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Background and purpose: The aim of this study was to assess the neurological complications of SARS‐CoV‐2 infection and compare phenotypes and outcomes in infected patients with and without selected neurological manifestations. Methods: The data source was a registry established by the European Academy of Neurology during the first wave of the COVID‐19 pandemic. Neurologists collected data on patients with COVID‐19 seen as in‐ and outpatients and in emergency rooms in 23 European and seven non‐European countries. Prospective and retrospective data included patient demographics, lifestyle habits, comorbidities, main COVID‐19 complications, hospital and intensive care unit admissions, diagnostic tests, and outcome. Acute/subacute selected neurological manifestations in patients with COVID‐19 were analysed, comparing individuals with and without each condition for several risk factors. Results: By July 31, 2021, 1523 patients (758 men, 756 women, and nine intersex/unknown, aged 16–101 years) were registered. Neurological manifestations were diagnosed in 1213 infected patients (79.6%). At study entry, 978 patients (64.2%) had one or more chronic general or neurological comorbidities. Predominant acute/subacute neurological manifestations were cognitive dysfunction (N = 449, 29.5%), stroke (N = 392, 25.7%), sleep–wake disturbances (N = 250, 16.4%), dysautonomia (N = 224, 14.7%), peripheral neuropathy (N = 145, 9.5%), movement disorders (N = 142, 9.3%), ataxia (N = 134, 8.8%), and seizures (N = 126, 8.3%). These manifestations tended to differ with regard to age, general and neurological comorbidities, infection severity and non‐neurological manifestations, extent of association with other acute/subacute neurological manifestations, and outcome. Conclusions: Patients with COVID‐19 and neurological manifestations present with distinct phenotypes. Differences in age, general and neurological comorbidities, and infection severity characterize the various neurological manifestations of COVID‐19. [ABSTRACT FROM AUTHOR]

Background and purpose: COVID‐19‐related acute neurological phenotypes are being increasingly recognised, with neurological complications reported in more than 30% of hospitalised patients. However, multicentric studies providing a population‐based perspective are lacking. Methods: We conducted a retrospective multicentric study at five hospitals in Northern Portugal, representing 45.1% of all hospitalised patients in this region, between 1 March and 30 June 2020. Results: Among 1261 hospitalised COVID‐19 patients, 457 (36.2%) presented neurological manifestations, corresponding to a rate of 357 per 1000 in the North Region. Patients with neurologic manifestations were younger (68.0 vs. 71.2 years, p = 0.002), and the most frequent neurological symptoms were headache (13.4%), delirium (10.1%), and impairment of consciousness (9.7%). Acute well‐defined central nervous system (CNS) involvement was found in 19.1% of patients, corresponding to a rate of 217 per 1000 hospitalised patients in the whole region. Assuming that all patients with severe neurological events were hospitalised, we extrapolated our results to all COVID‐19 patients in the region, estimating that 116 will have a severe neurological event, corresponding to a rate of nine per 1000 (95% CI = 7–11). Overall case fatality in patients presenting neurological manifestations was 19.8%, increasing to 32.6% among those with acute well‐defined CNS involvement. Conclusions: We characterised the population of hospitalised COVID‐19 patients in Northern Portugal and found that neurological symptoms are common and associated with a high degree of disability at discharge. CNS involvement with criteria for in‐hospital admission was observed in a significant proportion of patients. This knowledge provides the tools for adequate health planning and for improving COVID‐19 multidisciplinary patient care. [ABSTRACT FROM AUTHOR]

Introduction: The management of multiple sclerosis during pregnancy has changed in recent years; it is no longer viewed as hindering the evolution of the disease. Lower relapse rates are expected during gestation, while the early puerperium may be more critical. Case report: We present two cases of active relapse‐remitting multiple sclerosis in pregnant women with different clinical courses, mirroring the disease heterogeneity and unpredictability. The first one, despite the ongoing activity, had two uneventful pregnancies. The second woman, previously controlled, experienced an increase in the disease activity during pregnancy. Discussions: The decision of maintaining disease‐modifying therapy throughout gestation could be a strategy to reduce postpartum relapses. [ABSTRACT FROM AUTHOR]

Objectives: The diagnostic criteria for inflammatory demyelinating diseases of the central nervous system have evolved over the past years. Close follow up and accurate multiple sclerosis (MS) diagnosis are key to optimal treatment. We retrospectively reviewed the patients who had a diagnostic revision. Methods: The medical records of patients referred to our MS clinic (Centro Hospitalar Universitário de São João, Porto, Portugal) (n = 635) between 2009 and 2016 were reviewed. A total of 62 patients were identified, 44 were misdiagnosed with MS; 18 had another diagnosis, later redefined as MS. A total of 44 controls (with MS diagnosis) were matched to the 44 misdiagnosed cases, and a statistical analysis was carried out to determine the predictors of misdiagnosis. Results: The mean age of our population (n = 44) was 51 ± 11 years; 77% were women. The mean duration of misdiagnosis was 13 years. Optic neuritis was the most frequent presenting symptom (25%), followed by spinal cord syndrome and non‐specific neurological symptoms (20% each). The most frequent MS misdiagnoses were non‐specific white matter hyperintensities, optic neuritis and cerebrovascular disease. The mean Extended Disability Status Scale and Multiple Sclerosis Severity Score was 0 (SD 2) and 0.6 (SD 2), respectively, in the misdiagnosed group, and 3.5 (SD 5.4) and 3.2 (SD 5.6), respectively, in the control group. From all the variables analyzed, we found that the presence of black holes and spinal cord lesions were predictors of a correct MS diagnosis (P < 0,05). Conclusions: Our series underlines the need to continuously rethink an MS diagnosis and to thoughtfully use paraclinical tests (oligoclonal bands and magnetic resonance imaging) avoiding an unfounded diagnosis. The first event might not be atypical, as has been reported in other series. [ABSTRACT FROM AUTHOR]

Objectives: To study the association between multiple sclerosis (MS) and other autoimmune diseases (AID). Methods: From a cohort of 635 patients with MS, 66 patients with an history of AID were selected. Controls, matched 1:1, were chosen from the population of patients with MS without autoimmune comorbidities. Further analysis aimed to compare these groups. Results: The prevalence of comorbid AID in our population was 10.4%. The mean age was 43 ± 13 years; 77% were women. A total of 15 different types of AID were identified, the predominant one was autoimmune thyroiditis (18/66; 27%). In 46% of patients, the AID was diagnosed before, and in 49%, it was diagnosed after the diagnosis of MS. The presence of family history of AID was not associated with the risk of developing AID. Our analysis found no association between the presence of AID and disease activity (new lesions, gadolinium enhancement, mean of annual relapse rate, initiation of a second‐line treatment), either when considering each variable individually or within a composite score. The median Extended Disability Status Scale and Multiple Sclerosis Severity Score was 1.5 (interquartile range [IQR] 3.0) and 1.0 (IQR 3.0), respectively, in the AID group, and 1.5 (IQR 3.0) and 2.0 (IQR 3.0), respectively, in the control group, and the difference was not statistically significant. The presence of a concomitant AID influenced therapeutic choice in nine patients. Conclusions: The present study underlines the varied range of AID that might coexist with MS. This common ground does not seem to have a negative effect on the disease course. [ABSTRACT FROM AUTHOR]

Objective: Clinically isolated syndrome (CIS) is a first demyelinating episode targeting the central nervous system. It can later fulfill the diagnostic criteria for multiple sclerosis (MS) or remain as a single event with no further clinical or paraclinical evidence of disease. Given the difficulty of predicting the long‐term course of the disease at the moment of the first episode, we intended to examine clinical and laboratory features associated with MS conversion, underlining the prognostic value of oligoclonal bands (OCBs) determination. Methods: We analyzed patients with an episode compatible with CIS (not fulfilling the 2010 McDonald criteria) as the first manifestation of MS (according to the same criteria), observed in our MS clinic (n = 82). Results: From our cohort, 76.8% of patients converted to MS. OCBs were present at baseline in 68.0% of MS patients, but also in 73.7% of stable CIS patients (P =.77). After stratification of the results by treatment, there was also no statistically significant association between the presence of OCBs and conversion to MS. Additionally, positive OCBs did not predict earlier conversion to MS. Conclusions: Our study outlines the high prevalence of positive OCBs in patients with CIS, even in those who, until now, do not fulfill the 2010 diagnostic criteria for MS. This raises questions about the early assumption of MS diagnosis based on OCB status. A very hasty diagnosis could result in chronic treatment initiation and a possibly lifelong stigma in patients who might never develop new lesions or have a second attack. [ABSTRACT FROM AUTHOR]

A 56-year-old man presented with painless impairment of muscle relaxation on vigorous contraction (eg, eyelid closure, hand grip, running). There were no episodes of paralysis, symptom progression, weakness or extramuscular symptoms. Five of his fifteen siblings had similar complaints. His serum creatine kinase was normal. Electromyography showed electrical silence on muscle relaxation, without myotonic discharges. DMPK, ClCN1 and SCN4A genetic testing was normal, but he had a homozygous pathogenic variant of ATP2A1 (c.1315G>A; pGlu439Lys). Brody disease is a rare autosomal recessive myopathy due to ATP2A1 mutations that reduce sarcoplasmic reticulum calcium-ATPase1 activity, hence delaying muscle relaxation. [ABSTRACT FROM AUTHOR]