isabel; Maruta, Carolina; Morgado, Joana; Loureiro, Clara; Tavares, Joana; Freitas, Vanda; Lunet, Nuno; Viana, Pedro; Marques, PauloWe aimed to identify the early predictors of cognitive decline, and primary care physicians' (PCP) ability to diagnose cognitively impaired subjects, in a cohort of individuals recruited in primary care centers. Independent adults, aged ≥50 years at inception, with an overall low level of education, undertook a prospective clinical and cognitive evaluation targeting memory, attention and executive functions. At follow-up subjects were classified as cognitively normal (CN) or impaired (CI). Of 275 subjects (70.4 ± 8.3 years old, 176 females, 7.5 ± 4.4 education, 162 with MRI), 31 (11.2%) presented CI 4.9 years later, the majority (64.5%) presenting subjective cognitive complaints. PCP could correctly identify 40% of CI individuals, particularly if they presented current cognitive complaints. Male sex (OR = 3.117; CI95%: 1.007-9.645), age (OR = 1.063; CI95%: 1.004-1.126) and baseline scores on TMT-B (OR = 0.225; CI95%: 0.073-0.688) and Vocabulary (OR = 0.940; 95% CI: 0.894-0.986) predicted CI. This study shows that measures indicating poor cognitive reserve and low executive performance (as shown by low vocabulary and executive test scores, respectively) can be early indicators of the risk of decline, stressing the role of cognitive assessment as part of prevention/early intervention programs. The results also underline the need to help PCP to improve the detection of subjects with cognitive decline.
- Characteristics and Outcomes of Cerebral Venous Thrombosis Associated With COVID-19.Publication . Scutelnic, Adrian; van de Munckhof, Anita; Miraclin, Angel T; Aaron, Sanjith; Hameed, Sajid; Wasay, Mohammad; Grosu, Oxana; Krzywicka, Katarzyna; Sánchez van Kammen, Mayte; Lindgren, Erik; Moreira, Tiago; Acampora, Roberto; Negro, Alberto; Karapanayiotides, Theodoros; Yaghi, Shadi; Revert, Anna; Cuadrado Godia, Elisa; Garcia-Madrona, Sebastian; La Spina, Paolino; Grillo, Francesco; Giammello, Fabrizio; Nguyen, Thanh N; Abdalkader, Mohamad; Buture, Alina; Sofia Cotelli, Maria; Raposo, Nicolas; Tsivgoulis, Georgios; Candelaresi, Paolo; Ciacciarelli, Antonio; Mbroh, Joshua; Batenkova, Tatiana; Scoppettuolo, Pasquale; Zedde, Marialuisa; Pascarella, Rosario; Antonenko, Kateryna; Kristoffersen, Espen S; Kremer Hovinga, Johanna A; Jood, Katarina; Aguiar de Sousa, Diana; Poli, Sven; Tatlisumak, Turgut; Putaala, Jukka; Coutinho, Jonathan M; Ferro, José M; Arnold, Marcel; Heldner, Mirjam RIntroduction: Previous reports and meta-analyses derived from small case series reported a mortality rate of up to 40% in patients with coronavirus disease 2019 associated cerebral venous thrombosis (COVID-CVT). We assessed the clinical characteristics and outcomes in an international cohort of patients with COVID-CVT. Patients and methods: This was a registry study of consecutive COVID-CVT patients diagnosed between March 2020 and March 2023. Data collected by the International Cerebral Venous Thrombosis Consortium from patients with CVT diagnosed between 2017 and 2018 served as a comparison. Outcome analyses were adjusted for age and sex. Results: We included 70 patients with COVID-CVT from 23 hospitals in 15 countries and 206 controls from 14 hospitals in 13 countries. The proportion of women was smaller in the COVID-CVT group (50% vs 68%, p < 0.01). A higher proportion of COVID-CVT patients presented with altered mental state (44% vs 25%, p < 0.01), the median thrombus load was higher in COVID-CVT patients (3 [IQR 2-4] vs 2 [1-3], p < 0.01) and the length of hospital stay was longer compared to controls (11 days [IQR 7-20] vs 8 [4-15], p = 0.02). In-hospital mortality did not differ (5/67 [7%, 95% CI 3-16] vs 7/206 [3%, 2-7], aOR 2.6 [95% CI 0.7-9]), nor did the frequency of functional independence after 6 months (modified Rankin Scale 0-2; 45/58 [78%, 95% CI 65-86] vs 161/185 [87%, 81-91], aOR 0.5 [95% CI 0.2-1.02]). Conclusion: In contrast to previous studies, the in-hospital mortality rate and functional outcomes during follow-up did not differ between COVID-CVT patients and the pre-COVID-19 controls.
- The Role of Disease Modifying Therapies in Late-Onset Multiple Sclerosis: A Portuguese Multicentric Characterization Study.Publication . Faustino, Patrícia; Cruz, Diana Marques; Fernandes, Catarina; Pereira, Andressa; Franco, Roberto; Costa, Sara; Matos, Sara; Morganho, Armando; Fraga, Carla; Santos, Ernestina; Ladeira, Filipa; Santos, Mónica; Abreu, Pedro; Batista, Sónia; Vale, José; Sá, Maria José; Santos, MarianaIntroduction: Knowledge about the effect of disease modifying treatment (DMT) in late-onset multiple sclerosis (LOMS, onset ≥50 years-old) is scarce. This study aims to evaluate the association between DMT use and multiple sclerosis (MS) evolution in a LOMS cohort. Methods: This multicentre, retrospective and observational study included LOMS patients with ≥2 years of follow-up. Data on demographics, clinical/paraclinical (baseline and follow-up), DMT and adverse events were collected. Primary outcomes were irreversible EDSS 4.0 and 6.0 achievement and first year ARR. Univariate and multivariate regression models were conducted, with treated and/or relapsing phenotypes (RMS) subgroups analyses. Results: We included 232 patients (53.4 % with RRMS phenotype; 84.9 % submitted to DMT; median follow-up time of 141.5 (IQR 92.7-193.1) months). Treatment versus non-treatment did not affect EDSS milestones in multivariate analysis (adjusted to phenotype, baseline EDSS, age, and ARR), but initially receiving monoclonal antibodies (MAbs) was associated with lower odds of EDSS 4.0 (OR 0.13). In treated patients, starting with high efficacy DMT (HE-DMT) was related to a lower chance of EDSS 4.0 (OR 0.05) and 6.0 (OR 0.26) compared with being exclusively treated with moderate efficacy DMT (ME-DMT), with similar results when analysing the subgroup of RMS treated patients. In multivariate models, initial treatment with MAbs (vs. non-treatment) and with HE-DMT (vs. ME-DMT) were related to a lower first year ARR; when considering only RMS patients, every DMT class analysed reduced first year ARR vs. non-treatment. During DMT, we documented a rate of 0.6 % serious infections, 0.07 % opportunistic infections and 0.7 % neoplasm diagnosis per patient year. Conclusion: DMT type and therapeutic strategy influenced LOMS disability accumulation and relapses in our cohort. Our findings support the importance of investment in LOMS treatment optimization.