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1.
Can J Neurol Sci ; : 1-21, 2024 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-38312020

RESUMO

Autoimmune encephalitis is increasingly recognized as a neurologic cause of acute mental status changes with similar prevalence to infectious encephalitis. Despite rising awareness, approaches to diagnosis remain inconsistent and evidence for optimal treatment is limited. The following Canadian guidelines represent a consensus and evidence (where available) based approach to both the diagnosis and treatment of adult patients with autoimmune encephalitis. The guidelines were developed using a modified RAND process and included input from specialists in autoimmune neurology, neuropsychiatry and infectious diseases. These guidelines are targeted at front line clinicians and were created to provide a pragmatic and practical approach to managing such patients in the acute setting.

2.
Can J Neurol Sci ; : 1-7, 2023 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-37458116

RESUMO

BACKGROUND: There is a paucity of data on the obstetrical outcomes of Canadian pregnant patients with epilepsy, which may differ from the average Canadian pregnancy and from other populations of pregnant patients with epilepsy. METHODS: Pregnant patients with epilepsy were identified from a prospectively collected database of patients seen at the maternal-fetal medicine obstetrics program of Mount Sinai Hospital (Toronto, Canada) between January 1, 2014, and November 20, 2020. Pregnancy, delivery, and neonatal outcome data were retrieved from this database and described using 95% binomial confidence intervals. Comparisons of obstetrical outcomes over the same period among the Canadian population average, obtained from publicly available national health data, were done using one-proportion Z-tests for nominal variables and one-sample t-tests for continuous variables. RESULTS: In total, 282 pregnancies, from 224 patients, were included, which resulted in 274 live births. Mean maternal age was 32.8 years (s.d. = 4.6; population average [µ] = 30.9; p < 0.01), and 53% were primiparous (CI95% = 49%-61%; µ = 43%; p < 0.01). The observed rates of obstetrical complications were gestational hypertension 9% (CI95%=6%-13%; µ=7%; p=0.19), gestational diabetes 5% (CI95% = 3%-8%; µ = 9%; p = 0.02), cesarean section 44% (CI95% = 38%-50%; µ = 28%; p < 0.01), postpartum hemorrhage 5% (CI95% = 3%-8%; µ = 0.5%; p < 0.01), stillbirth 1% (CI95% = 0%-2%; µ=1%; p > 0.99), and prematurity 9% (CI95% = 6%-13%; µ = 8%; p = 0.44). CONCLUSION: In this cohort of Canadian pregnant patients with epilepsy from an urban tertiary care center, observed rates of obstetrical complications were rare and no higher than in the Canadian population over the same period, with the exception of cesarean section and postpartum hemorrhage. Future prospective studies that include primary care and rural settings are needed to increase the generalizability of those results.

3.
Can J Neurol Sci ; 50(2): 214-220, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-35022090

RESUMO

BACKGROUND: The North American AED Pregnancy Registry (NAAPR) provides crucial data for understanding the risks of antiepileptic drug (AED) exposure in pregnancy. This study aims to quantify the Canadian contribution to NAAPR and compare AED usage in pregnancy in Canada and the USA. METHODS: Enrollment rate ratios (ERR) to NAAPR, adjusted for the populations of women of childbearing age, were calculated for the USA, Canada, and for the different Canadian provinces. Methods of enrollment to NAAPR and AED usage were compared between the two countries using chi-squared tests. RESULTS: Between 1997 and 2019, 10,215 pregnant women enrolled into NAAPR: 4.1% were Canadian (n = 432, ERR = 0.39, CI95% = 0.35-0.43). Within Canada, no patients were enrolled from the three northern territories or from Prince Edward Island. While fewer patients than expected enrolled from Quebec (ERR = 0.35, CI95% = 0.19-0.58), Nova Scotia had the highest enrollment rate (ERR = 1.55; CI95% = 0.66-3.11). Compared with their American peers, Canadians were less likely to have been enrolled by their healthcare provider and more likely to have been enrolled via social media (p < 0.01). Canadian women were more likely to be taking carbamazepine (24% vs. 15%; p < 0.01) or valproic acid (8% vs. 4%; p < 0.01). CONCLUSION: The proportion of Canadian enrollees into NAAPR was less than expected based on the relative population size of Canadian women of reproductive age. Greater Canadian enrollment to NAAPR would contribute to ongoing worldwide efforts in assessing the risks of AEDs use in pregnant women and help quantify rates of AED usage, major congenital malformations, and access to subspecialized epilepsy care within Canada.


Assuntos
Anormalidades Induzidas por Medicamentos , Complicações na Gravidez , Feminino , Gravidez , Humanos , Estados Unidos/epidemiologia , Anticonvulsivantes/uso terapêutico , Anormalidades Induzidas por Medicamentos/tratamento farmacológico , Anormalidades Induzidas por Medicamentos/epidemiologia , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/epidemiologia , Sistema de Registros , Nova Escócia
4.
Can J Neurol Sci ; 45(5): 540-544, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29936915

RESUMO

BACKGROUND: A need exists to characterise the long-term cognitive outcomes in patients who recovered from autoimmune encephalitis and to identify the modifiable factors associated with improved outcomes. METHODS: We retrospectively analysed data from patients diagnosed with autoimmune encephalitis in our outpatient autoimmune encephalitis clinic over a 5-year period, where the Montreal Cognitive Assessment (MoCA) is routinely administered. RESULTS: In total, 21 patients met the inclusion criteria, of whom 52% had persistent cognitive impairment at their latest follow-up (median delay to testing=20 months, range 13-182). Visuospatial and executive abilities, language, attention, and delayed recall were predominantly affected. Patients with status epilepticus at presentation had lower total MoCA scores at their last follow-up (median total score 21, range 15-29) compared with patients without status epilepticus at presentation (median total score 27.5, range 21-30; r 2=0.366, p=0.004). Patients who experienced delays of more than 60 days from symptom onset to initiation of treatment (either immunosuppression or tumour removal) were more likely to have a MoCA score compatible with cognitive impairment at their last follow-up (r 2=0.253, p=0.0239; z-score=-2.01, p=0.044). CONCLUSIONS: Our study suggests that the MoCA may be used to evaluate cognition in recovering patients with autoimmune encephalitis. Delays to treatment shorter than 60 days and absence of status epilepticus at onset were associated with better performance on the MoCA obtained more than 1 year after symptom onset, and may predict better long-term cognitive outcomes.


Assuntos
Transtornos Cognitivos/diagnóstico , Transtornos Cognitivos/etiologia , Encefalite/complicações , Doença de Hashimoto/complicações , Adolescente , Adulto , Idoso , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Adulto Jovem
5.
J Neurovirol ; 23(1): 158-159, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27473195

RESUMO

A 63-year-old female with no significant past medical history was presented with a 5-day history of progressive opsoclonus-myoclonus, headaches, and fevers. Her workup was significant only for positive West-Nile Virus serum serologies. She received a 2-day course of intravenous immunoglobulin (IvIG). At an 8-week follow up, she had a complete neurological remission. Adult-onset opsoclonus-myoclonus syndrome is a rare condition for which paraneoplastic and infectious causes have been attributed. To our knowledge, this is the first case reported of opsoclonus-myoclonus secondary to West-Nile Virus treated with intravenous immunoglobulin monotherapy.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Opsoclonia-Mioclonia/tratamento farmacológico , Febre do Nilo Ocidental/tratamento farmacológico , Vírus do Nilo Ocidental/imunologia , Feminino , Humanos , Pessoa de Meia-Idade , Síndrome de Opsoclonia-Mioclonia/diagnóstico , Síndrome de Opsoclonia-Mioclonia/etiologia , Síndrome de Opsoclonia-Mioclonia/imunologia , Resultado do Tratamento , Febre do Nilo Ocidental/complicações , Febre do Nilo Ocidental/diagnóstico , Febre do Nilo Ocidental/imunologia , Vírus do Nilo Ocidental/isolamento & purificação , Vírus do Nilo Ocidental/patogenicidade
6.
Can J Neurol Sci ; 43(4): 543-8, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26963444

RESUMO

BACKGROUND: The acquisition of a new 320-row multidetector computed tomography angiography (CTA) scanner at the Montreal Neurological Institute and Hospital has provided higher quality imaging with less radiation exposure and shorter time of acquisition. However, its reliability has not been fully proven in critical vascular lesions when it comes to replacing a more invasive examination such as cerebral angiography. We wished to validate the accuracy of this equipment to investigate four common indications for patients to undergo conventional digital subtraction angiography: subarachnoid hemorrhage, vasospasm, unusual intracerebral hemorrhage, and unruptured aneurysm. METHODS: Radiological reports and relevant imaging from 82 consecutive subjects who underwent a 320-row multidetector CTA followed by cerebral angiography from February 2010 to February 2014 were retrospectively analysed. A total of 102 cerebrovascular anomalies were found. Reports from both imaging modalities were compared to determine the diagnostic accuracy of CTA. RESULTS: The overall sensitivity and specificity of 320-row multidetector CTA for detecting cerebrovascular abnormalities were, respectively, 97.60% and 63.20%. Similar results were obtained for all four categories of clinical indications. CONCLUSION: Results obtained from CTA were consistent with those obtained on digital subtraction angiography regardless of the vascular pathology. To our knowledge, this study is the first validating the accuracy of 320-row CTA in diagnosing critical cerebrovascular lesions.


Assuntos
Angiografia Digital/métodos , Transtornos Cerebrovasculares/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Processamento de Imagem Assistida por Computador , Adulto , Idoso , Bases de Dados Factuais/estatística & dados numéricos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
7.
J Comput Assist Tomogr ; 39(4): 541-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26182224

RESUMO

OBJECTIVE: To objectively assess the accuracy of 320-row multidetector computed tomographic (CT) angiography to diagnose cerebral vasospasm after a subarachnoid hemorrhage using a new quantitative method. METHODS: Fifty-four arterial segments were measured in 8 patients who had subarachnoid hemorrhage and underwent digital subtraction angiography within 24 hours after CT angiography for clinical suspicion of cerebral vasospasm. RESULTS: A correlation between arterial diameters measurements made on CT angiography and digital subtraction angiography was observed. The degree of vasospasm tended to be overestimated in the anterior circulation, with arterial diameters that were between 0.05 and 0.72 mm smaller than those on digital subtraction angiography. CONCLUSIONS: A quantitative approach can be used to objectively evaluate the ability of multidetector CT angiography to assess arterial diameter in patients with clinical symptoms of postsubarachnoid hemorrhage cerebral vasospasm. This pilot study also suggests that CT angiography may overestimate the degree of cerebral vasospasm in the anterior circulation.


Assuntos
Angiografia Cerebral/métodos , Aneurisma Intracraniano/complicações , Tomografia Computadorizada Multidetectores/métodos , Hemorragia Subaracnóidea/complicações , Vasoespasmo Intracraniano/diagnóstico por imagem , Vasoespasmo Intracraniano/etiologia , Idoso , Idoso de 80 Anos ou mais , Angiografia Digital , Meios de Contraste , Feminino , Humanos , Iopamidol , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Intensificação de Imagem Radiográfica , Reprodutibilidade dos Testes
8.
Epilepsy Res ; 202: 107336, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38471245

RESUMO

PURPOSE: Studies have shown an increased risk of epilepsy in patients with neurofibromatosis type 1 (NF1). However, most reports focus on the pediatric population. In this study, we describe the trajectory of patients with NF1 and epilepsy beyond childhood. METHODS: Patients with NF1 ≥18 years-old consecutively seen at a multidisciplinary neurofibromatosis clinic during a four-year period were prospectively enrolled and offered routine EEG, MRI, and genetic testing. The lifelong and point prevalence of epilepsy in patients with NF1 were calculated. Demographic, genetic, radiological, and clinical features found to be statistically associated with having received a diagnosis of epilepsy were incorporated into a logistic regression model. RESULTS: Among 113 patients with NF1 included in this study (median age at study inclusion: 33 years), the lifelong prevalence of epilepsy was 11% (CI95%=6-18%) and point prevalence 7% (CI95%= 3-13%). Most patients (73%) were diagnosed with epilepsy before the age of 18 and achieved seizure-freedom by adulthood. At study inclusion, three-quarters of patients with a diagnosis of epilepsy had been seizure-free for more than one year and a third had resolved epilepsy. A routine EEG with epileptiform discharges had a sensitivity of 25% (CI95%=3-65) and specificity of 99% (CI95%=93-100) for identifying adult patients with NF1 and unresolved epilepsy. A history of epilepsy was associated with having a low-grade glioma (OR: 38.2; CI95%=2.2-674.7; p<0.01), learning disability (OR: 5.7; CI95%=1.0-31.5; p<0.05), and no plexiform neurofibroma (OR: 0.05; CI95%=0.0-0.8; p=0.04). No single mutation type was associated with the development of epilepsy. CONCLUSIONS: In patients with NF1, although resolution of epilepsy over time was observed in many cases, the prevalence of epilepsy was higher among adults with NF1 than that reported in the general population. Epileptogenesis in NF1 likely requires the combination of multiple genetic and environmental factors and suggests involvement of a network that spreads beyond the borders of a well-defined parenchymal lesion.


Assuntos
Eletroencefalografia , Epilepsia , Neurofibromatose 1 , Fenótipo , Humanos , Neurofibromatose 1/genética , Neurofibromatose 1/complicações , Neurofibromatose 1/epidemiologia , Epilepsia/genética , Epilepsia/epidemiologia , Masculino , Feminino , Adulto , Prevalência , Adulto Jovem , Pessoa de Meia-Idade , Genótipo , Adolescente , Imageamento por Ressonância Magnética , Estudos Prospectivos
9.
Neurol Neuroimmunol Neuroinflamm ; 11(4): e200225, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38838283

RESUMO

BACKGROUND AND OBJECTIVES: Patients with ongoing seizures are usually not allowed to drive. The prognosis for seizure freedom is favorable in patients with autoimmune encephalitis (AIE) with antibodies against NMDA receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), and the gamma-aminobutyric-acid B receptor (GABABR). We hypothesized that after a seizure-free period of 3 months, patients with AIE have a seizure recurrence risk of <20% during the subsequent 12 months. This would render them eligible for noncommercial driving according to driving regulations in several countries. METHODS: This retrospective multicenter cohort study analyzed follow-up data from patients aged 15 years or older with seizures resulting from NMDAR-, LGI1-, CASPR2-, or GABABR-AIE, who had been seizure-free for ≥3 months. We used Kaplan-Meier (KM) estimates for the seizure recurrence risk at 12 months for each antibody group and tested for the effects of potential covariates with regression models. RESULTS: We included 383 patients with NMDAR-, 440 with LGI1-, 114 with CASPR2-, and 44 with GABABR-AIE from 14 international centers. After being seizure-free for 3 months after an initial seizure period, we calculated the probability of remaining seizure-free for another 12 months (KM estimate) as 0.89 (95% confidence interval [CI] 0.85-0.92) for NMDAR, 0.84 (CI 0.80-0.88) for LGI1, 0.82 (CI 0.75-0.90) for CASPR2, and 0.76 (CI 0.62-0.93) for GABABR. DISCUSSION: Taking a <20% recurrence risk within 12 months as sufficient, patients with NMDAR-AIE and LGI1-AIE could be considered eligible for noncommercial driving after having been seizure-free for 3 months.


Assuntos
Autoanticorpos , Encefalite , Peptídeos e Proteínas de Sinalização Intracelular , Proteínas de Membrana , Proteínas do Tecido Nervoso , Receptores de GABA-B , Recidiva , Humanos , Feminino , Masculino , Adulto , Peptídeos e Proteínas de Sinalização Intracelular/imunologia , Autoanticorpos/sangue , Pessoa de Meia-Idade , Encefalite/imunologia , Estudos Retrospectivos , Receptores de GABA-B/imunologia , Proteínas do Tecido Nervoso/imunologia , Adulto Jovem , Proteínas de Membrana/imunologia , Receptores de N-Metil-D-Aspartato/imunologia , Convulsões/etiologia , Convulsões/imunologia , Doença de Hashimoto/imunologia , Doença de Hashimoto/sangue , Idoso , Adolescente , Seguimentos , Proteínas/imunologia , Estudos de Coortes
11.
Clin Neurol Neurosurg ; 224: 107559, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36549220

RESUMO

OBJECTIVES: It is assumed that autoimmune limbic encephalitis (ALE) demonstrates distinct neuropsychological manifestations with differential responses to immunotherapy according to which associated autoantibody (AAB), if any, is identified. Towards investigating whether this is the case, this study aims to summarize respective findings from the primary literature on ALE with AABs binding to cell surface neural antigens and ALE with AABs against intracellular neural antigens. METHODS: We chose ALE with AABs against leucine-rich, glioma inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) as the most frequent cell surface membrane antigens, and ALE with AABs to Embryonic Lethal, Abnormal Vision, Like 1 (ELAVL) proteins (anti-Hu) and glutamic acid decarboxylase 65 (GAD65) as the most frequent intracellular neural antigens. The PubMed and Scopus databases were searched on March 1st, 2021 for neuropsychological test and -screening data from patients with ALE of these AAB-types. Findings were reviewed according to AAB-type and immunotherapy status and are presented in a review section and are further statistically evaluated and presented in a meta-analysis section in this publication. RESULTS: Of the 1304 initial hits, 32 studies on ALE with AABs against LGI1, CASPR2, and GAD65 reporting cognitive screening data could be included in a review. In ALE with AABs against LGI1, CASPR2 and GAD65, memory deficits are the most frequently reported deficits. However, deficits in attention and executive functions including working memory, fluency, and psychological function have also been reported. This review shows that ALE patients with AABs against both LGI1 and CASPR2 show higher percentages of neuropsychological deficits compared to ALE patients with AABs against GAD65 before and after initiation of immunotherapy. However, the methodologies used in these studies were heterogenous, and longitudinal studies were not comparable. Moreover, 21 studies including ALE patients with AABs against LGI1 and GAD65 were also suitable for meta-analysis. No suitable study on ALE with AABs against ELAVL proteins could be identified. Meta-Analyses could be executed for cognitive screening data and only partially, due to the small number of studies. However, in statistical analysis no consistent effect of AAB or immunotherapy on performance in cognitive screening tests could be found. CONCLUSION: Currently, there is no definite evidence supporting the notion that different AAB-types of ALE exhibit distinct neuropsychological manifestations and respond differently to immunotherapy. Overall, we could not identify evidence for any effect of immunotherapy on cognition in ALE. More systematic, in-depth and longitudinal neuropsychological assessments of patients with different AAB-types of ALE are required in the future to investigate these aspects.


Assuntos
Autoanticorpos , Encefalite Límbica , Humanos , Glutamato Descarboxilase , Imunoterapia , Peptídeos e Proteínas de Sinalização Intracelular , Encefalite Límbica/complicações , Encefalite Límbica/terapia
12.
J Appl Lab Med ; 7(1): 81-98, 2022 01 05.
Artigo em Inglês | MEDLINE | ID: mdl-34996085

RESUMO

BACKGROUND: Autoimmune encephalitis (AE) is a common cause of encephalitis. We review the most recent evidence on this neuroimmune condition and autoantibody testing currently available. CONTENT: Clinical criteria, neuroimaging and electroencephalography can facilitate the diagnosis of AE prior to obtaining autoantibody testing results, and lead to a diagnosis of AE even in the absence of a recognized antibody. Early treatment of AE has been found to correlate with improved long-term functional and cognitive outcomes. We suggest a clinical approach to diagnosis based on the predominant area of nervous system involvement and the results of ancillary testing that are widely available. We also propose a 2-tiered approach to the acute management of probable or definite AE. We, finally, provide guidance on the long-term management of AE-a challenging and understudied area. SUMMARY: Much work remains to be done to improve the care of patients with AE. As understanding of the pathophysiology and predisposing factors underlying this condition steadily increases, a more evidence-based, targeted approach to the treatment of AE is still desired. Nonetheless, looking at the progress made over the past 2 decades, since the discovery of the first autoantibodies associated with AE, one cannot help but feel optimistic about the road ahead.


Assuntos
Encefalite , Doença de Hashimoto , Autoanticorpos , Eletroencefalografia , Encefalite/diagnóstico , Encefalite/terapia , Doença de Hashimoto/diagnóstico , Doença de Hashimoto/terapia , Humanos
13.
Front Neurol ; 13: 1043785, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36468045

RESUMO

Background: For patients with anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) and ovarian teratoma, "conservative" surgical approaches (complete or partial unilateral oophorectomy or bilateral partial oophorectomies) are associated with clinical improvement. "Aggressive" ovarian resections (complete bilateral oophorectomy or "blind" ovarian resections without pre-operative evidence of teratoma) are also reported, although the evidence supporting these approaches is unclear. Objective: To compare the one-year functional outcomes of patients with NMDARE who underwent conservative vs. aggressive ovarian resections. Methods: Patients with NMDARE undergoing ovarian resection between January 1st, 2012 and December 31st, 2021 were retrospectively identified from three North American tertiary care centers. Primary outcome was a modified Rankin Scale score of 0-2 one year after ovarian resection. Fisher exact and Wilcoxon rank sum tests were used to compare demographic features, disease characteristics, and functional outcomes between the two surgical groups. A fixed-effects meta-analysis of studies reporting functional outcomes based on surgical approach was also performed. Results: Twenty-three patients were included. Eight underwent aggressive surgical management. There was a non-significant trend toward an association between aggressive surgical management and younger age-at-onset, higher baseline disease severity, and longer delays to treatment. There was no difference between "aggressive" (3/8, 38%) and "conservative" (11/15, 73%) management groups in achieving the primary outcome (OR95% = <0.1-1.9; p = 0.18). Findings were similar when considering data from 52 patients in two published studies (RR = 0.74; CI95% = 0.48-1.13; p = 0.16). Conclusions: Aggressive ovarian resection was not associated with improved outcomes in patients with NMDARE in this series. Group differences may have contributed, recognizing that patients who underwent aggressive resection tended to be sicker, with procedures performed later in the disease course. Based on available evidence, we advocate for function-sparing resection in patients with imaging-confirmed/suspected teratoma, and repeated multi-modal imaging in at-risk patients with NMDARE refractory to conventional treatment.

14.
PLoS One ; 16(4): e0250214, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33861789

RESUMO

Research has repeatedly shown that familiar and unfamiliar voices elicit different neural responses. But it has also been suggested that different neural correlates associate with the feeling of having heard a voice and knowing who the voice represents. The terminology used to designate these varying responses remains vague, creating a degree of confusion in the literature. Additionally, terms serving to designate tasks of voice discrimination, voice recognition, and speaker identification are often inconsistent creating further ambiguities. The present study used event-related potentials (ERPs) to clarify the difference between responses to 1) unknown voices, 2) trained-to-familiar voices as speech stimuli are repeatedly presented, and 3) intimately familiar voices. In an experiment, 13 participants listened to repeated utterances recorded from 12 speakers. Only one of the 12 voices was intimately familiar to a participant, whereas the remaining 11 voices were unfamiliar. The frequency of presentation of these 11 unfamiliar voices varied with only one being frequently presented (the trained-to-familiar voice). ERP analyses revealed different responses for intimately familiar and unfamiliar voices in two distinct time windows (P2 between 200-250 ms and a late positive component, LPC, between 450-850 ms post-onset) with late responses occurring only for intimately familiar voices. The LPC present sustained shifts, and short-time ERP components appear to reflect an early recognition stage. The trained voice equally elicited distinct responses, compared to rarely heard voices, but these occurred in a third time window (N250 between 300-350 ms post-onset). Overall, the timing of responses suggests that the processing of intimately familiar voices operates in two distinct steps of voice recognition, marked by a P2 on right centro-frontal sites, and speaker identification marked by an LPC component. The recognition of frequently heard voices entails an independent recognition process marked by a differential N250. Based on the present results and previous observations, it is proposed that there is a need to distinguish between processes of voice "recognition" and "identification". The present study also specifies test conditions serving to reveal this distinction in neural responses, one of which bears on the length of speech stimuli given the late responses associated with voice identification.


Assuntos
Reconhecimento Fisiológico de Modelo/fisiologia , Percepção da Fala/fisiologia , Reconhecimento de Voz/fisiologia , Adulto , Percepção Auditiva/fisiologia , Potenciais Evocados , Feminino , Humanos , Masculino , Quebeque , Reconhecimento Psicológico/fisiologia , Fala/fisiologia , Voz/fisiologia
15.
Neurology ; 96(20): e2546-e2557, 2021 05 18.
Artigo em Inglês | MEDLINE | ID: mdl-33795390

RESUMO

OBJECTIVE: To determine whether neuronal and neuroaxonal injury, neuroinflammation, and synaptic dysfunction associate with clinical course and outcomes in antibody-mediated encephalitis (AME), we measured biomarkers of these processes in CSF from patients presenting with AME and cognitively normal individuals. METHODS: Biomarkers of neuronal (total tau, VILIP-1) and neuroaxonal damage (neurofilament light chain [NfL]), inflammation (YKL-40), and synaptic function (neurogranin, SNAP-25) were measured in CSF obtained from 45 patients at the time of diagnosis of NMDA receptor (n = 34) or LGI1/CASPR2 (n = 11) AME and 39 age- and sex-similar cognitively normal individuals. The association between biomarkers and modified Rankin Scale (mRS) scores were evaluated in a subset (n = 20) of longitudinally followed patients. RESULTS: Biomarkers of neuroaxonal injury (NfL) and neuroinflammation (YKL-40) were elevated in AME cases at presentation, whereas markers of neuronal injury and synaptic function were stable (total tau) or decreased (VILIP-1, SNAP-25, neurogranin). The log-transformed ratio of YKL-40/SNAP-25 optimally discriminated patients from cognitively normal individuals (area under the receiver operating characteristic curve 0.99; 95% confidence interval 0.97, >0.99). Younger age (ρ = -0.56; p = 0.01), lower VILIP-1 (ρ = -0.60; p < 0.01) and SNAP-25 (ρ = -0.54; p = 0.01), and higher log10(YKL-40/SNAP-25) (ρ = 0.48; p = 0.04) associated with greater disease severity (higher mRS score) in prospectively followed patients. Higher YKL-40 (ρ = 0.60; p = 0.02) and neurogranin (ρ = 0.55; p = 0.03) at presentation were associated with higher mRS scores 12 months following hospital discharge. CONCLUSIONS: CSF biomarkers suggest that neuronal integrity is acutely maintained in AME, despite neuroaxonal compromise. Low levels of biomarkers of synaptic function may reflect antibody-mediated internalization of cell surface receptors and may represent an acute correlate of antibody-mediated synaptic dysfunction, with the potential to inform disease severity and outcomes.


Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/líquido cefalorraquidiano , Proteína 1 Semelhante à Quitinase-3/líquido cefalorraquidiano , Neurocalcina/líquido cefalorraquidiano , Proteínas de Neurofilamentos/líquido cefalorraquidiano , Proteínas tau/líquido cefalorraquidiano , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Doenças Autoimunes do Sistema Nervoso/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/imunologia , Biomarcadores/líquido cefalorraquidiano , Estudos de Casos e Controles , Criança , Pré-Escolar , Encefalite/líquido cefalorraquidiano , Encefalite/imunologia , Feminino , Humanos , Peptídeos e Proteínas de Sinalização Intracelular/imunologia , Masculino , Proteínas de Membrana/imunologia , Pessoa de Meia-Idade , Proteínas do Tecido Nervoso/imunologia , Neurogranina/líquido cefalorraquidiano , Proteína 25 Associada a Sinaptossoma/líquido cefalorraquidiano , Adulto Jovem
16.
Artigo em Inglês | MEDLINE | ID: mdl-32847939

RESUMO

OBJECTIVE: To determine the observed and expected incidence rates of paraneoplastic neurologic syndromes (PNSs) and autoimmune encephalitides (AEs) diagnosed in France between 2016 and 2018, we conducted a population-based epidemiologic study. METHODS: Observed incidence rates were stratified by sex, age groups, region of care, year of diagnosis, and disease subgroups. National expected incidence rates were calculated based on rates obtained in the area directly adjacent to the Reference Center using a mixed Poisson model and compared with observed incidence rates. RESULTS: Six hundred thirty-two patients with definite PNS or AE met the inclusion criteria. The observed incidence rate of definite PNS and AE in France was 3.2 per million person-years (CI95%: 2.9-3.4) compared with an expected incidence rate of 7.1 per million person-years (CI95%: 3.9-11.4). The national observed incidence rate for the antibody-positive AE subgroup increased from 1.4 per million person-years (CI95%: 1.2-1.7) in 2016 to 2.1 per million person-years (CI95%: 1.7-2.4) in 2018, thus surpassing the incidence rate of classical PNS (1.2 per million person-years [CI95%: 1.0-1.5]) of 2018. CONCLUSIONS: There was a significant widespread year-to-year increase in the incidence of diagnoses registered with the Reference Center for all subgroups of PNS and AE studied. The national observed incidence rate is likely underestimated due to underdiagnosis and underreporting.


Assuntos
Doenças Autoimunes do Sistema Nervoso/epidemiologia , Encefalite/epidemiologia , Síndromes Paraneoplásicas do Sistema Nervoso/epidemiologia , Adolescente , Adulto , Idoso , Doenças Autoimunes do Sistema Nervoso/sangue , Doenças Autoimunes do Sistema Nervoso/imunologia , Bases de Dados Factuais , Encefalite/sangue , Encefalite/imunologia , Feminino , França/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Síndromes Paraneoplásicas do Sistema Nervoso/sangue , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Adulto Jovem
17.
J Neuroimmunol ; 345: 577285, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32563126

RESUMO

OBJECTIVE: To determine the prevalence of cerebrospinal fluid (CSF) markers associated with inflammation (i.e., elevated white blood cell count, protein concentration, and CSF-specific oligoclonal bands) in patients with early active autoimmune encephalitis (AE). METHODS: CSF characteristics, including WBC count, protein concentration, and oligoclonal banding, were analyzed in patients diagnosed with AE at two tertiary care centers. RESULTS: Ninety-five patients were included in the study. CSF white blood cell counts and protein levels were within normal limits for 27% (CI95%: 19-37) of patients with AE. When results of oligoclonal banding were added, 14% (CI95%: 6-16) of patients with AE had "normal" CSF. The median CSF white blood cell count was 8 cells/mm3 (range: 0-544) and the median CSF protein concentration was 0.42 g/L (range: 0.15-3.92). CONCLUSIONS: White blood cell counts and protein levels were within normal limits in the CSF of a substantial proportion of patients with early active AE. Inclusion of CSF oligoclonal banding identified a higher proportion of patients with an inflammatory CSF profile, especially when CSF was sampled early in the disease process.


Assuntos
Encefalite/líquido cefalorraquidiano , Encefalite/diagnóstico , Doença de Hashimoto/líquido cefalorraquidiano , Doença de Hashimoto/diagnóstico , Mediadores da Inflamação/líquido cefalorraquidiano , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Encefalite/sangue , Feminino , Doença de Hashimoto/sangue , Humanos , Mediadores da Inflamação/sangue , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Adulto Jovem
19.
J Can Acad Child Adolesc Psychiatry ; 22(4): 317-23, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24223052

RESUMO

OBJECTIVE: To investigate how attitudes towards psychostimulant medication influence the adherence to psychostimulant treatment in children with Attention-Deficit/Hyperactivity Disorder (ADHD). METHOD: Thirty-three children with ADHD were prospectively recruited to take part in this study. The children and their parents filled questionnaires at both baseline and at a three-month follow-up to assess the severity of ADHD symptoms in the child and attitudes towards psychostimulant medication. Adherence to medication was assessed through standardized interviews of parents. RESULTS: Parental perceived psychosocial benefits of psychostimulant medication at the three-month follow-up were found to be positive predictors of adherence to medication. Parental perceived psychosocial benefits of medication at the three-month follow-up was in turn predicted by parental medication acceptability at three months and child's perceived psychosocial benefits of medication at three-months. CONCLUSION: Improving parents' awareness of psychosocial benefits of psychostimulant medication may increase adherence to psychostimulant medication in children with ADHD. Age of the child and severity of symptoms did not significantly affect medication adherence.


OBJECTIF: Rechercher comment les attitudes à l'égard des psychostimulants influencent l'observance du traitement par psychostimulants chez les enfants souffrant du trouble de déficit de l'attention avec hyperactivité (TDAH). MÉTHODE: Trente-trois enfants souffrant du TDAH ont été recrutés prospectivement pour prendre part à cette étude. Les enfants et leurs parents ont répondu à des questionnaires au départ et au suivi de trois mois afin d'évaluer la gravité des symptômes du TDAH chez l'enfant et les attitudes à l'égard des psychostimulants. L'observance des médicaments a été évaluée par des entrevues normalisées avec les parents. RÉSULTATS: La perception des parents des avantages psychosociaux des psychostimulants au suivi de trois mois s'est révélée être un prédicteur positif de l'observance des médicaments. La perception des parents des avantages psychosociaux des médicaments au suivi de trois mois a été prédite à son tour par l'acceptation parentale des médicaments à trois mois et par la perception de l'enfant des avantages psychosociaux des médicaments à trois mois. CONCLUSION: Sensibiliser davantage les parents aux avantages psychosociaux des psychostimulants peut améliorer l'observance des psychostimulants chez les enfants souffrant du TDAH. L'âge de l'enfant et la gravité des symptômes n'influaient pas significativement sur l'observance des médicaments.

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