Registro de epilepsias na gravidez, complicações obstétricas e fetais e o uso de drogas antiepilépticas em Alagoas-BR / Registry of epilepsy during pregnancy, obstetric and fetal complications and the use of antiepileptic drugs in Alagoas-BR

Objetivos: artigo 1: Descrever a prevalência das nas mulheres gestantes com epilepsia (MGCE), perfil sociodemográfico e desfechos obstétricos e analisar associação dessas variáveis com a doença. Artigo 2: Descrever o perfil de MAC (medicação anticrise) e tipos de crises e analisar a associação desses perfis com a incidência de complicações maternas e fetais nas MGCE. Artigo 3: Descrever a prevalência, perfil sociodemográfico e clínico, perfil de MAC, tipos de crises e analisar a associação da incidência de complicações maternas e fetais com essas variáveis nas MGCE. Métodos: Artigos 1 e 2: coortes retrospectivas com 234 MGCE e 492 MGSE. Artigo 3 coorte prospectiva coletou dados de 95 MGCE e 380 sem epilepsia (MGSE). Ambas as coortes com idade 40 anos em prontuários e entrevistas em 4 maternidades de alto risco em Alagoas no período de 2008 a 2021 na coorte retrospectiva e 2021 e 2022 na prospectiva. Resultados: A prevalência de MGCE nas coortes retrospectiva e prospectiva foi de 0,49% (n = 224/44917) e 0,53% (n = 105/19.624) com médias de idade (24,94 ± 6,25 e 23,98 ± 6,89); (24,42 ± 5,64 e 24,42 ± 5,62) anos. MGCE procederam da zona rural (58,2%; 64,2%), eram de cor parda (88,6%;98%), (7%; 3,2%) analfabetas e com ensino fundamental (40%; 52,1%), solteiras (47,3%; 49,5%) e (76,9%; 78,9%) donas de casa, respectivamente. Em relação a partos, a maioria (60,3%; 54,8%) eram multíparas, (74,6%; 70,8%) tiveram parto cesáreo, respectivamente. Na coorte prospectiva 15,8% sem renda, 54,7% com menos de 1 salário mínimo, 44,2% eram de religião católica e 87.4% não planejou a gravidez. A análise dos desfechos obstétricos e neonatais mostrou um risco maior nas MGCE para hipertensão relacionada à gravidez (HRG) (OR =6.29; 95% CI =3.50-11.30), pré-eclâmpsia (OR=8.04; 95% CI=2.22-29.10) na coorte retrospectiva, e em ambas coortes um risco de sangramento vaginal (OR=2.54; 95% CI=1.15-5.59);(OR=4.13; 95% CI=1.45-9.11), aborto espontâneo (OR=1.75; 95% CI=1.16-2.63); (OR=1.50; 95% CI=1.00-2.22) e natimorto (OR=11,16; 95% CI=2.22-29.10); (OR=5.27; 95% CI=2.29-10.30). Nas coortes retrospectiva e prospectiva (14%; 14,7%) não usaram MAC, (50,2%; 85,3%) monoterapia (35,8%; 12,6%) politerapia, respectivamente. O fenobarbital foi o MAC mais prescrito seguido pela carbamazepina em ambas coortes. Na prospectiva MGCE que usaram MAC e em politerapia tiveram maior risco de hemorragia vaginal, admissão em UTI materna e natimorto. Analisando os tipos de epilepsia a maioria 40% tinha o tipo generalizada. A respeito do tipo de crise, a maioria 53,3% apresentou crise focal na coorte retrospectiva, enquanto na prospectiva a maioria delas 48,4% teve CTCG e 19% estado de mal epiléptico associados a desfechos obstétricos e neonatais adversos. Conclusão: Ambos os estudos relatam um perfil sociodemográfico da MGCE de alta vulnerabilidade social e alto risco de desfechos obstétricos e neonatais adversos, provavelmente devido à procedência de uma região pobre do Brasil. Foi constatado algumas limitações na distribuição de MACs apropriados para essa população.

Objectives: article 1: To describe the prevalence of epilepsy in pregnant women, sociodemographic profile and obstetric outcomes and analyze the association of these variables with a disease. Article 2: Describe the profile of ASM (anti-crisis medication) and types of seizures and analyze the association of these profiles with the incidence of maternal and fetal complications in pregnat women with epilepsy (PWWE). Article 3: Describe the prevalence, sociodemographic and clinical profile, types of ASM used and seizures and analyze the association of the incidence of maternal and fetal complications with these variables in PWWE. Methods: Articles 1 and 2: retrospective cohorts with 234 PWWE and 492 pregnant women without epilepsy (PWNE). Article 3 prospective cohort collected data from 95 PWWE and 380 PWNE. Both cohorts aged 40 years in medical records and interviews from four high-risk maternity hospitals in Alagoas. The period from 2008 to 2021 in the cohort retrospective and 2021 and 2022 in the prospective. Results of the articles: The prevalence of PWWE in the retrospective and prospective cohorts were 0.49% (n = 224/44917) and 0.53% (n = 105/19,624) with mean ages (24.94 ± 6.25 and 23 .98 ± 6.89); (24.42 ± 5.64 and 24.42 ± 5.62) years, respectively. PWWE came from the countryside (58.2%; 64.2%), had brown skin (88.6%; 98%), illiterate (7%; 3.2%) and had primary education (40%; 52 .1%), single (47.3%; 49.5%) and (76.9%; 78.9%) homemakers, respectively. Regarding deliveries, the most of them (60.3%; 54.8%) were multiparous, (74.6%; 70.8%) had cesarean delivery, respectively. In the prospective cohort, 15.8% had no income, 54.7% earned less than 1 minimum wage, 44.2% were Catholic religious and 87.4% had not planned the pregnancy. Analysis of obstetric and neonatal outcomes showed a higher risk in PWWE for pregnancy-related hypertension (PrH) (OR=6.29; 95% CI=3.50-11.30), preeclampsia OR=8.04; 95% CI=2.22-29.10) in the retrospective cohort, and in the retrospective and prospective cohorts a risk of vaginal bleeding (OR=2.54; 95% CI=1.15-5.59);(OR=4.13; 95% CI=1.45-9.11), miscarriage (OR=1.75; 95% CI=1.16-2.63); (OR=1.50; 95% CI=1.00-2.22) and stillbirth (OR=11.16; 95% CI=2.22-29.10); (OR=5.27; 95% CI=2.29-10.30), respectively. In both cohorts (14%; 14.7%) they did not use MAC, (50.2%; 85.3%) monotherapy (35.8%; 12.6%) polytherapy, respectively. Phenobarbital was the most prescribed ASM followed by carbamazepine in both cohorts. In prospective PWWE who used ASM and polytherapy had a higher risk of vaginal bleeding, maternal ICU admission and stillbirth. Analyzing the types of epilepsy, most of them 40% had the generalized type. Regarding the type of seizure, most 53.3% had focal seizures in the retrospective cohort, while in the prospective cohort, most of them 48.4% had GTCS (tonic-clonic generalized) and 19% had status epilepticus, associated with adverse obstetric and neonatal outcomes. Conclusion: Both studies report a sociodemographic profile of PWWE with high social vulnerability and higher risk of adverse obstetric and neonatal outcomes, probably due to the origin of a poor region of Brazil. Some limitations were found in the distribution of appropriate ASM for this population

Seizures and COVID-19: Results from the Spanish Society of Neurology's COVID-19 registry.

We describea series of patients with COVID-19 who presented with seizures, reported in the Spanish Society of Neurology's COVID-19 Registry. This observational, descriptive,multicentre, registry-based study includes patients with confirmed COVID-19 who experienced seizures during active infection.Wedescribe theclinicalpresentation of COVID-19,seizures,and resultsof complementary tests.Wealsodescribe the suspectedaetiologyof the seizures. Of 232 reported cases, 26 (11.2%) presented with seizures;7 of these patients (26.9%) had prior history of epilepsy, whereas the remaining 19 (73.1%) had no history of seizures.In most cases, seizures presented on days 0 and 7 after onset of COVID-19. By seizure type, 8 patients (30.7%) presentedgeneralised tonic-clonic seizures, 7 (26.9%) status epilepticus, 8 (30.7%) focal impaired-awareness seizures, and 4 (11.7%) secondary generalised seizures.Six patients (23.1%) also presented other neurological symptoms, includingaltered mental status and decreased level of consciousness. Predisposing factors for seizures (eg, dementia, tumour, cerebrovascular disease) were observed in 10 of the 19 patients with no prior history of epilepsy (52.6%). Patients with COVID-19 may present with seizures over the course of the disease,either alone or in the context of encephalopathy.Seizures may present in patients with no prior history of epilepsy; however, most of these patients present predisposing factors.

Cost-effectiveness of perampanel for the treatment of primary generalized tonic-clonic seizures (PGTCS) in epilepsy: A Spanish perspective.

BACKGROUND: Persistent seizures are associated with physical injury, reduced quality of life, and psychosocial impairment. Perampanel is approved for the adjunctive treatment of primary generalized tonic-clonic seizures (PGTCS). OBJECTIVE: This study aimed to determine the cost-effectiveness of perampanel as adjunctive therapy to other antiepileptic drugs (AED) compared with AED maintenance therapy alone for the treatment of PGTCS. METHODS: We developed a Markov model for PGTCS where transitions were based on treatment response rates. The analysis was conducted over a 33-year time horizon from the Spanish National Health Service (NHS) and societal perspectives. Efficacy data were derived from clinical studies. Resource use, market shares, costs, and utilities were obtained from Kantar Health's National Health and Wellness Survey. Drug costs were obtained from the Consejo General de Colegios Oficiales de Farmacéuticos. One-way and probabilistic sensitivity analyses were performed. RESULTS: In the base case analysis from the NHS perspective, perampanel was associated with an incremental cost-effectiveness ratio (ICER) of €16,557/quality-adjusted life year (QALY) relative to AED maintenance therapy for the treatment of PGTCS. Incremental costs were €5475 and incremental QALYs were 0.33. In one-way sensitivity analyses, the ICERs were strongly influenced by discounting rate for costs and health effects, with little influence of other parameters, including perampanel cost and utilities. In probabilistic sensitivity analyses, the probability of perampanel being cost-effective at a willingness-to-pay threshold of €30,000/QALY was 89.3%. From the societal perspective, perampanel provided a cost-savings of €5288 per patient compared with AED maintenance therapy alone. CONCLUSION: Our study demonstrates that perampanel is likely to be a cost-effective option.

Antiepileptic drug reduction and increased risk of stimulation-evoked focal to bilateral tonic-clonic seizure during cortical stimulation in patients with focal epilepsy.

INTRODUCTION: Stimulation-evoked focal to bilateral tonic-clonic seizure (FBTCS) can be a stressful and possibly harmful adverse event for patients during cortical stimulation (CS). We evaluated if drug load reduction of antiepileptic drugs (AEDs) during CS increases the risk of stimulation-evoked FBTCS. MATERIAL AND METHODS: In this retrospective cohort study, we searched our local database for patients with drug-resistant epilepsy who underwent invasive video-EEG monitoring and CS in the University Hospital la Fe Valencia from January 2006 to November 2016. The AED drug load was calculated with the defined daily dose. We applied a uni- and multivariate logistic regression model to estimate the risk of stimulation-evoked FBTCS and evaluate possible influencing factors. Furthermore, we compared patients whose AEDs were completely withdrawn with those whose AEDs were not. RESULTS: Fifty-eight patients met the inclusion criteria and were included in the analysis. Stimulating 3806 electrode contact pairs, 152 seizures were evoked in 28 patients (48.3%). Ten seizures (6.6%) in seven patients (12.1%) evolved to FBTCS. In the univariate and multivariate analysis, a 10% reduction in drug load was associated with an increase of the odds ratio (OR) of stimulation-evoked FBTCS by 1.9 (95%-CI 1.2, 4.0, p-value=0.04) and 1.9 (95%-CI 1.2, 4.6, p-value=0.04), respectively. In patients, whose AEDs were completely withdrawn the OR of FBTCS increased by 9.1 (95%CI 1.7, 69.9, p-value=0.01) compared with patients whose AEDs were not completely withdrawn. No other factor (implantation type, maximum stimulus intensity, number of stimulated contacts, history of FBTCS, age, gender, or epilepsy type) appears to have a significant effect on the risk of stimulation-evoked FBTCS. CONCLUSIONS: The overall risk of stimulation-evoked FBTCS during CS is relatively low. However, a stronger reduction and, especially, a complete withdrawal of AEDs are associated with an increased risk of stimulation-evoked FBTCS.

Esclerosis tuberosa / Tuberous sclerosis

Se presenta el caso de paciente masculino de 15 años de edad que es llevado a sala de urgencia s del Hospital Roosevelt por convulsiones tónico clónico generalizadas de 2 minutos de duración, con período postictal, con antecedentes familiares de convulsiones mostradas en el árbol genealógico (grafico 2 ). Además presenta antecedente de síndrome convulsivo desarrollado a los tres años de edad, tratado con múltiples anticonvulsivantes sin llegar a un dia gnóstico. Paciente al examen físico se presenta normocéfalo, cabello adecuada implantación, alerta, orientado en tiempo, espacio y persona, escleras y mucosas normales, con lesiones angiofibromatosas de distribución en alas de mariposa en región de puente nasal, alas nasales, mejillas y frente , frecuencia respiratoria de 14 por minuto, con ruidos respiratorios conservados, frecuencia cardíaca 70 por minuto, presión arteria l 100/60 mmHg, ritmo cardíaco normal, sincrónico con el pulso, abdomen sin alteraciones, extremidades y evaluación de sistema nervioso central sin presentar alteraciones...(AU)

We present the case of a 15-year-old male patient who is taken to the emergency room s Roosevelt Hospital for generalized 2-minute clonic tonic convulsions, with a period postictal, with family history of seizures shown in the pedigree (chart 2). It also presents a history of convulsive syndrome developed at three years of age, treated with multiple anticonvulsants without reaching a Gnostic day. Patient to the physical examination presents normocephalus, adequate hair implantation, alert, oriented in time, space and person, normal scleras and mucous membranes, with angiofibromatosis lesions of distribution in butterfly wings in nasal bridge region, nasal wings, cheeks and forehead, frequency respiratory rate of 14 per minute, with preserved respiratory sounds, heart rate 70 per minute, artery pressure l 100/60 mmHg, normal heart rate, synchronous with the pulse, abdomen without alterations, extremities and evaluation of the central nervous system without presenting alterations. . (AU)

Bevacizumab in recurrent glioblastoma: five informative patient scenarios.

Glioblastoma is the most common and malignant primary brain tumour in adults. Maximum feasible surgical resection, radiotherapy and temozolomide chemotherapy at initial diagnosis have improved prognosis but rapid recurrence is typical and survival remains brief. There is an urgent need for effective new treatments and approval of the antiangiogenic agent bevacizumab for recurrent glioblastoma by Health Canada in 2009 has been the most notable recent therapeutic advance for this disease. This review with illustrative case studies highlights how bevacizumab has been incorporated into the treatment of glioblastoma in Canada and describes the ongoing controversies surrounding its clinical application.

[Seizures revealing phosphocalcic metabolism abnormalities]. / Crises épileptiques révélant des anomalies du métabolisme phosphocalcique.

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.

Presumed mitochondrial disease manifesting with recurrent syncopes.

OBJECTIVES: Loss of consciousness may be due to neurological or cardiac involvement in mitochondrial disease, and is often difficult to attribute to either cause, as in the following case. CASE REPORT: A 67-year-old man with hypertension, diabetes, elevated serum creatine kinase, glaucoma, optic atrophy, and vertigo had experienced recurrent losses of consciousness since 63 years of age. Diagnostic work-up revealed paroxysmal supraventricular arrhythmias, hyperlipidemia, steatosis hepatis, renal insufficiency, polyneuropathy, first-degree atrio-ventricular block, orthostasis, and cataract. From the age of 66 years, he developed tonic-clonic seizures. Electrocardiography loop recording showed some losses of consciousness as associated with supraventricular tachycardias and others with epileptic activity or arterial hypotension. Neurological investigations and muscle biopsy were indicative of mitochondrial disease with multisystem involvement. Losses of consciousness disappeared after catheter ablation and treatment with levetiracetam. CONCLUSION: Recurrent loss of consciousness in mitochondrial disease may not only be due to arrhythmias but also seizure activity, or autonomic neuropathy. Arrhythmias, seizures, and polyneuropathy may have a common underlying cause affecting various tissues.

Alternative tacrolimus and sirolimus regimen associated with rapid resolution of posterior reversible encephalopathy syndrome after lung transplantation.

BACKGROUND: Neurotoxicity is a significant complication of calcineurin inhibitor use, and posterior reversible encephalopathy syndrome has been reported. Limited data exist on the use of alternative immunosuppression regimens in the management of posterior reversible encephalopathy syndrome in transplant recipients. METHODS: We present the immunosuppression management strategy of a girl who underwent bilateral lung transplantation for cystic fibrosis 6 months earlier, then suddenly developed a grand mal seizure due to posterior reversible encephalopathy syndrome diagnosed by magnetic resonance imaging of the brain. In an effort to reduce her tacrolimus dose, an alternative immunosuppressant regimen combining tacrolimus and sirolimus was used. RESULTS: After the modification of her immunosuppressant regimen, there was rapid clinical improvement with no further seizures. Her brain findings had resolved on magnetic resonance imaging 2 months later. Over the next 6 months, allograft function remained stable and surveillance transbronchial biopsies found no allograft rejection on the combined sirolimus and tacrolimus therapy. CONCLUSIONS: Tacrolimus-associated neurotoxicity resolved in a lung transplant recipient with a combined tacrolimus and sirolimus regimen. This combined therapy appears to be an effective alternative for lung transplant recipients that allow them to receive the benefits of both drugs but at lower doses, which reduces the risk for adverse effects.

Atlanto axial rotatory dislocation in adults: a rare complication of an epileptic seizure--case report.

Atlanto Axial Rotatory Dislocations (AARDs) are a heterogeneous group of post-traumatic pathologies typical of the pediatric age, and rare in adults. We describe the case of a 34-year-old woman, developing Atlanto Axial Rotatory Fixation (AARF) after a generalized tonic-clonic epileptic seizure, an extremely rare traumatic cause never described in literature. AARF was detected only 1 month after the accident and nonsurgical treatment was attempted at the beginning. The patient underwent surgery only 2 months after the accident. The best treatment should be conservative reduction within 1 month; when it is not possible, it is advisable to perform surgery as soon as possible. C1-C2 fixation with Harm's technique is the gold standard for fixed luxations. Delay of treatment makes intraoperative reduction more difficult and increase the establishment of the chronic permanent change of neck muscles and ligaments.

Clinical course of intoxication with the new anticonvulsant drug perampanel.

Perampanel has recently been approved as an anticonvulsant drug for focal epilepsies. Phase III trials have shown good tolerability, although data regarding effects of high doses of perampanel are not available. Here, we describe the first case of a 34-year-old patient with perampanel intoxication and attempted suicide, in which the recommended daily dose of perampanel was exceeded ten-fold. Clinical signs of the intoxication and possible psychotropic effects are described.

Surgical treatment of recurrent torcular meningiomas: case report and review of the literature.

Torcular meningiomas intimately involving major dural sinuses such as superior sagittal sinuses, transverse sinuses, and straight sinuses and the confluence are a great challenge for neurosurgeons. Simpson grade I total resection, which can bring complete cure, is the ultimate goal, but under many circumstances, it is difficult to achieve. The patency of the sinuses around these tumors is the key factor for successful tumor resection, and decides the surgical strategy. We report the experience of complete resection of a huge recurrent torcular meningioma. Related literature is reviewed.

Adverse effects of antiepileptic drugs in North Indian pediatric outpatients.

PURPOSE: The present study investigates the pattern and predictors of treatment-emergent adverse drug reactions (ADRs) in children diagnosed with epilepsy. METHODS: We conducted prospective observational study in a tertiary care teaching hospital on 277 epileptic children. Antiepileptic drug (AED)-associated ADRs, demographic and clinical characteristics, AED regimen, and so on were recorded. Causality, severity, and preventability were performed by World Health Organization-Uppsala Monitoring Center scale, Hartwig's severity scale, and Schumock and Thornton questionnaire, respectively. RESULTS: Of the enrolled population, 53% children had symptomatic epilepsy, and 51% were in 5- to 10-year age group. More than two-thirds of children were on monotherapy, with phenytoin (n = 176, 63.5%) being the most common AED. Three hundred fifty-three AED-related ADRs were recorded in 175 children (63.2%). Poor scholastic performance (19%) was the most common ADR, followed by gum hypertrophy (13.3%), headache (10.2%), behavioral problems (5.7%), drowsiness (5.7%), and others. Two hundred sixteen ADRs were probable, and 126 ADRs were possible. Severe ADRs were noted in 6 children. Girls (odds ratio [OR], 1.93; 95% confidence interval [95% CI], 1.07-3.45; P = 0.03), children with secondary epilepsy (OR, 3.31; 95% CI, 1.76-6.23; P ≤ 0.001), children older than 5 years (5-10 years; OR, 6.28; 95% CI, 2.79-14.12; P ≤ 0.001), and those older than 10 years (OR, 9.04; 95% CI, 3.69-22.17; P ≤ 0.001) were found to be at higher risk of experiencing ADRs. CONCLUSIONS: Monotherapy was the preferred treatment. Phenytoin was the most common ADR causative agent. Female sex, symptomatic epilepsy, and older age (> 5 years) were found to be associated with higher probability of ADR development.

Malformations of cortical development with good clinical outcome: a case report and review of literature.

Malformations of cortical development (MCD) are important causes of chronic epilepsy. MCD encompass many varied disorders with diverse clinical manifestations. Schizencephaly, one of the MCD, is known to be complicated by various types of epilepsy, most of which are intractable. We treated a 28-year-old man with epilepsy, characterised by unprovoked generalised tonic-clonic seizures accompanied by MCD. Brain MRI detected multiple malformations including septum pellucidum defect, partial corpus callosum defect, schizencephaly, right hippocampal atrophy, cerebellar atrophy and cerebellum atrophy. 99m Tc-ethylcysteinate dimer single photon emission CT showed decreased cerebral blood flow (CBF), and the morphology of CBF defects overlapped with the anomalous findings on MRI. The clinical outcome of the patient was good despite his severe brain malformations. Although the reason of this discrepancy is unclear, the outcomes of social function and epileptic seizures may be relatively good despite severe MCD in some patients.

Posterior reversible encephalopathy syndrome in childhood: report of four cases and review of the literature.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a recently described disorder with typical radiological findings of bilateral gray and white matter abnormalities in the posterior regions of the cerebral hemispheres and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting, and abnormalities of visual perception such as cortical blindness. In this study, the clinical and radiological findings of 4 children with this syndrome due to a variety of conditions are reported. METHODS: The records of 4 children with a diagnosis of PRES were retrospectively analyzed. RESULTS: PRES is associated with a disorder of cerebrovascular autoregulation of multiple etiologies. Four patients with PRES who had primary diagnoses of severe aplastic anemia, nephritic syndrome, Henoch-Schönlein purpura, and acute poststreptococcal glomerulonephritis are presented. This syndrome has been described in numerous medical conditions, including hypertensive encephalopathy, eclampsia, and with the use of immunosuppressive drugs. CONCLUSIONS: Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate precise diagnosis and appropriate treatment.

[Isolated intracranial Rosai-Dorfman disease: a case report and literature review]. / Enfermedad de Rosai-Dorfman intracraneal. Presentación de un caso y revisión de la literatura.

INTRODUCTION: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is an idiopathic histiocytosis that usually affects the lymph nodes. Occasionally it may affect the CNS, being exceptional intracranial involvement without lymph node lesions. In the absence of typical radiological signs, affected patients are generally operated under the suspicion of a meningioma. The histological diagnosis is obtained after the surgical procedure. It is a clinicopathological entity not well known, controversy exists about its pathogenesis, clinical course and therapeutic management. CASE REPORT: We report the case of a 40-year-old male presented two generalized tonic-clonic seizures and brain MRI showed a left parieto-occipital extra-axial lesion extending into the posterior fossa, without presenting lesions at other levels. A partial resection of the lesion was performed and the histological findings were reported as Rosai-Dorfman disease. CONCLUSIONS: Despite its low frequency, the ERD should be included in the differential diagnosis of dural-based masses, compared to more common, such as meningiomas. Due to lack of specificity of additional studies its diagnosis is fundamentally histologic. More research is needed to define the best therapeutic option.