RESUMO
BACKGROUND: Patients with anti-N-methyl-d-aspartate (NMDA) receptor encephalitis (ANMDARE) show a wide range of behavioral abnormalities and are often mistaken for primary psychiatric presentations. We aimed to determine the behavioral hallmarks of ANMDARE with the use of systematic neuropsychiatric and cognitive assessments. METHODS: A prospective study was conducted, with 160 patients admitted to the National Institute of Neurology and Neurosurgery of Mexico, who fulfilled criteria for possible autoimmune encephalitis and/or red flags along a time window of seven years. Cerebrospinal fluid (CSF) antibodies against the NR1 subunit of the NMDAR were processed with rat brain immunohistochemistry and cell-based assays with NMDA expressing cells. Systematic cognitive, neuropsychiatric, and functional assessments were conducted before knowing NMDAR antibodies results. A multivariate analysis was used to compare patients with and without definite ANMDARE according to antibodies in CSF. RESULTS: After obtaining the CSF antibodies results in 160 consecutive cases, 100 patients were positive and classified as having definite ANMDARE. The most frequent neuropsychiatric patterns were psychosis (81%), delirium (75%), catatonia (69%), anxiety-depression (65%), and mania (27%). Cognition was significantly impaired. A total of 34% of the patients had a predominantly neuropsychiatric presentation without seizures. After multivariate analysis, the clinical hallmarks of ANMDARE consisted of a catatonia-delirium comorbidity, tonic-clonic seizures, and orolingual dyskinesia. CONCLUSIONS: Our study supports the notion of a neurobehavioral phenotype of ANMDARE characterized by a fluctuating course with psychotic and affective symptoms, catatonic signs, and global cognitive dysfunction, often accompanied by seizures and dyskinesia. The catatonia-delirium comorbidity could be a distinctive neurobehavioral phenotype of ANMDARE.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato , Catatonia , Delírio , Discinesias , Humanos , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/líquido cefalorraquidiano , Catatonia/etiologia , Estudos Prospectivos , N-Metilaspartato , Receptores de N-Metil-D-Aspartato , Convulsões/complicações , Delírio/complicações , Discinesias/complicaçõesRESUMO
INTRODUCTION: Primary lymphoma of the central nervous system is a variety of non-Hodgkin's lymphoma that accounts for 4-5% of intracranial tumours and 5% of all lymphomas. It has its origin in the brain, the eyes, the leptomeninges and the spinal cord with no systemic evidence of lymphomatoid activity; the subtype of lymphoma is predominantly of B-type cells. PATIENTS AND METHODS: We conducted a descriptive study of the patients diagnosed with primary brain lymphoma who were attended to at third-level centres in Mexico between the years 1980 and 2016. Patients who had been screened for systemic lymphoma were included. The results were analysed by means of simple frequencies, and disease-free and overall survival time was analysed by Kaplan-Meier curves; the differences among curves were analysed by means of log rank. RESULTS: Of a total of 215 patients, there were only 74 cases. By sex, 45% were females and 55% were males. Regarding age, 36.7% were over 60 years old. The most frequent clinical manifestations were motor loss (60%) and cognitive disorders (52%). Most patients received some form of chemotherapy (89%). The only significant factor for radiological response and clinical prognosis was the combined use of radiochemotherapy (p = 0.04493). CONCLUSION: Lymphoma is a tumorous condition with a high clinicoradiological response to treatment, although the response is not long-lasting. Its early identification and multidisciplinary management are essential for a more favourable prognosis in these patients.
TITLE: Linfoma primario del sistema nervioso central: experiencia clinica en un centro neurologico.Introduccion. El linfoma primario del sistema nervioso central es una variedad de linfoma no Hodgkin que representa el 4-5% de los tumores intracraneales y el 5% de todos los linfomas. Se origina en el encefalo, los ojos, la leptomeninge y la medula espinal sin evidencia sistemica de actividad linfomatoide; el subtipo de linfoma mayoritariamente es de celulas de tipo B. Pacientes y metodos. Estudio descriptivo de los pacientes diagnosticados con linfoma cerebral primario que fueron atendidos en centros de tercer nivel en Mexico entre los años 1980 y 2016. Se incluyo a los pacientes que contaran con cribado para busqueda de linfoma sistemico. Los resultados se analizaron mediante frecuencias simples; en el caso del tiempo libre de enfermedad y supervivencia global, mediante curvas de Kaplan-Meier, y las diferencias entre curvas, mediante log rank. Resultados. En un total de 215 pacientes solo hubo 74 casos. El 45% fueron mujeres y el 55%, hombres. El 36,7% eran mayores de 60 años. Las manifestaciones clinicas mas frecuentes fueron deficit motor (60%) y alteraciones cognitivas (52%). La mayoria recibio alguna forma de quimioterapia (89%). El unico factor significativo para respuesta radiologica y pronostico clinico era el uso combinado de radioquimioterapia (p = 0,04493). Conclusion. El linfoma representa una patologia tumoral con alta respuesta clinicorradiologica al tratamiento, aunque la respuesta no es duradera. Es fundamental su identificacion temprana y el tratamiento multidisciplinario para el mejor pronostico de estos pacientes.
Assuntos
Neoplasias do Sistema Nervoso Central/epidemiologia , Linfoma não Hodgkin/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Sistema Nervoso Central/complicações , Neoplasias do Sistema Nervoso Central/diagnóstico por imagem , Neoplasias do Sistema Nervoso Central/terapia , Quimiorradioterapia , Transtornos Cognitivos/epidemiologia , Transtornos Cognitivos/etiologia , Irradiação Craniana , Doenças dos Nervos Cranianos/epidemiologia , Doenças dos Nervos Cranianos/etiologia , Epilepsias Parciais/epidemiologia , Epilepsias Parciais/etiologia , Humanos , Estimativa de Kaplan-Meier , Linfoma não Hodgkin/complicações , Linfoma não Hodgkin/terapia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Transtornos dos Movimentos/epidemiologia , Transtornos dos Movimentos/etiologia , Neuroimagem , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricosRESUMO
INTRODUCTION: Radiotherapy with procarbazine, lomustine, and vincristine (PCV) improves overall survival in patients with anaplastic oligodendroglioma 1p19q codeleted. PATIENTS AND METHODS: This retrospective analysis investigated outcomes in patients with anaplastic oligodendroglioma 1p19q codeleted compared two different protocols (radiotherapy plus temozolomide or PCV). The primary end points were overall survival and progression-free survival. Secondary endpoint was the radiological response. RESULTS: A total of 48 patients were included. Mean age was 43 years (range: 19-66 years), 26 were male (54.1%). Twenty-one patients received PCV and 27 temozolomide. The baseline characteristics were not difference between the groups. The progression-free survival and overall survival in the PCV group were 7.2 and 10.6 years respectively and temozolomide were 6.1 and 9.2 years, both statistically significant. The radiological response was present in 80.9% in PCV arm and 70.2% in temozolomide arm there was not statistical differences. The multivariate Cox model showed only the significant parameters the use of PCV protocol. The toxicity grade 3 or 4 was present in 42.8% in PCV arm and 11.1% in temozolomide arm. CONCLUSIONS: The most common strategy in the Latin America community is the substitution of the PCV for temozolomide. This retrospective study showed superior efficacy of PCV than temozolomide. The Latin American community effort must be made to be able to have the drugs to available for using as a first line of treatment.
TITLE: Radioterapia mas temozolomida o PCV en pacientes con oligodendroglioma anaplasico con codelecion 1p19q.Introduccion. La radioterapia con procarbacina, lomustina y vincristina (PCV) mejora la supervivencia global en pacientes con oligodendroglioma anaplasico con codelecion 1p19q, pero no esta disponible en America Latina. Pacientes y metodos. Analisis retrospectivo comparando dos protocolos diferentes, radioterapia mas temozolomida o PCV, en pacientes con oligodendroglioma anaplasico con codelecion 1p19q. Los objetivos primarios fueron la supervivencia global y la supervivencia libre de progresion, y el objetivo secundario, la respuesta radiologica. Resultados. Se incluyo a 48 pacientes, 26 de ellos varones (54,1%), con una edad media de 43 años (rango: 19-66 años). Veintiun pacientes recibieron PCV, y 27, temozolomida. Las caracteristicas iniciales no tuvieron diferencias entre los grupos. La supervivencia libre de progresion y la supervivencia global en el grupo con PCV fueron de 7,2 y 10,6 años, y en el grupo de temozolomida, de 6,1 y 9,2 años, respectivamente, unos resultados estadisticamente significativos. Hubo respuesta radiologica en el 80,9% en el brazo de PCV y el 70,2% en el brazo de temozolomida. El analisis multivariado de Cox mostro como unico parametro significativo el uso del protocolo PCV. El grado de toxicidad 3-4 estuvo presente en el 42,8% en el brazo de PCV y en el 11,1% en el brazo de temozolomida. Conclusiones. La estrategia mas comun en America Latina es la sustitucion de PCV por temozolomida. Este estudio retrospectivo mostro una eficacia superior de PCV que de la temozolomida. La diferencia obliga a la comunidad latinoamericana a hacer un esfuerzo colectivo para poder tener acceso a los medicamentos para su uso como primera linea de tratamiento.
Assuntos
Antineoplásicos Alquilantes/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/radioterapia , Oligodendroglioma/tratamento farmacológico , Oligodendroglioma/radioterapia , Temozolomida/uso terapêutico , Adulto , Idoso , Neoplasias Encefálicas/genética , Terapia Combinada , Feminino , Deleção de Genes , Humanos , Lomustina/uso terapêutico , Masculino , Pessoa de Meia-Idade , Oligodendroglioma/genética , Procarbazina/uso terapêutico , Estudos Retrospectivos , Vincristina/uso terapêutico , Adulto JovemRESUMO
INTRODUCTION: Some previous studies have suggested familial aggregation of gliomas, although the results have not always been replicated. SUBJECTS AND METHODS: In the present study of a Mexican population, we compared 100 cases of glioma with 124 healthy unrelated controls, as well as their 1st, 2nd and 3rd degree relatives (n = 3,575 and 4,520 respectively). RESULTS: The relatives of the cases had a significantly higher risk of developing brain tumors than the relatives of controls (OR = 5.3; p < 0.05; 95% CI = 1.1-25.7), and their risk of developing any cancer was also increased (OR = 2; p < 0.05; 95% CI = 1.16-3.51), this risk was twofold for men when compared to females (OR = 2; p < 0.05; 95% CI = 1.15-3.37). CONCLUSION: The present study supports familial aggregation of brain tumors and warrants further research into their genetic etiology.