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1.
Neurology ; 2023 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-36599697

RESUMO

BACKGROUND AND OBJECTIVES: Modified Atkins Diet (MAD) has emerged as an adjuvant therapy in drug-resistant epilepsy (DRE). Most studies are in children, there is limited evidence for DRE in in adults. The present study aimed to investigate if MAD along with standard drug therapy (SDT) was indeed more effective than SDT alone in reducing seizure frequency and improving psychological outcomes at 6 months in Adolescents and adults with DRE (non-surgical). METHODS: A prospective randomized controlled trial was conducted at tertiary care referral centre, in India. Persons with DRE aged 10-55 years attending outpatient epilepsy clinics between August 2015 and April 2019, who had more than two seizures/month despite using at least three appropriate anti-seizure medications (ASMs) at their maximum tolerated doses and had not been on any form of diet therapy for the past one year, were enrolled. Patients were assessed for the eligibility and randomly assigned to receive SDT plus MAD (intervention arm) or SDT alone (control arm).The primary outcome was>50% reduction in seizure- frequency, and the secondary outcomes were quality of life (QOL), behaviour, adverse events and rate of withdrawal at six months. Intention to treat analysis was performed. RESULTS: 243 patients were screened for eligibility; 160 patients (80 adults and 80 adolescents) were randomized to either intervention or Control arm. Demographic and clinical characteristics in both groups were comparable at baseline. At six months > 50% seizure reduction was seen in 26.2% in the intervention group versus 2.5 % in the control group (95% CI 13.5-33.9; p<0.001). Improvement in QOL was 52.1±17.6 in the intervention group versus 42.5±16.4 in the control group (mean difference, 9.6; 95%CI 4.3 to 14.9, p<0.001). However, behaviour scores could be performed in 49 patients and improvement was seen in intervention versus control group (65.6±7.9 versus 71.4±8.1, p=0.015) at the end of the study. One patient had weight loss; two patients had Diarrhoea. DISCUSSION: MAD group demonstrated improvement in all aspects (reduction in seizure-frequency, and behavioural problems) compared to control group at the end of the study. MAD is an effective modality in controlling seizures, further research is required to assess its efficacy in terms of biomarkers along with descriptive metabolomics studies.

2.
Neurosci Lett ; 796: 137065, 2023 Feb 06.
Artigo em Inglês | MEDLINE | ID: mdl-36638954

RESUMO

Focal cortical dysplasia (FCD) represents a group of malformations of cortical development, which are speculated to be related to early developmental defects in the cerebral cortex. According to dysmature cerebral development hypothesis of FCD altered GABAA receptor function is known to contribute to abnormal neuronal network. Here, we studied the possible association between age at seizure onset in FCD with the subunit configuration of GABAA receptors in resected brain specimens obtained from patients with FCD. We observed a significantly higher ratio of α4/α1 subunit-containing GABAA receptors in patients with early onset (EO) FCD as compared to those with late onset (LO) FCD as is seen during the course of development where α4-containing GABAA receptors expression is high as compared to α1-containing GABAA receptors expression. Likewise, the influx to efflux chloride co-transporter expression of NKCC1/KCC2 was also increased in patients with EO FCD as seen during brain development. In addition, we observed that the ratio of GABA/Glutamate neurotransmitters was lower in patients with EO FCD as compared to that in patients with LO FCD. Our findings suggest altered configuration of GABAA receptors in FCD which could be contributing to aberrant depolarizing GABAergic activity. In particular, we observed a correlation of age at seizure onset in FCD with subunit configuration of GABAA receptors, levels of NKCC1/KCC2 and the ratio of GABA/Glutamate neurotransmitters such that the patients with EO FCD exhibited a more critically modulated GABAergic network.


Assuntos
Malformações do Desenvolvimento Cortical , Simportadores , Humanos , Cloretos/metabolismo , Ácido gama-Aminobutírico/metabolismo , Malformações do Desenvolvimento Cortical/metabolismo , Receptores de GABA-A/metabolismo , Convulsões/complicações , Simportadores/metabolismo , Idade de Início
4.
Epilepsy Res ; 189: 107056, 2022 Nov 23.
Artigo em Inglês | MEDLINE | ID: mdl-36469977

RESUMO

Benzodiazepines (BDZ) such as diazepam and lorazepam are popular as first-line treatment for acute seizures due to their rapid action and high efficacy. However, long-term usage of BDZ leads to benzodiazepine resistance, a phenomenon whose underlying mechanisms are still being investigated. One of the hypothesised mechanisms contributing to BDZ resistance is the presence of mutations in benzodiazepine-sensitive receptors. While a few genetic variants have been reported previously, knowledge of relevant pathogenic variants is still scarce. We used Sanger Sequencing to detect variants in the ligand-binding domain of BDZ-sensitive GABAA receptor subunits α1-3 and 5 expressed in resected brain tissues of drug-resistant epilepsy (DRE) patients with a history of BDZ resistance and found two previously unreported predicted pathogenic frameshifting variants - NM_000807.4(GABRA2):c.367_368insG and NM_000810.4(GABRA5):c.410del - significantly enriched in these patients. The findings were further explored in resected DRE brain tissues through cellular electrophysiological experiments.

5.
Epilepsy Res ; 188: 107037, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36332541

RESUMO

OBJECTIVE: To compare efficacy and safety of Intranasal and Intramuscular routes of midazolam administration in terminating seizures. METHOD: This was an open label Randomized controlled trial (RCT). People with drug resistant epilepsy (DRE) undergoing Video Electroencephalogram (VEEG) monitoring, were randomized in a 1:1 ratio to receive either Intranasal (IN) or Intramuscular (IM) midazolam, for prolonged seizures: longer than 5 min for focal, and longer than 2 min for focal to bilateral tonic-clonic. Outcome assessor was blinded to the allocation arm. Primary outcome was time to electrographic seizure termination after administration of midazolam. All adverse events in both the groups were noted. RESULT: A total of 1108 seizures were recorded in 130 subjects, of which 110 (65 seizures in 23 subjects in IN group; 45 seizures in 18 subjects in IM group) seizures required midazolam administration and were included in final analysis. Mean time to electrographic seizure termination after midazolam administration was 45.1 ± 23.8 s in the IM group and 90.4 ± 59.0 s in the IN group (p = 0.0014); mean time to clinical seizure termination was 53.9 ± 25.8 s in IM group and 104.3 ± 66.4 s in the IN group (p = 0.002). Local side effects were more in IN group; hypotension as serious adverse event was noted in the IM group. SIGNIFICANCE: Though mean time to electrographic and clinical seizure termination was significantly lesser in Intramuscular group for both adults and pediatric population, it was still under 2 min in the Intranasal midazolam group.IN midazolam is a useful option for terminating seizures.


Assuntos
Epilepsia Resistente a Medicamentos , Estado Epiléptico , Criança , Adulto , Humanos , Midazolam/uso terapêutico , Midazolam/efeitos adversos , Anticonvulsivantes/efeitos adversos , Convulsões/tratamento farmacológico , Convulsões/induzido quimicamente , Estado Epiléptico/tratamento farmacológico , Administração Intranasal , Epilepsia Resistente a Medicamentos/tratamento farmacológico
6.
Trials ; 23(1): 943, 2022 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-36397081

RESUMO

BACKGROUND: A Core Outcome Set (COS) is a standardised list of outcomes that should be reported as a minimum in all clinical trials. In epilepsy, the choice of outcomes varies widely among existing studies, particularly in clinical trials. This diminishes opportunities for informed decision-making, contributes to research waste and is a barrier to integrating findings in systematic reviews and meta-analyses. Furthermore, the outcomes currently being measured may not reflect what is important to people with epilepsy. Therefore, we aim to develop a COS specific to clinical effectiveness research for adults with epilepsy using Delphi consensus methodology. METHODS: The EPSET Study will comprise of three phases and follow the core methodological principles as outlined by the Core Outcome Measures in Effectiveness Trials (COMET) Initiative. Phase 1 will include two focused literature reviews to identify candidate outcomes from the qualitative literature and current outcome measurement practice in phase III and phase IV clinical trials. Phase 2 aims to achieve international consensus to define which outcomes should be measured as a minimum in future trials, using a Delphi process including an online consensus meeting involving key stakeholders. Phase 3 will involve dissemination of the ratified COS to facilitate uptake in future trials and the planning of further research to identify the most appropriate measurement instruments to use to capture the COS in research practice. DISCUSSION: Harmonising outcome measurement across future clinical trials should ensure that the outcomes measured are relevant to patients and health services, and allow for more meaningful results to be obtained. CORE OUTCOME SET REGISTRATION: COMET Initiative as study 118 .


Assuntos
Epilepsia , Projetos de Pesquisa , Adulto , Humanos , Técnica Delfos , Revisões Sistemáticas como Assunto , Avaliação de Resultados em Cuidados de Saúde , Epilepsia/diagnóstico , Epilepsia/terapia
7.
Neurol India ; 70(5): 2031-2038, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36352605

RESUMO

Background: Although epilepsy is a common neurological condition, there is paucity of nationwide data on treatment patterns and sociodemographic and clinical factors affecting treatment decisions in India. Objective: To assess clinical profiles, usage pattern of antiepileptic drugs (AEDs), and seizure control among patients with epilepsy in India. Methods: This was a cross-sectional, observational, multicenter study on adult patients with epilepsy who were on AEDs for at least six months before enrollment. Data were collected from patient interviews and medical records. Results: Out of 800 enrolled patients, a majority (69.0%) had generalized onset seizure in the six months before enrollment. The median age at epilepsy onset was 20.0 (1.0-64.0) years; 40.0% of the patients were females, 48.5% were married, 99.1% were literate, and 67.0% belonged to the lower or upper-middle socioeconomic class. Overall, 459 patients (57.4%) received AEDs as combination therapy. Most patients received levetiracetam (37.0%), sodium valproate (18.5%), carbamazepine (17.3%), or phenytoin (13.8%) as monotherapy, and clobazam (59.7%), levetiracetam (52.9%), carbamazepine (26.4%), sodium valproate (24.8%), or phenytoin (24.0%) in combination therapy. Quality of life was comparable for first- and third-generation AEDs. Adverse drug reactions were mostly attributed to dose modification or switching between drugs. No serious adverse drug reactions or new safety concerns were identified. Conclusions: Findings from this large, cross-sectional, observational, multicenter study indicate that first-generation AEDs sodium valproate and phenytoin continued to be used in a substantial number of patients on monotherapy and combination therapy in India, even though an increasing trend toward use of second-generation AEDs was noted in clinical practice.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Epilepsia , Adulto , Feminino , Humanos , Adulto Jovem , Pessoa de Meia-Idade , Masculino , Fenitoína/uso terapêutico , Levetiracetam/uso terapêutico , Ácido Valproico/uso terapêutico , Estudos Transversais , Qualidade de Vida , Epilepsia/tratamento farmacológico , Epilepsia/epidemiologia , Anticonvulsivantes/efeitos adversos , Carbamazepina/uso terapêutico , Convulsões/tratamento farmacológico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/tratamento farmacológico
8.
Clin Med (Lond) ; 22(6): 575-577, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36427894

RESUMO

The pharyngeal-cervical-brachial (PCB) variant is a rare presentation of Guillain-Barré syndrome (GBS), and there is a handful of case reports that overlap with the Miller-Fisher syndrome (MFS) variant of GBS. This overlap produces varied symptoms that may be confusing and challenging for physicians to diagnose timely and start appropriate treatment. In this article, we present a case report and review of the rare overlap of the PCB variant with the MFS variant of GBS.


Assuntos
Transtornos de Deglutição , Síndrome de Guillain-Barré , Síndrome de Miller Fisher , Oftalmoplegia , Adolescente , Humanos , Transtornos de Deglutição/diagnóstico , Transtornos de Deglutição/etiologia , Síndrome de Miller Fisher/diagnóstico , Síndrome de Guillain-Barré/diagnóstico , Pescoço
9.
Cells ; 11(22)2022 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-36428989

RESUMO

Glutamate-receptor-mediated hyperexcitability contributes to seizure generation in temporal lobe epilepsy (TLE). Tryptophan-kynurenine pathway (TKP) metabolites regulate glutamate receptor activity under physiological conditions. This study was designed to investigate alterations in the levels of TKP metabolites and the differential regulation of glutamatergic activity by TKP metabolites in the hippocampus, anterior temporal lobe (ATL), and neocortex samples of a lithium-pilocarpine rat model of TLE. We observed that levels of tryptophan were reduced in the hippocampus and ATL samples but unaltered in the neocortex samples. The levels of kynurenic acid were reduced in the hippocampus samples and unaltered in the ATL and neocortex samples of the TLE rats. The levels of kynurenine were unaltered in all three regions of the TLE rats. The magnitude of reduction in these metabolites in all regions was unaltered in the TLE rats. The frequency and amplitude of spontaneous excitatory postsynaptic currents were enhanced in hippocampus ATL samples but not in the neocortex samples of the TLE rats. The exogenous application of kynurenic acid inhibited glutamatergic activity in the slice preparations of all these regions in both the control and the TLE rats. However, the magnitude of reduction in the frequency of kynurenic acid was higher in the hippocampus (18.44 ± 2.6% in control vs. 30.02 ± 1.5 in TLE rats) and ATL (16.31 ± 0.91% in control vs. 29.82 ± 3.08% in TLE rats) samples of the TLE rats. These findings suggest the differential regulation of glutamatergic activity by TKP metabolites in the hippocampus, ATL, and neocortex of TLE rats.


Assuntos
Epilepsia do Lobo Temporal , Neocórtex , Ratos , Animais , Neocórtex/metabolismo , Cinurenina/metabolismo , Triptofano/metabolismo , Ácido Cinurênico/farmacologia , Ácido Cinurênico/metabolismo , Lobo Temporal/metabolismo , Hipocampo/metabolismo , Modelos Animais de Doenças
10.
Heliyon ; 8(10): e11073, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36281370

RESUMO

Objective: The goal of this study was to determine the effects of mono-, bi-, and polytherapy anti-seizure medications (ASMs) in terms of seizure reduction and quality of life (QOL) in persons with epilepsy (PWE). Methods: A cross-sectional observational study was conducted. All PWE with age <75 years were recruited and further classified into two groups: responders and non-responders, based on the response of the ASMs to the treatments for reduced seizure frequency since the last one year. Other demographic and clinical data such as seizure frequency, type of seizures, age at onset of seizures, and information about ASMs with their daily doses were assessed for the descriptive analysis. The quality of life was assessed in randomly selected PWE (n = 100) using the quality of life in epilepsy inventory-31 (QOLIE-31) in adults. Results: With a total of 486 PWE, the median age (years) was comparable in both groups. Out of these the non-responders group was found to be significantly higher (77.8%) than the responders group (22.2%). In the responders group, the percentage of PWE who were on monotherapy was significantly higher (51.85 %) than those who were on polytherapy (17.59%), whereas in the non-responders group, 21.16% of PWE were on monotherapy and 44.86% were on polytherapy. The duration of epilepsy was similar in both groups, but the average seizure frequency was significantly higher in the non-responders. In QOL assessments, 43% of PWE were observed in the responders group, whereas 57% of PWE were found in the non-responders group. The overall comparative QOL scores were also significantly higher (p < 0.0001) in the responders group as compared to the non-responders group. Conclusion: Our findings revealed that those PWE who were on monotherapy showed better reduction in seizure frequency and improved QOL in responder groups as compared to non-responder groups.

11.
Ann Indian Acad Neurol ; 25(4): 715-717, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36211156

RESUMO

Carotid artery stenting (CAS) is performed in patients with minor strokes and transient ischemic attacks (TIAs) to prevent further strokes. However, most operators do not intervene in older adults. We had a 92-year patient with recurrent minor strokes with two possible proximate causes - cardioembolism and significant symptomatic left carotid stenosis. This patient continued to have recurrent ischemic events in the left carotid territory despite optimum management of the cardioembolic source with dual antiplatelets and anticoagulation and was successfully treated with left CAS. The role of carotid revascularization in older patients with high-grade symptomatic carotid stenosis and cardiac comorbidities is discussed.

12.
Int J Geriatr Psychiatry ; 37(10)2022 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-36069187

RESUMO

INTRODUCTION AND OBJECTIVES: Early dementia diagnosis in low and middle-income countries (LMIC) is challenging due to limited availability of brief, culturally appropriate, and psychometrically validated tests. Montreal Cognitive Assessment (MoCA) is one of the most widely used cognitive screening tests in primary and secondary care globally. In the current study, we adapted and validated MoCA in five Indian languages (Hindi, Bengali, Telugu, Kannada, and Malayalam) and determined the optimal cut-off points that correspond to screening for clinical diagnosis of dementia and MCI. METHODS: A systematic process of adaptation and modifications of MoCA was fulfilled. A total of 446 participants: 214 controls, 102 dementia, and 130 MCI were recruited across six centers. RESULTS: Across five languages, the area under the curve for diagnosis of dementia varied from 0.89 to 0.98 and MCI varied from 0.73 to 0.96. The sensitivity, specificity and optimum cut-off scores were established separately for five Indian languages. CONCLUSIONS: The Indian adapted MoCA is standardized and validated in five Indian languages for early diagnosis of dementia and MCI in a linguistically and culturally diverse population.

13.
Brain Commun ; 4(5): fcac215, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36072647

RESUMO

Oxidative stress has been implicated in Alzheimer's disease, and it is potentially driven by the depletion of primary antioxidant, glutathione, as well as elevation of the pro-oxidant, iron. Present study evaluates glutathione level by magnetic resonance spectroscopy, iron deposition by quantitative susceptibility mapping in left hippocampus, as well as the neuropsychological scores of healthy old participants (N = 25), mild cognitive impairment (N = 16) and Alzheimer's disease patients (N = 31). Glutathione was found to be significantly depleted in mild cognitive impaired (P < 0.05) and Alzheimer's disease patients (P < 0.001) as compared with healthy old participants. A significant higher level of iron was observed in left hippocampus region for Alzheimer's disease patients as compared with healthy old (P < 0.05) and mild cognitive impairment (P < 0.05). Multivariate receiver-operating curve analysis for combined glutathione and iron in left hippocampus region provided diagnostic accuracy of 82.1%, with 81.8% sensitivity and 82.4% specificity for diagnosing Alzheimer's disease patients from healthy old participants. We conclude that tandem glutathione and iron provides novel avenue to investigate further research in Alzheimer's disease.

14.
Seizure ; 101: 253-261, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36116284

RESUMO

BACKGROUND: Available anti-seizure medications (ASMs) target the symptomatology of the disease rather than any significant disease/epileptogenesis modifying actions. There are critical concerns of drug resistance and seizure recurrence during epilepsy management. So, drug repurposing is evolving as a paradigm change in the quest for novel epilepsy treatment strategies. Metformin, a well-known anti-diabetic drug has shown multiple pieces of evidence of its potential antiepileptic action. OBJECTIVE: This review elucidates various mechanisms underlying the beneficial role of metformin in seizure control and modulation of the epileptogenesis process. METHODS: Preclinical and clinical evidence involving metformin's role in epilepsy and special conditions like tuberous sclerosis have been reviewed in this paper. The putative mechanisms of epileptogenesis modulation through the use of metformin are also summarised. RESULTS: This review found the efficacy of metformin in different seizure models including genetic knockout model, chemical induced, and kindling models. Only one clinical study of metformin in tuberous sclerosis has shown a reduction in seizure frequency and tumor volume compared to placebo. The suggested mechanisms of metformin relevant to epileptogenesis modulation mainly encompass AMPK activation, mTOR inhibition, protection against blood-brain-barrier disruption, inhibition of neuronal apoptosis, and reduction of oxidative stress. In addition to seizure protection, metformin has a potential role in attenuating adverse effects associated with epilepsy and ASMs such as cognition and memory impairment. CONCLUSION: Metformin has shown promising utility in epilepsy management and epileptogenesis modulation. The evidence in this review substantiates the need for a robust clinical trial to explore the efficacy and safety of metformin in persons with epilepsy.


Assuntos
Epilepsia , Metformina , Esclerose Tuberosa , Proteínas Quinases Ativadas por AMP , Anticonvulsivantes/farmacologia , Anticonvulsivantes/uso terapêutico , Epilepsia/tratamento farmacológico , Humanos , Metformina/farmacologia , Metformina/uso terapêutico , Serina-Treonina Quinases TOR , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológico , Esclerose Tuberosa/genética
16.
Neurol India ; 70(4): 1593-1600, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36076664

RESUMO

Background and Objective: Hemispherotomy surgery in adults is shrouded in doubts regarding the functional outcome. The age at surgery alone should not be the deciding factor for surgery. Language paradigms were used in functional magnetic resonance imaging (fMRI) to confirm the role played by the age at the onset of seizures to predict the postoperative functional outcome. The objective of the study was to formulate an optimal strategy for patient selection for the left-sided hemispherotomy in adults, based on functional outcome analysis. Materials and Methods: A retrospective analysis of 20 participants (age at surgery 1-26 years) who underwent left hemispherotomy (over a 5-year period) was conducted. The language and motor functional assessments of 18 participants (13 pediatric and five adult participants; attrition of participants- two) were recorded at presentation and during follow-up visits. After approval was obtained from the Institutional Ethics Committee, 13 cooperative participants (eight pediatric and five adult participants) underwent language fMRI. Motor fMRI with both active and passive paradigms was done in 16 participants. Results: All 18 participants with a mean follow-up of 24 months had class I seizure-free outcome. Of these 18, five were adults (mean age = 21 years, range: 18-22 years) and 13 were in the pediatric age group (mean age = 8 years, range: 2-15 years). Postoperatively, four adults retained both verbal fluency and language comprehension at a mean follow-up period of 38 months (range: 24-48 months). Their pre- and post-op language fMRI showed word generation and regional activations for semantic comprehension in the right hemisphere. The motor area activations were seen in the right hemisphere in two and in the left hemisphere in two participants. Among the pediatric participants, four (group I [n = 4/13]) who had good language outcome showed activations in the right hemisphere. In two participants (group II [n = 2/13]) who deteriorated postoperatively, the activations were in the left hemisphere. Five participants (group III [n = 5/13]) who retained the telegraphic language postoperatively had bilateral activations of semantic comprehension areas in fMRI. All 13 pediatric participants had motor area activations seen in the left hemisphere, similar to controls. Conclusion: Left hemispherotomy can be advised to adults with comparably good postoperative language and motor outcome as in the pediatric age group, provided the weakness is acquired perinatally or below the age of 7 years. The fMRI is a valuable tool to aid in patient selection.


Assuntos
Epilepsia , Transtornos da Linguagem , Adolescente , Adulto , Criança , Pré-Escolar , Epilepsia/cirurgia , Humanos , Idioma , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Adulto Jovem
19.
Sci Rep ; 12(1): 13494, 2022 08 05.
Artigo em Inglês | MEDLINE | ID: mdl-35931755

RESUMO

Primary CNS Vasculitis (PCNSV) is a rare inflammatory disorder affecting the blood vessels of the central nervous system. Patients present with a combination of headaches, seizures, and focal neurological deficits. There is usually a diagnostic delay. Treatment is based on observational studies and expert opinion. Our objective was to identify clinical, laboratory, neuroimaging, pathologic or management-related associations with 2 year outcome in patients with primary CNS vasculitis. We conducted a cohort study at a single tertiary care referral centre of prospectively (2018-2019) and retrospectively (2010-2018) identified individuals with primary CNS vasculitis (diagnosis was proven by either brain biopsy or cerebral digital subtraction angiography). Clinical, imaging and histopathologic findings, treatment, and functional outcomes were recorded. Univariate and stepwise multiple logistic regression were applied. P-value<0.05 was considered statistically significant. The main outcome measures were documentation of clinical improvement or worsening (defined by mRS scores) and identification of independent predictors of good functional outcome (mRS 0-2) at 2 years. We enrolled eighty-two biopsy and/or angiographically proven PCNSV cases. The median age at presentation was 34 years with a male predilection and a median diagnostic delay of 23 months. Most patients presented with seizures (70.7%). All patients had haemorrhages on MRI. Histologically lymphocytic subtype was the commonest. Corticosteroids with cyclophosphamide was the commonest medication used. The median mRS at follow-up of 2 years was 2 (0-3), and 65.2% of patients achieved a good functional outcome. Myelitis and longer duration of illness before diagnosis were associated with poorer outcomes. The presence of hemorrhages on SWI sequence of MRI might be a sensitive imaging marker. Treatment with steroids and another immunosuppressant probably reduced relapse rates in our cohort. We have described the third largest PCNSV cohort and multi-centre randomised controlled trials are required to study the relative efficacy of various immunosuppressants.Study registration: CTRI/2018/03/012721.


Assuntos
Vasculite do Sistema Nervoso Central , Angiografia Cerebral , Estudos de Coortes , Diagnóstico Tardio , Humanos , Imunossupressores/uso terapêutico , Masculino , Estudos Retrospectivos , Convulsões/complicações , Vasculite do Sistema Nervoso Central/diagnóstico por imagem , Vasculite do Sistema Nervoso Central/tratamento farmacológico
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